Majority of the patients were women (91%)

Majority of the patients were women (91%). and tongue mutilation are quite common. Seizures, are common and may be difficult to treat. The disease can be confirmed by serum and cerebrospinal fluid anti-NMDAR antibodies. Titers of these antibodies can also guideline response to treatment. Tumor removal is necessary if identified, followed by immunological treatment. Intravenous methylprednisolone and immunoglobulins aim to suppress/modulate immune response while plasma exchange attempts to remove antibodies and other inflammatory cytokines. JNJ 42153605 Rituximab and cyclophosphamide aim to suppress antibody production. Recovery is usually slow and often with neurological deficits if treatment is usually delayed. With many unique clinical features, a specific antibody that aids diagnosis, and early effective treatment with generally available drugs leading to good outcomes, NMDARE is usually a diagnosis that should be considered early in any case of unexplained encephalitis. Keywords: Autoimmune encephalopathy, autoimmune encephalitis, limbic encephalitis, N-methyl D-aspartate receptor encephalitis, paraneoplastic encephalitis Introduction Neurologists are often confronted with an encephalitic illness. They affect any age group with a wide spectrum of clinical presentation, the most common being headache, lethargy, fever, behavioral and personality changes leading on to drowsiness and seizures.[1] The annual incidence of encephalitis in the community is estimated to range from 3.5 to 7.4 cases per 100,000 populace.[2] Most are presumed to be infectious in origin and, in fact, more than 100 pathogens have been identified as a causative agent. However, often, no obvious pathogen is recognized. Autoimmune encephalitis (AIE) is an fascinating new group of disorders that is eminently treatable and should be considered in the routine differential diagnosis by every neurologist early on in the course of the illness. In this review, we provide the differential diagnosis of AIE and then focus on anti-NMDA (N-methyl D-aspartate) receptor antibody encephalitis (NMDARE). The first Indian case was recently described by one of us (BVM). Epidemiology A recent study has provided insights into the likely burden of encephalitic illness. Of 203 patients with an encephalitic illness, 42% experienced an JNJ 42153605 infectious cause (including 19% with Herpes simplex encephalitis, 5% with Varicella encephalitis and 5% with JNJ 42153605 Mycobacterium tuberculosis), 37% were of unknown cause and 21% experienced immune-mediated JNJ 42153605 encephalitis (IME). Of the last category, 11% were diagnosed as acute disseminated encephalomyelitis (ADEM), while 9% experienced other autoimmune causes. Among this subgroup, 1% of the patients were diagnosed with anti-NMDAR encephalitis,[3] a physique similar to that observed by Dalmau et al., who also found only six cases in a large series of 505 patients (1%).[4] Definitions and nosology It is easier to understand this category of illnesses in terms of neuroanatomical involvement. The common clinical presentations of AIE can be subdivided into limbic, diencephalic, brainstem encephalitis and encephalomyelitis. Patients with limbic encephalitis usually present with short-term memory loss, seizures, confusion, hallucinations, mood disorder and personality switch. The psychiatric manifestations can be prominent at the onset, the neurological features appearing later. The triad of anterograde amnesia, seizures and psychosis is fairly classic of limbic encephalitis. Diencephalic encephalitis presents with features of hypothalamicCpituitary dysfunction. Patients develop excessive daytime sleepiness (EDS), narcolepsyCcataplexy (with low cerebrospinal fluid hypocretin), hyperthermia, switch in excess weight (usually weight gain) or sexual dysfunction. In brainstem encephalitis (rhombencephalitis) cranial neuropathy, ophthalmoparesis, parkinsonism, dysarthria or dysphagia lead on to a lowered level of consciousness. In the encephalomyelitic variant, features of myelopathy and/or spasms and rigidity are also noted. However, it is essential Rabbit Polyclonal to APOL2 to note that patients may present with a forme fruste of a particular syndrome, and the full-blown picture can take time to develop. Table 1 lists some of the common AIEs. Table 1 Common autoimmune causes of encephalitis Open in a separate windows Anti-NMDA Receptor Encephalitis Background The original descriptions of anti-NMDA (N-methyl D-aspartate) receptor encephalitis (NMDARE) were confined to young women with ovarian teratomas, and was named acute juvenile non-herpetic encephalitis or ovarian teratoma-associated limbic encephalitis (OTLE) in Japan. Subsequently, Dalmau et al. published a seminal paper describing a series of 100 patients and detected the disorder in men and children also.[5] The disorder was correlated with the presence of an antibody directed against the extracellular N-terminal.