Crack the Case: Seizures, Fever, and a Fatal Outcome

Neuropathology for Neurologists

James W. Mandell, MD, PhD

Disclosures

September 09, 2013

Which Bodies?

Lewy bodies are the characteristic cytoplasmic neuronal inclusion of Parkinson disease; composed primarily of alpha-synuclein, they also found in dementia with Lewy bodies. Hirano bodies are brightly eosinophilic paracrystalline cytoplasmic intraneuronal inclusions; they are not disease-specific but accumulate with age. Marinesco bodies are intranuclear neuronal inclusions found specifically in substantia nigra neurons that are also related to aging, but are not disease-specific.

Negri bodies are characteristic of rabies virus infection and were first described in 1903 by Adelchi Negri, an Italian pathologist and microbiologist. Of interest, the search for a diagnostic marker of rabies (to be used on suspected rabid animal brains) was initiated by the much more famous neurologist/neuropathologist, Camillo Golgi; frustrated by a lack of success, however, Golgi gave this project to Dr. Negri, a recently graduated medical student in his laboratory.

Negri's discovery may be one of the first examples of a "theranostic," a diagnostic test linked to a specific therapy -- in this case, the therapy being the rabies vaccine developed by Louis Pasteur in 1885. Other fascinating historical details can be found in an excellent account of these and other discoveries in central nervous system infectious diseases.[1]

Negri bodies are most often found in hippocampal Ammon horn pyramidal neurons or cerebellar Purkinje neurons. However, their presence is variable and dependent on the duration of infection, and modern polymerase chain reaction-based molecular methods are now the preferred diagnostic tests. Recent research reveals an interaction of the innate immune receptor, toll-like receptor 3 (TLR3), with the rabies virus that is essential for the formation of Negri bodies.[2]

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