Demographic and clinical characteristics of patients and controls.

<p>ALS, amyotrophic lateral sclerosis; ALSFRS-R, revised ALS functional rating scale; ALSSS, ALS severity scale; HC, healthy controls; PMA, progressive muscular atrophy; the disease progression rate was calculated according to the formula of (48-ALSFRS-R score)/disease duration (month).</p><p>Data were means±SD.</p><p>Demographic and clinical characteristics of patients and controls.</p

Behavioral impairment and mood differences between PMA and ALS.

<p>ALS, amyotrophic lateral sclerosis; FBI, frontal behavioral inventory; HAMA, Hamilton Anxiety Scale; HAMD, Hamilton Depression Rating Scale; PMA, progressive muscular atrophy.</p><p>Data were means±SD.</p><p>Behavioral impairment and mood differences between PMA and ALS.</p

Comparison of neuropsychological performances among patients with PMA, ALS and HC.

<p>3 PMA patients did not complete Stroop Color-Word Task; 2 PMA in copy tests and 1 PMA in digit span and Symbol Digit Modalities Test. ABC, Aphasia Battery Chinese; ALS, amyotrophic lateral sclerosis; CDT, clock drawing test; CMT, Clinical Memory Test; HC, healthy controls; PMA, progressive muscular atrophy; SIE, Stroop interference effect; time for SIE was calculated according to the formula of (Stroop C time-Stroop B time) and correct number for SIE was calculated according to the formula of (Stroop B correct number- Stroop C correct number); WAIS, Wechsler Adult Intelligence Scale; WMS, Wechsler Memory Scale.</p><p>Data were means±SD.</p><p>*p value remained to be significant after Bonferroni correction (α = 0.05/3 = 0.017).</p><p>Comparison of neuropsychological performances among patients with PMA, ALS and HC.</p

Spectrum of MND registered in Peking Union Medical College Hospital (n = 143).

<p>ALS, amyotrophic lateral sclerosis; MND, motor neuron disease; PBP, progressive bulbar palsy; PLS, progressive lateral sclerosis; PMA, progressive muscular atrophy.</p

Age distribution of included ALS patients and the proportion of cognitive impairment and FTLD in each age group.

<p>(A) Age distribution of included ALS patients. (B) Prevalence of cognitive impairment in each age group. ALS, amyotrophic lateral sclerosis; ALS-CI, ALS with cognitive impairment; ALS-FTLD, ALS with frontotemporal lobe degeneration.</p

Demographic and clinical characteristics of ALS-NC, ALS-ECI and ALS-FTLD.

<p>ALS, amyotrophic lateral sclerosis; ALSFRS-R, revised ALS functional rating scale; ALSSS, ALS severity scale;ALS-NC, ALS with normal cognition; ALS-ECI, ALS with executive cognitive impairment; ALS-FTLD, ALS with frontotemporal lobe degeneration; the disease progression rate was calculated according to the formula of (48-ALSFRS-R score)/disease duration(months).</p><p>Data were means±SD.</p><p>Demographic and clinical characteristics of ALS-NC, ALS-ECI and ALS-FTLD.</p

Categorization of Chinese patients with sporadic ALS according to cognitive status (n = 106).

<p>ALS, amyotrophic lateral sclerosis; ALS-ECI, ALS with executive cognitive impairment; ALS-FTLD, ALS with frontotemporal lobe degeneration; ALS-NC, ALS with normal cognition; ALS-NECI, ALS with non-executive cognitive impairment.</p

Demographic and clinical characteristics of captured and non-captured ALS cases.

<p>ALS, amyotrophic lateral sclerosis; ALSFRS-R, revised ALS functional rating scale; ALSSS, ALS severity scale; the disease progression rate was calculated according to the formula of (48-ALSFRS-R score)/disease duration(month).</p><p>Data were means±SD.</p><p>Demographic and clinical characteristics of captured and non-captured ALS cases.</p

Comparison of proportion of impaired participants in each neuropsychological battery, cognitive domain and cognitive diagnosis between non-demented ALS and HC.

<p>ABC, Aphasia Battery Chinese; ALS, amyotrophic lateral sclerosis; CMT, Clinical Memory Test; HC, healthy controls;WAIS, Wechsler Adult Intelligence Scale; WMS, Wechsler Memory Scale.</p><p>N/A, chi-square could not be performed when numerators of both groups are zero.</p><p>Data were shown in the form of number of participants with impairment/total number.</p><p>Comparison of proportion of impaired participants in each neuropsychological battery, cognitive domain and cognitive diagnosis between non-demented ALS and HC.</p