Cerebellopontine angle lipoma in a teenager.

Lipomas of the cerebellopontine angle are very rare lesions. To date, 18 patients have been reported, 17 of whom were adults. A second child is described with cerebellopontine angle lipoma.

Treatment of infantile spasms with high-dose ACTH: efficacy and plasma levels of ACTH and cortisol.

Fifteen children with infantile spasms and a hypsarrhythmic EEG defined by EEG-videotelemetry monitoring received a regimen of high-dose (150 IU/m2/d) ACTH for their seizures. We carried out an endocrinologic evaluation before and after initiation of the ACTH and conducted a time course study of plasma ACTH and cortisol levels after ACTH dosing. Spasms were controlled and the EEG normalized in 14 of the 15 children. Prior to starting ACTH therapy all the patients had normal prolactin, insulin, cortisol, and ACTH levels in plasma and normal thyroid function. Although the pattern of rise of ACTH levels in plasma after ACTH dosing was similar in all the children, there was great individual variation in the absolute concentrations. However, both the pattern of rise and absolute level of cortisol in plasma after ACTH was highly predictable in all patients. Plasma cortisol rose rapidly within 1 hour of ACTH administration and continued a slower rise for 12 to 24 hours after the ACTH dose. High-dose ACTH therapy seems quite effective in infantile spasms, perhaps because of a sustained high level of plasma cortisol. This sustained plateau of cortisol may be more effective in controlling infantile spasms than the pulse effect expected with oral steroids or lower doses of ACTH.

ACTH and prednisone in childhood seizure disorders.

We treated 116 children with ACTH or prednisone. Fifty-two had infantile spasms with hypsarhythmia, and 64 had other types of intractable seizures. ACTH completely controlled seizures in all patients with infantile spasms and hypsarhythmia and 74% of those with other types of seizures. Prednisone controlled 51% of patients with infantile spasms and none with other seizures. Serious side effects were minimal for both drugs, and recurrent seizures occurred in 40 to 50% of patients within 4 to 14 months after completion of therapy.

Burn encephalopathy in children.

Among 287 children with burns treated over a recent two-year period, 13 (5%) showed evidence of encephalopathy. The major clinical symptoms were an altered sensorium and seizures. The majority of symptoms began later than 48 hours after the burn and were accompanied by multiple metabolic aberrations including hypocalcemia. Three children had a relapsing course, and 1 had temporarily enlarged cerebral ventricles. Eleven children improved to normal. In the majority of instances, burn encephalopathy probably reflects central nervous system dysfunction resulting from complex metabolic, hematological, and hemodynamic abnormalities rather than from a single metabolic abnormality.

High resolution computerized tomography with coronal and sagittal reconstruction in the diagnosis of brain tumors in children.

We present 4 children with brain tumors that were missed or incompletely characterized conventional computerized tomography (CT) scanning. Using high resolution CT with high dose contrast and three-dimensional reconstruction, we were able to define the anatomy of each tumor and its associated aberrant vasculature. High resolution CT scanning with reconstruction is very helpful in diagnostic problems in which conventional CT scanning fails to provide a clear diagnosis.

Ophthalmoplegia and ptosis in congenital fiber type disproportion.

Bilateral ophthalmoplegia and ptosis is reported for the first time in a patient with a rare neuromuscular disorder, congenital fiber type disproportion (CFTD). The importance of limb muscle biopsy in the diagnostic evaluation is emphasized. A summary is presented of other congenital neuromuscular diseases which may have associated ophthalmoplegia.

Steroid-responsive polyneuropathy with subacute onset in childhood.

Five pediatric patients with subacute onset polyneuropathy are presented, with electrophysiologic and pathologic data. All patients improved, the majority to resolution, with administration of prednisone. Distinguishing factors included (1) subacute onset polyneuropathy progressing gradually over weeks to months, (2) primarily motor neuropathy with little cranial nerve involvement, (3) elevated CSF protein concentration, (4) markedly delayed nerve conduction velocities, and (5) tendency toward relapse and recurrence. Although this disorder may share characteristics with the Guillain-Barré syndrome, its steroid responsiveness sets it apart clinically from the acute form of the disease. Because of the steroid responsiveness, it is important to recognize this entity.

Juvenile myasthenia gravis.

We studied 32 children with myasthenia gravis over a period of 12 years. The mean age at onset was 7.7 years. Presentation was ocular in 63% of patients. Another major disease in addition to myasthenia occurred in 44% of patients; a seizure disorder was the most commonly associated disease. Serum IgG antibody to nicotinic acetylcholine receptor was present in 53% of patients and did not correlate with severity of disease or treatment. Medical management was effective in 63%; thymectomy was effective in only 28%. We conclude that myasthenia gravis appears commonly before age 10 and is associated with the risk of some disease other than hyperthyroidism. Serum IgG nicotinic acetylcholine receptor antibody is present less frequently than in normal adults, and vigorous medical management should be attempted before thymectomy.

Christmas bow tragedies.

Two cases of fatal accidents involving plastic buttons from Christmas bows are described. Although accidents are the commonest cause of death in children less than 3 years old, governmental mechanisms, properly enforced, can be instrumental in preventing such catastrophies.