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Intellectual developmental disorder with paroxysmal dyskinesia or seizures(IDDPADS)

MedGen UID:
1727046
Concept ID:
C5436894
Disease or Syndrome
Synonyms: IDDPADS; INTELLECTUAL DEVELOPMENTAL DISORDER WITH PAROXYSMAL DYSKINESIA OR SEIZURES
 
Gene (location): PDE2A (11q13.4)
 
Monarch Initiative: MONDO:0030900
OMIM®: 619150

Definition

Intellectual developmental disorder with paroxysmal dyskinesia or seizures (IDDPADS) is an autosomal recessive complex neurologic disorder characterized by global developmental delay with impaired intellectual development and language delay. In addition, most patients develop a paroxysmal hyperkinetic movement disorder in the first months or years of life manifest as sudden falls or backward propulsion, eye or head deviation, and dystonic limb posturing followed by chorea and dyskinetic movements. The episodes are pharmacoresistant to anticonvulsant medication. EEG may show interictal abnormalities, but are usually not consistent with epilepsy. However, some patients may also develop epileptic seizures or only have seizures without a movement disorder (summary by Doummar et al., 2020). [from OMIM]

Clinical features

From HPO
Aggressive behavior
MedGen UID:
1375
Concept ID:
C0001807
Individual Behavior
Behavior or an act aimed at harming a person, animal, or physical property (e.g., acts of physical violence; shouting, swearing, and using harsh language; slashing someone's tires).
Bruxism
MedGen UID:
676
Concept ID:
C0006325
Mental or Behavioral Dysfunction
Bruxism is characterized by the grinding of the teeth including the clenching of the jaw and typically occur during sleep, but also can occur while the affected individual is awake.
Cerebellar ataxia
MedGen UID:
849
Concept ID:
C0007758
Disease or Syndrome
Cerebellar ataxia refers to ataxia due to dysfunction of the cerebellum. This causes a variety of elementary neurological deficits including asynergy (lack of coordination between muscles, limbs and joints), dysmetria (lack of ability to judge distances that can lead to under- or overshoot in grasping movements), and dysdiadochokinesia (inability to perform rapid movements requiring antagonizing muscle groups to be switched on and off repeatedly).
Chorea
MedGen UID:
3420
Concept ID:
C0008489
Disease or Syndrome
Chorea (Greek for 'dance') refers to widespread arrhythmic involuntary movements of a forcible, jerky and restless fashion. It is a random-appearing sequence of one or more discrete involuntary movements or movement fragments. Movements appear random because of variability in timing, duration or location. Each movement may have a distinct start and end. However, movements may be strung together and thus may appear to flow randomly from one muscle group to another. Chorea can involve the trunk, neck, face, tongue, and extremities.
Dystonic disorder
MedGen UID:
3940
Concept ID:
C0013421
Sign or Symptom
An abnormally increased muscular tone that causes fixed abnormal postures. There is a slow, intermittent twisting motion that leads to exaggerated turning and posture of the extremities and trunk.
Intellectual disability, moderate
MedGen UID:
7680
Concept ID:
C0026351
Mental or Behavioral Dysfunction
Moderate mental retardation is defined as an intelligence quotient (IQ) in the range of 35-49.
Seizure
MedGen UID:
20693
Concept ID:
C0036572
Sign or Symptom
A seizure is an intermittent abnormality of nervous system physiology characterized by a transient occurrence of signs and/or symptoms due to abnormal excessive or synchronous neuronal activity in the brain.
Motor stereotypies
MedGen UID:
21318
Concept ID:
C0038271
Individual Behavior
Use of the same abnormal action in response to certain triggers or at random. They may be used as a way to regulate one's internal state but must otherwise have no apparent functional purpose.
Falls
MedGen UID:
39084
Concept ID:
C0085639
Finding
A sudden movement downward, usually resulting in injury.
EEG abnormality
MedGen UID:
56235
Concept ID:
C0151611
Finding
Abnormality observed by electroencephalogram (EEG), which is used to record of the brain's spontaneous electrical activity from multiple electrodes placed on the scalp.
Inappropriate laughter
MedGen UID:
98407
Concept ID:
C0424304
Finding
Laughing that may be excessive and/or inappropriate in context (e.g., laughing at a funeral while others are crying).
Delayed speech and language development
MedGen UID:
105318
Concept ID:
C0454644
Finding
A degree of language development that is significantly below the norm for a child of a specified age.
Global developmental delay
MedGen UID:
107838
Concept ID:
C0557874
Finding
A delay in the achievement of motor or mental milestones in the domains of development of a child, including motor skills, speech and language, cognitive skills, and social and emotional skills. This term should only be used to describe children younger than five years of age.
Poor coordination
MedGen UID:
107874
Concept ID:
C0563243
Finding
Focal-onset seizure
MedGen UID:
199670
Concept ID:
C0751495
Disease or Syndrome
A focal-onset seizure is a type of seizure originating within networks limited to one hemisphere. They may be discretely localized or more widely distributed, and may originate in subcortical structures.
Paroxysmal dyskinesia
MedGen UID:
156242
Concept ID:
C0752210
Disease or Syndrome
Episodic bouts of involuntary movements with dystonic, choreic, ballistic movements, or a combination thereof. There is no loss of consciousness during the attacks.
Insomnia
MedGen UID:
214589
Concept ID:
C0917801
Sign or Symptom
Persistent difficulty initiating or maintaining sleep.
Focal motor status epilepticus
MedGen UID:
1716066
Concept ID:
C1396824
Disease or Syndrome
Status epilepticus with focal motor signs originating within networks limited to one hemisphere. Involves musculature in any form. The motor event could consist of an increase (positive) or decrease (negative) in muscle contraction to produce a movement.
Developmental regression
MedGen UID:
324613
Concept ID:
C1836830
Disease or Syndrome
Loss of developmental skills, as manifested by loss of developmental milestones.
Ventriculomegaly
MedGen UID:
480553
Concept ID:
C3278923
Finding
An increase in size of the ventricular system of the brain.
Axial hypotonia
MedGen UID:
342959
Concept ID:
C1853743
Finding
Muscular hypotonia (abnormally low muscle tone) affecting the musculature of the trunk.
Microcephaly
MedGen UID:
1644158
Concept ID:
C4551563
Finding
Head circumference below 2 standard deviations below the mean for age and gender.
Facial grimacing
MedGen UID:
65891
Concept ID:
C0234853
Finding
Facial grimacing is characterized by a distorted, distressed look. The brow is more wrinkled, as is the area around the mouth. Eyes may be squeezed shut.

