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)

Ten consecutive patients with a progressive pan-autonomic failure of the Shy-Drager syndrome were investigated. Movement disorders of the vocal cords were examined with a fiber-optic laryngoscope as well as a video-recorder. Moderate to severe vocal cord paralysis was present in five of ten patients. The vocal cords were almost immobile during inspiration, while there was no limitation of the adduction during phonation. In two cases, grade of vocal cord paralysis was asymmetric. One patient developed peculiar twisting-like dystonic movements of the vocal cord. Polygraphic studies revealed that SaO2 was lowered in spite of tachypnea during sleep. In two cases, the expiratory flow volume curve in effort-dependent portion near TLC showed a plateau and the inspiratory part of the curve also showed a plateau indicating constant flow. These functional disorders suggest an upper airway obstruction probably due to the vocal cord dysfunction. There was no vocal cord paralysis in two patients who had neither snore nor stridor. Development of a severe vocal cord dysfunction usually manifested itself clinically as stridor, snore or respiratory failure requiring tracheostomy. There was little information on the pathology of the vagal nerves and nuclei supplying motor control to the laryngeal muscles. The mechanism of the selective involvement of abductor muscle (posterior muscle) of the vocal cord (Gerhardt syndrome) remains unsolved. Vocal cord paralysis should be looked for since it can result in respiratory failure leading to death.
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PMID:[Bilateral abductor vocal cord paralysis (Gerhardt syndrome) in the Shy-Drager syndrome]. 260 29

Adductor spastic dysphonia (SD) is a laryngeal dystonia characterized by a choked, constrained voice pattern with breaks in vocal flow. Treatment with a variety of therapies including speech and pharmacotherapy have minimal benefit; only one-third of patients undergoing recurrent laryngeal nerve section have benefitted at 3 years. We have used local injections of botulinum toxin (Botox) bilaterally into vocalis muscles in 42 patients with SD. Injections were through a teflon-coated hollow electromyography (EMG) recording needle. Unilateral small doses (2.5-3.75 U) were of no clinical benefit. Bilateral small doses resulted in sustained improvement lasting 84.4 +/- 9.3 days. The degree of improvement was 61.1 +/- 4.6%. Common side-effects included a brief period of breathy hypophonia (8.5 +/- 2.5 days) and a mild sensation of choking/aspiration of fluids (1.7 +/- 0.6 days); there were no serious adverse effects. Vocal cord paralysis was not necessary for benefit. Follow-up vocalis muscle EMGs revealed denervation. All patients responded to retreatment (longest follow-up 3.5 years). Patients with prior recurrent laryngeal nerve surgery and residual uncomplicated dysphonia had similar results. Our results indicate that local injection of low-dose Botox is the treatment of choice for SD.
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PMID:Adductor laryngeal dystonia (spastic dysphonia): treatment with local injections of botulinum toxin (Botox). 281 88