Progressively intractable seizures, focal alopecia, and hemimegalencephaly

R. Pelayo, E. Barasch, H. Kang, Robert W. Marion, Solomon L. Moshe

Research output: Contribution to journalArticle

6 Citations (Scopus)

Abstract

We report a 3-year-old boy with the neurocutaneous combination of unilateral alopecia, ipsilateral hemimegalencephaly, and intractable seizures. He was born with an asymmetric hair pattern consisting of absent patches of hair, a small left eyebrow, and less eyelashes on the left eye; he had normal development until age 17 months, when he experienced right focal seizures with fever. Two months later, fever triggered new seizures characterized by flurries of head and body flexion and adduction of the right arm. He had left hand preference and language regression. EEG manifested left hemihypsarrhythmia, and MRI showed left hemimegalencephaly with marked enlargement of the temporal lobe with ventriculomegaly. Seizures were refractory to treatment with phenobarbital, adrenocorticotropic hormone, pyridoxine, sodium valproate, clonazepam, carbamazepine, phenytoin, and felbamate. This may represent a previously undescribed neurocutaneous syndrome.

Original languageEnglish (US)
Pages (from-to)969-971
Number of pages3
JournalNeurology
Volume44
Issue number5
StatePublished - May 1994

Fingerprint

Alopecia
Seizures
felbamate
Hair
Fever
Eyelashes
Neurocutaneous Syndromes
Eyebrows
Clonazepam
Pyridoxine
Carbamazepine
Valproic Acid
Phenytoin
Temporal Lobe
Phenobarbital
Adrenocorticotropic Hormone
Electroencephalography
Arm
Language
Hand

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Progressively intractable seizures, focal alopecia, and hemimegalencephaly. / Pelayo, R.; Barasch, E.; Kang, H.; Marion, Robert W.; Moshe, Solomon L.

In: Neurology, Vol. 44, No. 5, 05.1994, p. 969-971.

Research output: Contribution to journalArticle

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