Professional guidelines

PubMed

Charalambous M, Muñana K, Patterson EE, Platt SR, Volk HA
J Vet Intern Med 2024 Jan-Feb;38(1):19-40. Epub 2023 Nov 3 doi: 10.1111/jvim.16928. PMID: 37921621Free PMC Article
Zuberi SM, Wirrell E, Yozawitz E, Wilmshurst JM, Specchio N, Riney K, Pressler R, Auvin S, Samia P, Hirsch E, Galicchio S, Triki C, Snead OC, Wiebe S, Cross JH, Tinuper P, Scheffer IE, Perucca E, Moshé SL, Nabbout R
Epilepsia 2022 Jun;63(6):1349-1397. Epub 2022 May 3 doi: 10.1111/epi.17239. PMID: 35503712
Abboud H, Probasco JC, Irani S, Ances B, Benavides DR, Bradshaw M, Christo PP, Dale RC, Fernandez-Fournier M, Flanagan EP, Gadoth A, George P, Grebenciucova E, Jammoul A, Lee ST, Li Y, Matiello M, Morse AM, Rae-Grant A, Rojas G, Rossman I, Schmitt S, Venkatesan A, Vernino S, Pittock SJ, Titulaer MJ; Autoimmune Encephalitis Alliance Clinicians Network
J Neurol Neurosurg Psychiatry 2021 Jul;92(7):757-768. Epub 2021 Mar 1 doi: 10.1136/jnnp-2020-325300. PMID: 33649022Free PMC Article

Recent clinical studies

Etiology

Ziobro J, Shellhaas RA
Semin Neurol 2020 Apr;40(2):246-256. Epub 2020 Mar 6 doi: 10.1055/s-0040-1702943. PMID: 32143234
Shellhaas RA
Handb Clin Neurol 2019;162:347-361. doi: 10.1016/B978-0-444-64029-1.00017-5. PMID: 31324320
Gavvala JR, Schuele SU
JAMA 2016 Dec 27;316(24):2657-2668. doi: 10.1001/jama.2016.18625. PMID: 28027373
Patterson JL, Carapetian SA, Hageman JR, Kelley KR
Pediatr Ann 2013 Dec;42(12):249-54. doi: 10.3928/00904481-20131122-09. PMID: 24295158
Haut SR
Epilepsy Behav 2006 Feb;8(1):50-5. Epub 2005 Oct 24 doi: 10.1016/j.yebeh.2005.08.018. PMID: 16246629

Diagnosis

Wirrell E
Continuum (Minneap Minn) 2022 Apr 1;28(2):230-260. doi: 10.1212/CON.0000000000001074. PMID: 35393959
Vercueil L
Rev Neurol (Paris) 2022 Sep;178(7):654-658. Epub 2022 Feb 9 doi: 10.1016/j.neurol.2022.01.006. PMID: 35148906
Moosavi R, Swisher CB
Semin Neurol 2020 Dec;40(6):595-605. Epub 2020 Nov 5 doi: 10.1055/s-0040-1719075. PMID: 33155185
Ziobro J, Shellhaas RA
Semin Neurol 2020 Apr;40(2):246-256. Epub 2020 Mar 6 doi: 10.1055/s-0040-1702943. PMID: 32143234
Bollig KJ, Jackson DL
Obstet Gynecol Clin North Am 2018 Jun;45(2):349-367. doi: 10.1016/j.ogc.2018.02.001. PMID: 29747735

