Difference in neuropathogenetic mechanisms in human furious and paralytic rabies.
Whereas paralysis is the hallmark for paralytic rabies, the precise pathological basis of paralysis is not known. It is unclear whether weakness results from involvement of anterior horn cells or of motor nerve fibers. There is also no conclusive data on the cause of the neuropathic pain which occurs at the bitten region, although it has been presumed to be related to sensory ganglionopathy. In this study, six laboratory-proven rabies patients (three paralytic and three furious) were assessed clinically and electrophysiologically. Our data suggests that peripheral nerve dysfunction, most likely demyelination, contributes to the weakness in paralytic rabies. In furious rabies, progressive focal denervation, starting at the bitten segment, was evident even in the absence of demonstrable weakness and the electrophysiologic study suggested anterior horn cell dysfunction. In two paralytic and one furious rabies patients who had severe paresthesias as a prodrome, electrophysiologic studies suggested dorsal root ganglionopathy. Postmortem studies in two paralytic and one furious rabies patients, who had local neuropathic pain, showed severe dorsal root ganglionitis. Intense inflammation of the spinal nerve roots was observed more in paralytic rabies patients. Inflammation was mainly noted in the spinal cord segment corresponding to the bite in all cases; however, central chromatolysis of the anterior horn cells could be demonstrated only in furious rabies patient. We conclude that differential sites of neural involvement and possibly different neuropathogenetic mechanisms may explain the clinical diversity in human rabies.
Mitrabhakdi
Erawady
E
Neurology Division, Department of Medicine, Chulalongkorn University Hospital, Rama 4 Road, Bangkok 10330, Thailand. erawady.M@chula.ac.th
Shuangshoti
Shanop
S
Wannakrairot
Pongsak
P
Lewis
Richard A
RA
Susuki
Keiichiro
K
Laothamatas
Jiraporn
J
Hemachudha
Thiravat
T
D000328:Adult; D000368:Aged; D003711:Demyelinating Diseases / Q000473:pathology; D004568:Electrodiagnosis; D004576:Electromyography; D005260:Female; D005727:Ganglia, Spinal / Q000473:pathology; D006801:Humans; D007074:Immunoglobulin G; D008297:Male; D008875:Middle Aged; D018482:Muscle, Skeletal / Q000473:pathology / Q000503:physiopathology; D009417:Nerve Tissue / Q000473:pathology*; D009431:Neural Conduction / Q000502:physiology; D010243:Paralysis / Q000473:pathology; D010525:Peripheral Nerves / Q000473:pathology; D011818:Rabies / Q000276:immunology / Q000473:pathology*; D013116:Spinal Cord / Q000473:pathology
D007074:Immunoglobulin G