A 7-month-old infant had the infantile spasm syndrome, consisting of flexor and extensor spasms, developmental delay, and hypsarrhythmia. Corticotropin produced delay, and hypsarrhythmia. Corticotropin produced improvement in the clinical symptoms and reverted the generalized electroencephalographic abnormalities to more focal ones. Removal of a choroid… (More)
Venous angiomas are the rarest of all CNS vascular malformations. The association of a sublingual venous angioma with a similar ipsilateral cerebral lesion in an otherwise normal patient was observed.
A control system to convert a general-purpose stimulator into one which will generate premature stimuli at variable but controlled intervals is described. The control system was developed to sense spontaneous cardiac depolarizations and inject premature stimuli at preset elapsed times after every 10th or every spontaneous depolarization. The delay may be… (More)
Clinical, electrophysiological, and laboratory data are reported on a 21-year-old black woman with systemic lupus erythematosus, myasthenia gravis, and Ehlers-Danlos syndrome. The first two diseases are related by similar pathogenetic mechanisms. To our knowledge, this is the first patient reported with this unusual combination of rare diseases.
A female infant with neonatal myasthenia gravis remained weak for an excessively long period compared to the usual situation in this disease process. At 71 days of age, electrophysiological studies revealed evidence of a defect in neuromuscular transmission that improved with intravenous Tensilon therapy. Repeat study at 183 days of age was normal. This… (More)