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 99mTc labeled ethyl cysteinate dimer (99mTc-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). Conclusion: These results suggest a selective decline of frontal cerebral functions in the ALS-PBP group in relation to their bulbar symptoms and rCBF decline.
| Original language | English |
|---|---|
| Pages (from-to) | 96-101 |
| Number of pages | 6 |
| Journal | Journal of the Neurological Sciences |
| Volume | 319 |
| Issue number | 1-2 |
| DOIs | |
| Publication status | Published - Aug 15 2012 |
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Keywords
- Amyotrophic lateral sclerosis (ALS)
- Easy Z-score imaging system (eZIS)
- Frontal assessment battery (FAB)
- Regional cerebral blood ?ow (rCBF)
- Spinal and Bulbar Muscular Atrophy (SBMA)
ASJC Scopus subject areas
- Clinical Neurology
- Neurology
Cite this
Frontal dysfunctions of ALS-PBP patients in relation to their bulbar symptoms and rCBF decline. / Morimoto, Nobutoshi; Kurata, Tomoko; Sato, Kota; Ikeda, Yoshio; Sato, Shuhei; Abe, Koji.
In: Journal of the Neurological Sciences, Vol. 319, No. 1-2, 15.08.2012, p. 96-101.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Frontal dysfunctions of ALS-PBP patients in relation to their bulbar symptoms and rCBF decline
AU - Morimoto, Nobutoshi
AU - Kurata, Tomoko
AU - Sato, Kota
AU - Ikeda, Yoshio
AU - Sato, Shuhei
AU - Abe, Koji
PY - 2012/8/15
Y1 - 2012/8/15
N2 - 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 99mTc labeled ethyl cysteinate dimer (99mTc-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). Conclusion: These results suggest a selective decline of frontal cerebral functions in the ALS-PBP group in relation to their bulbar symptoms and rCBF decline.
AB - 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 99mTc labeled ethyl cysteinate dimer (99mTc-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). Conclusion: These results suggest a selective decline of frontal cerebral functions in the ALS-PBP group in relation to their bulbar symptoms and rCBF decline.
KW - Amyotrophic lateral sclerosis (ALS)
KW - Easy Z-score imaging system (eZIS)
KW - Frontal assessment battery (FAB)
KW - Regional cerebral blood ?ow (rCBF)
KW - Spinal and Bulbar Muscular Atrophy (SBMA)
UR - http://www.scopus.com/inward/record.url?scp=84862764143&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84862764143&partnerID=8YFLogxK
U2 - 10.1016/j.jns.2012.04.020
DO - 10.1016/j.jns.2012.04.020
M3 - Article
C2 - 22613760
AN - SCOPUS:84862764143
VL - 319
SP - 96
EP - 101
JO - Journal of the Neurological Sciences
JF - Journal of the Neurological Sciences
SN - 0022-510X
IS - 1-2
ER -