Preliminary evaluation of the sensitivity to change of DE-STA motor unit number estimation in the upper trapezius muscle in amyotrophic lateral sclerosis.

Objective: To compare the sensitivity to change of decomposition-enhanced spike-triggered averaging (DE-STA) motor unit number estimation (MUNE) in the upper trapezius (UT) to that of various clinical outcome measures in subjects with amyotrophic lateral sclerosis (ALS).

Methods: Ten patients with clinically probable or definite ALS were assessed at baseline, 2, 4 and 6months with the following outcome measures: manual muscle testing in five upper extremity muscle groups, scapular elevation and elbow flexion peak force measured with hand-held dynamometry, MUNE, forced vital capacity and the Revised ALS Functional Rating Scale (ALSFRS-R).

Results: ALSFRS-R was the only outcome measure for which there was a significant difference between baseline and 6months (p=0.

A quantitative measure of handgrip myotonia in non-dystrophic myotonia.

Introduction: Non-dystrophic myotonia (NDM) is characterized by myotonia without muscle wasting. A standardized quantitative myotonia assessment (QMA) is important for clinical trials.

Methods: Myotonia was assessed in 91 individuals enrolled in a natural history study using a commercially available computerized handgrip myometer and automated software.

Widespread cerebral haemodynamics disturbances occur early in amyotrophic lateral sclerosis.

We wished to longitudinally assess early changes in cerebral perfusion (CP) and its relationship to cognitive impairment (CI) in ALS. Fourteen ALS patients at time of diagnosis and 11 spousal controls, both without CI, were longitudinally assessed to determine a relationship between CP and incidence of CI in early stage disease. Neuropsychological testing and CP measurements were performed in both ALS and control groups at the initial assessment (T0) and two time-periods post initial assessment, T1 and T2, taken on average 6.

Neuropsychological functioning in PLS: a comparison with ALS.

Objective: In order to characterize the nature and extent of neuropsychological dysfunction in primary lateral sclerosis (PLS), we studied prospectively cognitive, emotional, and behavioral functioning in PLS, and compared performances to functioning in amyotrophic lateral sclerosis (ALS).

Methods: Eighteen patients with PLS and 13 patients with ALS completed a neuropsychological test battery assessing both cognitive skills and emotional/behavioral functioning.

Results: Both PLS and ALS groups scored broadly within normal limits (mean T-scores greater than 40) on all cognitive measures and no significant between-group differences were found with the exception of one variable.

Cerebral haemodynamic changes accompanying cognitive impairment in primary lateral sclerosis.

Our objective was to elucidate the relationship between cognitive decline and cerebral haemodynamics in patients with PLS. We examined 18 patients with PLS and contrasted both neuropsychological and cerebral perfusion findings with seven age- and education-matched non-PLS controls. PLS patients were stratified into two groups based on the number of abnormal neuropsychological test scores: 1) cognitively intact PLS patients (PLS; those having zero or one abnormal scores (n =14)), and 2) cognitively-impaired PLS patients (PLSci; those having two or more abnormal test scores (n =4)).

Differentiation between primary lateral sclerosis and amyotrophic lateral sclerosis: examination of symptoms and signs at disease onset and during follow-up.

Background: Motor neuron diseases can affect the upper motor neuron and/or the lower motor neuron. Both amyotrophic lateral sclerosis (ALS) and primary lateral sclerosis (PLS) are motor neuron diseases, and there is much debate as to whether these are 2 separate disorders or simply 2 points on a continuum.

Objective: To determine which clinical features at onset and during follow-up could help differentiate between PLS and ALS.

Prognosis of amyotrophic lateral sclerosis with respiratory onset.

Respiratory muscle involvement is a recognised, but often late, complication of amyotrophic lateral sclerosis (ALS). The clinical features and prognosis of 21 patients with respiratory onset ALS are reported here. On a retrospective chart review, it was found that 2.