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Pediatrics JIPMER 2019 d7911cf3

Which epileptic syndrome is associated with left hemispheric atrophy in child?

A
Dravet Syndrome
B
Dyke Davidson Masson syndrome
C
Lennox Gastaut syndrome
D
West Syndrome
High-Yield Explanation
All the above options are epileptic syndromes, among these only Dyke Davidson Masson syndrome have characteristic Childhood Cerebral Hemiatrophy features. Dyke Davidson Masson syndrome: Hemiatrophy or hypoplasia of unilateral cerebral hemisphere resulting from brain insult in fetal or early childhood period with compensatory ipsilateral calvarial hyperophy Insult could be vascular, congenital or acquired ischemic disease, trauma or inflammation In-utero gestational vascular occlusion involving middle cerebral vascular territory or coarctation of mid-aoic arch may decrease carotid aerial flow Dravet Syndrome- prolonged seizures, psychomotor delay, ataxia. It is characterized by cognitive impairment, behavioral disorders, and motor deficits. Lennox Gastaut syndrome- seizures, cognitive dysfunction, and EEG findings (slow spike wave). West Syndrome- epileptic/infantile spasms, abnormal brain wave patterns (hypsarrhythmia) and intellectual disability.

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