1.

Refer urgently adults with new-onset blackouts (transient loss of consciousness), accompanied by features that are strongly suggestive of epileptic seizures, for neurological assessment in line with the recommendation for people with suspected epilepsy in the NICE guideline on transient loss of consciousness (‘blackouts’) in over 16s.

2.

Do not routinely refer adults with blackouts if there are clear features of vasovagal syncope, even if associated with brief jerking of the limbs. See recommendation 1.1.4.3 on uncomplicated faint in the NICE guideline on transient loss of consciousness (‘blackouts’) in over 16s.

Recommendation 1 – Blackouts accompanied by seizure markers

A blackout associated with seizure markers may indicate the onset of epilepsy. Seizure markers include:

• a bitten tongue
• confusion following the event
• head-turning to one side during TLOC
• no memory of abnormal behaviour that was witnessed before, during or after TLOC by someone else
• prodromal déjà vu or jamais vu
• prolonged limb-jerking (note that brief seizure-like activity can often occur during uncomplicated faints)
• unusual posturing.

The committee had the benefit of being able to cross-refer to the NICE guideline on transient loss of consciousness, which includes a consideration of suspected epilepsy. Uncontrolled epileptic seizures could lead to injury or death, and new onset epilepsy can be the presenting feature of yet undiagnosed brain pathology. As such, urgent referral for assessment and treatment is advised.

Recommendation 2 – Transient loss of consciousness (TLOC) and vasovagal syncope

Vasovagal syncope is common and can often be diagnosed through the history or augmented by an eyewitness account. It requires minimal investigation unless accompanying ‘red flag’ features are present (NICE TLOC guideline CG 109). Of note, some jerking of limbs is common during a vasovagal episode. This alone should not be understood as indicating epilepsy or another serious pathology. Management is summarised in NICE TLOC guidance (CG 109).

The committee was provided with estimates of the number of referrals to neurologists from 2015/16 and their cost to the NHS. During 2015/16, there were 517,806 first-time neurological outpatient attendances. Using NHS reference costs where an outpatient visit cost £175 and a paediatric outpatient visit cost £285, first-time referrals to neurologists cost approximately £93 million in total, or £87 million for adults and £6 million for children. The committee used these estimates as a starting point for considering whether its recommendations caused additional referrals that would have a significant cost impact on the NHS.

The committee felt that the recommendations would not change the number of referrals given that they appear in other NICE guidance, and hence there will be no change from current costs.

Targeted engagement exercise

Both these recommendations received a high level of agreement ranging from 77.8% to 88.9%. Some helpful, minor adjustments to the wording were made following suggestions from the targeted engagement exercise group. Most importantly, responders indicated that these recommendations would only apply to people with a first presentation who have not been previously assessed by specialists. Therefore, ‘new onset’ was added to the recommendation to make this clear. For the sake of clarity, the original 3 recommendations have been edited into the current 2.

13.

Refer urgently adults with facial pain associated with persistent facial numbness or abnormal neurological signs for neuroimaging.

14.

Refer adults with unilateral facial pain that is triggered by touching the affected part of the face (trigeminal neuralgia) and is refractory to treatment, in line with the NICE guideline on neuropathic pain in adults.

15.

For adults with scalp tenderness or jaw claudication suggestive of temporal arteritis, consider blood tests and follow local pathways for suspected giant cell (temporal) arteritis. Be aware that a normal ESR (erythrocyte sedimentation rate) does not exclude a diagnosis of giant cell arteritis.

Measures of diagnostic accuracy including sensitivity, specificity, positive predictive value, negative predictive value, ROC, AUC as well as adjusted odds ratios were all considered important outcomes to determine whether the signs and symptoms in the presence of facial pain are indicative of a neurological condition that requires referral for a specialist assessment.

Sensitivity and specificity were considered to be of equal importance. Sensitivity was important because the consequences of missing a patient with a neurological condition would have serious implications, which can lead to rapid deterioration of health or even death. Specificity was important because incorrectly diagnosing an individual may result in inappropriate administration of medications or treatments. Only adjusted odds ratios from studies that had conducted a multivariate analysis including the predictors of interest were considered.

Recommendation 13 – Facial pain with abnormal neurological signs

Isolated facial pain is a common symptom and mostly the symptom is self-limiting and benign. Dental and facial sinus pain are common causes. The presence of persistent numbness accompanied by abnormal neurological signs on examination, such as sensory loss in the face which is persistent or progressive, an abnormality of eye movement or facial weakness, in people with facial pain suggests the presence of a mass lesion. Urgent neuroimaging according to the NICE guideline on suspected cancer is appropriate when these additional signs are detected.

Recommendation 14 – Trigeminal neuralgia

Trigeminal neuralgia is characterised by severe, stimulation-sensitive, shock-like pain in the distribution of the trigeminal nerve. People with trigeminal neuralgia do not necessarily need referral and can be managed in primary care according to the NICE guideline on neuropathic pain in adults. It is appropriate to refer people with more resistant pain to neurology services for assessment and treatment. Non-neurologic facial pain is commonly inappropriately attributed to trigeminal neuralgia but does not respond well to treatment of neuropathic pain.

Recommendation 15 – Temporal arteritis

Temporal arteritis can be difficult to diagnose and because of the risk of permanent neurological damage associated with untreated disease it should always be considered as a cause of headache and facial pain in older people. The diagnosis is usually confirmed by typical clinical features and abnormal blood tests such as elevated erythrocyte sedimentation rate, elevated C-reactive protein and abnormal liver function tests. It is exceptionally rare under the age of 50 years. People with symptoms suggestive of temporal arteritis should be treated urgently because of the risk of irreversible damage to eyesight and stroke,. Where there is uncertainty about the diagnosis, because of atypical clinical features or inconsistent blood test results, they should be referred urgently for consideration of a diagnostic temporal artery biopsy.

The committee was provided with estimates of the number of referrals to neurologists from 2015/16 and their cost to the NHS. During 2015/16, there were 517,806 first-time neurological outpatient attendances. Using NHS reference costs showing that an outpatient visit cost £175 and a paediatric outpatient visit cost £285, first-time referrals to neurologists cost approximately £93 million in total, or £87 million for adults and £6 million for children. The committee used these estimates as a starting point for the considering whether its recommendations caused additional referrals that would have a significant cost impact on the NHS.

Due to the seriousness of the symptom, the committee felt urgent referral is warranted in these specified cases. This is in line with current protocols; therefore, it is not believed to represent a dramatic shift in practice and should not lead to a significant increase in referrals.

16.

For recommendations on assessing sudden-onset unsteady gait in adults, see the NICE guideline on stroke and transient ischaemic attack in over 16s.

17.

Refer urgently adults with rapidly (within days to weeks) progressive unsteady gait (gait ataxia) for neurological assessment.

18.

Refer adults with gradually progressive unsteady gait (gait ataxia) for neurological assessment and:

• take an alcohol history and follow the recommendations in the NICE guideline on alcohol-use disorders: diagnosis, assessment and management of harmful drinking and alcohol dependence.
• Check thyroid function
• Check for vitamin B12 and folate deficiency
• consider serological testing for gluten sensitivity as recommended in the NICE guideline on coeliac disease.

19.

Refer adults who have difficulty initiating and coordinating walking (gait apraxia) to neurology or an elderly care clinic to exclude normal pressure hydrocephalus.

20.

For adults with unsteadiness of gait who are at risk of falling, follow the recommendations on multifactorial falls risk assessment in the NICE guideline on falls in older people and consider referring to a falls prevention team.

This symptom was not prioritised for an evidence review because the committee considered the referral decision to be non-contentious and an evidence review is unlikely to change this.

Diagnosis of the various different causes of unsteadiness demands clinical skills, but the requirement for referral is non-contentious once the type of unsteadiness is recognised.

Unsteadiness of gait can be caused by either gait ataxia or gait apraxia. Ataxia of gait is caused by impaired voluntary co-ordination of movement, whereas apraxia of gait is a difficulty in correctly sequencing the automatic components (including balance, gait initiation, stride length and speed) necessary to walk. The committee recognised that it is difficult to distinguish unsteadiness due to ataxia from unsteadiness due to apraxia. Therefore, differentiation may require referral for an expert assessment.

Recommendation 16 – Sudden-onset unsteady gait

Sudden onset of unsteady gait can signify a vascular event such as stroke, especially if it is an isolated symptom. The committee therefore agreed that referrals should be urgent. The committee noted the NICE guideline on stroke (CG68) and agreed to cross-refer.

Recommendation 17 – Rapidly progressive unsteadiness of gait

Rapidly progressive unsteadiness of gait (ataxia) that presents and progresses over a course of days or weeks is an unusual presentation and should be referred on the 2-week neurology pathway. The most common cause in a young person is multiple sclerosis (MS). The differential diagnoses for rapidly progressive ataxia includes brain tumour, paraneoplastic syndrome, atypical infections (for example, herpes zoster and legionella), autoimmune disorders and very rare conditions (for example, acute disseminated encephalomyelitis [ADEM]). These may require a quick diagnosis in order to treat the condition in the best manner; thus, people presenting with rapidly progressive gait ataxia should be referred to neurology urgently.

Clinicians should be aware that cancer is a risk in this population. Ovarian, breast and lung cancer can cause paraneoplastic syndromes, which can present with neuromuscular symptoms. Tests such as chest radiographs and pelvic ultrasounds can be used to search for cancers that may be driving a suspected paraneoplastic syndrome, but under these circumstances, the committee considered that referral to a specialist for a differential diagnosis in these rapidly progressive cases is more important than non-specialists undertaking investigative tests.

