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Neuromuscular rehabilitation and electrodiagnosis. 3. Diseases of muscles and neuromuscular junction.

UNLABELLED: This self-directed learning module highlights formation of a differential diagnosis as well as electrodiagnostic evaluation for those patients who present with the common complaint of weakness. It is part of the chapter on neuromuscular rehabilitation and electrodiagnosis in the Self-Directed Physiatric Education Program for practitioners and trainees in physical medicine and rehabilitation. This article specifically focuses on the common symptoms and typical clinical findings that allow the clinician to narrow the differential diagnosis. This is followed by the diagnostic evaluation, with emphasis on the technical aspects and interpretation of electrodiagnostic studies. OVERALL ARTICLE OBJECTIVE: To summarize the clinical presentation and electrodiagnostic findings in persons with disorders of muscle or disorders of the neuromuscular junction.

Adult↗

Electrodiagnosis in spinal cord injured persons with new weakness or sensory loss: central and peripheral etiologies.

OBJECTIVE: To assess the prevalence and causes of late neurologic decline of persons with spinal cord injury (SCI). DESIGN: Retrospective review of persons with SCI over a 9-year period. Those with complaints of new weakness or sensory loss were grouped into three categories based on clinical examination, electrodiagnosis, and imaging: (1) central pathology (ie, brain, spinal cord, or nerve root); (2) peripheral pathology (plexus or peripheral nerve); or (3) no identifiable etiology. The specific diagnoses of late neurologic decline were identified. SETTING: Regional Veterans Affairs Spinal Cord Injury Service. PATIENTS: Five hundred two inpatient and outpatient adults with SCI. RESULTS: Nineteen percent of the study population complained of new weakness and/or sensory loss. Neurologic abnormalities were noted in 13.5%, 7.2% with central and 6.4% with peripheral causes. The most common pathologies were posttraumatic syringomyelia (2.4%) and cervical (1.6%) and lumbosacral (1.2%) myelopathy/radiculopathy. A specific etiology was not determined in 6 cases (1.6%). Peripheral involvement was mostly from ulnar nerve entrapment (3.4%) and carpal tunnel syndrome (3.0%). CONCLUSIONS: Late-onset neurologic decline is common after SCI and can result from central or peripheral pathology. Regular neurologic monitoring of SCI patients is recommended, since many with neurologic decline respond favorably if diagnosed and treated early.

Adult↗

The changing role of pediatric electrodiagnosis.

Electrodiagnosis is one of several useful diagnostic tests in infants and children who have anterior horn cell disease, neuropathy, neuromuscular junction disorders, or myopathy. It is also used for intraoperative monitoring in children. For hypotonic infants and for older children with a nonspecific presentation of weakness, EDX may provide direction for more specific diagnostic testing, such as DNA testing with or without muscle biopsy. Genetic testing has an increasingly important role in the diagnosis of children with neuromuscular disorders. Future improvements in motor unit quantitation, which do not require active patient cooperation and require less time than current methodologies, may make EDX more specific and useful for diagnosing neuromuscular disease in children.

Electrodiagnosis↗

Electrodiagnosis and recovery of function.

The study of electrodiagnosis with respect to recovery of function is in its infancy. There is a need for better understanding of the mechanisms of recovery and for better techniques to monitor recovery. This paper reviews the potential uses and limitations of current electrodiagnostic tests to predict and monitor neuromuscular recovery. It illustrates the use of these techniques in patients with spinal cord injury.

Adult↗

Use of the AAEM guidelines in electrodiagnosis of ulnar neuropathy at the elbow.

