Abstract

Cortical Aphasia and Apraxia as Main Clinical Presentation of Anti-NMDAR Encephalitis Relapse with a Positive CSF PCR for Cytomegalovirus

Author(s): Leon-Salas Jorge Mario, Rosas Ramiro, Diaz-Victoria Ana Ruth, Flores Jose, Ojeda-Lopez Carmen, Espinola-Nadurille Mariana

Objective:

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).

Results:

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.

Conclusion:

This case widens the clinical spectrum of anti-NMDAR encephalitis in adolescents.