A young child of anti-NMDA receptor encephalitis presenting with epilepsia partialis continua: the first pediatric case in Korea

Abstract

Anti-N-methyl D-aspartate receptor (anti-NMDAR) encephalitis, recently recognized as a form of paraneoplastic encephalitis, is characterized by a prodromal phase of unspecific illness with fever that resembles a viral disease. The prodromal phase is followed by seizures, disturbed consciousness, psychiatric features, prominent abnormal movements, and autonomic imbalance. Here, we report a case of anti-NMDAR encephalitis with initial symptoms of epilepsia partialis continua in the absence of tumor. Briefly, a 3-year-old girl was admitted to the hospital due to right-sided, complex partial seizures without preceding febrile illness. The seizures evolved into epilepsia partialis continua and were accompanied by epileptiform discharges from the left frontal area. Three weeks after admission, the patient's seizures were reduced with antiepileptic drugs; however, she developed sleep disturbances, cognitive decline, noticeable oro-lingual-facial dyskinesia, and choreoathetoid movements. Anti-NMDAR encephalitis was confirmed by positive detection of NMDAR antibodies in the patient's serum and cerebrospinal fluid, and her condition slowly improved with immunoglobulin, methylprednisolone, and rituximab. At present, the patient is no longer taking multiple antiepileptic or antihypertensive drugs. Moreover, the patient showed gradual improvement of motor and cognitive function. This case serves as an example that a diagnosis of anti-NMDAR encephalitis should be considered when children with uncontrolled seizures develop dyskinesias without evidence of malignant tumor. In these cases, aggressive immunotherapies are needed to improve the outcome of anti-NMDAR encephalitis.

Anti-NMDAR Encephalitis in a 13-Year-Old Female: A 24-Month Clinical Follow-Up

“…The findings of the brain MRI in our patient were consistent with findings of anti-NMDAR encephalitis. 12 , 18 In most cases (89%), brain MRI results are normal or reveal non-specific focal changes at the initial presentation, as in this case. 6 Another study reported that most patients with NMDAR encephalitis (66%) had normal brain MRI findings, and the remaining 44% had a wide variation in the distribution and degree of T2-FLAIR hyperintense signal changes throughout the brain.…”

“…The patient in this study presented with epilepsia partialis continua, which is rarely reported in pediatric anti-NMDAR encephalitis, 12 , 13 along with motor aphasia and unilateral sensory changes. According to a recent study, the first neurological symptoms observed in young children with anti-NMDAR encephalitis included seizure (72%), particularly focal seizure (42%), with a median onset of 15 days before other encephalitis symptoms appeared; other patients mostly exhibited behavioral disorders (26%).…”

Anti-NMDA Encephalitis: A Rare Encephalitis in a Child- A Case Report

“…Following prodrome, early phase manifests with psychiatric or seizure activity and later progressing to deterioration in the consciousness, disintegration of speech and language, mutism, irritability, catatonia, and autonomic instability within next three to four weeks. 1,4,6 If treated, patient's recovery occurs with symptoms in reverse manner. 4 Early diagnosis and treatment has full or near full recovery among up to 75-80% of cases.…”

“…7 Brain MRI is abnormal in 35% of cases with non specific cortical and subcortical hyperintense signal abnormalities in T2. 1,6 Follow-up imaging may reveal cerebral atrophy. 6 A characteristic "extreme delta brush" EEG pattern characterised by beta-delta complexes are seen in paediatric patients (53% of cases).…”

Anti-N-methyl-D-aspartate Receptor (NMDAR) Encephalitis: Neuronal Burden of a Comorbid Ovarian Teratoma

“…The patient had a progressive course like our case with progression from continuous partial seizures to dyskinesia and cognitive impairment. However, the patient had no evidence of tumors, including ovarian teratomas 8) .…”