Progressively intractable seizures, focal alopecia, and hemimegalencephaly

R. Pelayo, E. Barasch, H. Kang, R. Marion, S. L. Moshé

Research output: Contribution to journalArticlepeer-review

6 Scopus citations

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
DOIs
StatePublished - May 1994

ASJC Scopus subject areas

  • Clinical Neurology

Fingerprint Dive into the research topics of 'Progressively intractable seizures, focal alopecia, and hemimegalencephaly'. Together they form a unique fingerprint.

Cite this