title: Enlargement of the Nissl substance as a manifestation of early damage to spinal cord motoneurons in amyotrophic lateral sclerosis.
abstract: OBJECTIVE
  Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease of motoneurons. Recent studies indicate that in ALS, degeneration of motoneuron body is late in comparison to degeneration of axons. The morphological consequence of the axonal damage is chromatolysis. Therefore, loss of tigroid in motoneurons as a morphological manifestation of chromatolysis should be a prominent feature seen in an early stage of the disease. To verify that assumption we examined morphologically spinal cord motoneurons in patients with sporadic ALS.
  
  
  METHODS
  In anterior horn motoneurons of 33 patients tigroid were assessed at light microscopy and morphometrically analyzed. Material was divided into an "acute" ALS group with a duration of the disease of up to 1 year, and a "chronic" ALS group with a clinical course lasting for 4 - 9 years.
  
  
  RESULTS
  In the "acute" ALS group, loss of motoneurons was slight, and only a part of them showed central chromatolysis. Instead of chromatolysis the enlargement of the tigroid was found. This phenomenon was observed only in "acute" ALS and confirmed by morphometric analysis.
  
  
  CONCLUSIONS
  In ALS, enlargement of the tigroid seems to be an early morphological feature - occuring earlier than central chromatolysis. Its presence may be connected with endoplasmic reticulum stress, disturbed axonal transport or functional compensation of the neuronal deficit.
authors.forename: Dorota
authors.initials: D
authors.lastname: Dziewulska
authors.forename: Anna
authors.initials: A
authors.lastname: Gogol
authors.forename: Pawel
authors.initials: P
authors.lastname: Gogol
authors.forename: Janina
authors.initials: J
authors.lastname: Rafalowska
mesh_terms: D000328:Adult; D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000473:pathology*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009046:Motor Neurons / Q000473:pathology*; D009562:Nissl Bodies / Q000473:pathology*; D013116:Spinal Cord / Q000473:pathology*