Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0155339 (Brown)
12,436 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Cramp syndromes pose a challenge for neuroscientists. The motor disorders of Isaacs syndrome have been ascribed to peripheral neuropathy, and sometimes there is ample supporting evidence of neuropathy. However, signs of overt neuropathy are found in a minority of cases and the essential findings (carpal and pedal spasm, pseudomyotonia and myokymia) may arise from abnormal excitability of the perikaryon because similar manifestations are seen in tetany and multiple sclerosis. The Moersch-Woltman (stiffman) syndrome differs from Isaacs' syndrome in essential characteristics. Hyperventilation syndromes may mimic either simple cramps, the Isaacs syndrome, the Moersch-Woltman syndrome, or the Foley and Denny-Brown syndrome of benign fasciculation and cramps. New approaches are needed to define the etiology and pathogenesis of these neurogenic disorders because the results of peripheral nerve block and spinal anesthesia have not been consistent in cases of typical Isaacs syndrome. Occupational cramps can be regarded as a form of action dystonia but that statement is a clue, not an "explanation". Myopathic disorders are only rarely a cause of cramp syndromes. In the glycogen storage disorders, the chemical basis of the cramp is still unproven. Whether myoadenylate deaminase is a cause of cramps is debated.
...
PMID:Cramps, spasms and muscle stiffness. 299 4

This review of the differential diagnosis of amyotrophic lateral sclerosis focuses on two themes. The first is practical, how to establish the diagnosis based primarily on clinical findings buttressed by electrodiagnosis. The main considerations are multifocal motor neuropathy and cervical spondylotic myelopathy. The second theme is the relationship of motor neuron disease to other conditions, including benign fasciculation (Denny-Brown, Foley syndrome), paraneoplastic syndromes, lymphoproliferative disease, radiation damage, monomelic amyotrophy (Hirayama syndrome), as well as an association with parkinsonism, dementia and multisystem disorders of the central nervous system.
...
PMID:Diagnosis of amyotrophic lateral sclerosis. 985 43

Fasciculations and fasciculation potentials (FPs) have been long known as a characteristic feature of amyotrophic lateral sclerosis (ALS). In this article, the history of the researches on fasciculation and FPs is first reviewed. The word and concept of fasciculation was first properly defined by Denny-Brown and Pennybacker (1938). It is noteworthy that they already stressed the necessity of strict discrimination of FPs from the "contraction fasciculation", a remnant of large voluntary motor unit potentials (MUPs), in this early milestone paper. FPs are rarely observed in neurogenic diseases other than ALS or disorders presenting with conduction block, i. e. they retain a high specificity in ALS diagnosis. Despite such usefulness, FPs were devaluated in the revised El-Escorial criteria. It is welcome that their value has been restored in the newer Awaji criteria. In the actual practice, correct identification of FPs would be a critical point. Remnant of voluntary MUPs is the greatest FP mimic to be differentiated. The key point for differentiation is the firing rhythm. FPs are characterized by a low-frequency and quite irregular firing, showing clustering of discharges. In contrast, voluntary MUPs are characterized by a semiregular firing.
...
PMID:[Fasciculation potential and ALS diagnosis]. 2567 13

The early development of nerve conduction studies (NCS) and electromyography (EMG) was linked to the discovery of electricity. This relationship had been concluded by observing the effect of applying electricity to the body of an animal and discovering that nerves and muscles themselves could produce electricity. We attempt to review the historical evolution of NCS and EMG over the last three centuries by reviewing the landmark publications of Galvani, Adrian, Denny-Brown, Larrabee, and Lambert. In 1771, Galvani showed that electrical stimulation of animal muscle tissue produced contraction and, thereby, the concept of animal electricity was born. In 1929, Adrian devised a method to record a single motor unit potential by connecting concentric needle electrodes to an amplifier and a loud speaker. In 1938, Denny-Brown described the fasciculation potentials and separated them from fibrillations. Toward the end of World War II, Larrabee began measuring the compound muscle action potential in healthy and injured nerves of war victims. In 1957, Lambert and Eaton described the electrophysiologic features of a new myasthenic syndrome associated with lung carcinoma. Overall, research on this topic was previously undertaken by neurophysiologists and then later by neurologists, with Adrian most likely being the first neurologist to be involved. The field greatly benefited from the invention of equipment that was capable of amplifying small bioelectrical currents by the beginning of the 20th century. Significant scientific and technical advances were later made during and after World War II which provided a large patient population with nerve injuries to study.
...
PMID:History of electromyography and nerve conduction studies: A tribute to the founding fathers. 2862 78