Cortical Aphasia and Apraxia as Main Clinical Presentation of Anti-NMDAR Encephalitis Relapse with a Positive CSF PCR for CytomegalovirusAuthor(s): Leon-Salas Jorge Mario, Rosas Ramiro, Diaz-Victoria Ana Ruth, Flores Jose, Ojeda-Lopez Carmen, Espinola-Nadurille Mariana
We present a patient with severe aphasic and apraxic syndromes as her clinical manifestation of anti-NMDAR encephalitis. Nineteen months earlier she had a first neurological episode consistent of a first event of the disease. Distinctive paraclinical findings and treatment measures are highlighted.
Materials and Methods:
A 17 years old female with previous history of seizures presented with headache, dizziness, nausea, and vomiting, that progressed to inattention and reduced, non- fluent spontaneous speech, altered repetition and nomination with literal paraphasias and spared comprehension (motor aphasia).
Initial cerebrospinal fluid (CSF) showed mild pleocytosis. In spite of antiviral treatment with acyclovir, her cognitive performance worsened to severe global aphasia and an apraxic syndrome. MRI, EEG and 18-PET Scan showed left generalized cortical abnormalities. Anti-NMDAR autoantibodies by immunofluorescence were positive in serum and CSF. Pulses of methylprednisolone were started with improvement. Still her cognitive deficits were disabling, so steroids were combined with 5 sessions of plasma exchange, a combination regarded as first-line immunotherapy treatment in anti-NMDAR encephalitis. Prompt and strong improvement was achieved. A pelvic ultrasound and a contrast-enhanced CT scan were negative for ovarian teratoma. Retrospectively we concluded that she had had a first neurological episode of autoimmune encephalitis that went undiagnosed and was self-limited 19 months earlier. Cyclophosphamide was prescribed as an outpatient to reduce the risk of relapses.
This case widens the clinical spectrum of anti-NMDAR encephalitis in adolescents.