Future research should aim for a non-reductionistic integration of these two disciplines. Footnotes Source of Support: Nil Conflict of Interest: None. REFERENCES 1. is usually evident but an appeal to temper the enthusiasm by considering the historical lessons learnt from Karl Jaspers critique of General Paresis of Insane, is usually in place. Catatonic syndrome has to be conceptualised broadly and should be recognised with a separate nosological position. strong class=”kwd-title” Keywords: em Anti-NMDA receptor encephalitis /em , em catatonia /em , em neuropsychiatry /em , em NMDA receptor hypofunction hypothesis /em , em schizophrenia /em INTRODUCTION Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is usually a newly recognised autoimmune encephalitic syndrome with specific pattern of presentation, course, and outcome. After the initial reports by Dalmau em et al /em ., in 2007,[1] multiple centres around the world reported comparable cases. Initially conceptualised as a paraneoplastic syndrome, it was later defined as autoimmune encephalitis with varied immunological aetiologies like paraneoplastic condition, microdeletions in HLA system etc. The syndrome predominantly presents in young women with 60% of them using a neoplasm (usually, ovarian teratoma). The clinical course is usually characterised by five stages2 – prodromal stage, neuro-behavioural stage, nonresponsive stage, hyperactive stage and KD 5170 gradual recovery stage. Children may present with speech regression and irritability instead of catatonia or psychosis. Early identification and intervention is usually paramount in its management. Though magnetic resonance imaging (MRI) brain, electroencephalogram (EEG) and cerebro-spinal fluid (CSF) analysis are abnormal, the changes are not specific to this disease entity. IgG autoantibodies against NR1 subunit of NMDA receptor is usually taken as definitive for making the diagnosis. Paired serum and CSF sample has been found to be more useful for detection than either sample alone. Management is the prompt use of immunotherapy, with tumour resection if present. First line immunotherapy is with Intravenous (IV) Ig, Corticosteroids or Plasmapheresis. If there is less than adequate or no response, treatment is with second line therapy of Cyclophosphamide or Rituximab. After recovery, some recommend continued immunosuppression for at least 1 year in view of relapses. Up to 75 % of patients recover. Even after recovery, Dalmau em et al /em ., recommend periodic screening for ovarian teratoma for up to 2 years.[3] CASE REPORT A 27-year-old married lady, with no family history or personal history of psychiatric or neurological illness, with normal intellectual development presented to us with 2 years duration of illness. It was characterised by acute onset of posturing and involuntary movements of left toes, reduced arm swing and generalized slow movements, progressing to fearful and preoccupied attitude, crying spells and hallucinatory behaviour accompanied by functional deterioration. She was treated with antipsychotic brokers and electroconvulsive treatment which improved the psychotic symptoms but her motor symptoms worsened. At presentation to our centre, she was mute with episodes of agitation and hallucinatory behaviour. On examination, she was oriented and conscious with catatonic symptoms of mutism, negativism and gegenhalten. Tone was rigid in all four limbs without involuntary movements but had normal motor power. Baseline Bush-Francis catatonia rating scale score was 24. We made a presumptive diagnosis of a neurodegenerative disease with probable autoimmune aetiology, after ruling KD 5170 out other differentials like Schizophrenia with drug induced parkinsonism, SSPE, Wilsons disease, Nieman pick disease and Neuro-ferritinopathy with appropriate evaluation. MRI brain (T2W and FLAIR) images showed diffuse moderate atrophy in various subcortical and cortical areas along with long TR hyperintensities involving basal ganglia. CSF analysis and EEG were within normal limits. ESR, Thyroid antibodies and dsDNA were normal with antinuclear antibodies (ANA) being weakly positive, similar to a published case.[4] In view of persisting neurological Rabbit polyclonal to ACC1.ACC1 a subunit of acetyl-CoA carboxylase (ACC), a multifunctional enzyme system.Catalyzes the carboxylation of acetyl-CoA to malonyl-CoA, the rate-limiting step in fatty acid synthesis.Phosphorylation by AMPK or PKA inhibits the enzymatic activity of ACC.ACC-alpha is the predominant isoform in liver, adipocyte and mammary gland.ACC-beta is the major isoform in skeletal muscle and heart.Phosphorylation regulates its activity. symptoms and absence of any detectable neoplasm (evaluated with whole body FDG PET scan), we sent for anti-NMDA receptor antibody which came as positive. She showed gradual improvement after initiating immunotherapy in her psychotic and catatonic symptoms while continuing to have other deficits. DISCUSSION NMDA receptors maintain synaptic plasticity and their disruption causes seizures, memory deficits and behavioural problems. Intrathecally produced IgG autoantibodies against the NR1 subunit[5] of NMDA receptor, cap and internalise them, similar to acetylcholine receptor antibody action in myasthenia gravis, causing their hypofunction and subsequent symptoms. The pathophysiological mechanisms might have ethnic and genetic variability, highlighting the need for research in Indian population.[3] FDA has recently approved cell-based qualitative indirect immunoflourescence antibody test which has good specificity.[6] However, prevalence of anti-NMDA receptor antibodies in KD 5170 the general population, as is known for.
