Combined tendon reflex and motor evoked potential recordings in amyotrophic lateral sclerosis.

Objective: This retrospective (case-control) collaborative study evaluates tendon reflex recordings combined with transcranial magnetic stimulation motor evoked potentials recordings (T-MEPs) at lower limbs in amyotrophic lateral sclerosis (ALS).

Methods: T-MEPs were recorded in 97 ALS patients distinguished according to their patellar reflex briskness. Patients' electrophysiological data were compared with values measured in 60 control patients matched for age and height.

Motor-neuron-disease-like phenotype associated with IgLON5 disease.

A growing spectrum of neurological manifestations are being recognized in association with IgLON5 autoimmunity, including recent reports of motor-neuron-disease-like phenotype. Here we describe four cases of IgLON5 autoimmunity with motor neuron involvement and evaluate an additional 109 probable or definite amyotrophic lateral sclerosis cases seen in our neuromuscular clinic for IgLON5-IgG seropositivity. The presence of parasomnias, vocal cord dysfunction or hyperkinetic movements in a patient with motor-neuron-disease-like phenotype should prompt evaluation for IgLON5-IgG autoantibodies.

Early advance care planning in amyotrophic lateral sclerosis patients: results of a systematic intervention by a palliative care team in a multidisciplinary management programme - a 4-year cohort study.

Introduction: Although recommended, the implementation of early advance care planning is suboptimal in amyotrophic lateral sclerosis (ALS) patients. Barriers to advance care planning include healthcare professionals’ and patients’ reluctance, and uncertainty about the right time to initiate a discussion.

Aim Of The Study: To determine how often advance care planning was initiated, and the content of the discussion in a first routine palliative care consultation integrated within a multidisciplinary management programme.

Sporadic late onset nemaline myopathy with monoclonal gammopathy of undetermined significance: two cases with long term stability.

Monoclonal gammopathy of undetermined significance (MGUS) associated to sporadic late onset nemaline myopathy (SLONM) is a rare and severely disabling condition of quickly progressive limb girdle acquired myopathy. It is believed by some authors to be due to myotoxicity of light chain deposits. Two female patients were diagnosed with MGUS associated SLONM.

Impaired conduction of Ia sensory fibers in multifocal motor neuropathy: An electrophysiological demonstration.

Objectives: To report the clinical and electrophysiological findings in two patients with multifocal motor neuropathy (MMN) and bilateral absent patellar and Achilles tendon reflexes despite normal strength of quadriceps and calf muscles.

Methods: The medical history and clinical evaluation were completed by electrophysiological tests: sensory and motor nerve conduction studies, needle electromyography, motor-evoked potentials (MEPs) after transcranial magnetic stimulation, patellar T (tendon) responses, quadriceps and soleus H (Hoffman) reflex recordings.

Results: In the two patients, history, clinical evaluation, nerve conduction studies, favorable response to intravenous immunoglobulins, and positive anti-GM1 antibodies fulfilled the diagnosis of MMN.

Multidisciplinary care in amyotrophic lateral sclerosis: a 4-year longitudinal observational study.

Over a four-year period, ALS patients complied with the modalities of the multidisciplinary management follow-up without any drop-outs. The multidisciplinary management structure also contributes to increasing the experience and knowledge of the clinicians involved in managing patients suffering from this rare disease.

Symptomatic tandem spinal stenosis: a clinical, diagnostic, and surgical challenge.

Tandem spinal stenosis (TSS) is an entity which refers to spinal canal diameter narrowing in at least two distinct regions of the spine. When symptomatic, management of TSS is controversial. In this study, we present a consecutive series of patients with symptomatic TSS and report diagnostic and surgical challenges.

Contraction response to muscle percussion: A reappraisal of the mechanism of this bedside test.

Objective: To study whether the contraction evoked by muscle percussion stems from the excitation of the muscle or of the nerve and to discuss the changes of this response in neuromuscular disorders.

Methods: In 30 neurologically healthy patients undergoing surgery (for ear, nose, or throat problems unrelated to the study) under general anesthesia with propofol and sufentanil we measured with an electrogoniometer the maximal dorsiflexion of the ankle evoked by reflex hammer percussion of the tibialis anterior muscle before and under neuromuscular junction blockade with rocuronium bromide. In 3 additional healthy volunteers we searched for F-waves to disclose whether percussion excites axons within the muscle.

A modern multidisciplinary approach to patients suffering from cervical spondylotic myelopathy.

Background: The traditional approach to patients suffering from cervical spondylotic myelopathy (CSM) consists of mere assessment of radiological standard magnetic resonance (MR) images and evaluation of surgical indication, depending on clinical symptoms and degree of radiological stenosis. Identification of prognostic factors for surgery remains difficult. Surgery is thought to be able to stop the disease progression, while significant improvements of neurological symptoms are not predictable.

Ryanodine receptor fragmentation and sarcoplasmic reticulum Ca2+ leak after one session of high-intensity interval exercise.

