Abstract
Anti-leucine-rich glioma inactivated protein-1 (LGI-1) encephalitis is a rare autoimmune disease with a varied neuropsychiatric clinical manifestation and have potential for reversal with immunotherapy. Early identification and treatment are therefore of paramount importance. We present the case of a 43-year-old man admitted for acute psychosis with facio-brachial dystonic seizures not responding to anti-epileptic drugs (AEDs). His symptoms started approximately 5 months prior admission to the hospital and associated with progressive cognitive impairment (particularly verbal and visio-spatial domains) followed by altered behavior, irrelevant talk, and disturbance in daily activities of living and facio-brachial dystonic seizures. There was history of allergic reaction to valproate, carbamazepine and levetiracetam, and serum sample tested strongly positive for LGI-1 (leucine rich glioma inactivated-1) antibodies. He was treated with injection methylprednisolone (1 gm intravenous) brief pulse therapy for 7 days and then underwent four sessions of plasma exchange followed by oral steroids and immunosuppressant therapy. Treatment was successful and patient returned to his previous baseline functioning but patient develops steroid induced hypomania and diabetes mellitus in the due course of time. Patient was discharged after successful resolution of symptoms. Patient did not come up for follow-up. He was contacted telephonically and we get to know that he relapsed after 2 weeks and consulted at higher center where he died while on treatment. Prognosis of anti-LGI-1 encephalitis is usually good but our patient died.
Highlights
Limbic encephalitis (LE) is a prototype of autoimmune encephalitis which is a rare neurological disorder
leucine-rich glioma inactivated protein-1 (LGI-1) is a protein secreted by hippocampal neurons largely associated with epilepsy
In some cases of encephalitis, patients produce autoantibodies directed against LGI-1
Summary
Limbic encephalitis (LE) is a prototype of autoimmune encephalitis which is a rare neurological disorder. Case report Patient Mr R, a 43-years-old illiterate, married, male, and belonging to lower SES presented with altered behavior, visual hallucinations, jerky movements of face and arm, cognitive decline, ataxic gait, and multiple falls associated with physical injuries. His symptoms started approximately 5 months prior admission to our hospital. Normal muscle tone and bulk with elicitable deep tendon reflexes was noted in all four limbs with bilaterally flexor plantar. He had no other neurological deficits, and the remainder of his examination was unremarkable. Patient was followed up at 1 month postdischarge through telephone consultation when family members reported relapse of symptoms and patients’ demise about a week back
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