Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0026827 (hypotonia)
 5,860 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Baclofen, the most effective drug for treating spasticity, is a specific agonist of gamma-aminobutyric acid-B receptors, and is very abundant in the superficial layers of the spinal cord. Given orally, baclofen does not easily penetrate the blood-brain barrier, and is distributed equally to the brain and spinal cord. Direct intrathecal administration was given in order to change the distribution of the drug by preferentially perfusing the spinal cord. Eighteen patients presenting a severe spastic syndrome were treated with chronic intrathecal infusion of baclofen in the lumbar cerebrospinal fluid. After clinical preselection, 38 patients were implanted with a lumbar access port allowing long-term trials in order to determine the efficacy of baclofen therapy and the effective 12-hour dose. The 18 patients selected for chronic administration were implanted with a programmable pump. The pathology in these cases was: multiple sclerosis (6 cases), posttrauma spastic syndrome (eight cases), and (one case each) cerebral palsy, ischemic cerebral lesion, spinal ischemia, and transverse myelitis. The mean follow-up period was 18 months (range 4 to 43 months). The clinical results were evaluated according to muscular hypertony on Ashworth's scale (changed for occurrence of painful spasms) and functional improvement. Results were better for spastic syndrome secondary to traumatic medullary lesion than for demyelinating disease. Hypertonia was improved in all cases as confirmed by the registration of the Hoffman (H) reflex. Painful muscular spasms disappeared in 14 of the 16 affected patients. Significant functional improvement was noted in nine patients and was considerable in three. The risk of side effects secondary to overdose (such as excessive hypotonia or central depression) and the absence of a specific baclofen antagonist stresses the necessity for accurate determination of the efficient dose. After an initial titration period and adjustment of the therapeutic dose, the individual doses were from 21 to 500 micrograms/24 hrs (mean 160 micrograms/24 hrs). This new conservative method is very effective, perfectly reversible, and safe when administered in conditions favorable to its use.
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PMID:Chronic intrathecal baclofen administration for control of severe spasticity. 230 74

The role of clinical and magnetic resonance imaging (MRI) features on the prognosis of acute transverse myelitis has been studied, but the role of electromyography (EMG) changes, although reported, has not been investigated. Seventeen patients with acute transverse myelitis were subjected to clinical evaluation, MRI scanning and concentric needle EMG. The outcome was defined on the basis of a 3-month Barthel Index (BI) score as good or poor. The EMG changes in these groups were compared. All of the patients had complete paraplegia (power grade 0), except 1 who had grade III power. Mild upper limb weakness was present in 6 patients. Joint position and vibration sense were impaired in the lower limbs, and a horizontal limit to sensory loss to pinprick was present in all of the patients. Spinal MRI was abnormal in 12 of 14 patients. EMG of the lower limb muscles in the acute stage (within 15-30 days of onset) revealed fibrillations or sharp waves or both in 11 patients. At 3-month follow-up, the lower limb power had improved in 8 and upper limbs in all 6 patients. The EMG changes also improved in 6 patients; fibrillations either disappeared or were markedly reduced. The motor unit potentials (MUPs) were of long duration, polyphasic with reduced recruitment. In 5 patients, however, no MUPs could be recorded and fibrillations persisted. Lower limb hypotonia and fibrillations on EMG were significantly related to the 3-month outcome. EMG evidence of denervation in the lower limb muscles in acute transverse myelitis suggests a poor outcome as assessed by 3-month Barthel index score.
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PMID:Can electromyography predict the prognosis of transverse myelitis? 980 44

This report presents the case of a child with atopic dermatitis, who developed progressive muscular weakness and hypotonia of the four limbs. The cervical spinal cord magnetic resonance imaging revealed a C(4) lesion (T(2)-weighted images); the cerebrospinal fluid findings were normal. Treatment with intravenous immunoglobulins and methylprednisolone obtained rapid clinical improvement, and approximately 1 month later the small C(4) lesion disappeared. Various diagnostic hypotheses are discussed: acute myelitis by infective agents, acute disseminated encephalomyelitis, multiphasic disseminated encephalomyelitis, multiple sclerosis, and isolated postinfective myelitis. Another hypothesis relates to atopic myelitis, a form recently described in the Japanese literature, associated with atopic dermatitis, hyperIgEemia, and high levels of specific immunoglobulin E to Dermatophagoides farinae and Dermatophagoides pteronyssinus. This diagnosis is difficult to confirm without biopsy evidence of eosinophilic inflammation.
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PMID:Acute myelitis in a child: current hypotheses. 1713 14