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Adult-Onset Anti-N-methyl-D-aspartate-receptor Encephalitis Presenting as a Non-Fluent Aphasia

In the late 1990s and early 2000s, cases were described of previously healthy young women who presented with a benign ovarian teratoma and subacute limbic encephalitis, which reversed at least partially following teratoma resection and immunosuppression. In 2007, Dalmau et al. coined the term “anti-N-methyl-D-aspartatereceptor (NMDAR) encephalitis” after discovering that these patients had antibodies against the NMDAR. Most patients initially present with a non-specific prodrome of headaches, fever, nausea, and psychiatric symptoms. Many also develop short-term memory impairment, seizures, and movement disorders, often progressing to decreased level of consciousness and autonomic instability requiring intensive care unit admission. Poor outcome, usually defined as a modified Rankin Scale of 3-5, occurs in ~10% of patients and death in another 10%. Early identification and treatment of the disorder is thought to be of prime importance in improving outcomes. Aphasia as a sign of anti-NMDAR encephalitis has been poorly characterized and is neither typically thought to be a localizing sign nor a presenting feature of this disorder. We describe a case of anti-NMDAR encephalitis that presented as a progressive non-fluent aphasia. A 29-year-old English-speaking female with no significant past medical history reported a 6-day history of progressive wordfinding difficulties followed by a 3-day history of multiple stereotypic episodes. The latter episodes lasted a few minutes each and were characterized by a prodrome of metallic taste, and a “scratching” sensation in her teeth, which then evolved into right-sided facial twitching, right hand cramping, and right face and arm paresthesiae without loss of consciousness, presumably representing simple partial seizures. She was brought to the emergency department following a first witnessed generalized tonic-clonic seizure. Initial neurological examination revealed agrammatic nonfluent speech and writing (see Figure 1A). She could name only