Abstract
Encephalitis has been known as a distinct entity for a long time but its exact cause has remained undetected in about half the cases in spite of extensive investigations. During the last few years there has been reports of cases of encephalitis in which antibodies has been detected against deep grey matter neurons. Antibodies have been detected against the N-methyl-D-aspartate (NMDA) receptors or voltage-gated potassium channels (VGKC). VGKC antibody positive encephalitis usually presents in middle aged or elderly people and is twice as common in men. The main symptoms are memory loss and seizures. Cognitive impairment, confusion, disorientation, personality change and behavioral disturbances are also common. Encephalitis associated with NMDA receptor antibodies is mainly seen in children and young adults, with women being affected about three to four times more often than men. Initially there is a period of cognitive impairment and psychiatric features like hallucinations, agitation and depression but later there is gradual deterioration in conscious level with autonomic features often requiring intensive care. The most striking feature of both these types of encephalitis is their uniform good response to immunomodulating therapies. Intravenous immunoglobulin (IVIG), glucocorticosteroids, plasma exchange and drugs like cyclophosphamide, rituximab, mycophenolate mofetil, azathioprine, singly or in combination have all produced good results. It is this surprisingly easily available effective treatment which makes diagnosis of this condition imperative. Encephalitis is a condition which is encountered by physicians at all levels and so a high level of awareness about the condition will ensure that the condition is not missed. This review about this new condition is being presented to make physicians aware about this devastating but easily treatable disease. DOI: http://dx.doi.org/10.3329/jom.v13i2.12755 J Medicine 2012; 13 : 179-189
Highlights
With the development of modern medical science, diagnosis of various neurological disease is becoming easier day by day
In 2009, the first 10 cases of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis identified at CEP were reported and compared with encephalitis cases resulting from viral etiologies.[44]
Ten anti-NMDAR+ patients were profiled with a median age of 18.5 years
Summary
With the development of modern medical science, diagnosis of various neurological disease is becoming easier day by day. In 2009, the first 10 cases of anti-NMDAR encephalitis identified at CEP were reported and compared with encephalitis cases resulting from viral etiologies.[44] Ten anti-NMDAR+ patients were profiled with a median age of 18.5 years (range 11-31 years) They had a characteristic progression with prominent psychiatric symptoms, autonomic instability, significant neurologic abnormalities, and seizures. In patients with features of Encephalitis they proposed that patients should ideally be tested for serum (paired with CSF if possible) antibodies to voltage-gated potassium channel (VGKC) complex antigens (leucine rich glioma inactivated protein 1 (LGI1), contactin-associated protein-2 (CASPR2) and contactin-2), á-amino-3-hydroxy-5-methyl-4isoxazolepropionic acid receptors (AMPARs), ãaminobutyric acid-B receptors (GABABRs), glutamic acid decarboxylase (GAD), N-methyl-D-aspartate receptor (NMDAR) and for onconeural antibodies ( antiHu, anti-Ma 1/2, CV-2, and amphiphysin). About 60% of cases have low serum sodium consistent with the syndrome of inappropriate antidiuretic hormone (SIADH).[23, 30,67] A similar proportion have medial temporal lobe high signal on magnetic resonance imaging, consistent with localised inflammation, which if untreated may lead to focal hippocampal atrophy,[23] a substrate for both memory impairment and adult onset medial temporal lobe epilepsy.[69]
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