Isaacs' syndrome with muscle hypertrophy reversed by phenytoin therapy.

A 16-year-old boy was seen for severe episodic muscle cramps and generalized myokymia, consistent with Isaacs's syndrome. Bilateral calf hypertrophy (46-cm calf circumference) and ankle areflexia were noted. He was treated with phenytoin sodium, 300 mg/day. Within three months there was marked decrease in myokymia, total relief of cramps, return of ankle reflexes, and 6-cm reduction in calf circumference. We suggest that the excess muscle activity in Isaacs's syndrome may be responsible for the associated phenomena of muscle hypertrophy and areflexia.

Acute hemiplegia associated with HIV infection.

An acute hemiplegia secondary to a large cerebral infarct is described in a 16-month-old infant with congenitally-acquired human immunodeficiency virus infection. Serial imaging studies during the next year documented improvement in his hemiplegia and a static underlying human immunodeficiency virus encephalopathy. Acquired immunodeficiency syndrome should be included in the differential diagnosis of children with acute hemiplegia.

Pediatric brain stem tumor presenting as a pure motor hemiparesis.

The clinical syndrome of pure motor hemiparesis occurs with lacunar lesions in the internal capsule or in the basis pontis in hypertensive adults. It has been described in three patients with brain stem tumors of which two were children. A 3 1/2 years old boy with a brain stem tumor which presented as pure motor hemiparesis is described.

Schwannoma presenting as lumbar disc disease in an adolescent boy.

Lumbar disc disease and spinal cord tumors occur relatively rarely in the pediatric age group. We report on a 16-year-old boy who presented with signs and symptoms of lumbar disc disease that failed to respond to conservative treatment. He was diagnosed preoperatively to have schwannoma of the S-1 root.

Hypothermia and systemic lupus erythematosus.

We describe an 18-year-old black woman with systemic lupus erythematosus (SLE) who presented with an exacerbation of polymyositis and neuropsychiatric symptoms. After starting prednisone she became hypothermic for 5 days. Hypothermia associated with SLE has been described in only 3 patients.

Congenital Horner's syndrome does not alter Lisch nodule formation.

A 21-year-old woman with neurofibromatosis type 1 (NF-1) had a unilateral congenital Horner's syndrome with resultant hypopigmentation of the affected iris. Lisch nodules, which are melanocytic hamartomas, were similar in number, size, and pigmentation in both eyes. The present findings suggest that the formation of Lisch nodules is not influenced by the presence or absence of sympathetic innervation of the iris.

Alkaline phosphatase activity in cultured skin fibroblasts from fibrodysplasia ossificans progressiva.

Alkaline phosphatase activity in four strains of cultured skin fibroblasts obtained from a patient with fibrodysplasia ossificans progressiva was at the low normal range. The enzyme activity in normal fibroblasts significantly increased at late confluency. It appears that the high levels of alkaline phosphatase activity reported in biopsies of lesions are not genetically determined but are secondary events of local tissue reaction.

Neurocysticercosis in an Orthodox Jewish community in New York City.

BACKGROUND AND METHODS: From June 1990 through July 1991, intracerebral infection with the larval stage of the pork tapeworm Taenia solium was diagnosed in four unrelated persons in an Orthodox Jewish community in New York City. None of the patients had eaten pork, and only one had traveled to a country in which T. solium infection was endemic. We investigated this outbreak, screened serum samples from family members and household contacts for antibodies to cysticercosis, and examined stool specimens from household employees for eggs of taenia species. RESULTS: The four patients had recurrent seizures and brain lesions that were radiologically consistent with the presence of cysticerci. The diagnosis was confirmed in two patients by a brain biopsy, and in two by immunoblot assays for cysticercus antibodies. Of 17 immediate family members screened serologically, 7 from two families had cysticercus antibodies. Magnetic resonance imaging of the brain showed cystic lesions in two of the seropositive family members, one of whom had had a seizure. Examinations of six domestic employees from all four households revealed an active infection with taenia species in one and a positive serologic test in another. Since these women had recently emigrated from Latin American countries where T. solium infection is endemic, they were the most likely sources of infection in the members of these households. CONCLUSIONS: A diagnosis of neurocysticercosis should be considered in patients with seizures and radiologic evidence of cystic brain lesions, even in those who do not eat pork and who have not traveled to a country in which T. solium infection is endemic. Recent emigrants from countries in which T. solium infection is endemic should be screened for tapeworm infection in their stools before they are employed as housekeepers or food handlers.