title: Autoimmune encephalitis: A potentially reversible cause of status epilepticus, epilepsy, and cognitive decline.
abstract: OBJECTIVE
  To review clinical characteristics and response to immunomodulation therapy in autoimmune encephalitis presenting with status epilepticus (SE), epilepsy, and cognitive decline.
  
  
  METHODS
  Observational, prospective case series.
  
  
  METHODS
  All India Institute of Medical Sciences, New Delhi, India.
  
  
  METHODS
  Prospective analysis of 15 patients, who presented with SE, epilepsy, cognitive decline, and other neurological symptoms with positive autoantibodies. Demographic and clinical characteristics were recorded. Brain magnetic resonance imaging (MRI), cerebrospinal-fluid analysis (CSF), and tumor screening were done periodically. Treatment received and responses (categorized as per patients and treating doctor's information) were noted.
  
  
  RESULTS
  There were 15 (males = 10) patients of autoimmune encephalitis. The mean age of presentation was 24 years (range: 2-64 years). The most common onset was subacute (64%) and four (29%) patients presented as SE. Predominant clinical presentations were seizures (100%) almost of every semiology. CSF was done in 10 patients; it was normal in 60%. Brain MRI was done in all patients, in six (40%) it was normal, six (40%) showed T2W and FLAIR hyperintensities in bilateral limbic areas. Antibodies found were the N-methyl-D-aspartate receptor antibody in seven (50%), voltage-gated potassium channel antibody in five (36%), two of antiglutamic acid decarboxylase, and one patient with double stranded DNA (dsDNA) antibodies. None showed evidence of malignancy. Patients received immunotherapy, either steroids, intravenous immunoglobulin, or both. Follow-up showed significant improvement in majority of cases, neither further seizures nor relapse in nine (67%) cases. One death occurred, due to delayed presentation.
  
  
  CONCLUSIONS
  Uncommon but potentially reversible causes of SE, epilepsy, and cognitive decline may be immune-related and high index of suspicion will prevent missing the diagnosis.
authors: Department of Neurology, All India Institute of Medical Sciences, New Delhi, India.
authors: Awadh Kishor
authors: AK
authors: Pandit
authors: Kavish
authors: K
authors: Ihtisham
authors: Ajay
authors: A
authors: Garg
authors: Sheffali
authors: S
authors: Gulati
authors: Madakasira Vasantha
authors: MV
authors: Padma
authors: Manjari
authors: M
authors: Tripathi
keywords: Autoimmune encephalitis; cognitive decline; drug refractory epilepsy; seizures; status epilepticus