OBJECTIVE: The goal of this study was to determine if practice patterns in Washington State were altered by the development and publication of the American Association of Electrodiagnostic Medicine practice parameter for electrodiagnosis of ulnar neuropathy at the elbow. DESIGN: Medical records from 350 workers' compensation patients who underwent surgery for ulnar neuropathy at the elbow in Washington State were reviewed for the consistency of the electrodiagnosticians' report and the practice parameter. Statistical analysis (chi2 or Fischer's exact test) compared prepublication and postpublication patterns of test use. RESULTS: Thirty-one percent of the 153 studies (n = 47) were not consistent with American Association of Electrodiagnostic Medicine guidelines before its publication vs. 34% of the 138 studies (n = 47) after publication. This was not a statistically significant change (P = 0.63). CONCLUSION: Evaluation of the usefulness and dissemination of the guidelines is warranted. The practice parameter may need to be simplified and educational efforts extended to improve provider use of new guidelines.

Adult↗

Fuzzy classification of patient state with application to electrodiagnosis of peripheral polyneuropathy.

A methodology which accounts for uncertainty or imprecision in experimental observations and both norm and pathology definitions is developed on the basis of a distance measure between fuzzy numbers. These fuzzy numbers may represent, respectively, the measurements, norm, and pathology. The distance measure, called normalized fuzzy pathology index (NFPI), evaluates the difference of distance between observed experimental values for a given patient and norm on the one hand, and pathology on the other hand. The NFPI characterizes patient state as a continuous index; however, to conform to medical usage, categories of values are defined. Each of these categories corresponds to a linguistic variable. The case study used to illustrate the methodology is the electrodiagnosis of peripheral polyneuropathy in diabetic patients. Here, four initial linguistic categories are defined by a physician, namely: normal state, borderline state, clear-cut, and severe pathology. The NFPI is calculated in three cases that provide a sensitivity analysis on measurement fuzziness and distance function weighting. The model is calibrated using 203 cases and validated using 291 different cases. The results correspond very closely to the physician's diagnosis. The loss of information in discretizing the continuous state of patients is discussed. Transferring this fuzzy approach to other cases where the concept of distance is relevant offers no difficulty.

Diabetic Neuropathies↗

Electrodiagnosis of infantile botulism.

Analysis of the literature on the electrophysiologic features of infantile botulism was undertaken. Small compound muscle action potential amplitude is a very sensitive feature but lacks specificity. The decremental response to 2- to 3-Hz repetitive nerve stimulation is inconsistent and not a reliable sign. Tetanic and posttetanic facilitation are highly sensitive and highly specific. Absence of posttetanic exhaustion is also highly specific for infant botulism and shared only by hypermagnesemia. We conclude that the findings of low compound muscle action potential amplitude in combination with tetanic facilitation or posttetanic facilitation and absence of posttetanic exhaustion constitute the triad on which the electrodiagnosis of infantile botulism can be supported.

Botulism↗

Telematic electrodiagnosis.

Using the Electrodiagnostic Neurophysiological Automated Analysis (ENAA) telematic system, 1560 standardized electrodiagnostic tests have been performed on 70 normal subjects and 320 patients in five laboratories in three European countries. The data from each patient have been transmitted from the five satellite laboratories to the Bristol Telematic Electrodiagnostic Centre (BRITEC), housed in the Bristol Eye Hospital, where they were evaluated and the conclusions transmitted to the place of origin. The results from the normal subjects were not significantly different between laboratories. Also patients with specific types of disease had similar results in all laboratories. The results from both the normal subjects and the patients show that a working prototype for providing telematic electrodiagnosis is available and has been well received by both clinicians and patients.

Adult↗

Neuromuscular rehabilitation and electrodiagnosis. 1. Central neurologic disorders.

This self-directed learning module highlights the medical treatment and rehabilitation intervention of certain central neurologic disorders encountered in physiatric practice. It is part of the chapter on neuromuscular rehabilitation and electrodiagnosis in the Self-Directed Physiatric Education Program for practitioners and trainees in physical medicine and rehabilitation. This article contains sections on multiple sclerosis, Parkinson's disease, and amyotrophic lateral sclerosis. Information covered in these sections includes discussions of the current medical management and the benefits of comprehensive rehabilitation and interventions for specific impairments seen in these conditions.

