Reduced Occipital Cortex Excitability in Amyotrophic Lateral Sclerosis.

Purpose: In addition to motor cortex involvement, sensory abnormalities have been demonstrated in amyotrophic lateral sclerosis (ALS), including structural and metabolic alterations in the occipital cortex. The aim of this study was to examine occipital excitability changes in ALS.

Methods: Twenty-one patients with ALS and 16 healthy subjects were enrolled into the study.

A new parameter to discriminate amyotrophic lateral sclerosis patients from healthy participants by motor cortical excitability changes.

Introduction: We sought a combination of abnormalities to define a more sensitive measure of cortical excitability in amyotrophic lateral sclerosis (ALS).

Methods: The automatic threshold tracking method was employed to assess the resting motor threshold, intracortical facilitation (ICF), short-interval intracortical inhibition (SICI), and short-interval intracortical faciilitation (SICF) in patients and controls.

Results: SICF at interstimulus intervals (ISI) between 1 and 1.

Impaired short- and long-latency afferent inhibition in amyotrophic lateral sclerosis.

Introduction: To test the hypothesis of impaired cholinergic activity in amyotrophic lateral sclerosis (ALS), we studied short- and long-latency afferent inhibition (SAI and LAI).

Methods: The ulnar nerve was stimulated at the wrist preceding transcranial magnetic stimulation (TMS), 21 ms for SAI and 200 ms for LAI, in 21 patients and 17 control subjects. Short-interval intracortical inhibition (SICI) and cognitive function was assessed in ALS patients using automatic threshold tracking and the Montreal Cognitive Assessment (MoCA).

Spinal excitability changes do not influence the mechanisms of split-hand syndrome in amyotrophic lateral sclerosis.

Introduction: We studied spinal excitability and its relation to motor unit action potential (MUAP) changes in the thenar and hypothenar muscles in patients with amyotrophic lateral sclerosis (ALS).

Methods: Quantitative MUAP and peak ratio interference analyses were performed, and cutaneous silent period (CutSP) was measured in the abductor digiti minimi (ADM) and first dorsal interosseous (FDI) muscles of patients with ALS and controls.

Results: Patients with ALS revealed more prominently increased duration (P = 0.

Autonomic nervous system dysfunction and serum levels of neurotoxic and neurotrophic cytokines in patients with cobalamin deficiency.

Objective: The imbalance between neurotoxic cytokine tumor necrosis factor-α (TNF-α) and neurotrophic cytokines epidermal growth factor (EGF) and interleukin-6 (IL-6) plays a role in the pathogenesis of cobalamin (Cbl) deficiency-induced neuropathy. The aim of this study was to evaluate autonomic nervous system dysfunction and to look for any relationship between autonomic nervous system disturbances and serum cytokine levels (TNF-α, EGF, IL-6) in patients with Cbl deficiency.

Methods: Serum levels of TNF-α, EGF and IL-6 were studied in patients with Cbl deficiency (n=41) and a healthy control group (n=17) and after 3 months in patients who underwent Cbl replacement therapy (n=22).

Clinical and electrophysiological evaluation of patients with thalidomide-induced neuropathy.

Background: Whether thalidomide induces a sensory ganglionopathy or a length-dependent axonal neuropathy is disputed. Moreover no agreement exists concerning the effects of thalidomide dosage on the clinical and electrophysiological findings.

Objective: We examined the effect of age, gender disease duration, total cumulative dose on the clinical and electrophysiologic parameters.

Miller-Fisher syndrome associated with chronic lymphocytic leukemia.

Chronic lymphocytic leukemia (CLL) is a frequent hematological malignancy, with meningeal or peripheral nerve infiltrations being the most commonly encountered neurological complications. In this report, we describe a CLL patient with Miller-Fisher syndrome (MFS) who responded to immune modulation with plasmapheresis. A 47-year-old man diagnosed as B-cell CLL admitted with neutropenic fever.

Osteomalacic myopathy associated with coexisting coeliac disease and primary biliary cirrhosis.

Objective: To report a patient with coeliac disease (CD) associated with primary biliary cirrhosis (PBC) who presented with myopathy without classical symptoms of CD.

Clinical Presentation And Intervention: A 42-year-old woman presented with inability to walk and marked loss of motor function. She had elevated liver enzymes with a cholestatic pattern.

Multiple system atrophy presenting with acute respiratory failure due to diaphragmatic dysfunction.

