Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027066 (myoclonus)
 4,275 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Myoclonus dystonia (M-D) syndrome is a heritable movement disorder characterized by myoclonic jerks and dystonia primarily of the upper extremities. M-D remains poorly responsive to pharmacological treatment. Emerging reports suggest good response to DBS of the internal globus pallidus (GPi) and ventral intermediate nucleus (VIM) of the thalamus. This study aimed to appraise the value of these two DBS targets by evaluating reports available in the literature. A systematic search of published case reports and case series was performed on Medline and Embase. Responses to DBS were evaluated. Myoclonus was assessed with the Unified Myoclonus Rating Scale (UMRS) and dystonia by the Burke-Fahn-Marsden dystonia rating scale (BFMDRS). The primary outcome of interest was the relative improvements noted with GPi, compared to VIM stimulation. A total of 17 publications yielded 40 unique cases, with mean follow-up of 27.2 months. All patients demonstrated improvements in myoclonus scores, with 93.5% showing at least a 50% improvement in UMRS. The mean improvement in myoclonus scores was 72.6%. In contrast, dystonia scores were improved in 87.9% of patients, with 72.7% reporting at least a 50% improvement in BFMDRS. The mean improvement in dystonia scores was 52.6%. Improvements in myoclonus scores were similar for both GPi (75.7%) and VIM (70.4%; P = 0.27). However, the improvements in dystonia scores were greater with GPi (60.2%), compared to VIM (33.3%; P = 0.03). Although both targets achieve similar improvements in myoclonus, GPi stimulation may be a preferred target because it may achieve greater improvements in dystonia, compared to VIM stimulation.
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PMID:Surgical treatment of myoclonus dystonia syndrome. 2340 Nov 50

Tics and myoclonus are phenomenologically similar given that both are jerk-like movements, but, in contrast to myoclonus, tics are often preceded by premonitory sensations and are typically associated with a variety of behavioral comorbidities, including attention deficit and obsessive-compulsive disorder. There are many other clinical features that help differentiate these two hyperkinetic disorders. Whereas behavioral and antidopaminergic therapies are most effective in the management of tics, clonazepam, other anticonvulsants, and serotonergic drugs are often used to control myoclonic movements. Botulinum toxin may also be helpful in focal tics and in segmental forms of myoclonus. DBS plays an increasingly important role in the treatment of these disorders, particularly when they are generalized and are disabling despite optimal medical therapy.
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PMID:Therapeutic Developments for Tics and Myoclonus. 2631 14

Myoclonus dystonia is a rare movement disorder that often causes significant disability. Deep brain stimulation of the internal pallidum (GPi DBS) is a recommended treatment for those who do not respond to pharmacotherapy or who have intolerable side effects. This paper reports on the case of a 17-year-old male who was admitted to a tertiary level mental healthcare facility for treatment of psychiatric and behavioral symptoms thought to be related to GPi DBS. Prior to GPi DBS insertion, the patient was diagnosed with anxiety and mild obsessive compulsive disorder (OCD). Following insertion, his OCD became severe and he developed depression, Tourette syndrome, and stuttering. His first admission to a psychiatric unit was for management of a manic episode following treatment for depression with fluoxetine, and he began to exhibit severe aggressive behavior. GPi DBS was turned off, but there were neither changes in dystonic movements nor improvement in aggressive behavior or psychiatric symptoms, though stuttering improved. The patient was transferred to a secure treatment centre where he was able to gain control over his behaviors with intense dialectical behavior therapy, but the aggressive behavior and safety concerns continue to persist today.
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PMID:Psychiatric and Behavioral Complications of GPi DBS in an Adolescent with Myoclonus Dystonia. 3127 87