Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0013421 (dystonia)
 8,418 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Intramuscular neutrolysis with phenol has been used for 10 years in the management of spasticity in children. Best results depend on fastidious technique and realistic use of the procedure. Sedation or anesthesia was used in all cases -- 5% phenol in water was used for all procedures. The main indications were spasticity which interfered with function, either actual or potential, or with care. Where uninhibited vestibular or tonic neck reflexes affect muscle tone, or there is dystonia or athetosis, the procedure is less effective than where spasticity alone is present. Duration of relief of spasticity ranged from 1 month to more than 2 years. About one half of the lower extremity muscle treated required tenotomy later. Generally training was required after the procedure to obtain improved function. A representative sample of muscles treated, repeat procedures, and later surgery is discussed. The procedure is recommended for use in the management of spasticity in children as a way of improving function and/or care.
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PMID:Intramuscular neurolysis for spasticity in children. 47 67

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

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.
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PMID:Cramps, spasms and muscle stiffness. 299 4

In 131 patients treated exclusively by selective denervation during the past 10 years, all or almost all the abnormal movements of spasmodic torticollis were suppressed in 115 (88%) while preserving posture and mobility. This approach was also used in certain forms of adult-onset dystonia. An appreciable amount of abnormal movements remained in the other patients, either due to residual innervation or because of limitation of denervation necessary to preserve normal movements (laterocollis) or neck stability (retrocollis). A medio-lateral approach to the posterior cervical region in the sitting position using stimulation under light anaesthesia is recommended.
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PMID:Observations and analysis of results in 131 cases of spasmodic torticollis after selective denervation. 345 Feb 40

Beside the importance of its prehensile function the human hand plays an essential role in the conveyance of expression. Manual gestures support the mimic and confirm the spoken word. Based on the psychofunctional connection between mimic and gesture the hand becomes an outlet for psychogenic disorders. The resultant projection of emotional processes into motor disorders has been a matter of conjecture etiologically. Psychogenic manifestation in isolated motor disorders of the hand is considered to be either a functional nervous disorder or a focal dystonia. In comparison to the conversion phenomena reported up until now, in which flexor muscle units of the hand are involved with increased muscular activity (spasm), the cases presented here are characterized by a functional motor disorder manifested as an isolated paralysis of a selective extensor muscle unit (M. extensor pollicis longus) of the hand. After a multidisciplinary approach including clinical morphology, neurology, EMG and psychiatry the final diagnosis of a psychogenic paralysis of the hand is made using direct electro-stimulation of the neuromuscular unit with a needle electrode under simultaneous elimination of the psychic influence (general anesthesia without muscular relaxation). Through such confirmed motor inactivity the psychic genesis of the clinically existing paralysis is proven. Through immediate introduction of a multimodal therapy including physical and psychiatric treatment psychogenic paralyses of the hand can be restored totally.
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PMID:[Diagnosis and therapy of psychogenic paralyses in the hand region]. 406 16

6 cases with tremor-athetotic type cerebral palsy and 2 cases with moderate dystonia-tremor type cerebral palsy were treated by selective stereotactic thalamotomy. In the former group, postural-movement type tremor in the upper limb gradually progressed with age while athetosis remained unchanged. In the latter group, dystonia in the truncal muscles predominated over the irregular tremulous movement of the upper limbs. In all cases, the intelligence was almost normal. Stereotactic selective thalamotomy (Vim for tremor athetosis, VL-Vim for dystonia tremor) was performed under local anesthesia with the aid of radiological and neurophysiological control methods. The results of the operations were satisfactory in regard to the tremor relief and concomitant improvement of motor performances in most of the cases. Stereotactic treatment might be an effective way to make possible a one-step progress in these handicapped cases. The importance of postoperative physical therapy is also emphasized.
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PMID:Stereotactic selective thalamotomy for the treatment of tremor type cerebral palsy in adolescence. 634 49