Sleep related respiratory dysfunction and vocal cord paralysis are considered to be the major factors responsible for respiratory failure in multiple system atrophy (MSA). We report a patient initially presenting with alveolar hypoventilation culminating in respiratory failure, ultimately diagnosed as MSA. No central sleep apnea or marked paralysis of the vocal cords was noted.

Jitter analysis utilizing a high speed FM tape recorder.

Jitter analysis in single fiber EMG (SFEMG) is usually done on-line during recording. However, this technique frequently prolongs the study and makes re-analysis impossible. We attempted to measure jitter with a high speed FM tape recorder and compare the results with the previously published values.

Turns-amplitude analysis and motor unit potential analysis in myasthenia gravis.

Objectives: The aims of this study were to investigate myopathic changes in myasthenia gravis (MG) by using turns-amplitude analysis (TAA) and quantitative motor unit potential duration analysis (MUPan), to correlate myopathic changes with severity and duration of the disease and the results of diagnostic tests including repetitive nerve stimulation test (RNS), single fiber electromyography (SFEMG), and anti-acetylcholine receptor antibody (AChR-ab), and to compare the sensitivities of these two methods in detecting myopathic changes in MG.

Materials And Methods: We studied both MUPan and TAA in 32 patients with MG.

Results: The MUPan study showed myopathic changes in 12 patients (37.

Hypereosinophilic syndrome: progression of peripheral neuropathy despite controlled eosinophil levels.

Idiopathic hypereosinophilic syndrome (HES) is a disorder of the hematopoietic system, characterized by persistent elevation in the total eosinophil count (> 1500/microliter) for over 6 months, associated with organ damage and no detectable underlying cause. Treatment is centered on the reduction of total circulating eosinophils, which generally leads to remission of symptoms. We report a 68-year-old female patient with HES and peripheral neuropathy, presenting with cutaneous lesions, mental changes, cardiac and pulmonary symptoms, followed by right foot drop and eventually paraparesis, which caused an inability to ambulate.

Botulism with sensory symptoms diagnosed by neuromuscular transmission studies associated with edrophonium responsiveness.

A 43-year-old female patient developed bilateral ophtalmoplegia, bulbar paresis and weakness of the respiratory and extremity muscles. Additionally she had decreased sensation on the left half of the body. Repetitive nerve stimulation (RNS) test confirmed the diagnosis of botulism and edrophonium injection caused a reduction in the decremental response.

Quantitation of tendon reflexes in normal volunteers.

Ankle (ATR), patellar (PTR) and triceps (TTR) T-reflex responses were recorded in 48 limbs of 24 normal volunteers by delivering tendon taps with a hand operated electronic hammer. Latency to the onset of the triggered response, peak to peak amplitude and duration of the wave were measured after several repetitions. In all individuals, ATR and PTR were observed, while in 27% of limbs, TTR was absent.

"Chronic sensory demyelinating neuropathy": chronic inflammatory demyelinating polyneuropathy presenting as a pure sensory neuropathy.

The clinical electrophysiological and histological features of 10 cases of "chronic sensory demyelinating neuropathy" (CSDN) are reported. This entity is characterised by: 1) subacute or chronic progression; 2) pure sensory neuropathy; 3) high spinal fluid protein in the majority of cases; 4) electrophysiological evidence of demyelination affecting motor as well as sensory nerve fibres; 5) demyelination on sural nerve biopsy and 6) good response to immunotherapy in progressive phase. It is believed that this entity represents chronic inflammatory demyelinating polyneuropathy (CIDP) presenting as pure sensory neuropathy.

Chronic limb-girdle myasthenia gravis.

The existence of chronic "limb-girdle" form of myasthenia gravis (MG) has been questioned. We report here 12 such patients (10 women and two men) who constituted 3.8% of 314 MG patients in our study.

Diagnostic sensitivity of the laboratory tests in myasthenia gravis.

The diagnostic sensitivity of three laboratory tests [serum antiacetylcholine receptor antibody (AChR-ab) assay, the repetitive nerve stimulation (RNS) test, and, the single fiber EMG (SFEMG)] for myasthenia gravis (MG) was compared in 120 patients. In all cases, at least one of the tests was abnormal. SFEMG was the most sensitive test, being abnormal in 92% of cases, followed by the RNS test (77%) and the AChR-ab assay (73%).