Narcolepsy, REM sleep behavior disorder, and supranuclear gaze palsy associated with Ma1 and Ma2 antibodies and tonsillar carcinoma

Arch Neurol. 2011 Apr;68(4):521-4. doi: 10.1001/archneurol.2011.56.


Objective: To describe a patient with diencephalic and mesencephalic presentation of a Ma1 and Ma2 antibody-associated paraneoplastic neurological disorder.

Design: Case report.

Setting: The Colorado Neurological Institute Movement Disorders Center in Englewood, Colorado, and the Mayo Clinic in Rochester, Minnesota.

Patient: A 55-year-old man with a paraneoplastic neurological disorder characterized by rapid eye movement sleep behavior disorder, narcolepsy, and a progressive supranuclear palsy-like syndrome in the setting of tonsillar carcinoma.

Intervention: Immunotherapy for paraneoplastic neurological disorder, surgery and radiotherapy for cancer, and symptomatic treatment for parkinsonism and sleep disorders.

Main outcome measures: Polysomnography, multiple sleep latency test, and neurological examination.

Results: The cancer was detected at a limited stage and treatable. After oncological therapy and immunotherapy, symptoms stabilized. Treatment with modafinil improved daytime somnolence.

Conclusions: Rapid onset and progression of multifocal deficits may be a clue to paraneoplastic etiology. Early treatment of a limited stage cancer (with or without immunotherapy) may possibly slow progression of neurological symptoms. Symptomatic treatment may be beneficial.

Publication types

  • Case Reports

MeSH terms

  • Antigens / immunology*
  • Antigens, Neoplasm / immunology*
  • Autoantibodies / biosynthesis
  • Carcinoma, Squamous Cell / complications
  • Carcinoma, Squamous Cell / diagnosis
  • Carcinoma, Squamous Cell / immunology
  • Humans
  • Male
  • Middle Aged
  • Narcolepsy / complications
  • Narcolepsy / diagnosis*
  • Narcolepsy / immunology
  • Nerve Tissue Proteins / immunology*
  • Ocular Motility Disorders / complications
  • Ocular Motility Disorders / diagnosis*
  • Ocular Motility Disorders / immunology
  • Paraneoplastic Polyneuropathy / complications
  • Paraneoplastic Polyneuropathy / diagnosis*
  • Paraneoplastic Polyneuropathy / immunology
  • REM Sleep Behavior Disorder / complications
  • REM Sleep Behavior Disorder / diagnosis*
  • REM Sleep Behavior Disorder / immunology
  • Supranuclear Palsy, Progressive / complications
  • Supranuclear Palsy, Progressive / diagnosis*
  • Supranuclear Palsy, Progressive / immunology
  • Tonsillar Neoplasms / complications
  • Tonsillar Neoplasms / diagnosis*
  • Tonsillar Neoplasms / immunology


  • Antigens
  • Antigens, Neoplasm
  • Autoantibodies
  • Ma1 antigen
  • Ma2 antigen
  • Nerve Tissue Proteins