Use of laryngeal mask in combination with gastric intubation permitted anesthesia for laparoscopic cholecystectomy in a female patient with disseminated osteochondrosis of the spine, expected difficult intubation, manifest polyvalent allergy, neurocirculatory dystonia of the hypertensive type, and high myopia. The risk of anesthesia was higher than surgical risk because of manifest concomitant diseases and aggravated anesthesiologic anamnesis. The operation was effective, anesthesia involved no complications, hemodynamics and gas exchange were stable, the patient awakened on the operation table and after recovery of adequate spontaneous respiration and muscular tone was transferred to resuscitation ward.
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PMID:[Use of laryngeal mask in a female patient with expected difficult intubation, polyvalent allergy and high grade myopia]. 764 84

Adriamycin, an anthracycline antineoplastic agent, can swiftly be transported to the sensory or somatic motor neurons by way of axoplasmic transport when injected into the subepineurium of the trigeminal nerve or sciatic nerve in experimental animals, and is consequently able to induce degeneration of the neurons without any systemic side effects. Intraneural injection of this agent was carried out for the treatment of a total of 22 patients presenting with intractable neural dysfunction (12 with neuralgia, including 7 with post-herpetic neuralgia and 10 with facial dystonia). The nerve which innervated the affected site was exposed under local anesthesia and approximately 10-60 microliters of 1-20% adriamycin was injected into the subepineurium. Results of the treatment after average follow-up periods of 21.5 months were as follows: Out of 12 patients with neuralgia, good results were obtained in 2 cases (16.7%), fair results in 6 cases (50.0%) (overall effective rate 67.7%). There were no changes in symptoms in 4 cases (33.3%). Out of 10 patients with facial dystonia, good results were obtained in 2 cases (20.0%), fair in 2 cases (20.0%) (overall effective rate 40.0%), and no changes in symptoms in 6 cases (60.0%). No major complications were encountered during these procedures and, once symptoms had disappeared after the treatment, no recurrence of symptoms was experienced. This method clearly differs from other various kinds of simple peripheral neurotomy, since transection of the peripheral nerve does not cause any, destructive changes in the sensory ganglion or motor nucleus and, hence, symptoms may recur.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Treatment of intractable postherpetic neuralgia and blepharospasm: intraneural injection of adriamycin]. 787 32

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

Midazolam has GABAergic effects in children that may modify propofol-induced involuntary movements, yet delay recovery. In a double-blind, randomized study, 24 children (2-7 yr of age, ASA physical status I or II) undergoing short surgical procedures received midazolam 0.5 mg/kg (Group M) or placebo (Group P) per os 20-30 min before propofol anesthesia (5 mg/kg intravenously followed by an infusion). Blind observers scored sedation and anxiety levels (scale 1-4) before premedication, at separation from parents, and at induction of anesthesia. Induction and emergence were videotaped, and body movements were recorded. During recovery, times to eye opening and maximum Steward (SS = 6) and Vancouver Sedative Recovery (VSRS = 22) scores were noted. Parents were questioned about side effects that may have occurred during the following week. Both groups were similar in age, sex, weight, timing of premedication, propofol dose, and duration of surgery. The incidence of involuntary movements did not differ between groups but was higher at induction (79%) than on emergence (25%) (P < 0.05). Anxiety and sedation scores were similar in Group P and Group M, but recovery took longer after midazolam, with eye opening (mean +/- SD) 24 +/- 7 vs 43 +/- 18 min, maximum SS (median and range) 27 (13-37) vs 55 (24-138) min, and maximum VSRS 51 (30-100) vs 80 (50-130) min. Children returned to normal activity in 1 (0-5) day, and none exhibited neurological complications. We conclude that an oral premedicant dose of midazolam prolongs recovery from anesthesia in children without affecting dystonic movements after propofol.
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PMID:Midazolam premedication delays recovery after propofol without modifying involuntary movements. 921 21


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