Case Report
A fatal encephalitis Lancet 2005; 365: 358 Department of Neurology (C J Schankin MD, T Birnbaum MD, A Straube MD) and Neuroradiology ( J Linn MD, R Bruning MD), University of Munich Hospital—Großhadern, Marchioninistr. 15, 81377 Munich, Germany; Center for Priondisease and Neuropathology, (H A Kretzchmar MD, B Krebs MD), University of Munich, Feodor-Lynen-Str. 23, 81377 Munich, Germany Correspondence to: Dr B Krebs
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Christoph J Schankin, Tobias Birnbaum, Jennifer Linn, Roland Brüning, Hans A Kretzschmar, Andreas Straube, Bjarne Krebs
In May, 2004, a 51-year-old engineer was referred to our clinic agitated and disoriented. Previously healthy, he first noticed fever and pain in his left shoulder 4 days earlier, followed by a refusal to drink anything, a fear of draughts, and involuntary contractions of his limb and neck muscles. 4 weeks before he came to the clinic, he had returned from India, a country he visited regularly in the course of his work on solar energy installations. On examination, he was awake but did not respond to instructions. There was an intermittent difference in the diameters of his pupils together with absent corneal reflexes. After several hours he developed respiratory insufficiency requiring intubation and was transferred to the neurological intensive care unit. When his sedation was reduced either he developed hypertension up to 280 mm Hg systolic or his heart stopped. He was given a pacemaker. From the tenth day after his admission, he had no cephalic reflexes, did not breathe off the ventilator, and his EEG showed nonreactive delta-coma, suggesting a very severe encephalopathy. An MRI of his brain on day 7 was normal. However, on day 19, MRI showed extensive lesions in the brainstem and diencephalon (figure). Cerebrospinal fluid (CSF) taken on day 4 had a normal white count but contained 14-3-3 proteins, elevated tauprotein, and reduced -amyloid 1-42, but normal neuronspecific enolase and S100 protein. Only a slight pleocytosis was found in the CSF on day 16. Other laboratory investigations were non-contributory. Progressive loss of consciousness, agitation, pharyngeal and skeletal muscle spasms, and phobic fear of draughts and fluids are found in encephalitic rabies.1 The patient’s history of a visit to India where he helped look after some young dogs could have explained this infection. By report, he had neither been bitten nor suffered from any skin disease or injury, but skin contact with saliva would be expected. A diagnosis of rabies was not supported by the absence of rabies antibodies in saliva, serum, or CSF. He died on day 21 without a definitive diagnosis being established. An autopsy showed severe rabies encephalitis and hypoxic damage with substantial intracranial hypertension. By this time, the young dogs in India had all died after refusing food. They were not investigated by autopsy. After the onset of typical neurological symptoms of rabies no treatment is known to prevent death. Only postexposure prophylaxis with passive and active vaccination applied on time can save a person’s life.2 However, there has been a report of a 15-year-old girl surviving without post-exposure vaccination after treatment with ribavirin and drug-induced coma.3 Many countries where rabies is endemic have reduced the impact of the disease by prompt treatment of bites and vaccination of animal
Figure: Coronal FLAIR-weighted MRI (day 19) Hyperintense signal changes bilaterally in the caudate nucleus, thalamus, mesencephalon, pons and medulla oblongata. On administration of contrast agent (not shown), a pathological enhancement of the leptomeninges and the basal ganglia was seen.
vectors. In countries with a high incidence of human rabies such as India (more than 30 000 cases per year), infection is mainly transmitted by domestic animals such as dogs, but vaccination programmes have not yet been successfully established.4 This first case of rabies in Germany for 8 years5 and another case reported in Austria from Morocco are a reminder that imported rabies is a risk in European countries. This is also relevant for North America, although the most common vectors there are bats. Physicians, especially general practitioners, should bear in mind the possibility of rabies infection in travellers who have visited countries where rabies is endemic and been exposed to animals that might be rabid. References 1 Hemachudha T, Laothamatas J, Rupprecht CE. Human rabies: a disease of complex neuropathogenetic mechanisms and diagnostic challenges. Lancet Neurol 2002; 1: 101–09. 2 Jackson AC, Warrell MJ, Rupprecht CE, et al. Management of rabies in humans. Clin Infect Dis 2003; 36: 60–63. 3 Recovery of a patient from clinical rabies—Winsconsin 2004. MMWR Morb Mortal Wkly Rep 2004; 53: 1171–73. 4 World Health Organization. World survey of rabies no 34 for the year 1998. WHO/CDS/CSR/APH/99.6 http://www.who.int/emc/ diseases/zoo/wsr98/HTML_version/wsr98index.html, 2000 (accessed June 20, 2004). 5 World Health Organization. World survey of rabies no 32 for the year 1996. WHO/EMC/ZDI/98.4 http://www.who.int/emcdocuments/rabies/whoemczdi984c.html, 1998 (accessed Sept 14, 2004).
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