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)

Delayed neurological deterioration following anoxia is known to result from carbon monoxide exposure. However, it may also occur with anoxia of other types as well. The present report describes a case of delayed postanoxic encephalopathy with bilateral striatal lesions demonstrated by magnetic resonance imaging. A 27-year-old man exhibited anoxic anoxia caused by upper airway obstruction following general anesthesia for shoulder fracture surgery. Initially he was delirious and markedly excited for one day and became apparently normal for the following three days. Then he relapsed into delayed neurological deterioration with speech and gait disturbance, clumsiness of hand, pyramidal signs and metamorphopsia. Thereafter, he became bed-ridden and fell into semicomatose state with marked motor restlessness, involuntary movement of the tongue and decorticate posture. Twenty-five days later he had a second recovery period after hyperbaric oxygenation that lead to the sequelae with speech and motor disturbances and mild mental changes. I examined the present case as an expert witness in a civil suit eleven years after initial anoxia. The patient showed slight intellectual impairment and personality change. Impairment in figure-ground differentiation and disorders of spatial thought were also observed. Neurological examination revealed anisocoria, dysarthria with acquired stuttering, disturbances of fractionated movement of fingers, writer's cramp and Babinski's sign bilaterally. Postural dystonia of both hands and fingers, rigidity and spasticity of all extremities were also present. Magnetic resonance imaging (MRI) showed bilateral lesions of the corpus striatum, especially of the putamen. Some portion of the caudate nucleus was also involved. Cerebral cortices and white matter were slightly atrophic. From the above clinical course and neurological findings, we diagnosed the present case as delayed postanoxic encephalopathy. Ginsberg (1979) noted that in cases of anoxia not related to carbon monoxide, diffuse demyelinative changes of cerebral hemispheral white matter tended to be associated with relapsing clinical course, and gray matter injury was only seen in a few cases. MRI findings in the present case suggest that main site of the lesion to be in gray matter of the corpus striatum. In this respect, the present case is considered to be noteworthy.
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PMID:[A case of delayed postanoxic encephalopathy with bilateral lesions of the corpus striatum]. 281 6

Of 365 spastic dysphonia patients treated by recurrent laryngeal nerve (RLN) section to date, 44 (12%) have experienced recurrence of the spastic dysphonia. Most of these 44 patients had moderately severe to very severe spastic dysphonia before the RLN section was performed. We believe, therefore, that preoperative severity is an important predictor of the likelihood of recurrence. Twenty-eight patients with recurrent spastic dysphonia following RLN section were further treated by one or more carbon dioxide laser thinnings of the paralyzed vocal fold. Following this procedure, 23 patients (50%) achieved eradication of spasticity, while a mild degree of spasticity remained in 17 (39%). These findings lead us to conclude that even though spastic dysphonia may recur following RLN section, a viable secondary surgical procedure is available.
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PMID:Evaluation and treatment of recurrent spasticity after recurrent laryngeal nerve section. A preliminary report. 646 74

Recurrent laryngeal nerve section for spastic dysphonia was first performed in 1975 because prior forms of treatment had failed. Virtually every patient has had a detailed postoperative follow-up which includes a tape recording and a self-assessment questionnaire. The majority of patients remain free of spasticity at this medium-term follow-up. In some, spasticity recurred with less than preoperative severity. A small percentage of patients have a persistent breathy phonation. The first group is treated with vocal fold lateralization procedure using the CO2 laser; the second, with Teflon. When needed, voice therapy is also given. This paper provides a basis for diagnosis, indications for surgery, primary and secondary surgical techniques, encountered problems, and medium-term follow-up results.
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PMID:Intermediate results of 306 recurrent laryngeal nerve sections for spastic dysphonia. 682 80

Lesch-Nyhan syndrome is a rare, x-linked, recessive disorder of purine metabolism resulting in hyperuricemia, spasticity, choreoathetosis, dystonia, self-injurious behavior, and aggression, without significant cognitive impairment. Anesthetic management of inpatients who demonstrate classic manifestations of Lesch-Nyhan syndrome and require surgical interventions have been described. There are no guidelines in the literature addressing the anesthetic management of the outpatient with Lesch-Nyhan syndrome. Specifically, sudden, unexplained death, abnormalities in respiration, apnea, severe bradycardia, and an increased incidence of vomiting and chronic pulmonary aspiration may preclude this patient population from receiving anesthesia for outpatient procedures. General anesthesia with spontaneous ventilation was performed for diagnostic, radiographic imaging in 11 outpatients with Lesch-Nyhan syndrome using intravenous propofol. A bolus dose of 1.5 to 2.0 mg/kg propofol was followed by maintenance doses of 60 to 160 mcg/kg/min. Results during and following sedation indicated end-tidal carbon dioxide ranges between 34 mmHg and 59 mmHg. Respiratory rates were never below 10 breaths/min and no partial/complete airway obstruction or labored breathing was clinically evident. Hemodynamics were within 30% of presedation values. No patient demonstrated nausea, vomiting, or pulmonary aspiration. Baseline neuropsychologic status was achieved following sedation, and patients were discharged from the hospital 35 to 90 minutes after sedation was completed. Potential risks and benefits of using propofol in this patient population are discussed.
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PMID:Use of propofol anesthesia during outpatient radiographic imaging studies in patients with Lesch-Nyhan syndrome. 905 48

Mitochondrial myopathies make up a group of rare disorders whose onset is in childhood or adolescence. Muscle and central nervous system involvement is variable. Mitochondrial respiratory chain complex III deficiency (coenzyme Q - cytochrome C reductase) can manifest as exercise intolerance, myopathy, encephalopathy, and myocardial disease. Approximately 38 patients with complex III deficiency have been described since 1966, yet only a single anesthetic experience (epidural analgesia for cesarean delivery) has been reported. We describe the case of an 11-year-old boy with mitochondrial respiratory chain complex III deficiency, severe myopathy, and moderate encephalopathy who underwent surgery to improve right ischiotibial muscle spasticity. Monitoring included electrocardiography, noninvasive blood pressure, oxygen saturation by pulse oximetry, end-tidal carbon dioxide pressure, esophageal temperature, spirometry, and neuromuscular block (Relaxograph Datex). Midazolam, fentanyl, and propofol were used for anesthetic induction; mivacurium was used during intubation. Anesthetic maintenance was with propofol in continuous infusion and fractionated doses of fentanyl and mivacurium on demand in a mixture of oxygen and air. The boy's response to mivacurium was abnormal but he could nevertheless be extubated in the operating room at a train-of-four ratio of 75% and with no need to reverse the neuromuscular blockade. There were no problems during the anesthetic procedure, so it could be a good technique for these patients, despite of considering individually every case and extension of syntomatology, due to the little experience in anesthesia with deficiency of Complex III.
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PMID:[Anesthesia for a patient with mitochondrial respiratory chain complex III deficiency]. 1729 35