Rabies Encephalomyelitis

Abstract

Background: Three patients received solid organ transplants from a common donor and were subsequently discharged from the hospital following an uneventful hospital course. Within 30 days, all 3 organ recipients returned to the hospital with varying symptoms that progressed to rapid neurological deterioration, coma, and death.Objective: To describe the clinical, neuroradiological, and pathological findings of rabies virus infection in organ transplant recipients infected from a common donor.Design: Case series involving a common donor and 3 organ recipients ascertained through review of clinical course and autopsy findings. A fourth case was determined by review of pending autopsy cases in which death occurred within the same time interval. Portions of postmortem central nervous system and organ tissues were frozen and formalin-fixed. Fluids and tissues were also collected for cultures, serology, and molecular studies. Postmortem fluids and tissues and antemortem fluids and tissues from all 4 transplant recipients and serum and banked lymphocyte or spleen cells from the donors were sent to the Centers for Disease Control and Prevention for further evaluation.Setting: Transplant unit of an urban teaching hospital.Results: Antemortem cerebrospinal fluid analysis for 3 of the 4 recipients was consistent with a viral etiology. Neuroimaging and electroencephalogram studies were suggestive of an infectious encephalitis or a toxic encephalopathy. Initial laboratory testing did not demonstrate an infectious etiology. Postmortem histologic analysis, immunohistochemistry, electron microscopy, and direct fluorescence antibody testing revealed rabies virus infection. Serological testing done postmortem confirmed rabies virus infection in the common donor.Conclusions: These cases demonstrate a risk for transmitting rabies virus infection through solid organ and tissue transplantation, and this diagnosis should be considered in any rapidly progressing neurological disease.

Intravenous Inoculation of a Bat-Associated Rabies Virus Causes Lethal Encephalopathy in Mice through Invasion of the Brain via Neurosecretory Hypothalamic Fibers

“…The transit from the vascular system to the brain via neurosecretory fibers of the ME and NPH might also present a possible explanation for the RV infection of immunosuppressed recipients of RV infected organs that was reported in 2004 in the U.S.A. and in 2005 in Germany [38],[39]. Transplanted tissue is deprived of direct nerval input for many months [40]; thus the classical retrograde pathway of CNS invasion by RV via visceromotor fibers of the autonomic nervous system would have still been unavailable at the time of the actual incubation for the reported transplantation incidents.…”

Epidemiological and clinical features of human rabies cases in Bali 2008-2010

“…Clinically, furious rabies should be easier to establish than paralytic rabies. The paralytic type is caused by virus infection in the spinal cord [18]. In some cases of human rabies in Bali, the patients showed paralytic symptoms on admission and then developed furious symptoms in the later stages.…”

Transmission of rabies virus from an organ donor to four transplant recipients

“…The organ donor, a 20‐year‐old man, had been seen at hospital emergency departments with complaints of nausea, vomiting, abdominal pain, and difficulty swallowing, rapidly progressing to include confusion and agitation and truncal ballistic movements 1, 2. Soon after hospital admission, he developed tachycardia and hypertension.…”

“…In the United States, human infection is rare. Only 55 cases have occurred in the last 25 years 2. The disease is endemic throughout Africa, Asia, Latin America, and the Indian subcontinent.…”