Amyotrophic Lateral Sclerosis↗

Neuromuscular rehabilitation and electrodiagnosis. 3. Generalized peripheral neuropathy.

This self-directed learning module provides an updated tool for establishing the differential diagnosis and subsequently designing a cost-effective workup for patients with peripheral neuropathy. It is part of the chapter on neuromuscular rehabilitation and electrodiagnosis in the Self-Directed Physiatric Education Program for practitioners and trainees in physical medicine and rehabilitation. Medication intervention for neuropathic pain is reviewed by medication class with recommendations for starting doses and review of side-effect profiles. This article also reviews the current treatment standards for a patient with juvenile-onset diabetes, including recommendations for glucose control, management of nephropathy and retinopathy, and care of foot complications.

Algorithms↗

Neuromuscular rehabilitation and electrodiagnosis. 5. Myopathy.

This self-directed learning module highlights hypotonia, facioscapulohumeral dystrophy, and herbal supplements causing muscle weakness. It is part of the chapter on neuromuscular rehabilitation and electrodiagnosis in the Self-Directed Physiatric Education Program for practitioners and trainees in physical medicine and rehabilitation. This section presents advances in the diagnosis of myotubular dystrophy, myoblast transfer, and problems associated with the increased use of herbal supplements.

Counseling↗

Electrodiagnosis of demyelinating neuropathies.

The inflammatory demyelinating neuropathies constitute a significant proportion of the acquired polyneuropathies. Major progress in finding the causes and in the treatment of these neuropathies has been made over the last decade. Early recognition is of paramount importance, because timely and appropriate treatment can largely reduce morbidity and disability. Electrodiagnosis plays a key role in the detection and characterization of the inflammatory demyelinating neuropathies. Electrodiagnostic criteria for primary demyelination have therefore been developed. They are empirically based on changes of nerve conduction parameters in populations of patients with a confirmed clinical and laboratory diagnosis of inflammatory demyelinating neuropathy. The challenge consists of defining criteria sets that are highly specific but also as sensitive as possible. Most of the hereditary demyelinating neuropathies are part of Charcot-Marie-Tooth disease type 1. The pattern of nerve conduction abnormalities usually provides valuable clues for the distinction from chronic inflammatory demyelinating neuropathies.

Action Potentials↗

[The UDK-87 device for the electrodiagnosis of caries].

A new scheme of an device for electrodiagnosis of caries is suggested, based on studies of dental hard tissue electric resistance. A highly sensitive M136 gage with 0.5 microA complete deviation current is employed in the scheme, this permitting measurements of high electric resistance of dental hard tissues within a range of 1-200 M omega, painless for patients. The device may be useful for the diagnosis of fluorosis, hypoplasia, incomplete enamel mineralization, enamel microfissures, abnormal wearing of dental hard tissue.

Dental Caries↗

The electrodiagnosis of the carpal tunnel syndrome.

The carpal tunnel syndrome is one of the most commonly discussed subjects among the public, as well as the medical community, these days. Even though this is a rather simple nerve compression, which can be easily corrected, the carpal tunnel syndrome can induce further complications, sometimes leading to total disability of the patient. Early, proper diagnosis and treatment is very vital. Unfortunately, the exact pathomechanism and proper diagnosis is yet to be seen. As electromyographers, the authors would like to re-visit this debatable carpal tunnel syndrome emphasizing the electrodiagnosis. Among the electrodiagnostic tests, comparison of distal motor or sensory latency of the median nerve to the ulnar nerve along with amplitude of the response, was found to be the most sensitive test. Also, the C6 cervical radiculopathy and the pronator syndrome can present similar clinical pictures and require a differentiation. After successful surgery many patients still complain of subjective residual symptoms. The electrophysiologic findings do not correspond to the clinical progress in many cases. The authors also experienced that most of the victims, following successful surgery, still would benefit from a proper exercise program including instruction for proper body mechanics before they return to their previous activities, to lessen the undesirable complications including the reflex sympathetic dystrophy.

