Electromyographic pattern in Duchenne and Becker muscular dystrophy. Part II. Electromyographic pattern in Becker muscular dystrophy in comparison with Duchenne muscular dystrophy.

Background: The aim of this study was to compare the electromyographic pattern in Becker muscular dystrophy (BMD) with that found in Duchenne muscular dystrophy (DMD).

Material And Method: Fourteen men with BMD and 51 boys with DMD were investigated. Proximal muscles were examined: m.

Electromyographic pattern in Duchenne and Becker muscular dystrophy. Part I: Electromyographic pattern in subsequent stages of muscle lesion in Duchenne muscular dystrophy.

Background: The study was aimed to evaluate the EMG pattern in myopathy, in the muscles with early, slight abnormalities and in the muscles severely affected, and to analyze the sequence of abnormalities appearing with the progress of the impairment.

Material And Method: Fifty one boys with Duchenne muscular dystrophy were studied. Ninety nine proximal muscles (biceps brachii and rectus femoris) were examined.

MuSK-positive myasthenia gravis is rare in the Polish population.

Background And Purpose: MuSK-positive myasthenia gravis (MG) is diagnosed in 0-48% of cases with generalized seronegative MG in different populations. The presence of anti-MuSK antibodies generally relates to a severe course and lack of response to thymectomy. We analyzed for the first time the serology and clinical characteristics of MuSK-positive MG in the Polish population.

[Impact of hypoglycemic episodes on nerves conduction and auditory and visual evoked potentials in children with type 1 diabetes].

Background: Hypoglycemia is an acute disturbance of energy, especially impacting the central nervous system, but direct influence on peripheral nervous function is not detected. The aim of the study was to establish the influence of hypoglycemic moderate and severe episodes on the function of peripheral nerves, hearing and visual pathway.

Material And Methods: 97 children with type 1 diabetes (mean age 15.

[A case of the Lambert-Eaton syndrome of non-neoplastic origin. Ten-year follow-up].

A case is presented of a 34-year-old man who developed weakness of the proximal muscles of the extremities, particularly lower, slight myalgia, and vegetative symptoms (dryness in the mouth). Those symptoms progressed within a month. On examination weakness of the muscles of the extremities was found as well as weak tendon reflexes, slight atrophy of muscles of the arms and thighs and apokamnosis.

[Myasthenia gravis and pregnancy].

We retrospectively analyzed the course and outcome of pregnancy in a group of 26 women with myasthenia gravis. Premature births were noted in 7.9% of pregnancies, the rate of cesarean section was 15.

[F-wave amplitude in peripheral nervous system lesions].

Background And Purpose: F-wave is a late response recorded from muscle elicited by electric impulse conveyed antidromically to alpha-motor neurons of the spinal cord. F-wave latency and frequency are assessed in routine electroneurography providing information of conduction in the proximal segment of the nerves. F-wave amplitude is rarely analyzed, while it could add valuable information on excitability of motor neurons in different disease states.

[Neurophysiological testing in myasthenia syndromes].

Myasthenic syndromes are a heterogeneous group of congenital or acquired disorders of neuromuscular junction. Despite major advance in genetics and molecular biology of disorders of neuromuscular junction, clinical diagnosis and choice of treatment largely depends on results of neurophysiological tests. Different protocols of repetitive nerve stimulation and single fibre EMG are indispensable in confirming neuromuscular junction defect, they can also give additional information on the level of abnormality and differentiate myasthenia gravis from Lambert-Eaton syndrome (pre- or postsynaptic defect).

The reorganisation of motor units in different motor neuron disorders.

A study was made of the degree and sequence of neurophysiological changes during motor unit reorganisation in motor neuron disease (MND), spinal muscular atrophy (SMA), and prior polio. Concentric needle EMG was used in conjunction with our own computerized EMG-LAB system. Motor unit action potential (MUAP) parameters were measured in 543 muscles on weak and maximum effort.

"Functional-QEMG" a new reliable method in daily routine investigation.

In classical quantitative analysis motor unit action potentials (MUAPs) parameters are useful in establishing diagnosis, but not in monitoring the functional status as expressed by clinical sings. This drawback is overcome by our new method called "Functional-QEMG", where the analysis is based on structural and functional changes of all acting motor units (MUs). The automatic classification of normal versus myogenic or neurogenic disorder together with the assessment of their severity is performed by special computer-assisted diagnostic procedures.

Motor unit hyperexcitability in amyotrophic lateral sclerosis vs amino acids acting as neurotransmitters.

Objectives: Electrophysiological studies of amyotrophic lateral sclerosis (ALS) patients reveal not only lower motor neuron involvement, but also widespread signs of its hyperexcitability. They might be the consequence of changes in the level of amino acids acting as neurotransmitters.

Material And Methods: Electrophysiological examination of 31 patients with sporadic ALS was performed.

Clinical, electrophysiological and immunological remissions after thymectomy in myasthenia gravis.

