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Biomedical subjects

Barbara E Shapiro

Publications and source records attributed to Barbara E Shapiro.

9 recordsLinked to original sources
PubMed2005

Adult polyglucosan body disease: a case report of a manifesting heterozygote.

A 62-year-old man developed progressive gait instability, bladder dysfunction, proximal weakness, distal sensory loss, and mild cognitive impairment over 6 years. Neurologic examination revealed upper and lower motor neuron dysfunction in the lower extremities, with distal sensory loss. Electrodiagnostic studies, magnetic resonance imaging of the brain, and sural nerve biopsy were consistent with adult polyglucosan body disease. Biochemical and genetic analyses demonstrated reduced glycogen brancher enzyme levels associated with a heterozygous point mutation (Tyr329Ser or Y329S) in the glycogen brancher enzyme gene on chromosome 3. Mutational heterozygosity in the glycogen brancher enzyme gene has not been previously reported as a cause for this rare disease. A review of the clinical presentation, pathogenesis, etiology, and diagnosis of this disease is presented.

1,4-alpha-Glucan Branching Enzyme↗
PubMed2003

Iatrogenic complications and risks of nerve conduction studies and needle electromyography.

Electrodiagnostic procedures are routinely performed in patients with a variety of neuromuscular disorders. These studies are generally well tolerated and rarely thought to be associated with any significant side effects. However, needle electromyography is an invasive procedure and under certain situations has the potential to be associated with iatrogenic complications, including bleeding, infection, nerve injury, pneumothorax, and other local trauma. Similar complications are possible if needles are used for either stimulating or recording. In addition, like all other electrical devices and monitoring equipment connected to patients, electrodiagnostic testing carries the risk of stray leakage currents that under certain circumstances can result in electrical injury, especially in patients in the intensive care setting. Similarly, certain precautions are required during nerve conduction studies (NCS) in patients with pacemakers and other similar cardiac devices. In this review, we address the known and theoretical complications of NCS and needle electrode examination, and the possible methods to avoid such hazards.

Electromyography↗
PubMed2003

Entrapment and compressive neuropathies.

Entrapment and compressive neuropathies of the upper and lower extremities are frequently encountered disorders in the office. Certain clinical clues in the history and examination, along with electrodiagnostic testing and imaging studies, often suggest the correct diagnosis. Some of the more common neuropathies are discussed, along with suggestions regarding testing and treatment.

Adolescent↗
PubMed2003

Repetitive nerve stimulation and exercise testing.

Repetitive nerve stimulation and exercise testing are useful in the evaluation of patients with suspected disorders of the NMJ and muscle membrane excitability when performed with close attention to technical factors. They can be very helpful in the diagnosis of myasthenia gravis. Lambert-Eaton myasthenic syndrome, and botulism, as well as rare disorders of skeletal muscle membrane excitability, including paramyotonia congenita, myotonia congenita, myotonic dystrophy, and the periodic paralyses.

Electric Stimulation↗
PubMed2002

Lumbrical and interossei recording in severe carpal tunnel syndrome.

Patients with severe carpal tunnel syndrome (CTS) may occasionally have absent median motor and sensory responses; in these cases, it is not possible to accurately localize the median mononeuropathy to the wrist using standard electrodiagnostic tests. We prospectively investigated the use of comparing the median motor latency to the second lumbrical and the ulnar motor latency to the interossei muscles in 28 patient hands with severe CTS and absent median motor and sensory responses. We found a prolonged latency difference in 92.8%. Along with its use in mild CTS, study of the lumbrical-interossei latency difference is helpful in patients with severe CTS with absent median motor and sensory responses.

Action Potentials↗
PubMed2002

Needle electromyography. Fundamentals, normal and abnormal patterns.

The needle electromyographic (EMG) examination is a challenging component of the electrophysiologic study. Knowledge of anatomy and physiology, sound EMG technique, and good patient rapport are required. Every muscle sampled by needle EMG is assessed for insertional activity, spontaneous activity, and voluntary motor-unit action potentials. Key pieces of information obtained from the needle EMG examination include the localization of the disorder, its chronicity, severity, and whether or not the underlying pathophysiology is neuropathic, myopathic, or associated with a neuromuscular junction disorder.

Electrodes↗