Recommendation 18 – Gradually progressive unsteady gait (gait ataxia)

Unsteady gait is common in the elderly but true gait ataxia is rare. It can be caused by cerebellar or proprioceptive sensory lesion due to peripheral neuropathy or spinal cord (dorsal column) pathology. These may be of sporadic or genetic origin and require a neurological assessment. An accurate diagnosis is important to look for treatable causes. However, the committee also wished to highlight some simple measures, which could be taken while awaiting a secondary care appointment.

Longstanding alcohol use can often lead to gait ataxia. Some people may not volunteer a history of excessive alcohol use, and assessment of alcohol intake can be problematic. Adults with gait ataxia and a history of excessive alcohol use should also be referred to neurology because, in a significant minority, the ataxia will be due to a condition not related to their alcohol intake.

Ataxia can be the presenting feature of an inflammatory neuropathy and treatable with immunomodulatory medicines. There will usually be some weakness, which can be distal or proximal, and deep tendon reflexes will be depressed. The clinical diagnosis is confirmed with nerve conduction studies.

The nutritional status of people with progressive gait ataxia is important and can be assessed using full blood count, vitamin B12 and folate tests prior to attending secondary care clinics. Test results can also help to expedite a diagnosis and optimise nutritional status prior to a neurological assessment. Occult coeliac disease should be considered as a cause of B12 and folate deficiency. This is particularly important for vitamin B12 and folate as malabsorption or deficiency can cause sensory ataxia due to peripheral neuropathy and spinal cord or dorsal column pathology.

Gluten sensitivity is one of the most common treatable causes of sporadic gait ataxia. It can present at any age and may be overlooked by non-specialists. If gluten sensitivity is suspected, the person should be referred for a gastroenterology opinion and associated nutritional deficiencies addressed at the same time as neurological referral for gait ataxia. The NICE coeliac guideline recommends considering tests for coeliac disease in people with unexplained neurological symptoms, particularly peripheral neuropathy or ataxia (NICE guideline on Coeliac disease: recognition, assessment and management, NG20).

Recommendation 19 – Normal pressure hydrocephalus

The committee wanted to raise awareness of normal pressure hydrocephalus (NPH) amongst both GPs and other non-specialists. Normal pressure hydrocephalus is a potentially treatable cause of gait apraxia and can easily be overlooked. It can also manifest with cognitive loss and urinary incontinence, and people with these features should be referred to a specialist with relevant expertise for consideration of a NPH diagnosis (local service arrangements may vary). A significant improvement in gait following lumbar puncture is usually required for diagnosis. Treatment options include ventricular shunting or repeated lumbar puncture. Surgery requires a certain level of fitness, and the gait disorder is more likely to improve than cognitive dysfunction. Therefore, the referral of very frail people and individuals with advanced cognitive decline may not be appropriate although referral to an elderly care or falls clinic and repeated lumbar puncture remains an option.

Recommendation 20 – Preventing falls

Unsteadiness of gait is more prevalent in older people, in whom it often has a multifactorial aetiology. While an early neurological assessment is important in younger people, if the patient is older and has multiple comorbidities, a referral to an elderly care or falls clinic may be more appropriate. The elderly care or falls clinic will refer to neurology if necessary. The guideline committee agreed that the recommendations on multifactorial falls risk assessment for people aged over 65 in the NICE guideline on falls in older people are applicable to adults aged under 65 who are at risk of falling.

The committee was provided with estimates of the number of referrals to neurologists from 2015/16 and how much this cost the NHS. During 2015/16, there were 517,806 first-time neurological outpatient attendances. Using NHS reference costs where an outpatient visit cost £175 and a paediatric outpatient visit cost £285, first-time referrals to neurologists cost approximately £93 million in total, or £87 million for adults and £6 million for children. The committee used these estimates as a starting point for considering whether its recommendations caused additional referrals that would have a significant cost impact on the NHS.

The committee agreed that the recommendations did not represent a change from current best practice, and that, viewed along with the rest of this guideline, the recommendations should not increase the total number of referrals or NHS costs.

The committee agreed that all individuals would be offered serological testing at some point in the pathway, so this did not represent an incremental cost if it was merely conducted sooner. The committee also agreed that such test results could expedite a diagnosis and optimise a referral, meaning that they could improve health outcomes.

Targeted engagement exercise

There was a very high level of agreement with this set of recommendations ranging from 81.3% to 100%. There were suggestions to remove gluten sensitivity testing from recommendation 18. However, the committee felt that it was important to include this as it is one of the most common and treatable causes of sporadic gait ataxia, which is easily missed by both specialists and non-specialists. The committee felt that this should remain in the recommendation and provided further detail above to support this.

21.

Refer adults who have sudden-onset difficulty with handwriting that has no obvious musculoskeletal cause for a neurological assessment according to local stroke pathways.

22.

Ask adults who have difficulty with handwriting that has no obvious musculoskeletal cause to demonstrate their handwriting and:

• if they have a problem with generating language rather than hand function, refer for neurological assessment
• if their handwriting is small and slow, consider referring for possible Parkinson’s disease
• if their difficulty is specific to the task of handwriting and examination shows no other abnormalities, consider referring for possible focal dystonia.

There are many neurological reasons for people to have writing difficulties besides those caused by weakness, which is covered elsewhere in this guideline. Examples of this include movement disorders (such as Parkinson’s disease) and task-based dystonias (such as writer’s cramp). Cognitive deficits (including those associated with dementia) that affect executive function and language can make it difficult for some people to write.

Recommendation 21 – Sudden-onset writing difficulty

Isolated difficulty in writing is a relatively unusual manifestation of a stroke. However, in the event of a very sudden onset of writing difficulties (that is, illegible writing that has no musculoskeletal explanation), it would be important to consider this possibility, and the committee therefore recommend referral using the relevant local pathway.

Recommendation 22 – Demonstrating writing

Patients may be concerned that their handwriting has changed to become less legible. By asking the patient to demonstrate writing, non-specialists can garner additional information that may help to clarify a potential diagnosis and determine whether the person needs to be referred for a suspected neurological condition.

Parkinson’s disease can occasionally present with writing difficulties, typically small writing (micrographia), and if this symptom is dominant, other features of Parkinson’s disease should be sought, including asymmetric slowness of rapid repetitive movements, a history of anosmia or REM behaviour sleep disorder. A referral for confirmation of the diagnosis and management advice should be made to a neurologist or a care of the elderly physician, in line with local pathways.

Dystonia is a task-specific movement disorder, and standard neurological examination of the hand and arm usually reveals no abnormality. Recognition of dystonia is currently sub-optimal, and there are often long delays before neurologists see people with this condition. The committee therefore considered it important to raise awareness amongst non-specialists about the possibility of focal dystonia as a diagnosis as well as the need for referral for neurological assessment. People in whom dystonia is suspected could be referred directly to a movement disorders clinic if available; otherwise, they can be referred to the local neurology service.

Cognitive deficits (including those associated with dementia) that affect executive function and language can make it difficult for some people to write and may be interpreted as a writing difficulty. Such people may benefit from referral for assessment to a neurology clinic or, where language difficulties occur in older people in the context of other cognitive features suggestive of dementia, a memory clinic. Be aware that ambivalence, poverty of speech and disorganised language can be the presenting features of a primary psychiatric disorder.

The committee was provided with estimates of the number of referrals to neurologists from 2015/16 and how much this cost the NHS. During 2015/16, there were 517,806 first-time neurological outpatient attendances. Using NHS reference costs when an outpatient visit cost £175 and a paediatric outpatient visit cost £285, first-time referrals to neurologists cost approximately £93 million in total, or £87 million for adults and £6 million for children. The committee used these estimates as a starting point for considerations whether its recommendations caused additional referrals that would have a significant cost impact on the NHS.

People with writing difficulties may seek advice from a general practitioner, but such symptoms are uncommon in general practice. The approach to interpreting the symptoms may be unfamiliar, and the symptoms may sometimes be misinterpreted or considered likely to be emotionally generated. Difficulty with writing can be caused by tremor, dystonia of the hand or Parkinson’s disease. In each case, the problem may compromise the patient’s ability to work or undertake other manual tasks. Such difficulties with writing may be considered as untreatable or the underlying neurological disorder may remain unrecognised. Prompt referral to a neurologist may facilitate diagnosis and symptomatic treatment. This improvement in quality of life would ensure referral was a cost-effective use of NHS resources.

Although the committee felt this might increase referrals it is not a symptom commonly seen in GP practices; therefore, the increase in referrals will not be significant.

Difficulty writing is more commonly due to a mechanical problem, particularly due to musculoskeletal disorders, and these should be excluded before considering a referral to a neurology service. The committee therefore draws attention to this in both recommendations.

Targeted engagement exercise

The committee discussed the results of the targeted engagement exercise, which showed that 66.7% of the 18 participants who responded to this section agreed with recommendation 21, 22.2% requested revisions and 11% (2 participants) disagreed. It was thought that the original wording of the recommendations needed clarification that not all people with a new of onset difficulty writing should be referred but only those where there were no obvious musculoskeletal causes. The committee agreed and amended the recommendations accordingly.

There was no disagreement with recommendation 22, but 50% of the 18 participants who responded requested some revisions on the link to Parkinson’s disease, the differentiation between difficulty writing and language as well as the differences in local services for which the committee provided further details in the table above and made minor edits to the recommendation.

23.

Be aware that sudden-onset weakness, even in restricted distribution (for example, sudden hand weakness), may be caused by a stroke or transient ischaemic attack. See the NICE guideline on stroke and transient ischaemic attack in over 16s for recommendations on assessing sudden-onset limb or facial weakness in adults.

24.

