A Novel Immune-Mediated Sleep-Related Hypermotor Syndrome with Sleep- Disordered Breathing

Clinicians should consider autoimmunity and perform antibody testing in patients with suspected neurodegenerative disease involving brain-stem and hypothalamic dysfunction, hypermotor sleep behavior, and sleep-disordered breathing.

This is an observational study of eight patients (aged 52–76 years; 3 men, 5 women) who presented with a unique neurological syndrome, abnormal sleep behavior, and serum and cerebrospinal fluid (CSF) antibodies against a previously unidentified neuronal cell surface antigen.

All patients presented with excessive motor events in non–rapid eye movement (NREM) sleep, characterized by a combination of jerks and complex elaborate movements mimicking identifiable daily-life activities in five patients on video polysomnography. Four patients had REM behavior disorder. All had sleep-disordered breathing. Other symptoms in several patients included variable gait instability, ataxia, dysarthria, and dysautonomia. Serum from all and CSF from five patie…

Reviewing Author

Sudhansu Chokroverty, MD, FRCP, FACP

A Novel Immune-Mediated Sleep-Related Hypermotor Syndrome with Sleep- Disordered Breathing

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Reviewing Author