De novo nose-pinching stereotypy with somnolence: Clues to autoimmune encephalitis

Autoimmune encephalitis (AE) is being increasingly recognized as a cause of new-onset movement disorders. Movement disorders in AE are diverse and range from hyperkinetic conditions such as oromandibular dyskinesias, tremors and chorea to hypokinetic ones such as bradykinesia and parkinsonism. Stereotypies have been described in association with anti-NMDAR encephalitis. Similarly, sleep dysfunction is an underrecognized feature in many AE subtypes, prominently anti-IgLON5 although the correlation of phenotype of sleep dysfunction with a particular antibody subtype in AE is unclear. Despite the recognition of both these features as part of an overreaching spectrum in any patient with AE, seldom are they the sole presenting manifestations. Additionally, the challenge is further compounded in a patient who has seronegative AE since neither sleep disturbances nor stereotypies have been well characterized with this condition yet, and the diagnosis is conditional to exhausting a list of ancillary supportive features. In this brief communication, we describe the case of a young man who presented with hypersomnolence and an unusual focal nose-pinching stereotypy of subacute onset who lacked the presence of other typical clinical characteristics such as cognitive/memory impairment and seizures and had negative autoimmune antibodies but responded to immune therapy dramatically. We propose that the presence of de novo hypersomnolence and stereotypy should inform a potential diagnosis of AE.