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Megalencephaly with dysmyelination

MedGen UID:
344470
Concept ID:
C1855309
Disease or Syndrome
Synonym: MEGALENCEPHALY WITH DIFFUSE WHITE MATTER HYPODENSITY
 
Monarch Initiative: MONDO:0009574
OMIM®: 249240

Clinical features

From HPO
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).
Spasticity
MedGen UID:
7753
Concept ID:
C0026838
Sign or Symptom
A motor disorder characterized by a velocity-dependent increase in tonic stretch reflexes with increased muscle tone, exaggerated (hyperexcitable) tendon reflexes.
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.
Megalencephaly
MedGen UID:
65141
Concept ID:
C0221355
Congenital Abnormality
Diffuse enlargement of the entire cerebral hemispheres leading to macrocephaly (with or without overlying cortical dysplasia).
Abnormal cerebral white matter morphology
MedGen UID:
181756
Concept ID:
C0948163
Pathologic Function
An abnormality of the cerebral white matter.
Cerebral dysmyelination
MedGen UID:
343222
Concept ID:
C1854885
Finding
Defective structure and function of myelin sheaths of the white matter of the brain.
EEG with photoparoxysmal response
MedGen UID:
765735
Concept ID:
C3552821
Finding
EEG abnormalities (epileptiform discharges) evoked by flashing lights or black and white striped patterns.

Recent clinical studies

Etiology

Santos AC, Escorsi-Rosset S, Simao GN, Terra VC, Velasco T, Neder L, Sakamoto AC, Machado HR
Childs Nerv Syst 2014 Nov;30(11):1813-21. Epub 2014 Oct 9 doi: 10.1007/s00381-014-2476-6. PMID: 25296542

Diagnosis

Santos AC, Escorsi-Rosset S, Simao GN, Terra VC, Velasco T, Neder L, Sakamoto AC, Machado HR
Childs Nerv Syst 2014 Nov;30(11):1813-21. Epub 2014 Oct 9 doi: 10.1007/s00381-014-2476-6. PMID: 25296542
Deprez M, D'Hooghe M, Misson JP, de Leval L, Ceuterick C, Reznik M, Martin JJ
Acta Neurol Scand 1999 Mar;99(3):158-65. doi: 10.1111/j.1600-0404.1999.tb07338.x. PMID: 10100959

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