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NEUROLOGY 1994;44:969
© 1994 American Academy of Neurology

Progressively intractable seizures, focal alopecia, and hemimegalencephaly

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

Departments of Neurology, Albert Einstein College of Medicine, Bronx, NY. (Drs. Pelayo, Kang, and Moshé, Radiology (Dr. Barasch)
Departments of Pediatrics, Albert Einstein College of Medicine, Bronx, NY. (Drs. Kang, Marion, and Moshé)
Departments of Neuroscience, Albert Einstein College of Medicine, Bronx, NY. (Dr. Moshé)
MontefiordAECOM Epilepsy Center, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, NY. (Drs. Kang and Moshé)

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.

Address correspondence and reprint requests to Dr. S.L. Moshé, Montefiore Medical Center, NW 7 EEG, 111 East 210th Street, Bronx, NY 10467.

Presented at t h e 46th Annual Eastern Association of Electroencephalographers, New York, NY, December 3, 1992.

Received August 30, 1993. Accepted for publication in final form November 16, 1993.




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