Objectives: Approximately 50% of patients treated with thymectomy have a chance for symptom-free life. However, immunological and neurophysiological abnormalities may be detected in patients with clinical remission. Although improvement usually parallels decrease in acetylcholine receptor antibody (AChRAb) levels and jitter values, there is a question what factors influence immunological and electrophysiological remission in a population of myasthenia gravis (MG) patients.

[Electrophysiological investigation s in diagnosis and evaluation of ALS progress].

In diagnostics of ALS the electrophysiologic investigation should be able to evaluate two fundamental processes: the primary process of the loss of some motoneuros--denervation, and secondary process of reinnervation. The most important diagnostic methods include the quantitative electromyography (EMG) evaluating several parameters of the motor unit potential (MUP) and of the maximal effort pattern. The earliest features are the signs of spontaneous activity (denervation) and the elevation of MUP amplitude and area (reinnervation).

[Is there true remission in myasthenia gravis? Long-term clinical and electrophysiological studies].

Twenty myasthenic patients were followed up who had in the years 1981-1982 full clinical remission (no drugs, no symptoms), lasting at that time at least several years. However, in 19 of them neuromuscular transmission defects were then found by single fibre electromyography (SFEMG). We then concluded that true remissions did not exist in myasthenia (J.

Reconsiderations about the abnormalities of somatosensory evoked potentials in motor neuron disease.

The frequency of involvement of sensory pathways in motor neuron disease (MND) remains the matter of controversy. For this reason the purpose of the present work was to test how often sensory system involvement might be detected by somatosensory evoked potentials (SEP) studies and then to verify the presence of alteration of the sensory conduction and to detect the frequency of abnormalities of somatosensory peripheral, spinal, subcortical and cortical potentials in MND. SEP were tested after median nerve stimulation at the wrist, recorded from Erb's point, Ce2, Ce7 and scalp.

[Dysphonia as the first sign of myasthenia gravis].

Ten cases of myasthenia are presented in which dysphonia was the initial sign. Isolated signs of dysphonia continued for several months or years, presenting diagnostic difficulties. Eventually neurologic examination followed by electrophysiological investigation (classical repetitive nerve stimulation and single fibre EMG) as well as edrophonium test allowed proper diagnosis of myasthenia, then confirmed by the clinical course.

[Hereditary sensorimotor neuropathy in electrophysiological studies].

We performed clinical and electrophysiological studies in 42 children with hereditary motor and sensory neuropathy type I and II (HMSN I and II) and in 103 members of their families. In 24 families with HMSN I the conduction velocity and latency were markedly changed in the nerves innervating the distal muscles (median, peroneal nerves) as well as proximal muscles (facial, axillary and musculocutaneous nerves). The changes were uniform in all motor and sensory nerves studied in a particular patient.

Sympathetic skin response in obstructive sleep apnea syndrome.

Examination of the sympathetic skin response (SSR), a non-invasive method of studying conduction in the sympathetic nervous system was performed in 15 male patients with obstructive sleep apnea syndrome (OSAS) evaluated by polysomnography who were compared with 7 non-apneic snorers and 26 controls. The aim of the study was to assess sympathetic nervous system function in OSAS, to compare the results with those found in non-apneic heavy snorers, to define the pattern of abnormalities and to study the correlations between SSR results and polysomnographic parameters. In the OSAS group the mean hand latency was significantly longer than in non-apneic snorers and healthy subjects.

[Median nerve electrophysiological assessment in amyotrophic lateral sclerosis].

Our material comparises 105 patients (62 men and 43 women) aged 26-73 years with amyotrophic lateral sclerosis (ALS). EMG examination confirmed the diagnosis of multilevel lesion of spinal motor neurons. Clinically, 94 of them had classical ALS, 3 had primary bulbar palsy (PBP), 6 had primary motor spinal atrophy (PSMA), and 2 had primary lateral sclerosis (PLS).

Electrophysiological findings in hereditary motor and sensory neuropathy type I and II--a conduction velocity study.

We performed clinical and electrophysiological studies in 42 children with hereditary motor and sensory neuropathy type I and II (HMSN I and HMSN II) and in 103 members of their families. In 24 families with HMSN I the conduction velocity and the latency were markedly changed in the nerves innervating the distal muscles (median, peroneal nerves), as well as proximal muscles (facial, axillary, and musculocutaneous nerves). The changes were uniform in all motor and sensory nerves studied in the particular patient.

The reorganization of motor units in motor neuron disease.

We studied 78 patients with motor neuron disease (MND) using concentric needle electromyography. Analysis on weak and maximal effort was performed using our own, fully automated, computer method, EMG-LAB. In addition to the conventional parameters of single motor unit action potentials (MUAPs) and interference pattern, new criteria were applied: the range of the acting motor units and the functional recruitment order.

[Myasthenia preceded by dysphonia. Clinical and electrophysical study].

The solely dysphonia symptom as atypical and relatively rare onset of myasthenia gravis is difficult to diagnose. We present 11 cases of myasthenia, where dysphonia was one and only one symptom of illness during several months to several years. The evidences of dysphonia were the fatigability and nasality of speech, as well as chronic hoarseness.