REM sleep behavior disorder and narcoleptic features in anti-Ma2-associated encephalitis

  • Compta Y
  • Iranzo A
  • Santamaría J
 et al. 
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A 69-year-old man with anti-Ma2 paraneoplastic encephalitis presented with subacute onset of severe hypersomnia, memory loss, parkinsonism, and gaze palsy. A brain magnetic resonance imaging study showed bilateral damage in the dorsolateral midbrain, amygdala, and paramedian thalami. Videopolysomnography disclosed rapid eye movement (REM) sleep behavior disorder, and a Multiple Sleep Latency Test showed a mean sleep latency of 7 minutes and 4 sleep-onset REM periods. The level of hypocretin-1 in the cerebrospinal fluid was low (49 pg/mL). This observation illustrates that REM sleep behavior disorder and narcoleptic features are 2 REM-sleep abnormalities that (1) may share the same autoimmune-mediated origin affecting the brainstem, limbic, and diencephalic structures and (2) may occur in the setting of the paraneoplastic anti-Ma2-associated encephalitis.

Author-supplied keywords

  • Anti-Ma2-associated encephalitis
  • Hypocretin-1
  • Narcolepsy
  • REM sleep behavior disorder

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  • Yaroslau Compta

  • Alex Iranzo

  • Joan Santamaría

  • Roser Casamitjana

  • Francesc Graus

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