Primary lateral sclerosis as a phenotypic manifestation of familial ALS.

نویسندگان

  • F Brugman
  • J H J Wokke
  • J M B Vianney de Jong
  • H Franssen
  • C G Faber
  • L H Van den Berg
چکیده

Primary lateral sclerosis (PLS) is a diagnosis of exclusion in patients with progressive spinobulbar spasticity and could be part of the clinical spectrum of ALS. Unlike ALS, which is familial in 5 to 10% of the cases, PLS has been described as a sporadic disorder in adults. The authors report two patients with PLS from unrelated SOD1-negative familial ALS families. These observations provide further evidence that PLS can be linked pathophysiologically to ALS.

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عنوان ژورنال:
  • Neurology

دوره 64 10  شماره 

صفحات  -

تاریخ انتشار 2005