Auto immune limbic encephalitis; clinical profile and treatment response – a single center experience (original) (raw)
2019, Journal of the Neurological Sciences
AI-generated Abstract
Background: Auto immune limbic encephalitis are usually antibody mediated and may be a paraneoplastic manifestation. Results: We report a case series of thirteen patients were admitted with features suggestive of auto-immune encephalitis from January 2014 and March 2019. Age range was 18-37, with male: female ratio of 1:4.5. All of the patients had prodromal respiratory catarrh a week or two preceding onset of neurological symptoms. Psychosis was a prominent initial symptom in all patients. Extrapyramidal symptoms were seen in six. Seizures occurred in eleven patients, three of whom were refractory. Three had associated autonomic instability. Antibody testing was positive for VGKC antibodies in five, anti NMDA antibodies in six and two patients were seronegative for any antibodies. MRI was normal in anti NMDA encephalitis. It showed mesial temporal swelling/hyper intensities in patients with anti VGKC encephalitis, two of whom developed mesial temporal sclerosis at one year follow up. Antibody titers did not correlate with clinical manifestations. Paraneoplastic work up was positive for ovarian teratoma in one patient with anti NMDA encephalitis who had refractory encephalopathy despite tumor removal and aggressive immunotherapy. Most patients had near complete recovery with treatment (methyl prednisolone, immunoglobulins and/or PLEX). Most patients with anti VGKC encephalitis required long term anti epileptic medications. Conclusion: Prominent psychosis, dysautonomia and extrapyramidal symptoms after a prodrome of respiratory catarrh warrant early consideration of autoimmune encephalitis since early treatment initiation determines the outcome. Paraneoplastic etiology portends a poor prognosis.
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