Relationship between ALS and the degree of cognitive impairment, markers of neurodegeneration and predictors for poor outcome. A prospective study
Rusina R et al. – A prospective study shows age, disease severity and CSF beta-amyloid levels together as a risk factor suggesting shorter survival times. Dementia is relatively frequent in amyotrophic lateral sclerosis (ALS) and may be a consequence of either frontotemporal lobar degeneration (FTLD) or result from co-existing Alzheimer disease. Methods- Prospective study of 67 pts, 42 women, and 25 men, with clinically defined ALS
- Assessment of disease severity
- SPECT, lumbar puncture with determination of tau, hyperphosporylated tau (p-tau) and beta-amyloid and detailed neuropsychological assessment by standardized test battery
- Detailed neuropathologic evaluation for deceased pts
Results- Mean survival duration: 26.8 mo
- Delay between first signs and confirmation of diagnosis: 12.75 mo
- Cognitive impairment did not affect disease evolution
- No correlation between neuropsychological and SPECT findings
- Higher age at onset, more pronounced handicap and elevated beta-amyloid in CSF associated with shorter survival times
- In brain tissue from 9 of the deceased patients with ALS and dementia, all showed signs of comorbidity, 6 had FTLD hallmarks, and 3 showed Alzheimer disease pathology
- Brain tissues from 11 deceased ALS pts who did not show signs of dementia had only changes compatible with diagnosis of motor neuron disease
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