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March 1971

Familial Lymphohistiocytosis of the Nervous System

Author Affiliations

Boston; St. Louis; Boston
From the Charles S. Kubik Laboratory for Neuropathology of the James Homer Wright Pathology Laboratories, Massachusetts General Hospital (Dr. Richardson), the departments of neurology-neuropathology and pathology, Harvard Medical School (Drs. Price, Rosman, and Richardson), Boston, the departments of neurology and pathology. Cardinal Glennon Memorial Hospital for Children and St. Louis University School of Medicine (Dr. Woolsey), St. Louis.

Arch Neurol. 1971;24(3):270-283. doi:10.1001/archneur.1971.00480330098010

Four children in a family had a progressive neurologic disease characterized by diffuse lymphohistiocytic infiltrations of the central nervous system (CNS) in association with multiple foci of parenchymatous destruction. The age range was 15 months to 12 years. The clinical features, although variable, comprised seizures, spastic weakness of limbs, and ataxia. The cerebrospinal fluid showed pleocytosis and increased protein. Histopathologically the disorder resembled what has been called familial hemophagocytic reticulosis, or familial erythrophagocytic lymphohistiocytosis, but unlike those disorders, was largely confined to the CNS. The cause and pathogenesis remain unknown. A genetically-determined atypical response to an undisclosed infection is possible.

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