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We reviewed the outcome (seizures and development) of 25 cases with West syndrome in which antiepileptic drugs (valproate sodium, clonazepam) or high doses of vitamin B6, instead of ACTH therapy, were administered for the initial treatment. Five of 9 cryptogenic cases (56%) and 4 of 16 symptomatic cases (25%) showed complete cessation of spasms. In cryptogenic cases, response to vitamin B6, valproate sodium or clonazepam was not predicted by clinical features (age of onset, seizure type, initial EEG finding or treatment lag). On the other hand, response to these drugs was correlated with some clinical findings in symptomatic cases; all infants with neurocutaneous syndrome (tuberous sclerosis, neurofibromatosis type 1) had controlled infantile spasms, while none of patients with severe neonatal asphyxia or with prior other seizures responded. Twenty of 25 patients have been followed-up. The average age at follow-up was 5 years and 8 months. Prognosis of both cryptogenic and symptomatic "responders" was favorable; all had seizures controlled, and 50% had normal psychomotor development or only mild impairment (DQ > 70). Symptomatic "nonresponders" had the worst prognosis. Our results suggest that choice of drug in the initial treatment of West syndrome should be determined by clinical features (especially etiology).

We reported a surviving case of 6-year-old boy with pontine hematoma. He complained of headache as an initial symptom and developed progressively Foville's syndrome with impairments of the IX-XII cranial nerves. Although brainstem tumor was suspected initially using CT scan, MRI revealed the existence of hematoma in the ventromedial pons. During the first 4 months of his clinical course, Gd-DTPA did not demonstrate any enhancement in that lesion. However, hemangiomatous lesion was suspected by subsequent serial MRIs with positive Gd-DTPA enhancement. Using conservative treatment including oral corticosteroids, all the neurological deficits disappeared in several months and he did not show any recurrence of clinical signs for 3 years. It was suggested that MRI was very useful in the differential diagnosis and the follow-up of hematoma in the posterior fossa.

A 5-year-old girl who showed congenital nystagmus and mental and motor developmental delay, is described. Auditory brainstem responses (ABR) revealed wave I at normal latency, but all of the following waves were absent. In T2-weighted images, magnetic resonance imaging (MRI) demonstrated diffuse high intensity area of cerebral white matter, suggesting extensive dysmyelination or demyelination. She has not shown any deterioration through her clinical course. Subsequent MRI examinations did not demonstrate a progressive disorder. These findings suggest the possibility of Pelizaeus-Merzbacher (P-M) disease in this patient, which is a rare form of sudanophilic leukodystrophy, transmitted by an X-linked recessive mutant gene. It is reported that the proteolipid protein, one of the major proteins of myelin, was absent in classical type P-M disease, resulting in dysmyelination. Because chromosomal study showed the normal female karyotype and no family history of a similar disease was found in this case, it might be different from classical P-M disease. Since P-M disease may be heterogeneous, more detailed chromosomal analysis in each case of congenital hypomyelination will give a clue to clarify the pathogenesis of P-M disease and other disorders showing failure in myelination.