A two-and-a-half-year-old girl presented at the Indus Hospital & Health Network, Korangi Campus, Karachi in 2021 with complaints of altered behaviour, decreased consciousness and seizures for the last 3 days. Her illness started with fever 1 week back which persisted for a few days, and then subsided when she arrived to the hospital. She had had intermittent episodes of generalized tonic-clonic seizures at home and was persistently drowsy according to her parents. She was a healthy girl before this illness. There was no history of trauma, diarrhoea or viral symptoms preceding the illness. She was a developmentally appropriate child, with no past history of seizures. Her birth, vaccination and family history were unremarkable. On admission, her physical examination revealed an unoriented child with height at 75th centile, weight at 50th centile and front occipital circumference of 49 cm. Her pulse was 122beats per min, RR 30 breaths per min and BP 99/55 mmHg. Neurological examination showed GCS 9/15, hypotonia with normal reflexes and decreased power 3/5 in all four limbs. Her cranial nerves were intact but higher mental functions including memory, speech and cognition were lost. Rest of the systemic examination was unremarkable.
She was admitted initially in high dependency unit as a case of suspected meningoencephalitis, was given ceftriaxone and acyclovir empirically and managed for seizures with IV Levetiracetam. Neuroprotective measures (Head in midline with 30-degree elevation, normal oxygenation, ventilation, euglycemia, normothermia, normotensive, monitoring of vitals, perfusion was done and ensured for the signs of raised ICP, seizures and electrolyte imbalance) Her baseline labs including blood counts, renal & liver functions and serum electrolytes were all within normal limits. Her lumbar puncture was done and CSF showed a sugar of 65mg/dl, protein of 25mg/dl and cells 3.0/ul. HSV PCR came out to be negative. There was no response to acyclovir. Her seizures remained uncontrolled despite giving maximum dose of levetiracetam and topiramate and phenobarbitone had to be added to control the seizures. MRI brain with contrast was done which was unremarkable.
After a few days of admission, she developed involuntary movements of limbs and face, suggestive of choreoathetosis and orofacial dyskinesias, respectively. She also exhibited psychiatric-behavioural symptoms with prominent anxiety, agitation and aggression; had bouts of such episodes during which she shouted out loudly and pulled out her hair. These symptoms were progressive and frequent along with insomnia. She was put on clonazepam, haloperidol and clonidine to control these worsening symptoms. There were no signs of autonomic instability. She also had a few manic episodes in between with excessive laughter. There was regression in her gross and fine motor skills. She could not make social or eye contact and could not speak. Her EEG was done which showed diffuse slow activity in the delta and theta range.
On the basis of these symptoms, a suspected diagnosis of autoimmune encephalitis was made, workup was sent and immunotherapy was started empirically. A repeat lumbar puncture was done to send samples for CSF anti-NMDA receptor antibodies and CSF oligoclonal bands. CSF again did not show pleocytosis or increased protein. Meanwhile, she was given IV methylprednisolone pulse therapy (30mg/kg) once a day for 5 days. There was no significant response to pulse therapy so, she was given intravenous immunoglobulins (IVIG) 1 gm/kg for 2 days. This resulted in improvement in some of her behavioural symptoms, insomnia and seizures, but her social, verbal, gross and fine motor skills did not improve. Her CSF anti NMDAR antibodies were reported positive (+++) and oligoclonal bands were present in CSF. This confirmed our diagnosis of anti NMDAR encephalitis. She was worked up for underlying teratoma by ultrasound abdomen and pelvis, which was found to be normal. She was discharged to home on oral medications and called for follow-up in out-patient clinic. Her parents were counselled regarding the disease prognosis and slow recovery. The patient lost to follow in opd.
