This initial phase is followed by decreased responsiveness that can alternate between periods of agitation and catatonia, abnormal movements and autonomic instability

This initial phase is followed by decreased responsiveness that can alternate between periods of agitation and catatonia, abnormal movements and autonomic instability. a distinct type of autoimmune encephalitis that may present without a tumor.[2] It is a multistage illness that progresses from psychosis, memory deficits, seizures and language disintegration into a state of unresponsiveness with catatonic features, abnormal movements, and autonomic and Resiquimod breathing instability. It predominantly affects children and young adults, and responds to treatment but can relapse.[3] The main epitope targeted by the antibodies is in the extracellular N-terminal domain of the GluN1 subunit. Persistence of these antibodies has been described as long as six years after clinical remission.[4] Since its discovery, many cases have been reported from our country.[5,6] We report a unique case of anti-NMDAR encephalitis from North East India, who had relapse after a gap of eight years. To the best of our knowledge, relapsing anti-NMDAR encephalitis of such a long duration has not been reported so far. Case Report $$A 13-year-old girl was admitted in our hospital with difficult-to-control seizures. Her symptoms started with worsening of handwriting. Parents noticed that she had involuntary, repetitive pill-rolling hand movements on the right side. She then developed seizures of multiple phenotypes (focal seizures, GTCS, Resiquimod facio-brachial dystonic seizures) and Resiquimod had distinct periods of agitation with unresponsiveness and mutism [Figure 1, Video 1]. She appeared lucid between these episodes, attempting to form words but without speech production. Subsequently, she developed bruxism and dystonic posturing, and at times full body rigidity approaching opisthotonic posturing. She had insomnia, appeared afraid and agitated and cried abnormally and spontaneously. Her reflexes were brisk with unsustained clonus bilaterally, and at times she had waxy catatonia. Opsoclonus was noted during some time. After 10 days of admission, she developed high-grade fever and her sensorium deteriorated. She developed orofacial dyskinesia, autonomic instability, and Cd34 irregular respiration. Her hospital stay was complicated by sepsis, urinary tract infection and colitis. During this time her modified Rankin Scale (mRS) score was 5. Blood investigations including hemogram, liver function tests, renal function tests, serum electrolytes, thyroid function and viral markers were normal. Initial MRI Brain and repeat MRI Brain two weeks later were normal. The electroencephalogram (EEG) showed delta slowing in left hemispheric regions. The cerebrospinal fluid (CSF) analysis showed normal cell count, sugar and protein and was negative Resiquimod for Herpes Simplex and Japanese Encephalitis viruses. Serum antinuclear antibody (ANA) was negative. The girl was examined thoroughly for evidence of malignancy. Chest X-ray, contrast enhanced computed tomography (CECT) of abdomen with screening of thorax, ultrasound pelvis and skeletal survey using X-rays were done, and they did not reveal any associated tumor. Tumor markers (CA-125, CEA, -fetoprotein) were advised, but could not be done due to financial constraints. She was initially started on empirical Acyclovir on benefit of doubt, and her seizures were being managed with multiple antiepileptic drugs (AEDs). Anti-NMDAR antibody was detected by indirect immunofluorescence using cell-based assay with substrate as transfected HEK (human embryonic kidney cells) with NMDA-NR1 receptor protein [Figure 2]. She was started on a combination of intravenous methylprednisolone and intravenous immunoglobulins followed by oral prednisolone. Her symptoms started improving and by four weeks her mRS became 4. She continued improving on oral prednisolone as the AEDs were being tapered. At four months follow-up, her mRS was 1 [Video 2] Resiquimod and her residual deficits included amnesia, mild.