Academic journal article Indian Journal of Psychological Medicine

Catatonic Syndrome in Anti-NMDA Receptor Encephalitis

Academic journal article Indian Journal of Psychological Medicine

Catatonic Syndrome in Anti-NMDA Receptor Encephalitis

Article excerpt

Byline: Starlin. Mythri, Vivek. Mathew

Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is a newly recognised autoimmune condition. With its typical clinical pattern, consistent association with the presence of auto antibodies and rapid improvement with immunotherapy, this condition is giving insights into the boundaries between psychiatry and other neurosciences, and is opening avenues for future research. In a young lady who presented with catatonia, we considered anti-NMDA receptor encephalitis, after ruling out other aetiologies. After a positive antibody test we treated her with immunotherapy. She showed gradual improvement in her psychotic and catatonic symptoms. Knowledge regarding the nature and function of NMDA receptors and pathophysiology of this particular encephalitis is important for psychiatric practice. The great opportunity for research in this area due to its association with psychotic disorders is evident but an appeal to temper the enthusiasm by considering the historical lessons learnt from Karl Jaspers' critique of General Paresis of Insane, is in place. Catatonic syndrome has to be conceptualised broadly and should be recognised with a separate nosological position.

INTRODUCTION

Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is a newly recognised autoimmune encephalitic syndrome with specific pattern of presentation, course, and outcome. After the initial reports by Dalmau et al ., in 2007, [sup][1] multiple centres around the world reported similar 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 having a neoplasm (usually, ovarian teratoma). The clinical course is characterised by five stages [sup][2] - prodromal stage, neuro-behavioural stage, nonresponsive stage, hyperactive stage and gradual recovery stage. Children may present with speech regression and irritability instead of catatonia or psychosis.

Early identification and intervention is 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 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 et al ., recommend periodic screening for ovarian teratoma for up to 2 years. [sup][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 agents 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. …

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