Which cytogenic findings are characteristic of primary lateral sclerosis (PLS)?

Updated: Apr 28, 2014
  • Author: Hidehiro Takei, MD; Chief Editor: Adekunle M Adesina, MD, PhD  more...
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Answer

Answer

Primary lateral sclerosis (PLS) is typically sporadic with no family history; however, rare familial forms are seen. Mutations in the ALSIN gene on chromosome 2q33 (ALS2) cause juvenile PLS, which may overlap with the juvenile-onset UMN-predominant ALS. This condition is inherited in an autosomal recessive pattern. A unique locus (chromosome 4ptel-4p16.1) for an autosomal dominant form of adult-onset PLS in a large French-Canadian family has been mapped. [56]


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