Refer immediately adults with rapidly (within 4 weeks) progressive symmetrical limb weakness for neurological assessment and assessment of bulbar and respiratory function.

25.

Refer immediately, in line with local pathways, adults who have severe low back pain radiating into the leg and new-onset disturbance of bladder, bowel or sexual function, or new-onset perineal numbness, to have an assessment for cauda equina syndrome.

26.

Refer urgently adults with rapidly (within hours to days) progressive weakness of a single limb or hemiparesis for investigation, including neuroimaging, in line with the recommendation on brain and central nervous system cancers in adults in the NICE guideline on suspected cancer.

27.

For adults with slowly (within weeks to months) progressive limb or neck weakness:

• refer for assessment of neuromuscular disorders in line with the recommendations on recognition and referral in the NICE guideline on motor neurone disease
• refer urgently if there is any evidence of swallowing impairment
• refer immediately if there is breathlessness at rest or when lying flat

28.

Be aware that lower limb claudication symptoms in adults with adequate peripheral circulation might be caused by lumbar canal stenosis and need specialist assessment and imaging.

29.

Be aware that, for adults who have been diagnosed with a functional neurological disorder by a specialist, recurrent limb weakness might be part of the disorder and the person might not need re-referral if there are no new neurological signs. New symptoms in adults who have been diagnosed with a functional neurological disorder by a specialist should be assessed as described in the relevant sections of this guideline.

30.

Advise adults with limb or facial weakness ascribed to a functional neurological disorder that their limb or facial weakness might fluctuate and evolve over time and might increase during times of stress.

31.

For adults with clear features of compression neuropathy and no features of a nerve root lesion (radiculopathy^a) of the radial nerve, common peroneal nerve or ulnar nerve:

• refer to orthotic services for a splint
• review the symptoms after 6 weeks, and refer for neurological assessment if there is no evidence of improvement
  For adults with features of radiculopathy see the section on cervical or lumbar radiculopathy.

32.

Advise adults with compression neuropathy to avoid any activity that might lead to further pressure on the affected nerve.

33.

Do not routinely refer adults with an uncomplicated episode of Bell’s palsy (unilateral lower motor neurone pattern facial weakness affecting all parts of the face and including weakness of eye closure) and no evidence of another medical condition such as middle ear disease.

34.

Advise adults with Bell’s palsy about eye care, and that the rate of improvement is variable and maximum recovery can take several months.

35.

Consider referring adults with Bell’s palsy who have developed symptoms of aberrant reinnervation (including gustatory sweating or jaw-winking) 5 months or more after the onset of Bell’s palsy for neurological assessment and possible treatment.

Recommendation 23 – Sudden onset limb weakness

Sudden onset transient limb weakness could indicate a vascular event such as a transient ischaemic attack (TIA) even if it is present only in a restricted distribution, for example, hand weakness. The NICE stroke guideline provides recommendations for recognition and referral. Stroke or TIA should also be considered in unexplained isolated hand weakness of sudden onset, which, in the absence of a history of trauma, is sometimes incorrectly attributed to a compression neuropathy.

Recommendation 24 – Rapidly progressive symmetrical weakness

Rapidly progressive lower motor neurone weakness could be an indication of Guillain−Barré syndrome (GBS), which progresses rapidly over the course of days – up to 4 weeks. Such symptoms may be a feature of other acute neuromuscular disorder (for example, myasthenia gravis), in which case other features may be found such as double vision or drooping of the eyelids that can fluctuate during the course of the day. These are highly treatable but potentially life-threatening conditions, as they can affect the respiratory muscles causing respiratory failure requiring intensive levels of supportive care in a hospital setting. The committee noted that the initial presenting features of neuromuscular disorders may not be recognised by non-specialists and people might be inappropriately reassured. GPs should feel confident to re-refer for immediate neurological re-assessment, where there is concern about progressive symptoms as respiratory compromise may rapidly ensue.

Rapidly progressive upper motor neurone symmetrical weakness could be an indication of spinal cord compression or demyelination. This may require treatment to preserve function and so requires an immediate referral for neurological assessment, including cervical spine imaging.

Recommendation 25 – Cauda equina syndrome

Cauda equina syndrome is an emergency requiring immediate assessment for imaging and consideration of surgical decompression. The committee noted that identical symptoms are common in the context of, or as a new presentation of, functional neurological disorder but these people still require emergency imaging.

Recommendation 26 – Rapidly progressive weakness of a single limb or hemiparesis

Rapidly progressive (hours to days) weakness of a single limb or hemiparesis indicates potentially serious neurological disease and would require referral for urgent assessment, including neuroimaging in line with the recommendations on brain and central nervous system cancers in adults in the NICE guideline on Suspected cancer: recognition and referral – NG12.

Recommendation 27 – Slowly progressive weakness

Slowly progressive lower motor neurone weakness could be due to a global weakness of muscle. Appropriate haematological investigations for causes of myositis and myopathy should be conducted before referral. A routine referral is acceptable.

Slowly progressive weakness could also be indicative of anterior horn cell disease, such as motor neurone disease. The typical rate of disease progression and currently available treatment options requires a prompt rather than urgent neurological review. Accurate diagnosis is important in order to initiate appropriate multidisciplinary supportive care, which may include physiotherapy, occupational therapy and respiratory support.

People with slowly progressive weakness should be kept under review by the referrer until they see a neurologist. Occasionally, people’s conditions can deteriorate rapidly (over weeks), and these people may be more appropriately managed through an acute hospital admission. In addition, the Committee recognised that a more urgent referral is required when slowly progressive weakness includes signs of bulbar involvement, with the attendant risk of aspiration, or of respiratory impairment. The recommendation was designed to cover these possibilities.

Recommendation 28 – Lumbar canal stenosis

Limb weakness that comes on after walking and improves with rest (claudication), in which vascular causes have been excluded, requires a referral for a specialist assessment of possible lumbar canal stenosis. Depending on local pathways, this could be to an extended scope practitioner, neurosurgeon or orthopaedic surgeon. Because of variation in local pathways a directed referral recommendation was not possible.

Recommendation 29 – Recurrent limb weakness

Functional symptoms are complaints that are not primarily explained based on physical or physiological abnormalities. They may mimic neurological disorders. Diagnosis may depend on exclusion of a medical explanation of the symptoms and require a high level of clinical expertise and judgement. Functional symptoms can complicate a medically-explained illness and cause difficulties in diagnosis and delineation. Recurrent episodes of limb weakness, hemiparesis or paraparesis are not uncommon in people with an existing functional neurological disorder; and with other disorders for example, chronic fatigue syndrome and fibromyalgia. The committee considered it inappropriate to refer people with such episodes for investigation after each episode, and considered that psychological support would often be more appropriate than a neurological services re-investigation. The committee emphasised that normal and symmetrical tone and deep tendon reflexes significantly reduce the likelihood of an underlying organic disorder. A recommendation which covered these considerations was thought likely to help GPs to direct these patients to more appropriate channels than neurology services.

Recommendation 30 – Advice for suspected functional neurological disorders

As functional neurological symptoms usually fluctuate and evolve with time, the committee agreed that it was important to address the nature of the condition with people with this condition in order to allay concern about the presence of physical illness and to reduce pressure for onward referral for further specialist opinion.

Recommendation 31 and 32 – Suspected compression neuropathy

Compression neuropathies can be suspected based on a suggestive history of prolonged pressure on the nerve and the pattern of weakness and numbness. Such neuropathies are expected to improve spontaneously without treatment within 6 weeks in most cases, although a splint may be required to support and preserve functionality in a weak wrist or dropped foot during recovery. Where a history of compression is unclear, or where there is evidence of progressive neurological deficit, urgent referral is required to exclude mononeuritis multiplex, a condition that may require systemic treatment.

Reducing pressure or trauma on the affected nerve can increase the chance of recovery. Some activities to be avoided include leg crossing, sleeping on a sore shoulder, excessive kneeling, leaning on elbows, and sleeping deeply following ingestion of alcohol and sedatives.

Recommendations 33, 34 and 35 – Unilateral lower motor neurone facial nerve (Bell’s) palsy

A diagnosis of unilateral lower motor neurone facial nerve palsy (Bell’s palsy) is usually straightforward. Lyme disease should be considered in areas where it is endemic.

The presentation is sometimes confused with stroke. However, unilateral lower motor neurone facial nerve palsy (Bell’s palsy) affects all divisions of the facial nerve, including the forehead and weakens eye closure on 1 side. The condition usually evolves over hours, but may be present on wakening. Primary care clinicians can diagnose Bell’s palsy without referring to neurology, and offer high-dose steroids if seen within 72 hours of onset. Corneal exposure may be managed with taping the eyelids shut or lubrication.

Although the outcome is usually excellent, recovery is frequently incomplete. Aberrant re-innervation of the facial nerve can lead to a number of troublesome symptoms, including gustatory sweating or jaw-winking. These symptoms can occur months after the initial recovery period. As there are potential management techniques for these symptoms available in secondary care, including botulinum toxin injections, these people need to be referred to a neurologist.

The committee was provided with estimates of the number of referrals to neurologists from 2015/16 and their cost to the NHS. During 2015/16, there were 517,806 first-time neurological outpatient attendances. Using NHS reference costs showing that an outpatient visit cost £175 and a paediatric outpatient visit cost £285, first-time referrals to neurologists cost approximately £93 million in total, or £87 million for adults and £6 million for children. These estimates were the committee’s starting point for considering whether its recommendations caused additional referrals that would significantly affect the cost impact of the NHS.

