| BackgroundAmyotrophic lateral sclerosis (ALS), a fatal neurodegenerative disease characterized by weakness, muscle atrophy, fascilations and dyspnea, is the most common type of motor neuron disease(MND). Hudson in1981published the article pointed out:sporadic and familial ALS patients had executive functional, behavior and language deficits. severe cases could progress to FTD. Therefore there is a view that ALS involving not only the nervous system, but also the frontal temporal lobe cortex and hippocampus neurons, is a multisystem degeneration disease.ObjectiveTo discovery ALS patients’cognitive impairment status and the relationship between the age of onset, the location, the course of disease and cognitive impairmentMethodsThe study collected32cases of the Second Affiliated Hospital. Zhejiang University, from January2012to March2013who were diagnosed of definite or probable amyotrophic lateral sclerosis. A total of45health person were enrolled as control group. All the objects were separated into two groups:Group1,32patients with ALS, including18males,14females, aged from34to76, school aged from0to15years, the course of disease varies from2to24months. The mean age of this group is54.5±11.4, the mean school age of this group is7.8±4.1years, the mean course of disease if11.1±6.7months.32cases of ALS patients included10with limb-onset,9with unilateral upper limb-onset,9with unilateral lower limb-onset and4with bilateral upper limbs or lower limbs-onset. All recruited patients met the criteria for probable or definite ALS as defined by the El Escorial diagnostic criteria for ALS. Exclusion criteria included history of other neurologic conditions that could affect cognition (stroke, dementia), dyspnea, color blindness, deafness and patients who was suffering serious illness.Group2,45healthy people as control group, including26males,18females, aged from32to77, school aged from1to17years. The mean age of this group is53.4±11.3, the mean school age of this group is8.2±4.2years. The control group was collected after exclusion of color blindness, deafness, alcohol dependence syndrome, use of high-dose psychoactive medication and patients who was suffering serious illness. The study used ALS functional rating scale, mini-mental state examination, frontal assessment battery, verbal category fluency test, clock drawing test and stroop color word interference test were used to evaluate the ALS patients and normal controls’cognitive function.23cases of ALS patients were collected to do the event related potentials.17patients and17healthy people with out using drugs, did the quantitative EEG examination. All statistical analyses were performed using the Statistical Program for Social Sciences (SPSS) statistical software(version21.0). All measurement data are presented as mean±SD, Comparisons were made using χ2test for categorical variables and two-sample T test. correlation analysis using Pearson correlation analysis; P<0.05was considered statistically significant.ResultsThe effect of gender age and school age of different groups were analyzed, and the differences were not statistically significant between each two groups(P>0.05). There were4patients according with the Petesen’diagnostic criteria of mild cognitive impairment. ALS functional rating scale score (22.8±5.7) of ALS patients with bilateral upper limbs or lower limbs-onset was significantly lower than the score(30.6±3.7) of patients with bulbar-onset, lower than the score (30.9±4.6) of patients with unilateral lower limb-onset, and also lower than the score (32.2±5.2) of patients with unilateral upper limb-onset, and the differences were statistically significant (P<0.05). Mini-mental state examination score (26.7±2.9) of ALS group was significantly lower than the control group (28.8±1.7), and the difference was statistically significant (P <0.01). Mini-mental state examination score of ALS patients and ALS functional rating scale score were positively correlated (r=0.352), with a statistically significant (P<0.05). Mini-mental state examination score (24.0±4.55) of ALS patients with bilateral upper limbs or lower limbs-onset was significantly lower than the score (28.0±1.63) of patients with bulbar-onset, and the difference was statistically significant (P <0.05). Frontal assessment battery score of ALS patients (15.8±2.3) was significantly lower than the control group (17.5±2.2), and the difference was statistically significant (P<0.01). Verbal category fluency test score (1.6±0.8) of ALS patients was lower than the control group (1.9±0.3), and the difference was statistically significant (P <0.05). The Ta time (46.4±30.7)s of stroop color word test of ALS patients was significantly longer than the control group (28.0±6.1)s. The Tb time (65.3±43.3)s of ALS patients was significantly longer than the control group (25.4±10.1)s and the difference was significant significant (P<0.01). The Tcl-Ta time (29.2±49.6)s of stroop color word test time of ALS patients was significantly longer than the control group (9.3±8.3)s, the Tc2-Tb time (55.4±51.7)s of ALS patients was significantly longer than the control group (37.2±13.7)s, and the difference was statistically significant (P<0.05). Clock drawing test score of two groups was analyzed, and the difference was not statistically significant (P>0.05). The latency (364.7±44.2) ms of P300of ALS group was significantly longer than the control group (306.0±18.9) ms. The amplitude (9.3±8.1) uv of ALS group was significantly less than the control group (15.4±2.3) uv. Reaction tiem (453.0±122.6) ms of ALS group was significantly longer than the control group (359.1±31.8) ms. And three differences were statistically significant (P<0.01). Quantitative EEG’s (δ+θ)/(a+(3) value (1.015±0.375) of ALS group was significantly less than the control group (0.479±0.112), and the difference was statistically significant (P<0.01).ConclusionNewly diagnosised ALS is often associated with mild cognitive impairment, including impaired executive function, language function, and an interference control, visual-spatial ability is relatively complete. ALS patients with bilateral upper limbs or lower limbs-onset progress faster and suffer more severe cognitive impairment, compared with patients with other onset form. P300and QEEG examination helped us to find ALS patients who have under clinical cognitive impairment. |
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