幼児期に発症し,両側大脳白質病変,難治性てんかんを合併したParry-Romberg症候群の1例(<特集>小児科学教室大澤真木子教授開講5周年記念)

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  • A Case of Early Onset Parry-Romberg Syndrome with Bilateral White Matter Abnormalities and Intractable Epilepsy(Papers for Cerebrating the Fifth Aniversary of the Directorship of Prof. M. Osawa at the Department of Pediatrics, Tokyo Women's Medical University)

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小児科学教室大澤真木子教授開講5周年記念特集

A 7-year-old girl with Parry-Romberg syndrome (progressive hemifacial atrophy) was described. She had cutaneous changes on her right forehead that began at 3 years of age, and slowly progressive hemifacial atrophy. Epileptic seizures were first noted at 6 years of age and were intractable including various type of myoclonic seizure, myoclonic astatic seizure, atypical absence, and generalized tonic clonic seizures. EEG showed frequent bi-frontal dominant diffuse spike and wave activity both when awake and asleep. Brain CT revealed multiple intracranial calcifications and frontal bone change ipsilateral to hemifacial atrophy. MRI revealed increased signals on T2 weighted images mainly in the white matter of the right hemisphere and also in the left temporal lobe. Antinuclear antibody (ANA) was positive in serum and local IgG production in the cerebrospinal fluid was increased. Ketogenic diet provided partially control of her epileptic seizures. Previous descriptions of Parry-Romberg syndrome of early onset complicated with epilepsy were reviewed in relation to the present case, and the etiology, neurological complications and intracranial findings in Parry-Romberg syndrome were discussed.

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