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NEUROLOGY 1989;39:1530
© 1989 American Academy of Neurology

Primary lateral sclerosis in a child

M. L. Grunnet, MD, C. Leicher, MD, A. Zimmerman, MD, E. Zalneraitis, MD and M. Berwick, MD

From the Departments of Neurology (Drs. Grunnet, Zalneraitis, and Barwick), Pathology (Dr. Grunnet), and Pediatrics (Dr. Zalneraitis), University of Connecticut Health Center, Farmington, CT; Department of Pediatric Neurology (Dr. Leicher), Hartford Hospital, Hartford, CT; and the Knoxville Neurology Clinic (Dr. Zimmerman), Knoxville, TN.

Primary lateral sclerosis (PLS), previously undescribed in children, is characterized by a spastic motor deterioration and pathologic demonstration of corticospinal tract degeneration. We report an infant who, before 12 months of age, developed a progressive motor disease characterized by generalized spasticity. He died at 47 months, and neuropathologic examination revealed only corticospinal tract degeneration. We conclude that this child had typical clinical and pathologic findings for PLS.

Address correspondence and reprint requests to Dr. Grunnet, Department of Pathology, University of Connecticut Health Center, 263 Farmington Avenue, Farmington, CT 06032.

Received February 15, 1989. Accepted for publication in final form May 11, 1989.




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