An Important and Treatable Cause of Encephalitis: anti-NMDR Encephalitis from Lancet Neurology

The book, Brain on Fire: My Month of Madness, 2013 tells the story of a young reporter who suddenly developed psychosis, movement disorder, and other terrifying mental symptoms over a few weeks. But at that time, the cause and treatment of her disease was not widely known. She had anti-NMDR encephalitis, a brain disease caused by antibodies to a specific type of brain receptor, the N-methyl-D-aspartate receptor (NMDAR).

An excellent article on anti-NMDR encephalitis is Clinical experience and laboratory investigations in patients with anti-NMDAR encephalitisLancet Neurol. 2011 Jan;10(1):63-74. [PubMed Abstract] [Free PMC Full Text] What follows is a quote from the article:

“Approach to diagnosis and proposal of a treatment strategy”

“Anti-NMDAR encephalitis should be suspected in any individual, usually younger than 50 years and especially a child or a teenager, who develops a rapid change of behaviour or psychosis, abnormal postures or movements (mostly orofacial and limb dyskinesias), seizures, and variable signs of autonomic instability, hypoventilation, or both. Supportive findings include CSF lymphocytic pleocytosis or oligoclonal bands; electroencephalogram with infrequent spikes, but frequent, slow, disorganised, sometimes rhythmic activity that does not relate with most abnormal movements; and brain MRI that is often normal or shows transient FLAIR or contrast-enhancing abnormalities.

“Antibody studies should be done in both serum and CSF. Such tests allow comparison of antibody concentrations during the course of the disease. Periodic screening of serum and CSF is useful to assess the effects of treatment, especially in the CNS. All patients should be examined for the presence of an underlying tumour, mainly an ovarian teratoma or a testicular germ-cell tumour. The very low frequency of other tumours suggests that periodic whole-body screening, as recommended for classic paraneoplastic syndromes, is unnecessary.88 However, we do recommend periodic screening for ovarian teratomas for at least 2 years, even if patients have recovered from encephalitis.17

“On the basis of previous experience and data from this Review, a proposal for treatment is shown in figure 7. Although no standard of care exists, we prefer concurrent IVIG (0·4 g/kg per day for 5 days) and methylprednisolone (1 g/day for 5 days) to plasma exchange. Plasma exchange is more difficult to do in children, poorly cooperative patients, or patients with autonomic instability. If no response is seen after 10 days, we start second-line therapy. In adults, we use rituximab (375 mg/m2 every week for 4 weeks) combined with cyclophophamide (750 mg/m2 given with the first dose of rituximab), followed by monthly cycles of cyclophosphamide. This treatment is discontinued when patients have had substantial clinical recovery, which is usually accompanied by a decrease of CSF and serum antibody concentrations. Paediatricians often use only one of these drugs—mostly rituximab. After substantial improvement, antiepileptics are not needed in most patients. Because relapses occur in 20–25% of patients, often in those without teratoma, in such patients we recommend continued immunosuppression (mycophenolate mofetil or azathioprine) for at least 1 year after initial immunotherapies are discontinued.”

 

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