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Biomedical subjects

D B Sanders

Publications and source records attributed to D B Sanders.

At least 55 records · Page 3Linked to original sources

Repetitive nerve stimulation studies in the Lambert-Eaton myasthenic syndrome.

We compared changes in amplitude and area of surface recorded compound motor action potentials (CMAPs) during 20-Hz repetitive nerve stimulation and after maximum voluntary contraction in patients with the Lambert-Eaton myasthenic syndrome (LEMS), myasthenia gravis (MG), and normal controls. There was greater potentiation of CMAP amplitude after voluntary contraction than during 20-Hz stimulation in 10 of 14 LEMS patients; CMAP area increased more after exercise than during 20-Hz stimulation in all LEMS patients. Although abnormal potentiation of CMAP area and amplitude was seen in equal numbers of LEMS patients, more LEMS patients demonstrated a greater than 100% potentiation of CMAP area than of CMAP amplitude. We conclude that maximum voluntary contraction is preferable to brief 20-Hz RNS to demonstrate potentiation in LEMS because it is at least as sensitive and is less painful. Measurement of CMAP area in LEMS patients is not better than measuring the change in CMAP amplitude in demonstrating abnormal potentiation. Testing of a single hand muscle for potentiation in LEMS does not demonstrate abnormal potentiation in all LEMS patients.

Action Potentials↗

Race, sex, and puberty influence onset, severity, and outcome in juvenile myasthenia gravis.

We assessed the influence of race, sex, and puberty upon clinical features and outcome in 115 patients with autoimmune juvenile myasthenia gravis (JMG). These demographic variables influenced not only disease incidence but also disease severity, response to therapy, and outcome, despite comparable therapeutic strategies. Among white patients, those with prepubertal onset had low incidence and equal sex ratio; the incidence in females increased during and after puberty; males had lesser disease severity than females during and after puberty (p < 0.05); spontaneous remissions were most frequent (44%, p = 0.001) and persistence of active JMG for more than 10 years was least frequent (p = 0.05) in patients with prepubertal onset; remissions were more frequent after early than late thymectomy (p = 0.03); and final disease severity was less after early than late thymectomy. Black patients had similar incidence, disease severity, and sex ratio (F:M = 2:1) with pre-, peri-, or postpubertal disease onset; infrequent spontaneous or treatment-induced remissions; and the same final disease severity after early or late thymectomy. These observations imply that race and sex hormones modify the clinical features and outcome of JMG; spontaneous remissions are common in white patients with prepubertal disease onset; early thymectomy may be more beneficial than late thymectomy in white patients; and the role of thymectomy in the youngest patients is uncertain. We suggest that demographic factors should be considered when evaluating past and future therapeutic strategies for JMG.

Adolescent↗

The electrodiagnosis of myasthenia gravis and the Lambert-Eaton myasthenic syndrome.

Electrodiagnostic studies are valuable in confirming the diagnosis of a disorder of neuromuscular transmission. They are used to distinguish presynaptic and postsynaptic abnormalities. These studies provide an objective measure of the severity of the illness and may be useful in assessing the response to therapy. This article reviews the electrodiagnostic techniques that are commonly used today and highlights their specificity, sensitivity, and pitfalls.

Electromyography↗

The treatment of patients with myasthenia gravis.

Although there is general agreement among experienced physicians on the usefulness of different treatments for myasthenia gravis (MG), so many variations are possible that in practice, similar patients treated by different physicians rarely are treated the same. Because of these divergent opinions one may read papers in reputable journals that present and propose extremely variable programs for treating MG. Scientific controversies, although stimulating, often result in confusion, especially for less experienced physicians seeking treatment guidelines for their patients. This article offers the authors' opinions on the benefits and limitations of the therapeutic modalities used in MG and their proposals of therapeutic plans for specific situations.

Adrenal Cortex Hormones↗

Single fiber electromyography in myasthenia gravis during pregnancy.

We report the use of single fiber electromyography (SFEMG) to demonstrate changes in the physiologic abnormality of myasthenia gravis (MG) during pregnancy. A 23-year-old became pregnant 15 months after the onset of mild ocular weakness. On initial evaluation, SFEMG jitter measurements demonstrated a slight abnormality of neuromuscular transmission. There was no change in severity of clinical disease or jitter measurements until the third trimester, when she improved. Jitter measurements at that time were normal. Labor was normal and she delivered a normal male. Three days postpartum, myasthenic weakness recurred temporarily and jitter measurements showed worsening. At 16 days and 6 weeks postpartum, she had only minimal medial rectus weakness and jitter studies were normal. Three months postpartum, ocular symptoms recurred and jitter measurements were slightly abnormal. She continued to worsen, developing limb muscle and severe ocular muscle weakness at 4 months postpartum. She was treated with plasma exchange and thymectomy. Prednisone was added 2 months after thymectomy due to continued worsening and development of oropharyngeal weakness. Three years postpartum she was taking prednisone 10 mg every other day and had only slight weakness of neck flexors, and jitter studies were again normal.

