Can Treatment of Sudden Speech Impairment Be Life-Saving?

Background/Purpose: Pediatric anti-NMDA receptor encephalitis is a rare and life-threatening disease. Timely diagnosis and treatment initiation directly influences outcome. Therefore, a high level of suspicion—particularly in mild new focal neurological deficits—is of utmost importance to prevent neurological sequelae.

Case Presentation: We report on a 12.6-year-old female, who initially presented with two self-limiting focal seizures. Wake and sleep EEG were normal. Brain MRI revealed a leptomeningeal enhancement in the right central region. CSF showed a mild pleocytosis (total white blood cells 85/mm³; lymphocytes 62/mm³; monocytes 23/mm³), pointing towards the diagnosis of viral meningitis. The patient recovered completely and control MRI of the brain 4 weeks later was normal. However, at that time she developed a sudden speech impairment with predominant dysarthria, headache, and tiredness. EEG was again unremarkable, ruling out nonconvulsive status epilepticus. Extensive laboratory workup revealed positive anti-NMDA receptor antibodies in CSF (1:10) and serum (1:10). After 1 g of intravenous methylprednisolone daily for 5 days, dysarthria improved significantly and complete recovery was documented within 4 weeks after symptom onset. The course of dysarthria is documented in a series of videos.

Conclusion: Pediatric anti-NMDA receptor encephalitis can present with a wide spectrum of symptoms, resulting in a life threatening condition, if untreated. Diagnostic workup of rapid onset of speech dysfunction should include immediate testing of anti-NMDA-receptor antibodies in serum and CSF, as timely treatment initiation in this condition can be life-saving.

Publication History

Article published online:
28 October 2021