The Sleepy Sickness

The Sleepy Sickness

This article will address three broad areas of viral encephalitis—its causes, differential diagnosis, and management. To our knowledge, this is one of the largest cohorts examining the diverse etiologies and presentations of BE. Infection or inflammation in the brain can lead to permanent damage. When an individual was stricken, the first signs were typically a sore throat and fever accompanied by a headache; but these discomforts soon developed into more alarming problems such as double-vision and severe weakness. Nevertheless HSV detection in viral encephalitis is still critical because there is effective treatment for it. Moreover, encephalitis associated with HSV, the most frequently identified agent of infectious encephalitis, typically affects the temporal and cingulate cortices with only rare involvement of the brainstem [11–13]. Often, we may find a bruise and not know what caused it; the same is true for encephalitis.

Many became comatose and completely unresponsive. Thus, St Louis virus encephalitis, which is caused by a mosquito borne arbovirus, occurs in the midwestern and eastern states of the USA, and not in the UK, while Japanese encephalitis is a major problem in Asia, and is the most important cause of epidemic encephalitis worldwide, causing up to 15000 deaths annually.5 Two “emerging” viral infections of the nervous system which have received much attention recently are West Nile virus encephalitis and Nipah virus encephalitis. The one patient who had normal brain biopsy was subsequently found to have an immune-mediated condition. It can be the normal (and healthy) reaction of the body to a viral or bacterial infection. Although full recoveries were not unheard of, they were a rarity. Once a diagnosis of infective viral encephalitis has been established it is then necessary to have a clear investigative plan to try to determine the likely cause. It is important to closely monitor these patients on immunosuppressive therapies, and any deterioration clinically or on neuroimaging should prompt a brain biopsy, to exclude infection or tumor.

The Sleepy Sickness
Most children have been exposed to this virus, and your child may be infected with it even if she does not have a cold sore or blister around her mouth, or other sign of the virus. In some cases, individuals retained their hearing, intelligence, and reasoning, but were left in a catatonic state, unable to respond to stimuli. In patients recently returning from abroad, particular vigilance must be paid to the possibility of such non-viral infections as cerebral malaria which may be rapidly fatal if not treated early. We found that elevated CSF glucose was tightly associated with elevated serum glucose, suggesting that poor systemic glycemic control likely drives increased CSF glucose, and that both are predictors of poor outcome in patients with BE. In the past, physicians assumed that if we couldn’t identify the cause of a case of encephalitis, the cause must be a virus that we weren’t able to detect. In 1969, over forty years after the strange disease disappeared, some catatonic victims were treated with a newly developed antiparkinson drug called Levodopa. While the distinction may not always be straightforward, there are a number of clues which may indicate that the patient has an infective viral encephalitis rather than a non-viral encephalopathy.

In a recent study, factors associated with a poor outcome following infectious encephalitis included the presence of comorbid conditions and increasing age [17]. Q: Will my child be okay? Oliver Sacks. ADEM, also known as postinfectious encephalomyelitis, usually follows either a vaccination within the preceding four weeks, or an infection which may be a childhood exanthema such as measles, rubella or chickenpox, or else a systemic infection characteristically affecting the respiratory or gastrointestinal systems.7 There is very good evidence from various sources to suggest an immune pathogenesis of this disorder, with an abnormal immune reaction directed against normal brain. In our series, seven biopsies were performed on the brainstem itself, four of which resulted in a specific diagnosis. A: No, but it may be caused by a contagious virus. Despite his advanced molecular probes, he found no evidence of viruses in the tissue.

As is the case for table 3, these various diagnostic pointers should be taken together and not in isolation when trying to make the diagnosis of ADEM. Thus, biopsy of the brainstem itself yielded a specific diagnosis in over 50 % of cases and was quite safe in our cohort. Here at Boston Children’s, we have a “neurology step-up unit,” where your child can receive additional specialized care from nurses trained in caring for children with neurological disorders if needed after she leaves the emergency room or the intensive care unit, and before she transfers to the general neurology inpatient unit. Though the evidence is insufficient to be certain, the findings of these researchers strongly suggests that the sleepy sickness epidemic was caused by the body’s massive over-reaction to these bacteria. However, it should be appreciated that in approximately half of cases the cause of viral encephalitis is not found. Our data suggest that CLIPPERS is an uncommon cause of BE. It may take a few months for the brain to heal, although some children recover much more quickly.

It is likely that most cases are minor, and go undiagnosed. For example, both cerebral malaria and human African trypanosomiasis should be considered in a patient who has recently travelled to Africa, Japanese encephalitis in the case of travel to Asia, and Lyme disease in the case of travel to high risk regions of Europe and the USA. Thus, although outcomes of BE are variable and can be devastating, the majority of our patients with inflammatory/autoimmune and undefined causes of BE recovered with favorable outcomes. Lots of parents find it helpful to jot down questions as they arise – that way, when you talk to your child’s doctors, you can be sure that all of your concerns are addressed.

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