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November 1973

Familial Striatal Degeneration

Author Affiliations

Cleveland; Atlanta
From the Institute of Pathology, Case Western Reserve University, Cleveland (Dr. Roessmann), and the Department of Pediatrics (Pediatric Neurology), Emory University, Atlanta (Dr. Schwartz).

Arch Neurol. 1973;29(5):314-317. doi:10.1001/archneur.1973.00490290054005

Clinical history and pathologic findings are described in a disease occurring in two siblings and characterized by onset in childhood, subacute progression, movement disorder, increased muscle tone, and dysphagia. Pathologically, there is marked loss of neurons from the caudate nucleus and putamen. The disease is familial.