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Cerebral cortical hemiatrophy

MedGen UID:
867763
•Concept ID:
C4022153
•
Disease or Syndrome
HPO: HP:0100308

Definition

Atrophy of one side of the brain, characterized by findings including thinning of the cerebral cortex, reduced volume of the cerebral white matter with abnormal myelination, and enlargement of the ispilateral fourth ventricle. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCerebral cortical hemiatrophy

Professional guidelines

PubMed

Differential diagnosis of cerebral hemispheric pathology: multimodal approach.
Moritani T, Smoker WR, Lee HK, Sato Y
Clin Neuroradiol 2011 Jun;21(2):53-63. Epub 2011 Apr 29 doi: 10.1007/s00062-011-0072-7. PMID: 21528369

Recent clinical studies

Etiology

Epileptic spasms in clusters with focal EEG paroxysms: A study of 12 patients.
Caraballo RH, Reyes G, Falsaperla R, Ramos B, Ruiz AC, Fernandez CA, Peretti G, Beltran L
Seizure 2016 Feb;35:88-92. Epub 2016 Jan 15 doi: 10.1016/j.seizure.2016.01.012. PMID: 26803282
Right cerebral hemiatrophy: neurocognitive and electroclinical features.
Demirtas-Tatlidede A, Yalcin AD, Uysal E, Forta H
Epilepsy Behav 2010 Apr;17(4):536-40. Epub 2010 Mar 16 doi: 10.1016/j.yebeh.2010.02.006. PMID: 20236868
Left hemisphere and male sex dominance of cerebral hemiatrophy (Dyke-Davidoff-Masson Syndrome).
Unal O, Tombul T, Cirak B, Anlar O, Incesu L, Kayan M
Clin Imaging 2004 May-Jun;28(3):163-5. doi: 10.1016/S0899-7071(03)00158-X. PMID: 15158218
Etiology, timing of insult, and neuropathology of cerebral palsy evaluated with magnetic resonance imaging.
Jaw TS, Jong YJ, Sheu RS, Liu GC, Chou MS, Yang RC
J Formos Med Assoc 1998 Apr;97(4):239-46. PMID: 9585674
Hemimegalencephaly: diagnosis and treatment.
Trounce JQ, Rutter N, Mellor DH
Dev Med Child Neurol 1991 Mar;33(3):261-6. doi: 10.1111/j.1469-8749.1991.tb05116.x. PMID: 1902803

Diagnosis

Clinico-radiological approach to cerebral hemiatrophy.
Tan AP, Wong YLJ, Lin BJ, Yong HRC, Mankad K
Childs Nerv Syst 2018 Dec;34(12):2377-2390. Epub 2018 Aug 6 doi: 10.1007/s00381-018-3934-3. PMID: 30083798
Differential diagnosis of cerebral hemispheric pathology: multimodal approach.
Moritani T, Smoker WR, Lee HK, Sato Y
Clin Neuroradiol 2011 Jun;21(2):53-63. Epub 2011 Apr 29 doi: 10.1007/s00062-011-0072-7. PMID: 21528369
Hemiparkinsonism-hemiatrophy syndrome.
Ayromlou H, Najmi S, Arami MA
Arch Iran Med 2011 Mar;14(2):152-4. PMID: 21361726
Infectious acute hemicerebellitis.
García-Cazorla A, Oliván JA, Pancho C, Sans A, Boix C, Campistol J
J Child Neurol 2004 May;19(5):390-2. doi: 10.1177/088307380401900514. PMID: 15224713
MR of craniocerebral hemiatrophy.
Sener RN, Jinkins JR
Clin Imaging 1992 Apr-Jun;16(2):93-7. doi: 10.1016/0899-7071(92)90119-t. PMID: 1547482