High-intensity interval training (HIIT) is a time-efficient way of improving physical performance in healthy subjects and in patients with common chronic diseases, but less so in elite endurance athletes. The mechanisms underlying the effectiveness of HIIT are uncertain. Here, recreationally active human subjects performed highly demanding HIIT consisting of 30-s bouts of all-out cycling with 4-min rest in between bouts (≤3 min total exercise time).

Anti-TNF alpha medications and neuropathy.

We studied the clinical, electrophysiological, and pathological features, outcome, and frequency of anti-tumor necrosis factor alpha (a-TNF) medications-induced neuropathies (ATIN) in patients with inflammatory disorders. Of 2,017 patients treated with a-TNF medication, 12 patients met our inclusion criteria for a prevalence of 0.60% and an incidence of 0.

Acute painful diabetic neuropathy: an uncommon, remittent type of acute distal small fibre neuropathy.

Introduction: Acute painful diabetic neuropathy (APDN) is a distinctive diabetic polyneuropathy and consists of two subtypes: treatment-induced neuropathy (TIN) and diabetic neuropathic cachexia (DNC). The characteristics of APDN are (1.) the small-fibre involvement, (2.

Multifocal motor neuropathy with high titers of anti-MAG antibodies.

Multifocal motor neuropathy (MMN) and anti-myelin-associated glycoprotein (anti-MAG)-associated neuropathy are clinically and electrophysiologically distinct entities. We describe a patient with characteristic features of both neuropathies, raising the possibility of an overlap syndrome. A 49-year-old patient reported a history of slowly progressive predominantly distal tetraparesis, with mild sensory deficits.

Experimental study of a late response recorded from the thoracic wall after phrenic nerve stimulation.

Objectives: The phrenic nerve cervical stimulation induces an early motor diaphragmatic M response that may be recorded from the 7th ipsilateral intercostal space (ICS). Some responses with prolonged latency and of unclear origin can be recorded from the same recording site. The aim of the study was to determine the electrophysiological characteristics and the neuroanatomical pathways underlying the long-latency responses (LLRs) recorded from the 7th ICS.

Peripheral autoimmune neuropathy assessed using corneal in vivo confocal microscopy.

Background: Corneal nerves can be examined using in vivo confocal microscopy (IVCM). This new technique permits sequential observation of the corneal subbasal nerve plexus and detects early signs of diabetic peripheral neuropathy.

Objective: To describe a patient with autoimmune peripheral neuropathy followed up using corneal IVCM.

Mild clinical expression of Lambert-Eaton myasthenic syndrome in a patient with HIV infection.

Neuromuscular complications of HIV are related to immunodeficiency, direct cytotoxicity of the virus or side effect of the treatments. Autoimmune disorders involving the nervous system, including Guillain-Barre syndrome, myositis and vasculitis, have been described in association with HIV. Neuromuscular junction autoimmune diseases such as myasthenia gravis have been occasionally reported in patients with HIV, whereas the Lambert-Eaton myasthenic syndrome (LEMS) has never been described.

A clinical study of corticospinal and peripheral conductions to proximal lower limb muscles.

Objective: To investigate the clinical utility of the newly developed "quadriceps combined technique" (QCT), which provides a global evaluation of the central and peripheral conduction to the proximal muscles of lower limbs, in a variety of central and peripheral neurological disorders.

Methods: Using surface recordings from the vastus medialis of the quadriceps muscle, we analyzed amplitudes and latencies of M response, patellar T reflex and motor evoked potentials (MEPs) after transcranial magnetic stimulation. We studied 180 patients with disorders impairing proximal strength of one or both lower limbs and compared them with 100 controls reported previously.

[Leprosy, a neurologic disease].

Leprosy is a treatable chronic infectious disease, caused by Mycobacterium leprae, not highly transmittable that affects mainly the skin and peripheral nerves. Often neglected because it is rare in western countries, it may be encountered in patients coming back from endemic areas. Diagnostic criteria include underpigmented patches with loss of sensation, thickened peripheral nerves and acid-fast bacilli on skin smears or biopsy material.

[Sensory neuropathies].

Peripheral sensory neuropathies are rare. Their clinical and electrophysiological pictures vary mainly with the course of the disorder (acute, subacute or chronic), with the size of the nerve fibres (large or small diameter) and the nervous structure involved (sensory axon or dorsal root ganglion). We discuss the characteristics and aetiologies of the various sensory neuropathies.

Electrophysiological evaluation of motor pathways to proximal lower limb muscles: a combined method and reference values.

Objective: In contrast with their important functional and clinical role, motor pathways to proximal muscles of lower limbs are rarely investigated in clinical neurophysiology. We describe a method to evaluate central and peripheral pathways to these muscles and report reference values.

Methods: Recording of both quadriceps was performed in 100 subjects.

Myasthenia gravis associated with HTLV-I infection and atypical brain lesions.

We report a patient who experienced progressive diplopia and distal weakness of the upper limbs. Magnetic resonance imaging of the brain showed extensive white matter lesions and analysis of cerebrospinal fluid revealed acute human T-lymphotropic virus type I (HTLV-I) infection. Myasthenia gravis (MG) was evidenced by electromyography (EMG) and antibodies against acetylcholine receptor.