We report clinical and pathologic findings from two kindreds afflicted with a familial form of progressive subcortical gliosis. The disorder segregated as an autosomal dominant trait. Onset was in the presenium and the course was slowly progressive. Affected individuals initially manifested personality change, degeneration of social ability, disinhibition, psychotic symptoms, memory impairment, or depression. Later, all developed progressive dementia, frequently associated with verbal stereotypy, decreased speech output, echolalia, or manifestations of the human Klüver-Bucy syndrome. Terminal clinical manifestations included profound dementia, frequently with mutism, dysphagia, and extrapyramidal signs. Autopsy of seven end-stage patients revealed generalized cerebral atrophy, predominantly involving the white matter of the frontal and temporal lobes. Microscopically, prominent fibrillary astrocytosis was present in the subcortical white matter and in the subpial and deep layers of the overlying cerebral cortex. These changes were most pronounced in the frontal and temporal lobes, especially in the cingulate gyri and insulae. Mild cortical neuronal loss accompanied the gliosis, but no myelin loss was evident. The claustra and substantia nigra also showed severe astrocytosis and degenerative changes. Amyloid deposits and neuronal cytoskeletal inclusions were absent.

We studied a man affected with facioscapulohumeral muscular dystrophy (FMD) and multiple sclerosis (MS). This association has not been previously reported, to our knowledge. As a chance occurrence, it would be encountered less than once in 10 9 in the world population. REPORT OF A CASE At age 16 years, the patient noticed weakness of the proximal parts of the upper extremities and atrophy of both pectoralis muscles. At age 23 years he had an episode of temporary (six weeks) loss of vision in the left eye. On examination at 26 years of age there was moderate atrophy and weakness of the pectoralis, biceps, and triceps muscles, with winging of the scapulas. At this time, his physician (J.M.H.) commented that the patient appeared to have both dystrophy and MS. This was met with disbelief by one of us (R.D.C.). Results of the neurologic examination were otherwise normal. A lumbar puncture