title: Olfactory dysfunction related to TDP-43 pathology in amyotrophic lateral sclerosis.
abstract: OBJECTIVE
  To clarify a possible contribution of TDP-43 pathology to odor dysfunction in amyotrophic lateral sclerosis patients.
  
  
  METHODS
  An 83-year-old woman suffered from muscle weakness, which started to deteriorate during the previous half year. In addition to her pyramidal signs, lower motor involvement was shown by needle electromyography; this upper and lower motor neuron involvement was suggestive of probable ALS. She presented with severe odor impairments but relatively preserved cognitive functions. Her autopsy findings revealed TDP-43-positive inclusions in the spinal motor neurons and cerebral limbic system without significant tau or α-synuclein deposits.
  
  
  CONCLUSIONS
  This case showed evidence suggesting that olfactory dysfunction was probably related to limbic TDP-43 pathology and was possibly independent of her Alzheimer pathology. Olfactory dysfunction does not necessarily indicate the presence of tau or α-synuclein pathology and could be an early sign of ALS with the limbic involvement of TDP-43 pathology even when cognitive functions are preserved.
authors.forename: Takahiro
authors.initials: T
authors.lastname: Takeda
authors.forename: Toshiki
authors.initials: T
authors.lastname: Uchihara
authors.forename: Shunji
authors.initials: S
authors.lastname: Kawamura
authors.forename: Takashi
authors.initials: T
authors.lastname: Ohashi
mesh_terms: D000369:Aged, 80 and over; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications* / Q000378:metabolism / Q000473:pathology; D004268:DNA-Binding Proteins / Q000502:physiology*; D005260:Female; D006801:Humans; D000857:Olfaction Disorders / Q000209:etiology*
chemical_list: D004268:DNA-Binding Proteins