The committee acknowledged the cost of an outpatient referral for neurological problems as £178 for adults and £285 for children. Therefore, the committee only recommended referral of an individual when it was felt that this would lead to sufficiently great health benefits to compensate for the additional pressures placed on neurological services. The committee also acknowledged the additional costs that would be incurred following referral but that these would constitute a cost-effective use of NHS resources, meaning that individuals would not be placed on treatment pathways that were not cost effective.

The committee suggested that an individual should not be referred where it was felt that there would be no, or minimal, reductions in health, thus justifying a reduction in referrals which would lead to cost savings.

The committee did not feel that the recommendations for face and limb weakness would change the number of referrals or increase costs to the health service. The committee felt that cost savings may arise through an ending of unnecessary referral for diagnosis of Bell’s palsy, which can be successfully managed in primary care.

Targeted engagement exercise

Recommendation 33 on non-referral of isolated Bell’s palsy scored 70.6% agreement. Some responders to the targeted engagement exercise were concerned that no treatment advice for Bell’s palsy is included in the recommendations. This has been omitted because to include such recommendations would require a full evidence review of the available treatment options, which is beyond the scope of this guideline. The consensus of the committee is that current standard treatment is appropriate, including local measures (for example, eye protection) and high dose prednisolone, offered early in the disease course.

There was some disagreement (5.9%, 1 participant) on recommendation 25. It was thought that this recommendation was not helpful and should not be included in the guideline as it wasn’t clear what the recommendation addressed. The committee felt that it was important to keep this recommendation and refined the wording and LETR to support it and explain the reasoning further in the LETR.

Recommendation 29 achieved 58.8% agreement. A suggestion was made to change the wording of ‘functional disorders’ to ‘patient weakness describes may not be explained anatomically’. However, the committee preferred to keep the original wording as it was more concise and they wanted to raise awareness of ‘functional disorders’ and the suggested wording may not be clear enough.

All other recommendations had a high level of agreement ranging from 76.5% to 88.2% and therefore remained unchanged.

42.

Suspect cervical dystonia in adults who have persistent abnormalities of head or neck posture, with or without head tremor, especially if the symptom improves when the person touches their chin with their hand.

43.

Do not offer cervical imaging to evaluate suspected cervical dystonia in adults.

44.

Be aware that dystonia in adults can affect other parts of the body (for example, it can cause writer’s cramp or in-turned posture of the foot).

45.

Refer adults with suspected dystonia to have an assessment for diagnosis and possible botulinum toxin treatment.

46.

Be aware that antipsychotic and antiemetic medicines can trigger or exacerbate dystonia in adults.

Recommendation 42 – Cervical dystonia

Cervical dystonia is the most common type of dystonia and is characterised by a tendency of the neck to twist, flex, extend or be pulled laterally. Head tremor is a common feature of cervical dystonia; however, cervical dystonia can present without tremor. Painful lesions and nerve root irritation in the neck can also cause pain and muscle spasm but cervical dystonia can be painful, so the presence or absence of pain is not discriminatory. People with dystonia sometimes find that temporary relief from muscle spasm is achieved by touch or light pressure, classically to the chin. Postural abnormalities of the head and neck that could be due to cervical dystonia require a referral to a neurologist if sufficiently troublesome.

Recommendation 43 – Cervical imaging

Non-specialists may incorrectly assume that cervical nerve entrapment causes persistent distorted neck posture; thus, cervical imaging is inappropriately requested. The diagnosis of dystonia is based on clinical features rather than imaging. Neuroimaging is usually non-contributory. The delay in waiting for imaging results prolongs the time taken for people to access specialist treatment and incurs unnecessary medical costs. If imaging is appropriate, a neurologist will order it after referral.

Recommendation 44 – Dystonia

The muscle group affected with dystonia may cause involuntary muscle contractions resulting in a change in posture or distortion of a limb. Some focal dystonias are task-specific and only become apparent when that specific activity is undertaken, for example, writing in writer’s cramp. Others are characterised by involuntary contractions of the eyes, tongue, face, neck, trunk, limbs or larynx, which can be sustained or fluctuating (spasmodic). Other examples include oculogyric crisis, tongue twisting or protrusion and in-turning of the ankle. The committee was unable to capture every possibility within the recommendation and chose to couch this in general terms aimed at reminding healthcare professionals of the possibility of dystonia.

Recommendation 45 – Assessment for botulinum toxin treatment

Non-specialists may misinterpret distortions of neck and foot posture and not refer to neurology or inappropriately refer to orthopaedic specialists first. Idiopathic dystonia is the most common cause of such abnormalities and is responsive to treatment with botulinum toxin. Spasticity associated with stroke, structural lesions of the brain and spinal cord, and cerebral palsy are also sometimes responsive to botulinum toxin injections. Secondary dystonia (or acquired dystonia) can arise from specific underlying neurodegenerative conditions or medication. This requires a specialist review for assessment, as the relationship between medication and dystonia is complex and often the discontinuation of medication is not an effective solution by itself.

Recommendation 46 – Antipsychotic and antiemetic medication

A variety of movement disorders can occur as a side effect to anti-psychotic and some antiemetic (for example, metoclopramide, prochlorperazine) medications, typically within a few days of initiation of treatment. These include acute dystonic reactions, motor restlessness (akathisia) and parkinsonism. If this happens, the prescriber of the medication should review it and consider symptomatic treatment with anticholinergic agents or benzodiazepines. Tardive dyskinesias and dystonias are involuntary movements of the face, lips, tongue, trunk and limbs that occur after a delay following treatment with antipsychotic medication. These medications are used widely, and the possibility that they have caused dystonia may not be recognised, generating an inappropriate neurology referral. The committee considered that a recommendation pointing out the possibility of an iatrogenic cause might reduce some unnecessary referrals.

The committee was provided with estimates of the number of referrals to neurologists from 2015/16 and how much this cost the NHS. During 2015/16, there were 517,806 first-time neurological outpatient attendances. Using NHS reference costs where an outpatient visit cost £175 and a paediatric outpatient visit cost £285, first-time referrals to neurologists cost approximately £93 million in total, or £87 million for adults and £6 million for children. The committee used these estimates as a starting point for consideration of whether its recommendations caused additional referrals that would significantly cost impact the NHS.

The committee is recommending that diagnosis of dystonia should be based on clinical features rather than imaging. The reduction in imaging will create cost savings for the NHS without having a negative impact on health.

As dystonia can be treated, the committee felt that a strong recommendation concerning referral should be made, as this will have a positive impact on the individual’s quality of life. The committee also felt that the symptom is sometimes inappropriately referred to orthopaedic specialists first which, in this case, represents an inappropriate use of NHS resources. The suggested assessment of botulin toxin ensures that the individual is assessed and, if appropriate, initiated on the most appropriate treatment pathway in the most timely and cost-effective manner.

Targeted engagement exercise

As the committee anticipated, there was no contention with this set of recommendations and the large majority of those consulted agreed with them. The level of agreement ranged from 83–94.4 %. The recommendation on antipsychotic and antiemetic medication exacerbating dystonia received 72.2 % agreement from a total of 18 participants, which was slightly below the threshold (27.8% thought it needed revision). Comments were mainly requesting clarifications on why these drugs may exacerbate dystonia and what the prescriber could do pending the review by neurological services. The committee felt that this would be too much detail in a recommendation and therefore added further information to the relevant LETR section.

• adults, young people and children (>5 years)
• children (<5 years old) and babies

• alcohol use
• diabetes
• distribution of symptoms (for example, peripheral or particular nerve)
• duration of symptoms
• loss of reflexes
• pain
• periodicity (transience) and focality
• sensory loss
• vitamin deficiencies
• weakness.

• Sensitivity (%) and specificity (%)
• Area under the ROC curve (AUROC) – measure of predictive accuracy
• Positive and negative predictive values

• Adjusted odds ratios for the presence of the following conditions:

  • compression neuropathy (for example, carpal tunnel syndrome and Meralgia parasthetica)
  • demyelination
  • drug toxicity – chemotherapy, alcohol, platinum-based drugs
  • functional (hyperventilation)
  • mononeuropathy multiplex
  • peripheral neuropathy
  • radiculopathy
  • seizures
  • small fibre neuropathy
  • TIAs
  • tethering of the spinal cord.

47.

Assess sudden-onset transient unilateral numbness in adults in line with the NICE guideline on stroke and transient ischaemic attack in over 16s.

48.

Refer immediately adults with rapidly progressive (within hours to days) symmetrical numbness and weakness or imbalance to have a neurological assessment.

49.

Refer urgently adults with recurrent, brief (less than 2 minutes), fixed-pattern disturbances of sensation to have an assessment for epilepsy.

50.

Refer adults with persistent, distally predominant altered sensation in the limbs, and brisk deep tendon reflexes, to have an assessment for possible brain or spine disease.

51.

Suspect migraine with aura in adults who have sensory symptoms that occur with or without headache and:

• are fully reversible and
• develop over at least 5 minutes and
• last between 5 and 60 minutes.

For recommendations on diagnosing and managing migraine with aura, see the NICE guideline on headaches in over 12s.

52.

For adults with persistent, distally predominant (‘stocking’ or ‘glove and stocking’) altered sensation in the limbs and depressed deep tendon reflexes:

• be alert to the possibility of peripheral neuropathy and consider checking:

  • vitamin B12 deficiency
  • thyroid function
  • for evidence of coeliac disease in line with the NICE guideline on coeliac disease.
  • renal function
  • blood glucose
  • ESR (erythrocyte sedimentation rate)
  • alcohol consumption, using a tool such as AUDIT (Alcohol Use Disorders Identification Test), in line with the NICE guideline on alcohol-use disorders: diagnosis, assessment and management of harmful drinking and alcohol dependence
• if no causes of peripheral neuropathy are found, refer for neurological assessment.

53.