Therapy

Epileptic spasms in clusters with focal EEG paroxysms: A study of 12 patients.
Caraballo RH, Reyes G, Falsaperla R, Ramos B, Ruiz AC, Fernandez CA, Peretti G, Beltran L
Seizure 2016 Feb;35:88-92. Epub 2016 Jan 15 doi: 10.1016/j.seizure.2016.01.012. PMID: 26803282
Hemiparkinsonism-hemiatrophy syndrome.
Ayromlou H, Najmi S, Arami MA
Arch Iran Med 2011 Mar;14(2):152-4. PMID: 21361726
Parry-Romberg syndrome and Rasmussen encephalitis: possible association. Clinical and neuroimaging features.
Longo D, Paonessa A, Specchio N, Delfino LN, Claps D, Fusco L, Randisi F, Genovese E, Vigevano F, Fariello G
J Neuroimaging 2011 Apr;21(2):188-93. doi: 10.1111/j.1552-6569.2009.00398.x. PMID: 19555404
Dyke-Davidoff-Masson syndrome in postcerebral malaria.
Karuppiah S, Rodgman C, Lombard J
J Child Neurol 2009 Apr;24(4):487-90. Epub 2009 Feb 9 doi: 10.1177/0883073808324541. PMID: 19204321
An open study of tacrolimus therapy in Rasmussen encephalitis.
Bien CG, Gleissner U, Sassen R, Widman G, Urbach H, Elger CE
Neurology 2004 Jun 8;62(11):2106-9. doi: 10.1212/01.wnl.0000128044.94294.87. PMID: 15184626

Prognosis

Clinico-radiological approach to cerebral hemiatrophy.
Tan AP, Wong YLJ, Lin BJ, Yong HRC, Mankad K
Childs Nerv Syst 2018 Dec;34(12):2377-2390. Epub 2018 Aug 6 doi: 10.1007/s00381-018-3934-3. PMID: 30083798
Fatal outcome in hemiconvulsion-hemiplegia syndrome.
Jayakody H, Joshi C
J Child Neurol 2014 Mar;29(3):406-11. Epub 2012 Dec 26 doi: 10.1177/0883073812469053. PMID: 23271756
October 2002: 27-year-old female with epilepsy.
Mourelatos Z, McGarvey M, French JA, Wells G
Brain Pathol 2003 Apr;13(2):233-4, 239. PMID: 12744477
The natural history of Rasmussen's encephalitis.
Bien CG, Widman G, Urbach H, Sassen R, Kuczaty S, Wiestler OD, Schramm J, Elger CE
Brain 2002 Aug;125(Pt 8):1751-9. doi: 10.1093/brain/awf176. PMID: 12135966
Hemimegalencephaly: diagnosis and treatment.
Trounce JQ, Rutter N, Mellor DH
Dev Med Child Neurol 1991 Mar;33(3):261-6. doi: 10.1111/j.1469-8749.1991.tb05116.x. PMID: 1902803

Clinical prediction guides

Cerebral hemiatrophy (Dyke-Davidoff-Masson syndrome) in childhood: clinicoradiological analysis of 19 cases.
Atalar MH, Icagasioglu D, Tas F
Pediatr Int 2007 Feb;49(1):70-5. doi: 10.1111/j.1442-200X.2007.02299.x. PMID: 17250509
Unilateral cerebral polymicrogyria with ipsilateral cerebral hemiatrophy.
Hayakawa K, Kanda T, Yamori Y
Eur Radiol 2002 Oct;12(10):2542-7. Epub 2002 Apr 17 doi: 10.1007/s00330-002-1329-y. PMID: 12271397
The natural history of Rasmussen's encephalitis.
Bien CG, Widman G, Urbach H, Sassen R, Kuczaty S, Wiestler OD, Schramm J, Elger CE
Brain 2002 Aug;125(Pt 8):1751-9. doi: 10.1093/brain/awf176. PMID: 12135966
Magnetic resonance imaging. Its role in the neuroradiologic evaluation of neurofibromatosis, tuberous sclerosis, and Sturge-Weber syndrome.
Truhan AP, Filipek PA
Arch Dermatol 1993 Feb;129(2):219-26. doi: 10.1001/archderm.129.2.219. PMID: 8434982
MR of craniocerebral hemiatrophy.
Sener RN, Jinkins JR
Clin Imaging 1992 Apr-Jun;16(2):93-7. doi: 10.1016/0899-7071(92)90119-t. PMID: 1547482

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