Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026838 (spasticity)
 6,471 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Familial spastic paraparesis with amyotrophy of the hands was reported, and its significance in the literature was reviewed. Case 1: An 18 year-old boy, who had been suffering from spastic gait since 12 years old, noticed his hand muscle wasting distributed bilateral first interosseous muscle, thenar and hypothenar muscle at age 17. Case 2: A 20 year-old man, elder brother of case 1, who also walked in spastic manner from his childhood, developed bilateral hand muscle atrophy similar to case 1 at age 19. Clinical features of these two cases could be summarized as familial spastic paraparesis with amyotrophy characterized by hand muscle atrophy, spasticity of lower extremities with hyperreflexia and bilateral positive pathological reflexes and spastic gait. Their younger sister was also examined, who showed only minimal spastic paraparesis. The electrophysiological examination including EMG and SEP suggested the pathological process could involve not only lateral column, but also posterior column and anterior horn. Slight but generalized spinal cord atrophy was demonstrated on metrizamide CT myelography. The muscle biopsy performed from left gastrocnemius in case 2, confirmed neurogenic changes. Although the association of retinal degeneration, cataracta, mental retardation, pes cavus or even generalized amyotrophy has been reported in familial spastic paraparesis, only limited cases are available, dealing with the amyotrophy of limbs. As far the cases with amyotrophy localized to the hands are concerned, it is absolutely rare and only the cases reported by Silver could be regarded as similar or same clinical entities to our cases.
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PMID:[Two cases of familial spastic paraparesis with amyotrophy of the hands]. 267 23

Clinical neurophysiological tests have been introduced for the sacral neuromuscular system to aid with diagnosis of neurogenic conditions involving the lower urinary tract, anorectal and sexual dysfunction. The tests have, however, the potential to be of value in different interventions outside of the neurophysiological laboratory. EMG monitoring can be used for exact application of botulinum toxin by the relatively non-invasive transcutaneous approach in treatment of male detrusor sphincter dyssynergia. Checking for compound muscle action potentials of the external anal sphincter is proposed as the best method for exact placement of wire electrodes close to the 3rd sacral roots in treating lower urinary tract dysfunction by 'neuromodulation'. Presently the most established use of clinical neurophysiological techniques--outside the laboratory--as related to the sacral neuromuscular system is in the operating theatre. These tests have been introduced to identify relevant structures, for instance pudendal afferents within dorsal sacral roots, which should be spared during rhizotomy procedures for treatment of spasticity. Modified techniques are used intraoperatively to monitor the integrity of the lower sacral reflex arc (the bulbocavernosus reflex) or the lower sacral afferents throughout the spinal cord (pudendal SEP). Clinical neurophysiological tests are expected to become established in several interventions involving the sacral neuromuscular system.
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PMID:Interventional neurophysiology of the sacral nervous system. 1159 31