title: Frontal dysfunctions of ALS-PBP patients in relation to their bulbar symptoms and rCBF decline.
abstract: BACKGROUND
  Amyotrophic lateral sclerosis (ALS) is a progressive and fatal disease characterized by progressive degeneration of spinal and bulbar motor neurons. However up to 50% ALS patients may also have cognitive dysfunction which has not been fully examined.
  
  
  METHODS
  35 ALS patients [23 patients presenting with limb-type ALS (ALS-limb) and 12 patients presenting with primary bulbar palsy (PBP)-type ALS (ALS-PBP)] and 5 Spinal and Bulbar Muscular Atrophy (SBMA) patients have participated. To assess cognitive function mini-mental state examination (MMSE), Hasegawa dementia scale-revised (HDS-R), and frontal assessment battery (FAB) were performed. Additionally the time to complete card flipping with a computerized touch panel-type screening test was also examined. To investigate regional cerebral blood flow (rCBF), single-photon emission computed tomography (SPECT) using a (99m)Tc labeled ethyl cysteinate dimer ((99m)Tc-ECD) were performed. Statistical image analysis was performed using the easy Z-score imaging system (eZIS).
  
  
  RESULTS
  HDS-R and FAB scores were significantly lower in the ALS-PBP group than in age- and gender-matched control subjects or ALS-limb groups (p<0.01). The time to complete card flipping was significantly longer in the ALS-PBP group than in the control and ALS-limb groups (p<0.01). Although MMSE, HDS-R and FAB scores and the time to complete card flipping had no correlation with ALS functional rating scale-revised (ALSFRS-R) or Norris-limb scale scores, FAB score (r=0.63, p <0.01) and the time to complete card flipping (r=-0.66, p<0.01) were strongly correlated with Norris-bulbar score. The eZIS showed that rCBF of the frontal lobe was more severely declined in ALS-PBP patients than in ALS-limb patients (p<0.05).
  
  
  CONCLUSIONS
  These results suggest a selective decline of frontal cerebral functions in the ALS-PBP group in relation to their bulbar symptoms and rCBF decline.
authors.affiliation: Department of Neurology, Graduate School of Medicine, Dentistry and Pharmaceutical Science, Okayama University, Okayama, Japan.
authors.forename: Nobutoshi
authors.initials: N
authors.lastname: Morimoto
authors.forename: Tomoko
authors.initials: T
authors.lastname: Kurata
authors.forename: Kota
authors.initials: K
authors.lastname: Sato
authors.forename: Yoshio
authors.initials: Y
authors.lastname: Ikeda
authors.forename: Shuhei
authors.initials: S
authors.lastname: Sato
authors.forename: Koji
authors.initials: K
authors.lastname: Abe
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications / Q000000981:diagnostic imaging / Q000503:physiopathology*; D010244:Bulbar Palsy, Progressive / Q000150:complications / Q000000981:diagnostic imaging / Q000503:physiopathology*; D002560:Cerebrovascular Circulation / Q000502:physiology*; D003072:Cognition Disorders / Q000150:complications / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000503:physiopathology; D018450:Disease Progression; D005260:Female; D005625:Frontal Lobe / Q000000981:diagnostic imaging / Q000503:physiopathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D020966:Muscular Disorders, Atrophic / Q000150:complications / Q000000981:diagnostic imaging / Q000503:physiopathology*; D009483:Neuropsychological Tests; D011877:Radionuclide Imaging