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April 1976

Negri Bodies in a Case of Ataxia Telangiectasia

Author Affiliations

Dept of Neuropathology Beilinson Medical Center Petah Tikva, Israel

Arch Neurol. 1976;33(4):306. doi:10.1001/archneur.1976.00500040090021

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To the Editor.—  In reference to Dr Derakhshan's article, "Is the Negri Body Specific for Rabies?" (Arch Neurol 32:75, 1975), we encountered a case of ataxia telangiectasia in which typical Negri bodies were found at autopsy.A 5-year-old boy suffered from psychomotor retardation. As soon as he began to walk, ataxia was obvious. On examination he had telangiectasias in both eyes, vitiligo, and hyperreflexia. Results of laboratory tests showed hypogammaglobulinemia, especially IgA. The patient suffered periodically from pneumonia and diarrhea and finally died of intractable sepsis. There was no history of exposure to dogs nor was the clinical picture even vaguely reminiscent of rabies.The autopsy findings revealed intracytoplasmic inclusion bodies in the Purkinje cells of the cerebellum. These inclusion bodies were eosinophilic and homogeneous, with a diameter between 2μ and 7μ (Figure). They stained a bright red with Masson trichrome. No signs of inflammation were found in any

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