Carpal Tunnel Syndrome↗

[Electrodiagnosis of caudal cranial nerve disorders in infants and small children].

The endoscopic application of electrodes for electromyography of laryngeal and pharyngeal muscles is routinely performed in adults and very helpful in electrodiagnosis of voice and speech disturbances. Dysfunctions of the lower cranial nerves in infancy and childhood are congenital diseases in most of the cases. Up to now the most helpful diagnostic tool has been direct laryngoscopy which, however, could not classify the type of peripheral or central neural or even muscular disease. It is only by electrophysiological methods that the laryngologist can answer these questions. Electromyography and neuromyography as well as reflex myography of the lower cranial nerves have been performed in ten children since 1979, enabling diagnosis and classification of congenital and acquired lesions in the muscles of tongue, soft palate, pharynx and larynx. In particular accurate diagnosis of laryngeal malacia with intact peripheral innervation and voluntary activity during electromyography is always possible. In addition, the method is helpful in the planning of therapy which consists in either conservative or surgical treatment.

Adolescent↗

[Electrodiagnosis of neuromuscular diseases--a syndrome of monomelic amyotrophy with spinal hemiatrophy, anti-GM1 antibodies and abnormal F-waves (MASH)].

Electrodiagnosis rests upon sound anatomical and physiological bases of the peripheral nervous system, which are utilized for the dynamic process of differential diagnosis. If clinical and electrophysiological findings do not conform to any of the previously known diseases, there may be a chance of identifying a new entity. As an example, we report nine Japanese patients with unilateral atrophy of the distal upper limb and the cervical cord. Symptoms typically began insideously in young adulthood, progressed for a few years and then stabilized. Distribution of the muscle weakness usually corresponded to the territories of a few peripheral nerves, although not accompanied by sensory deficits. Serum anti-GM1 IgG antibodies were elevated in 6 out of 9 patients. Electrophysiological testings disclosed absent or delayed F-waves in the nerve involved. No long tract signs of the cord were demonstrated, although the spinal cord atrophy was extensive in some patients. Three showed partial clinical improvement after cyclophosphamide therapy or intravenous immunoglobulin. These findings may suggest that this entity is immune-mediated as in multifocal motor neuropathy, with its blunt of immune attack directed not only to peripheral motor nerves but also to spinal motor neurons.

Adolescent↗

Advances in electrodiagnosis in Zimbabwe. Part II: "Evoked potentials".

This paper is a short review of the electrodiagnostic technique of "evoked potentials", a technique recently introduced to Zimbabwe. The types of evoked potentials recorded, the method of recording and their clinical applications are discussed. Abnormal evoked potentials from a variety of neurological disorders are illustrated. "Advances in electrodiagnosis in Zimbabwe" Part I discussed peripheral nerve conduction measurements and electromyography. This paper (Part II) will review the physiological basis of "Evoked Potentials" and their clinical applications.

Electrodiagnosis↗

Electrodiagnosis of compression of individual nerve roots of the cauda equina.

Segmental electrodiagnosis of compression of individual nerve roots of the cauda equina has been attempted in 45 patients, including cases of disc hernia, spondylsis and spondylolisthesis. The needle electrode was inserted into the nerve root either just lateral to the intervetebral foramen or through the posterior sacral foramen. The recordings made on stimulating a single nerve root were somatosensory evoked potential, the cauda equina action potential and the M and H waves. Mixed spinal nerve root action potentials were also recorded by stimulating the sciatic, peroneal and tibial nerves. It was shown that a diagnosis could be made from the somatosensory evoked potential, the H wave and root pain reproduction, and also the diagnosis of a subclinical compression involvement. In other words, the neurophysiological state of dysfunction of individual roots in each aspect of compression can be expressed.

Action Potentials↗