Be aware that, for adults who have been diagnosed with a functional neurological disorder by a specialist, recurrent numbness and tingling might be part of the disorder and the person might not need re-referral if there are no new neurological signs. New symptoms in adults who have been diagnosed with a functional neurological disorder by a specialist should be assessed as described in the relevant sections of this guideline.

54.

Advise adults with tingling and a diagnosis of functional neurological disorder that the tingling might fluctuate and evolve over time and could increase at times of stress.

55.

Refer in line with local pathways if symptoms of carpal tunnel syndrome are severe or persistent after initial management.

56.

Reassure adults with unilateral or bilateral numbness, tingling or pain in the distribution of the lateral cutaneous nerve of the thigh (meralgia paraesthetica) that the condition is benign and might improve spontaneously. Consider referring for pain management only if the symptoms are severe.

57.

Do not routinely refer adults with symptoms of cervical radiculopathy that have remained stable for 6 weeks or more unless:

• pain is not controlled with analgesics or
• the symptoms are disabling or
• one of the following factors is present:

  • age under 20
  • gait disturbance
  • clumsy or weak hands or legs
  • brisk deep tendon reflexes (triceps and lower limbs)
  • extensor plantar responses
  • new-onset disturbance of bladder or bowel function.

58.

Do not routinely refer adults with symptoms of lumbar radiculopathy that have remained stable for 6 weeks or more unless pain is not controlled with analgesics or symptoms are disabling, in line with the NICE guideline on low back pain and sciatica in over 16s..

59.

Do not routinely refer adults with recurrent episodes of tingling or sensory disturbance in the limbs that are present on waking from sleep and last less than 10 minutes.

Measures of diagnostic accuracy including sensitivity, specificity, positive predictive value, negative predictive value, ROC, AUC as well as adjusted odds ratios were all considered important outcomes to determine whether the signs and symptoms in the presence of sensory symptoms are indicative of a neurological condition that requires referral for a specialist assessment.

Sensitivity and specificity were considered to be of equal importance. Sensitivity was important because the consequences of missing a patient with a neurological condition would have serious implications that can lead to rapid deterioration of health or even death. Specificity was important because incorrectly diagnosing an individual may result in inappropriate administration of medications or treatments. Only adjusted odds ratios from studies that had conducted a multivariate analysis including the predictors of interest were considered.

Recommendation 47 – Sudden-onset loss of sensation

Sudden-onset unilateral transient sensory symptoms raise the possibility of stroke or TIA and require urgent assessment and possibly immediate referral (see NICE CG68).

Recommendation 48 – Rapidly progressive symmetrical numbness

Rapidly progressive symmetrical numbness may indicate the development of post infective polyneuropathy, a rapidly progressive syndrome that may result in respiratory failure. Immediate neurological assessment is required for respiratory support and treatment.

Recommendation 49 – Disturbances of sensation

Disturbances of sensation even without loss of consciousness may be a result of epileptic seizures. Although not the most common presentation of epilepsy, it is important not to miss this diagnosis and referral should follow the recommendations of NICE CG 137.

Recommendation 50 – Possible brain or spine disease

Sensory disturbance in the limbs can be caused by lesions in the central nervous system (brain and spinal cord), for example, those caused by multiple sclerosis or tumours. In contrast to sensory disturbance caused by damage to peripheral nerves, those caused by lesions of the brain or spinal cord characteristically produce exaggerated deep tendon reflexes and extensor plantar responses (rather than depressed deep tendon reflexes and flexor plantar responses as found in neuropathies). The presence of exaggerated deep tendon reflexes and extensor plantar responses should trigger referral in people with undiagnosed neurological disease.

Recommendation 51 – Migraine aura

Tingling and sensory distortion can occasionally be part of the aura of migraine and may be misconstrued as suggestive of stroke. Typical aura features include visual symptoms such as flickering lights, spots or lines, or partial loss of vision as well as numbness or tingling which usually spreads gradually from its starting point to adjacent body parts over a period of a few minutes, in contrast to stroke, which comes on very rapidly. Sensory migraine aura may be associated with speech and language disturbance. It is important to be aware of the possibility of migraine (see also CG150).

Recommendation 52 – Suspected neuropathy

Distally predominant sensory loss may be caused by nerve damage (neuropathy). This should be suspected if it has a ‘glove and stocking’ distribution covering the distal parts of the affected limbs and if the deep tendon reflexes are depressed. If reflexes are brisk, then neuropathy is very unlikely, and a central nervous system cause should be considered. The committee considered that a straightforward screening for common causes of neuropathy would facilitate direction of the patient to the most appropriate service, which may not be neurology in the first instance. If people have diabetes, gluten sensitivity or alcohol excess, they may need referral to the appropriate non-neurological specialist service. In other cases, neurological referral for diagnostic clarification will be appropriate.

Recommendation 53–54 – Recurrent numbness and tingling

Functional symptoms are complaints that are not primarily explained by physical or physiological abnormalities. They may mimic neurological disorders. Diagnosis may depend on exclusion of a medical explanation of the symptoms and require a high level of clinical expertise and judgement. Functional symptoms can complicate a medically explained illness and cause difficulties in diagnosis and delineation.

Transient sensory symptoms are common in functional neurological disorders. The committee considered that these might not require neurological assessment, although this will depend on the experience of the healthcare professional to whom the symptom is presented, and their certainty regarding the diagnosis. People with functional disorder may require medical or psychological support for their condition. The committee considered that people with functional disorder might benefit from an understanding that their symptoms are likely to fluctuate and evolve with time and acceptance of this may reduce the overall load on clinical services.

Recommendation 55 – Carpal tunnel syndrome

Carpal tunnel syndrome is caused by compression of the median nerve at the wrist, which produces pain in the arm, often at night, and sensory symptoms in the palm of the hand and sometimes hand weakness. It is a common condition and, providing the diagnosis is considered, a provisional diagnosis can be made without necessarily requiring referral to neurology for confirmation. Many regions have well-established management pathways for this common condition (which may avoid the involvement of neurological services) and referral should follow these local arrangements.

Recommendation 56 – Meralgia parasthetica

This common entrapment neuropathy gives rise to symptoms of numbness and pain in the outer aspects of the thigh. People with meralgia parasthetica should be reassured that the symptoms will usually improve with time and may benefit from weight loss and simple analgesia. Symptoms are largely self-limiting and do not normally require referral.

Recommendation 57 – Cervical radiculopathy

Compressive cervical radiculopathy is usually caused by disc herniation, osteophytes arising in cervical spondylosis, or a combination of the 2. Stable compressive radiculopathy can be managed conservatively in the first instance, with analgesia and physical therapy or exercise. It usually settles spontaneously within a few weeks. Referral is required if surgery is to be considered for progressive or unrelenting symptoms or if there are atypical features suggesting damage to the spinal cord (myelopathy). Urgent surgical referral should be considered if there are symptoms or signs of myelopathy or spinal cord involvement with, for example, gait disturbance, brisk deep tendon reflexes, extensor plantar responses or disturbance of bladder or bowel function. Clumsy or weak hands could indicate a high cervical or intrinsic cord lesion and again should be referred urgently for specialist assessment and imaging.

Recommendation 58 – Lumbar radiculopathy

This is a common condition of nerve root entrapment and usually settles spontaneously within a few weeks. Stable lumbar radiculopathy is usually managed conservatively and does not require referral unless there are progressive unrelenting or disabling symptoms or symptoms of conus or cauda equina involvement (for example, bladder or bowel dysfunction or perineal numbness in the case of cauda equina involvement). Urgent referral is required if surgery is to be considered or if there are signs of cauda equina compromise. For guidance on management of low back pain with or without sciatica, see NICE NG59.

Recommendation 59 – Transient tingling on waking from sleep

Transient tingling on waking is usually caused by nerve compression related to sleeping posture. It recovers rapidly and many people who experience this symptom will not report it. Those who do should be reassured that it does not represent a serious illness. It does not require referral to a neurologist.

The committee was provided with estimates of the number of referrals to neurologists from 2015/16 and their cost to the NHS. During 2015/16, there were 517,806 first-time neurological outpatient attendances. Using NHS reference costs showing that an outpatient visit cost £175 and a paediatric outpatient visit cost £285, first-time referrals to neurologists cost approximately £93 million in total, or £87 million for adults and £6 million for children. The committee used these estimates as a starting point for considering whether its recommendations caused additional referrals that would have a significant cost impact on the NHS.

The committee agreed that these recommendations were generally similar to current practice. Taken together, they would be likely to reduce the total number of referrals by reducing some current unnecessary referrals. This will consequently be cost saving.

60.

Offer advice on sleep hygiene to adults with insomnia.

61.

Do not routinely refer adults with insomnia, jerks on falling asleep or isolated brief episodes of sleep paralysis.

62.

Refer urgently adults with symptoms suggestive of new-onset epileptic seizures in sleep for neurological assessment in line with the NICE guideline on epilepsies.

63.

For adults with excessive sleepiness:

• use the Epworth score together with history of obstructive symptoms in sleep to assess the likelihood of sleep apnoea
• refer in accordance with local policy
• if appropriate, offer advice on weight reduction, alcohol consumption and smoking cessation, in line with NICE guidance on obesity, alcohol-use disorders, and smoking and tobacco.

64.

Refer adults with narcolepsy, with or without cataplexy, for neurological assessment.

65.

Consider referring adults with persistent symptoms suggestive of sleep behaviour disorders (such as agitated or violent movements that are more complex than a simple jerking motion) for neurological assessment.