Therapy

Silbergleit R
Epilepsy Behav 2023 Apr;141:109051. Epub 2022 Dec 26 doi: 10.1016/j.yebeh.2022.109051. PMID: 36577548
von Wrede R, Helmstaedter C, Surges R
Clin Drug Investig 2021 Mar;41(3):211-220. Epub 2021 Feb 9 doi: 10.1007/s40261-021-01003-y. PMID: 33559102Free PMC Article
Moosavi R, Swisher CB
Semin Neurol 2020 Dec;40(6):595-605. Epub 2020 Nov 5 doi: 10.1055/s-0040-1719075. PMID: 33155185
Hoy KE, Fitzgerald PB
Expert Rev Med Devices 2011 Nov;8(6):723-32. doi: 10.1586/erd.11.55. PMID: 22029469
Glass HC, Wirrell E
J Child Neurol 2009 May;24(5):591-9. Epub 2009 Feb 13 doi: 10.1177/0883073808327832. PMID: 19218527

Prognosis

Grünewald R
Br J Hosp Med (Lond) 2019 Nov 2;80(11):652-657. doi: 10.12968/hmed.2019.80.11.652. PMID: 31707888
Williams AM, Park SH
CNS Drugs 2015 Feb;29(2):101-11. doi: 10.1007/s40263-014-0222-y. PMID: 25537107
Patterson JL, Carapetian SA, Hageman JR, Kelley KR
Pediatr Ann 2013 Dec;42(12):249-54. doi: 10.3928/00904481-20131122-09. PMID: 24295158
Judge BS, Rentmeester LL
Psychiatr Clin North Am 2013 Jun;36(2):245-60. Epub 2013 Apr 11 doi: 10.1016/j.psc.2013.02.004. PMID: 23688690
Martindale JL, Goldstein JN, Pallin DJ
Emerg Med Clin North Am 2011 Feb;29(1):15-27. doi: 10.1016/j.emc.2010.08.002. PMID: 21109099

Clinical prediction guides

Baud MO, Proix T, Rao VR, Schindler K
Curr Opin Neurol 2020 Apr;33(2):163-172. doi: 10.1097/WCO.0000000000000798. PMID: 32049738
Debicki DB
Seizure 2017 Jul;49:69-73. Epub 2017 Apr 25 doi: 10.1016/j.seizure.2017.03.001. PMID: 28532713
van Mierlo P, Papadopoulou M, Carrette E, Boon P, Vandenberghe S, Vonck K, Marinazzo D
Prog Neurobiol 2014 Oct;121:19-35. Epub 2014 Jul 8 doi: 10.1016/j.pneurobio.2014.06.004. PMID: 25014528
Stacey W, Le Van Quyen M, Mormann F, Schulze-Bonhage A
Epilepsy Res 2011 Dec;97(3):243-51. Epub 2011 Aug 31 doi: 10.1016/j.eplepsyres.2011.07.012. PMID: 21885253
So EL
J Clin Neurophysiol 2010 Aug;27(4):227-8. doi: 10.1097/WNP.0b013e3181eaa60e. PMID: 20634714

Recent systematic reviews

Pressler RM, Abend NS, Auvin S, Boylan G, Brigo F, Cilio MR, De Vries LS, Elia M, Espeche A, Hahn CD, Inder T, Jette N, Kakooza-Mwesige A, Mader S, Mizrahi EM, Moshé SL, Nagarajan L, Noyman I, Nunes ML, Samia P, Shany E, Shellhaas RA, Subota A, Triki CC, Tsuchida T, Vinayan KP, Wilmshurst JM, Yozawitz EG, Hartmann H
Epilepsia 2023 Oct;64(10):2550-2570. Epub 2023 Sep 1 doi: 10.1111/epi.17745. PMID: 37655702
Steinert T, Fröscher W
Pharmacopsychiatry 2018 Jul;51(4):121-135. Epub 2017 Aug 29 doi: 10.1055/s-0043-117962. PMID: 28850959
Whelan H, Harmelink M, Chou E, Sallowm D, Khan N, Patil R, Sannagowdara K, Kim JH, Chen WL, Khalil S, Bajic I, Keval A, Greydanus D
Dis Mon 2017 Jan;63(1):5-23. Epub 2017 Jan 12 doi: 10.1016/j.disamonth.2016.12.001. PMID: 28089358
Mewasingh LD
BMJ Clin Evid 2014 Jan 31;2014 PMID: 24484859Free PMC Article
Beghi E, Carpio A, Forsgren L, Hesdorffer DC, Malmgren K, Sander JW, Tomson T, Hauser WA
Epilepsia 2010 Apr;51(4):671-5. Epub 2009 Sep 3 doi: 10.1111/j.1528-1167.2009.02285.x. PMID: 19732133

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