[Psychological and cognitive interventions in end-of-life support of patients with amyotrophic lateral sclerosis. A review.].

Amyotrophic lateral sclerosis (ALS) is an incurable disease characterized by muscle atrophy leading to complete paralysis. Once diagnosed, the average life expectancy is three to five years. In this context, palliative and end-of-life care are essential, as well as the development of cognitive and/or psychological therapies to improve the quality of life of patients.

iMAX: A new tool for assessment of motor axon excitability. A multicenter prospective study.

Objective: This study was undertaken to establish by a multicentric approach the reliability of a new technique evaluating motor axon excitability.

Methods: The minimal threshold, the lowest stimulus intensity allowing a maximal response by 1 mA increments (iUP) and then by 0.1 mA adjustments (iMAX) were prospectively derived from three nerves (median, ulnar, fibular) in four university centers (Liège, Marseille, Fraiture, Nice).

[Postherpetic abdominal pseudohernia].

Postherpetic neuralgia is the most feared complication of herpes zoster (HZ). The incidence increases with age. Nevertheless, around 5 % of HZ patients will experience a motor deficiency known as segmental zoster paresis (SZP) according to the affected dermatome.

Improving fatigue in multiple sclerosis by smartphone-supported energy management: The MS TeleCoach feasibility study.

Background: Fatigue is a frequently occurring, often disabling symptom in MS with no single effective treatment. In current fatigue management interventions, personalized, real-time follow-up is often lacking. The objective of the study is to assess the feasibility of the MS TeleCoach, a novel intervention offering telemonitoring of fatigue and telecoaching of physical activity and energy management in persons with MS (pwMS) over a 12-week period.

Safety and efficacy of natalizumab in Belgian multiple sclerosis patients: subgroup analysis of the natalizumab observational program.

Natalizumab (Tysabri(®)) is highly efficacious in controlling disease activity in relapsing multiple sclerosis (MS) patients. As it is one of the more recent therapies for MS, there remains a need for long-term safety and efficacy data of natalizumab in a clinical practice setting. The Tysabri observational program (TOP) is an open-label, multicenter, multinational, prospective observational study, aiming to recruit up to 6,000 patients with relapsing-remitting MS from Europe, Canada and Australia.

[F-waves].

F-waves result from the discharge of the motoneurons following their antidromic activation. The F-wave appears, as an indirect (the F-wave latency decreases when the stimulation site moves away from the muscular detection) and late response (occurring after the M response). In practice, the most useful parameter is the F-wave minimal latency, provided that at least seven distinct F-waves are evoked.

Novel SACS mutation in a Belgian family with sacsin-related ataxia.

The authors describe the four patients in the first known Belgian family with autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS). A novel homozygous missense mutation, NM_014363.3: c.

[How I explore. . . vasculitis of the peripheral nervous system].

Vasculitic neuropathies require early diagnosis and therapy since their prognosis is potentially severe. The following features are usually suggestive of a vasculitic neuropathy: painful multiple mononeuropathy with an acute/subacute course, signs and symptoms of multisystem involvement (arthritis, asthma, renal and skin involvement), biological evidence of an inflammatory syndrome. In one third of patients however, vasculitic neuropathies present as a polyneuropathy.

[Advantages and limitations of the motor unit number estimation techniques].

It is now 30 years since the first motor unit number estimation (MUNE) technique was introduced by Allan McComas as a way of providing an objective, sensitive and reproducible means of measuring the number of motor axons in living human muscle or muscle group. MUNE techniques have substantially evolved over the past decade and have been applied, with increasing frequency, to the study of age effects on motoneurone population and muscle denervating disorders such as amyotrophic lateral sclerosis (ALS), spinal muscular atrophy, poliomyelitis and different types of inherited and acquired peripheral neuropathies. In the future, one of the most important topics involving MUNE, will probably be its use in monitoring the progress of ALS patients undergoing experimental drug trials.

[Symmetry and temporal variability of neurography].

Unlabelled: The aims of the present study are to document side-to-side differences and temporal variability, between two trials (T1 and T2 at a time interval of 3 months) of nerve conduction measurements collected from 30 healthy subjects (mean age 22 +/- 2 years).

Methods: The protocol at T1 consisted of motor nerve conduction studies of median, ulnar, peroneal and tibial nerves bilaterally, with measurement of (a) motor response size (amplitude and area); (b) terminal latency; (c) minimal, mean and maximal F-wave latency; (d) motor conduction velocity; and (e) F-wave occurrence. T1 also involved sensory nerve conduction studies of median, ulnar, radial, lateral and medial cutaneous, sural and superficial peroneal nerves bilaterally, with measurement of sensory potential size (amplitude and area) and computation of sensory conduction velocity.

Tibial muscular dystrophy in a Belgian family.

We report a Belgian family with autosomal dominant, late-onset, distal myopathy with selective foot extensor muscle involvement of the lower legs. Linkage to the tibial muscular dystrophy (TMD) locus 2q31 was not evident at first because of incomplete disease penetrance in a 50-year-old asymptomatic family member. An abnormal tibialis anterior muscle biopsy established her subclinical status and linkage of the family to the TMD locus.

Changes in motor unit numbers in patients with ALS: a longitudinal study using the adapted multiple point stimulation method.

Method: The adapted multiple point stimulation (AMPS) method for calculating motor unit numbers (MUNE) was applied in 12 patients with amyotrophic lateral sclerosis (ALS) before riluzole therapy (T(0)) and again after 4, 8 and 12 months of treatment.

Results: Paired Student's t-test indicated a significant decrease of thenar MUNE and compound muscle action potential (CMAP) size at each 4-monthly interval, while average surface motor unit potential (SMUP) size did not change significantly over time. The rate of motor unit (MU) loss at month 4 was more than 20% in six patients (group 1) and less than 20% in six other patients (group 2).