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Neurological features

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

Authoring team

The neurological manifestations of type I neurofibromatosis include:

  • increased incidence of peripheral nerve and CNS tumours, particularly:
    • meningioma
    • acoustic neuroma
    • optic nerve glioma
    • malignant peripheral nerve sheath tumours (MPNST) (1,2)
      • MPNST is the term now used to refer to malignant schwannomas, neurogenic sarcomas, malignant neurilemomas, neurofibrosarcomas and spindle cell sarcomas
      • NF1 patients show an 8–13% lifetime risk of developing MPNSTs (1)
      • MPNSTs in NF-1 have a male predilection and occur approximately a decade earlier than in the general population
    • central nervous system tumours
    • spinal root tumours with or without compression

  • seizures

Reference:

  1. Frahm S et al. Genetic and phenotypic characterization of tumor cells derived from malignant peripheral nerve sheath tumors of neurofibromatosis type 1 patients. Neurobiol of Dis 2004;16 (1): Pages 85-91.
  2. Hillier JC, Moskovic E. The soft-tissue manifestations of neurofibromatosis type 1.Clin Radiol 2005; 60 (9): 960-967

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