New York Association of Neuropathologists
A 50 year old woman presented with chorea
of the face, head, and all extremities and cognitive impairment. Her clinical
problems had begun at least 4 years earlier and she had gone downhill rapidly
over the last year. CSF 14-3-3 protein was positive. Genetic tests for
Huntington's Disease, HDL-2, and DRPLA (DentatoRubroPallidoLuysian Atrophy,
remember?) were all negative, however. She had a gammopathy of unknown
significance, had hospital admissions for fever of unknown origin, and
secondary to urinary tract infections.
The autopsy slide of brain showed perivascular myelin rarefaction with an
infiltrate of mononuclear inflammatory cells together with small
multinucleated giant cells. The appearance was typical for HIV encephalitis.
Apparently this diagnosis was so doubted by the clinicians, who had not
suspected AIDS, that Dr Chin did HIV p245 protein immunostains, which were
nicely positive. It subsequently was learned that antemortem studies had also
shown that she had lymphadenopathy and pneumonias. Epidemiological
investigation disclosed that her first husband, who had been known to have
died of "lymphoma", died of AIDS-related lymphoma.