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November 1968

Alexander's Disease in an Adult: Report of a Case

Author Affiliations

USAR, USAR Washington, DC
From the Neuropathology Branch, Armed Forces Institute of Pathology, Washington, DC.

Arch Neurol. 1968;19(5):494-502. doi:10.1001/archneur.1968.00480050064006

PREVIOUS descriptions of Alexander's disease have generally been of cases in young children.1-8 The clinical picture most often associated with this disease has been one of developmental retardation and enlargement of the head. Vogel and Hallervorden,9 however, described a case in a 15-year-old girl that began with weakness of the left leg and foot at age 7 and progressed to spasticity and contractures of the lower extremities, eventual weakness of the upper extremities, and development of blurred vision, nystagmus, dysarthria, and dysphagia. The brain was atrophic, weighing 990 gm at autopsy. Stevenson,10 referring to this case, stated that the patient "presented the picture that one would think might be multiple sclerosis." Rewcastle11 presented a case of a 26-year-old man whose symptoms began at age 14 with weakness of the legs, followed by arm weakness, incontinence, diplopia, and difficulty with speech and swallowing. Findings on examination included

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