Adult↗

Clinical neurophysiology of disorders of the neuromuscular junction.

This is a review of the neurophysiological techniques used clinically to demonstrate abnormalities of neuromuscular transmission. The use of these tests in myasthenia gravis, Lambert-Eaton myasthenic syndrome, congenital myasthenic syndromes, and other conditions with disturbed neuromuscular transmission is presented.

Acetylcholine↗

Acetylcholine receptor antibodies in juvenile myasthenia gravis.

We analyzed relationships among pubertal stage at disease onset, sex, disease severity, and acetylcholine receptor antibody (AChR Ab) levels in 46 patients with autoimmune juvenile myasthenia gravis (JMG). Female predominance was least in children with prepubertal disease onset (F:M = 1.3:1) and increased in patients with peripubertal (F:M = 1.8:1) and postpubertal (F:M = 14:1) onset. Seronegative JMG was most common in children with early disease onset: 4 of 9 (44%) with prepubertal, 4 of 22 (18%) with peripubertal, and 0 of 15 (0%) with postpubertal onset were seronegative. The rapid therapeutic response to plasmapheresis was useful in distinguishing some patients with seronegative JMG from those with congenital myasthenia gravis (CMG). The high frequency of seronegative JMG in patients with prepubertal onset indicates that AChR Ab assays do not adequately discriminate between JMG and CMG in young children. Furthermore, the different sex distribution in patients with different pubertal stages at disease onset suggests that sex hormones play an important modulating role in JMG.

Adolescent↗

On the shape of the normal turns--amplitude cloud.

Factors that affect the shape of the so-called "normal cloud" of the turns and amplitude measurements of the electromyographic interference pattern are investigated. As the force of voluntary contraction increases from low to moderate levels, the number of turns in the signal increase faster than does the mean amplitude change between turns. This results in a cloud that is concave downward. At higher force levels, the pattern is reversed. The overall shape of the cloud thus depends on the maximum effort at which recordings are made, which is determined by the procedure of muscle activation.

Adult↗

Recording characteristics of monopolar EMG electrodes.

Motor unit action potentials (MUAPs) and the electromyographic (EMG) interference pattern (IP) were recorded from the biceps muscle of 5 normal subjects using both a concentric needle (CN) and a disposable monopolar needle (MN) electrode. The MUAPs recorded by the MN electrode had higher amplitude and area and were more frequently complex than those recorded with the CN electrode. The MUAP duration and area: amplitude ratio were similar for both electrodes. Although the MN electrode had a larger recording surface, its dimensions (maximum diameter and length of the cone shaped tip) were similar to those of the CN electrode (minor and major axes of the elliptical recording tip). Based on these observations, we infer that the MN electrode may be more selective than the CN electrode, ie, the AP amplitude recorded by the MN electrode decreases faster than the AP amplitude recorded by a CN electrode when the distance of the muscle fiber from the recording electrode increases. Photomicrographs of the MN electrode after use demonstrated no evidence that the insulating material had peeled off. There was also no evidence that MUAP measurement values changed during the recordings as would be expected if the recording surface changed due to peeling of the insulating material.

Action Potentials↗

Single-fiber EMG demonstrates reinnervation dynamics after nerve injury.

Serial single-fiber EMG (SF-EMG) studies in the frontalis muscle showed increased jitter with normal fiber density 15 days after facial nerve trauma. Both measurements were increased thereafter. The maximum increases occurred at 37 days, coincident with the initial return of function, and persisted until 67 days, when function had returned to normal. Thereafter, both features returned toward normal values, but were still elevated 34 months after injury.

Adult↗

Quantitative EMG in inflammatory myopathy.

Fifty-four quantitative electromyographic (EMG) studies were made in 37 patients with inflammatory myopathy (IM) at different points in their clinical course and treatment. All studies were performed in the biceps brachii which varied in clinical strength. Motor unit action potentials (MUAPs) in 45 studies and EMG interference pattern (IP) in 48 studies were recorded using a concentric needle electrode. Macroelectromyographic (Macro-EMG) MUAPs were recorded from 10 patients in 14 studies. MUAP analysis revealed a myopathic pattern (decreased duration and/or area: amplitude ratio) in 69% of studies. IP analysis was more sensitive than MUAP analysis, demonstrating a myopathic pattern in 83% of studies. Macro-EMG MUAP amplitudes were reduced in two studies, minimally increased in one study and normal in the remainder; in 6 (40%) studies, fiber density was slightly increased. Thus, reinnervation does not seem to play an important role in motor unit remodeling in IM.

Action Potentials↗