Recommendation 60 – Sleep hygiene advice for insomnia

Difficulty sleeping is not usually a neurological problem and the committee also noted that most forms of insomnia do not require medical treatment. However, people with insomnia may be referred inappropriately to sleep clinics, which can place a strain on these services. Insomnia can be addressed by improved sleep hygiene, about which advice is easily accessible to non-specialists.

Recommendation 61 – Benign sleep phenomena

Brief involuntary movements in sleep (such as hypnic jerks, which happen when falling asleep and being jerked awake) are common. People with such symptoms should be reassured that they do not indicate an underlying neurological disease and do not need referral unless severe. Some people find sleep hygiene measures helpful in managing these symptoms Isolated brief episodes of sleep paralysis are also benign but should prompt enquiry about symptoms of narcolepsy.

Recommendation 62 – Sleep epilepsy

While seizures from sleep may remain unrecognised for long periods and may result in only minor injuries, the risk of sudden unexpected death in epilepsy is substantial in this group. The committee noted the existing NICE epilepsy guideline, which recommends that people who have had a seizure be referred after their first seizure to see an epileptic management specialist as soon as possible. This includes night time seizures. The committee therefore reflected the existing guidance in this recommendation.

Symptoms suggestive of nocturnal epilepsy include tonic clonic movements, stertorous breathing, tongue biting, urinary incontinence, postictal drowsiness, and confusion.

Recommendation 63 – Excessive sleepiness

The Epworth score is an appropriate, simple, well-established measure for screening people with excessive sleepiness.

Raising awareness amongst non-specialists of the possibility of obstructive sleep apnoea as a diagnosis should help to reduce inappropriate referrals to neurology. Local policies and pathways will generally already be in place for referral of sleep apnoea based on a person’s Epworth score, although the committee noted that local thresholds might vary. Some sleep disorders such as sleep apnoea and excessive sleepiness can be reduced with advice on weight reduction, and in some areas dietary advice is recommended as a first-line response. Limiting alcohol and smoking can also increase sleep quality. Clinicians should refer to national guidance regarding alcohol consumption and smoking cessation

Sleep services will identify narcolepsy if it is present, but the committee noted that good communication does not always exist between neurology and sleep services, which may create a disservice to those in need of care. Clinicians should refer according to local policies unless there is a strong suspicion that the person has narcolepsy or another serious neurological condition

Recommendation 64 – Narcolepsy with or without cataplexy

Although narcolepsy and cataplexy are rare conditions, the committee felt it was important to highlight these conditions to raise awareness amongst non-specialists that these could be potential diagnoses. Symptoms suggestive of narcolepsy include excessive daytime sleepiness, hypnagogic hallucinations, sleep paralysis and cataplexy. Cataplexy should be suspected in people complaining of transient muscle weakness triggered by emotion (for example, laughter, anger and grief).

Recommendation 65 – Sleep behaviour disorders

Sleep behaviour disorders vary in severity and on rare occasions can endanger life if they cause a person to undertake potentially harmful behaviours while asleep. People with complex and severe sleep behaviour disorders should be referred, but milder forms may not necessitate referral to neurology. The committee agreed that clinical judgement should be used to determine the appropriateness of a referral on an individual basis; therefore, the committee decided upon a recommendation to consider referral. The committee recognised that clinicians sometimes face pressure from people to refer, and a weaker recommendation may help to emphasise that milder sleep behaviour disorders do not always need to be referred.

People with REM sleep behaviour disorder (RBD) are at increased risk of future Parkinson’s disease and related neurodegenerative conditions. The committee noted that people with such sleep behaviour disorders may present at sleep clinics first but they will then be referred to neurology.

The committee was provided with estimates of the number of referrals to neurologists from 2015/16 and their cost to the NHS. During 2015/16, there were 517,806 first-time neurological outpatient attendances. Using NHS reference costs where an outpatient visit costs £175 and a paediatric outpatient visit costs £285, first-time referrals to neurologists cost approximately £93 million in total, or £87 million for adults and £6 million for children. The committee used these estimates as a starting point for considering whether its recommendations caused additional referrals that would have a significant cost impact on the NHS.

The committee agreed that the recommendations did not represent a change from current practice; therefore, the recommendations will not increase the number of referrals. The committee recommended referral in situations where there is a standard treatment pathway in place that will enable individuals to cope better with their symptoms. In addition, as sleep disorders can be chronic, the committee believed that appropriate timely referral and treatment may prevent future, unscheduled healthcare utilisation.

Targeted engagement exercise

Recommendations 60 and 61 received 100% agreement and recommendation 64 had 93.8% agreement. Therefore, the committee did not discuss these recommendations; they remain unchanged.

Recommendation 63 received 81.3% agreement. The participants highlighted that the Epworth score on its own should not be the sole criteria for referral. The committee felt that this was a simple test and that local policies which are generally already be in place use this score as a basis for referral. There was also some doubt about whether there was evidence to suggest that weight loss helps with sleep apnoea. However, the committee felt that in their experience this was helpful as a first-line response.

Recommendation 62 reached 75% agreement. However, 6.3% (1 participant) disagreed and 18.8% (3 participants) suggested revisions. The participants did not agree with the use of the term ‘nocturnal epilepsy’ stating that these types of seizures were not necessarily linked to particular time of day or night but occur with sleep. Therefore, the committee changed the wording to ‘epileptic seizures in sleep’. The participants also highlighted that people fulfilling these criteria are at high risk of sudden unexpected death from epilepsy (SUDEP) and therefore should be referred urgently. The committee agreed and changed the wording of the recommendations to reflect that.

Recommendation 64 reached 87.5% agreement but 6.3% (1 participant) disagreed stating that these people should be referred to sleep specialists. The committee felt that these people would be best seen for neurological assessment and therefore did not change the recommendation.

66.

Be aware that sudden-onset distortion of sense of smell or taste in adults is rarely associated with structural neurological abnormality and usually resolves within a few months.

67.

Refer adults with transient, repetitive smell or taste hallucinations to have a neurological assessment for epilepsy.

68.

Consider neuroimaging for adults with unexplained loss of sense of smell or taste that lasts more than 3 months.

69.

Do not routinely refer adults with loss of sense of smell or taste and normal neuroimaging.

70.

Do not routinely refer adults who lose their sense of smell or taste immediately after a head injury.

Recommendation 66 – Sudden-onset distortion of sense of smell or taste

Sudden-onset distortion of sense of smell or taste is usually idiopathic and unlikely to indicate the presence of a neurological condition. Idiopathic dysgeusia is particularly common in the first trimester of pregnancy, and it is also commonly seen in people undergoing chemotherapy.

The committee noted that there is an issue in current practice regarding excessive imaging and referral for this symptom. The committee hopes that reassuring non-specialists that neurological assessment is unnecessary will help to reduce the number of inappropriate referrals.

Recommendation 67 – Transient fixed-pattern taste or smell hallucinations

The committee discussed the presentation of recurrent positive olfactory symptoms (olfactory hallucinations). These are unlikely to be associated with a brain tumour but can be a manifestation of temporal lobe epilepsy auras (that is, focal seizures), particularly if they are brief and stereotyped and should prompt the search for a witness account of an accompanying loss of awareness or other feature suggestive of epilepsy. People with recurrent olfactory hallucinations should therefore be referred for a neurological assessment.

Recommendation 68 – Unexplained persistent loss of sense of smell or taste

Progressive loss of sense of smell or taste can be a rare presentation of an olfactory groove meningioma or frontal lobe tumour. The vast majority of people presenting with progressive loss of sense of smell or taste will not have a brain tumour but imaging should be considered if the symptom is unexplained and persistent. Referral to an Ear, Nose and Throat specialist, may be required to exclude a local cause of sensory loss first. The committee considered that a loss of sense of smell that did not appear to have a rhinological cause could not be attributed to normal aging or neurodegenerative disease and should trigger neuroimaging if it persisted beyond 3 months without sign of recovery. This symptom is of particular concern in younger people. The committee noted that urgent neuroimaging should be undertaken where progressive loss of sense of smell or taste presents with other neurological deficits such as headaches, memory dysfunction or personality change in line with cancer guidelines. It was also highlighted that progressive loss of sense of smell or taste in older people can be an accompaniment of neurodegenerative disorders affecting the olfactory bulb (for example, Parkinson’s disease) but neuroimaging is usually unhelpful in these circumstances.

Recommendation 69 – Loss of sense of smell or taste and normal neuroimaging

Loss or distortion of sense of smell is a common referral to neurological services, but it only rarely has a serious neurological cause. It could be age-related, associated with Parkinson’s or Alzheimer’s disease or caused as a side effect of treatments such as chemotherapy. The committee therefore believe that neuroimaging is usually not required; if neuroimaging has been performed and the results are normal, the committee considered that referral to neurology is not necessary.

Recommendation 70 – Loss of sense of smell or taste after a head injury

Loss of sense of smell or taste is extremely common after a head injury and, although distressing, does not warrant referral. Impaired olfactory function following a head injury is an indication of damage of the fine olfactory nerve connections as they pass through the cribriform plate. It does not correlate with post-traumatic amnesia and is not a marker of a more extensive brain injury. Post-traumatic anosmia is untreatable and usually permanent so referral for neurological assessment is unnecessary if this is the only symptom present.

The committee was provided with estimates of the number of referrals to neurologists from 2015/16 and their cost to the NHS. During 2015/16, there were 517,806 first-time neurological outpatient attendances. Using NHS reference costs where an outpatient visit cost £175 and a paediatric outpatient visit cost £285, first-time referrals to neurologists cost approximately £93 million in total, or £87 million for adults and £6 million for children. The committee used these estimates as a starting point for considering whether its recommendations caused additional referrals that would have a significant cost impact on the NHS.

