To describe results of training and 1-year follow-up of brain-communication in a larger group of early and middle stage amyotrophic lateral sclerosis (ALS) patients using a P300-based brain–computer interface (BCI), and to investigate the relationship between clinical status, age and BCI performance. A group of 21 ALS patients were tested with a BCI-system using two-dimensional cursor movements. A four choice visual paradigm was employed to training and test the brain-communication abilities. The task consisted of reaching with the cursor one out of four icons representing four basic needs. Five patients performed a follow-up test 1 year later. The clinical severity in all patients were assessed with a battery of clinical tests. A comparable control group of nine healthy subjects was employed to investigate performance differences. Nineteen patients and nine healthy subjects were able to achieve good and excellent cursor movements' control, acquiring at least communication abilities above chance level; during follow-up the patients maintained their BCI-skill. We found mild cognitive impairments in the ALS group which may be attributed to motor deficiencies, while no relevant correlation has been found between clinical data and BCI performance. A positive correlation between age and the BCI-skill in patients was found. Time since training acquisition and clinical status did not affect the patients brain-communication skill at early and middle stage of the disease. A brain-communication tool can be used in most ALS patients at early and middle stage of the disease before entering the locked-in stage.

Objective. To propose a rehabilitation protocol able to produce immediate and long-term beneficial effects on level of disability and overall performance in ADLs. Materials and Methods. Forty-one FM patients were randomized to an exercise and educational-behavioral programme group (experimental group, EG = 21) or to a control group (CG = 20). Each subject was evaluated before, at the end (T1), and after 6 months (T6) from the conclusion of the rehabilitation treatment using the Fibromyalgia Impact Questionnaire (FIQ), the visual analogue scale (VAS), the Health Assessment Questionnaire (HAQ), the fatigue severity scale (FSS), the 6-minute walking test (6MWT), tender points count (TPC), and spinal active range of motion. The exercise protocol included 20 sessions consisting in self-awareness, stretching, strengthening, spine flexibility, and aerobic exercises, which patients were subsequently educated to perform at home. Results. The two groups were comparable at baseline. At T1, the EG showed a positive trend in FIQ, VAS, HAQ, and FSS scales and significant improvement in 6MWT and in most spinal active range of motion measurements (P between 0.001 and 0.04). The positive results were maintained at the follow-up. Conclusion. The proposed programme was well tolerated and produced immediate and medium-term beneficial effects improving function and strain endurance. This trial is registered with DRKS00005071 on DRKS.

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The distinction between primary lateral sclerosis (PLS) and amyotrophic lateral sclerosis (ALS) still remains debated. Recently, PLS patients displaying lower motor neuron (LMN) signs have been defined as 'upper motor neuron (UMN)-dominant ALS', using 'clinically pure PLS' diagnosis to those with no LMN signs. To further characterize the LMN involvement in UMN-dominant ALS we investigated the presence and the extent of neurogenic abnormalities in the skeletal muscle of patients affected with a pyramidal syndrome consistent with UMN-dominant ALS. A total of nine patients affected with UMN-dominant ALS were analysed. In all cases, muscle biopsies showed the presence of scattered or clustered atrophic angulated fibres in small groups, and a mild to moderate fibre type-grouping. Target and targetoid fibres were detected in two cases only. Three patients had a second muscle biopsy which demonstrated a roughly unchanged pattern of chronic denervation with still moderate reinnervation phenomena. This study suggests that in UMN-dominant ALS muscle denervation may be characterized by an early chronic impairment of a restricted number of LMNs. The extent rather than the presence of LMN signs may allow to categorize patients with motor neuron disease involving mainly UMN into distinct entities.

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