Recovery is slow and often with neurological deficits if treatment is delayed

Recovery is slow and often with neurological deficits if treatment is delayed. antibodies and other inflammatory cytokines. Rituximab and cyclophosphamide aim to suppress antibody production. Recovery is usually slow and often with neurological deficits if treatment is usually delayed. With many distinctive clinical features, a specific antibody that aids diagnosis, and early Ombitasvir (ABT-267) effective treatment with commonly available drugs leading to good outcomes, NMDARE is usually a diagnosis that should be considered Ombitasvir (ABT-267) early in any case of unexplained encephalitis. strong class=”kwd-title” 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 population.[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 clear pathogen is identified. PKCA Autoimmune encephalitis (AIE) is an exciting 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 Ombitasvir (ABT-267) 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% had an infectious cause (including 19% with Herpes simplex encephalitis, 5% with Varicella encephalitis and 5% with em Mycobacterium tuberculosis /em ), 37% were of unknown cause and 21% had immune-mediated encephalitis (IME). Of the last category, 11% were diagnosed as acute disseminated encephalomyelitis (ADEM), while 9% had 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 em et al /em ., who also found only six Ombitasvir (ABT-267) 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 change. 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, change in 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 Ombitasvir (ABT-267) noted. However, it is essential 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 window 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.