There is a cost implication for recommending neuroimaging for progressive loss of sense of smell or taste; however, the NICE guideline on suspected cancer (NG12) already recommends urgent neuroimaging in the event of progressive, sub-acute loss of central neurological function (recommendation 1.9.1).

Overall, the committee agreed that the recommendations did not represent a change from current practice. These recommendations will not increase the number of referrals or the costs to the health service.

Targeted engagement exercise

There was a high level of agreement with this set of recommendations, ranging from 76.5% to 94.1%. Although there was a very low level of disagreement with some recommendations, the committee felt that they raised important issues and warranted some consideration and discussion.

The level of disagreement with recommendation 70 was 5.9% (1 participant). It was felt that not referring people for imaging who have had a recent head injury may miss otherwise unidentified frontal fractures. The committee considered this but did not think it warranted a change in the recommendation when anosmia is the only symptom.

The level of disagreement with recommendation 68 was also 5.9% (1 participant). There was concern that this may lead to an increase in referrals for imaging. The committee believed this would not be the case, as this is in line with the suspected cancer guideline and the cost implications have already been considered. There was no change to the recommendation but further detail was provided in the trade-off between clinical benefits and costs section.

The level of disagreement with recommendation 69 was also 11.8% (2 participants). One participant expressed concern that this recommendation also indirectly suggests referral for imaging. The committee had already addressed this in recommendation 68 so no further action was taken. The other reason for disagreement was that this recommendation may miss people with neurodegenerative disorders, but the committee felt that referring people with loss of smell and no other neurological features was unnecessary.

71.

Refer urgently adults with sudden-onset speech or language disturbance to have an assessment for a vascular event, in line with local stroke pathways and following the recommendations on prompt recognition of symptoms of stroke and TIA in the NICE guideline on stroke and transient ischaemic attack in over 16s.

72.

For adults with progressive slurred or disrupted speech:

• refer for an assessment for neuromuscular disorders, in line with the recommendations on recognition and referral in the NICE guideline on motor neurone disease
• refer urgently if there is any evidence of swallowing impairment
• refer immediately if there is breathlessness at rest or when lying flat.

73.

Consider referring adults with isolated and unexplained persistent dysphonia (a quiet, hoarse or wobbly voice) to have an assessment for laryngeal dystonia (involuntary contractions of the vocal cords) if hoarseness caused by structural abnormality or malignancy has been excluded by ear, nose and throat examination.

74.

Be aware that persistent dysphonia in adults may be a presenting symptom of a neurological condition such as Parkinson’s disease. For recommendations on the diagnosis and management of Parkinson’s disease, see the NICE guideline on Parkinson’s disease in adults.

75.

Be aware that anxiety disorder and functional neurological disorders are the most common causes of minor word-finding difficulties in adults and people with a diagnosis of anxiety disorder or functional neurological disorder made by a specialist might not need a referral.

Recommendation 71 – Sudden onset of speech disturbance

New onset transient speech disturbance could indicate a vascular event such as a transient ischaemic attack. The NICE stroke guideline (CG68) provides recommendations for recognition and referral.

Recommendation 72 – Progressive slurred speech

Progressive disruption of speech over a period of months could indicate motor neurone disease (MND) because it is often associated with swallowing difficulties. Myasthenia gravis can also present with progressive slurred speech and swallowing difficulties; although, unlike in MND, these fluctuate significantly, are usually worse after chewing and towards the end of the day and are often accompanied by double vision or drooping of the eyelids. Other causes of progressive slurred speech include ataxia, extrapyramidal and dementing disorders.

Although MND prognosis is not influenced greatly by early diagnosis, the committee considered that a prompt referral should be made, partly to consider other diagnoses such as myasthenia gravis, which is highly treatable, and also to enable people with MND to access vital support services to maintain their independence and quality of life for as long as possible, including services such as physiotherapy, occupational therapy and respiratory support. It also enables people to make provision to prepare for the rapid progression of the disease, such as accessing home or vehicular adaptations, communication aids, or services such as voice banking which enables people to record their own voice for when they lose the ability to speak. In addition, there are safety considerations because people with bulbar difficulties may also run into problems with chewing and swallowing. The committee noted the recommendations on recognition and referral in the NICE guideline on MND (NG42) and agreed to cross-refer.

People with slowly progressive speech disturbance should be kept under review by the referrer until they are seen by a neurologist. Occasionally, people’s conditions can deteriorate rapidly (over days to weeks), and these people may require an urgent hospital admission.

Recommendation 73 – Persistent dysphonia

People with an unexplained new onset hoarse voice over the age of 45 years, particularly in smokers, should be referred urgently to ENT for exclusion of malignancy (see NICE NG12), and the committee agreed that this is an important first step.

In the absence of structural abnormalities identified by ENT services, dysphonia is commonly mislabelled as a functional neurological disorder. However, a quiet or wobbly voice can be a symptom of a neurological condition such as Parkinson’s or laryngeal dystonia. When dysphonia is accompanied by dysarthria or dysphagia, motor neurone disease and myasthenia gravis should be considered

People who present with dysphonia are usually appropriately referred to ENT first for exclusion of ENT causes. In the absence of laryngeal pathology and signs of parkinsonism, laryngeal dystonia should be considered and, depending on local pathways, referred to specialist ENT or neurology for diagnosis and consideration of botulinum toxin treatment.

The committee felt it was important to highlight potential neurological causes for non-specialists to bear in mind when assessing people whose symptoms persist despite no obvious problem being found on ENT examination. Examination for dysarthria and of the lower cranial nerves may be revealing. Dysphonia can be an early presenting symptom of a significant neurological condition such as Parkinson’s. In addition, a prompt diagnosis of laryngeal dystonia – while not an indicator of a more serious neurological condition – could increase the quality of life for those who present with the disabling voice condition. When dysphonia is accompanied by dysarthria or dysphagia, motor neurone disease and myasthenia gravis should be considered.

Recommendation 74 – Parkinson’s disease

Parkinson’s disease can occasionally present with dysphonia, typically a quiet voice. This is not appreciated by all clinicians, and the committee considered that it was appropriate to raise awareness of this as a presenting symptom to facilitate more prompt diagnosis. If this symptom is dominant, other features of Parkinson’s disease should be sought, including asymmetric slowness of rapid repetitive movements, a history of anosmia or REM behaviour sleep disorder. A referral for confirmation of the diagnosis and management advice should be made to a neurologist or a care of the elderly physician, in line with local pathways.

Recommendation 75 – Minor word-finding difficulties

Functional symptoms are complaints that are not primarily explained based on physical or physiological abnormalities. They may mimic neurological disorders. Diagnosis may depend on exclusion of a medical explanation of the symptoms and require a high level of clinical expertise and judgement. Functional symptoms can complicate a medically explained illness and cause difficulties in diagnosis and delineation. Minor word-finding difficulties are a very common presentation in functional neurological disorders, including anxiety states. The symptom may wax and wane and is often more apparent to the patient than to observers. The committee agreed that anxiety disorder should be considered in someone with word-finding difficulties that are not progressive or accompanied by other symptoms. Referral for psychological support may be appropriate. Common symptoms that may coincide with word-finding difficulties in people with anxiety include migraine, headaches, tingling, fatigue, memory and concentration difficulties. People may be unaware that they are experiencing functional symptoms or that functional symptoms may be exacerbated at times of stress.

The committee noted that isolated word-finding difficulties can rarely be the presenting symptom of semantic dementias, such as primary progressive aphasia. These are extremely rare conditions that would be distinctive because, rather than fluctuating, there are progressive language difficulties, for example, in reading and comprehension. In due course, impairments will become apparent in other cognitive domains, such as memory and orientation, suggestive of Alzheimer’s disease, or behaviour, indicating possible frontotemporal dementia.

Word-finding difficulty causes undue distress in some cases, and the committee decided that a recommendation to raise awareness of functional disorder as a common cause might help GPs reassure patients and prevent some inappropriate referrals.

The committee was provided with estimates of the number of referrals to neurologists from 2015/16 and their cost to the NHS. During 2015/16, there were 517,806 first-time neurological outpatient attendances. Using NHS reference costs where an outpatient visit cost £175 and a paediatric outpatient visit cost £285, the first-time referrals to neurologists cost approximately £93 million in total, or £87 million for adults and £6 million for children. The committee used these estimates as a starting point for considering whether its recommendations caused additional referrals that would have a significant cost impact on the NHS.

The committee agreed that these recommendations did not represent a change from current best practice, and that, viewed along with the rest of this guideline, the recommendations will not increase the total number of referrals or NHS costs.

The committee believed that the recognition of and potential referral for laryngeal dystonia would represent a cost-effective use of resources in some cases as potential treatment options can improve quality of life and may also highlight a potential neurological cause. Therefore, referral should be considered on an individual basis for these people.

Targeted engagement exercise

There was a high level of agreement with this set of recommendations ranging from 75–93.8%. Therefore, the recommendations remained mostly unchanged except for minor refinement of the wording to make them clearer.

76.

Do not routinely refer adults with tics (involuntary movements that can be temporarily suppressed at the expense of mounting inner tension) unless the tics are troublesome or accompanied by additional progressive neurological symptoms.

77.

Consider referring adults with a tic disorder for psychological therapy if the disorder distresses them.

78.

Consider referring adults who have completed psychological therapy for a tic disorder to have a neurological assessment if their symptoms are severe and the disorder continues to distress them, but tell the person that:

• there are not many medicines available to treat a tic disorder
• the medicines that are available don’t always work very well and can have serious side effects.

79.

Do not routinely refer adults with isolated involuntary movements of the eyelid unless the movements:

• cause involuntary tight eye closure of both eyes (blepharospasm) or
• have persisted for more than 3 months.

80.

In adults with involuntary movements of the face, neck, limbs or trunk that cannot be temporarily suppressed by mental concentration:

• refer for neurological assessment or
• refer to neurology or an eye clinic according to local provision, if the person has involuntary tight eye closure of both eyes (blepharospasm).

81.

Do not routinely refer people with small involuntary muscular twitches (fasciculations) unless these are associated with muscle wasting and weakness or muscle rigidity.

Recommendation 76 – Transiently suppressible movements

Tic disorder is a relatively common disorder in adults and mild forms frequently remain undiagnosed. Isolated tic disorder is benign and not indicative of neurodegenerative disease. Treatment and management options are limited. The drug treatment of the tic requires taking medication of limited efficacy and with significant side effects, for example, anti-dopaminergic drugs such as risperidone, tetrabenazine or botulinum toxin injections. There is no neurosurgical option.

Tics should be differentiated from other fleeting movements such as hemifacial spasm (a condition caused by irritation of the facial nerve producing simultaneous contraction of muscles in half the face) and blepharospasm (a form of dystonia condition causing involuntary persistent bilateral eye closure).

The committee noted that, once they receive a clear explanation, adults most often decide they are better off without treatment, unless it has a severe impact on their quality of life (for example, reduced social interaction, job performance or increased pain). Tics are generally managed most effectively by controlling stress. There is little value in referring to secondary care except for confirmation of the diagnosis. The committee therefore agreed upon a recommendation of ‘do not refer’ providing there are no other neurological signs or symptoms.

Recommendation 77 – Referral for psychological therapy

Anxiety and distress is a common comorbidity of tic disorder. The committee noted that, in these circumstances, a psychology referral may be appropriate, and clinical judgement should be used to determine appropriate circumstances. Treatment may reduce the psychological impact of the disorder including help with associated co-morbid psychiatric conditions such as obsessive compulsive and anxiety disorder. For particularly disabling tics, and where this is available, psychologists may be able to offer habit-reversal therapy, for which some efficacy has been demonstrated.

Recommendation 78 – Following psychological therapy

In people with very severe or socially disabling tics, medical treatment can occasionally be justified. A neurological referral would then be appropriate in order to explore further treatment options. It would be appropriate to warn the patient that there are few drug options, they are of limited efficacy, and involve a risk of serious side effects.

Recommendation 79 – Involuntary movements confined to the eyelids

Conditions treatable with botulinum toxin injections, such as blepharospasm, involuntary tight closure of both eyes, and hemifacial spasm, where there is distortion of other parts of the face on the same side as the eye closure, are often mistaken for tics. In contrast to tics, these are outside any voluntary control and cannot be suppressed.

Minor, insuppressible and flickering movements confined to one eyelid (myokymia) are a widely experienced self-limiting normal phenomenon, even more common with sleep-deprivation and stress. These involuntary eyelid movements can sometimes continue for months in which case, if they are distressing, a referral is justifiable.

Recommendation 80 – Involuntary movements that cannot be suppressed

Involuntary movements that cannot be suppressed could represent a number of neurological disorders, for example hemifacial spasm, tremor, chorea, epileptic myoclonus or tight eye closure of both eyes (blepharospasm). These movement disorders require a neurological assessment and diagnosis, and may require treatment in a movement disorder clinic to relieve symptoms.

Tight eye closure (blepharospasm) can be a form of dystonia in adults. It may be mild, but often causes significant disability through interference with vision. Treatment options, such as botulinum toxin injections, are available, which can be offered following a neurological or ophthalmological assessment.

Recommendation 81 – Involuntary muscular contractions (fasciculations) associated with muscle wasting and weakness or muscle rigidity

Muscle fasciculation is usually an innocent phenomenon, and is especially common in the calf muscles. As it is sometimes an indicator of serious underlying neuromuscular disease, it may cause patient concern. When indicating neuromuscular disease it is accompanied by weakness, muscle wasting or muscular rigidity (stiffness). In the absence of such features, it is usually appropriate to reassure the patient and review after an interval.

The committee was provided with estimates of the number of referrals to neurologists from 2015/16 and their cost to the NHS. During 2015/16, there were 517,806 first-time neurological outpatient attendances. Using NHS reference costs where an outpatient visit cost £175 and a paediatric outpatient visit cost £285, first-time referrals to neurologists cost approximately £93 million in total, or £87 million for adults and £6 million for children. The committee used these estimates as a starting point for considerations of whether its recommendations caused additional referrals that would have a significant cost impact on the NHS.

The committee acknowledged that tic disorders are a common symptom that is presented in general practice. The committee believe that the recommendations would reduce overall referrals to neurology and would ensure that only those who stand to benefit from further interventions are referred. This would reduce costs to the NHS without compromising treatment for those who could benefit from treatment.

Targeted engagement exercise

Of the 18 participants who commented on recommendation 76, 77.8% agreed with the recommendation where as 5.6% (1 participant) disagreed, as it did not seem clear why referral for psychological therapy had been recommended. The committee agreed that this is an appropriate therapy with some evidence of efficacy and therefore did not make any changes to the recommendation.

Recommendation 79 had a relatively lower level of agreement at 66.7% but only minor changes to the wording of the recommendation was required. The committee agreed to add ‘blepharospasm’ that ‘persistent for more than 3 months’ to the recommendation based on the targeted engagement exercise comments.

All other recommendations in this section had a high level of agreement ranging 77.8–94.4% and therefore did not require any changes to the recommendations. The committee discussed some of the helpful comments that were made and used them to elaborate further on the rationales provided.

82.

Refer adults with suspected parkinsonian tremor, other asymmetric tremor, or tremor associated with stiffness, slowness, balance problems or gait disorders for neurological assessment before treatment in line with the NICE guideline on Parkinson’s disease in adults.

83.

Suspect essential tremor in an adult with symmetrical postural tremor and no symptoms of parkinsonism.

84.

In adults with suspected essential tremor:

• review regular medication
• check thyroid function
• assess alcohol consumption using a tool such as AUDIT (Alcohol Use Disorders Identification Test), in line with the NICE guideline on alcohol-use disorders: diagnosis, assessment and management of harmful drinking and alcohol dependence.

Refer for neurological assessment only if the symptoms are disabling and first-line treatment as specified in the British national formulary is ineffective or not tolerated.

85.

Consider referring adults with troublesome tremor of the head to a movement disorder clinic.

Measures of diagnostic accuracy including sensitivity, specificity, positive predictive value, negative predictive value, ROC, AUC as well as adjusted odds ratios were all considered important outcomes to determine whether the signs and symptoms in the presence of tremor are indicative of a neurological condition that requires referral for a specialist assessment.

Sensitivity and specificity were considered to be of equal importance. Sensitivity was important because the consequences of missing a patient with a neurological condition would have serious implications, which can lead to rapid deterioration of health or even death. Specificity was important because incorrectly diagnosing an individual may result in inappropriate administration of medications or treatments. Only adjusted odds ratios from studies that had conducted a multivariate analysis including the predictors of interest were considered.

Recommendation 82 – Suspected parkinsonian tremor

A unilateral or predominantly unilateral tremor, especially if more prominent at rest and accompanied by slowness of rapid alternating movements, is particularly suggestive of parkinsonism. People who exhibit suspected parkinsonian tremor should see a neurologist for assessment and diagnosis, as disability may be progressive, the diagnosis may have a lifelong impact, and the person may require on-going medical and social support. There are other causes of asymmetric tremor, but the committee felt that referral of these people was required in order to differentiate from Parkinson’s.

Recommendation 83 – Symmetrical postural tremor

Essential tremor is an action or postural tremor of limbs, and sometimes head, of variable amplitude that tends to worsen with stress and deteriorate gradually with age. It is largely bilateral and not associated with any alteration of muscle tone or slowness of movement. It is very common and poorly responsive to drug treatment. People who show signs of essential tremor but do not demonstrate signs of a parkinsonian tremor do not need to be referred to neurology. The committee stressed the need to reduce referrals to neurology for people with essential tremor, as GPs can administer or prescribe first-line treatments without input from secondary care. Exaggerated physiological tremor and essential tremor are on a continuum and can be offered the same symptomatic treatment.

Recommendation 84 – Suspected essential tremor

Some medicines, such as sodium valproate, and thyrotoxicosis can cause tremor. Excessive alcohol use can be associated with tremor. Essential tremor may be transiently ameliorated by alcohol and some people use alcohol to self-medicate. Clinicians require a comprehensive medication and alcohol-intake history to assess people with essential tremor. Medical treatment of tremor is generally of low efficacy, and referral should be considered only if the tremor is significantly disabling. For people with severe essential tremor, neurosurgical treatment may be an option after review by a neurologist.

Recommendation 85 – Troublesome head tremor

Head tremor (titubation) is a common disorder, which responds poorly to drug treatment. The condition can be socially disabling. Botulinum toxin has been shown to help control these tremors and is now widely used in the UK. People with troublesome head tremor should be referred to a neurology movement disorder clinic to be assessed for suitability for this treatment.

The committee was provided with estimates of the number of referrals to neurologists from 2015/16 and their cost to the NHS. During 2015/16, there were 517,806 first-time neurological outpatient attendances. Using NHS reference costs showing that an outpatient visit cost £175 and a paediatric outpatient visit cost £285, first-time referrals to neurologists cost approximately £93 million in total, or £87 million for adults and £6 million for children. The committee used these estimates as a starting point for the considering whether its recommendations caused additional referrals that would have a significant cost impact on the NHS.

The committee agreed that these recommendations do not represent a change from current best practice, and that, viewed along with the rest of this guideline, the recommendations should not increase the total number of referrals or NHS costs.