Reciprocal inhibition of forearm flexor muscles in spasmodic torticollis

Deuschl, Günther; Seifert, C.; Heinen, Florian; Illert, M.; Lücking, Carl Hermann

doi:10.1016/0022-510x(92)90269-q

Cited by 70 publications

(29 citation statements)

References 19 publications

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“…Likewise, an isolated change in this late phase following a normal early disynaptic phase has also been reported in non-hereditary forms of primary dystonia and in secondary dystonia (Nakashima et al, 1989; Panizza et al, 1989; Boesch et al, 2007). Nonetheless, this apparent penetrance-related feature has also been reported in the unaffected limbs of patients with focal dystonia (Chen et al, 1995; Deuschl et al, 1992). The specificity of this finding is subject to further query by the presence of changes in the early and late phases of reciprocal inhibition in individuals with hemiplegia secondary to stroke as well as in psychogenic dystonia (Nakashima et al, 1989; Espay et al, 2006).…”

Section: Abnormal Brain-behavior Relationships In Dyt1 Carriersmentioning

confidence: 64%

Abnormal structure-function relationships in hereditary dystonia

2009

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Primary torsion dystonia (PTD) is a chronic movement disorder manifested clinically by focal or generalized sustained muscle contractions, postures, and/or involuntary movements. The most common inherited form of PTD is associated with the DYT1 mutation on chromosome 9q34. A less frequent form is linked to the DYT6 locus on chromosome 8q21-22. Both forms are autosomal dominant with incomplete (~30%) clinical penetrance. Extensive functional and microstructural imaging with PET and diffusion tensor MRI (DTI) has been performed on manifesting and nonmanifesting carriers of these mutations. The results are consistent with the view of PTD as a neurodevelopmental circuit disorder involving cortico-striatal-pallido-thalamocortical (CSPTC) and related cerebellar-thalamo-cortical pathways. Studies of resting regional metabolism have revealed consistent abnormalities in PTD involving multiple interconnected elements of these circuits. In gene carriers, changes in specific subsets of these regions have been found to relate to genotype, phenotype, or both. For instance, genotypic abnormalities in striatal metabolic activity parallel previously reported reductions in local D 2 receptor availability. Likewise, we have identified a unique penetrance-related metabolic network characterized by increases in the pre-SMA and parietal association areas, associated with relative reductions in the cerebellum, brainstem, and ventral thalamus. Interestingly, metabolic activity in the hypermetabolic areas has recently been found to be modified by the penetrance regulating D216H polymorphism. The DTI data raise the possibility that metabolic abnormalities in mutation carriers reflect adaptive responses to developmental abnormalities in the intrinsic connectivity of the motor pathways. Moreover, findings of increased motor activation responses in these subjects are compatible with the reductions in cortical inhibition that have been observed in this disorder. Future research will focus on clarifying the relationship of these changes to clinical penetrance in dystonia mutation carriers, and the reversibility of diseaserelated functional abnormalities by treatment. Keywordsprimary torsion dystonia; positron emission tomography; motor sequence learning; brain activation; DYT1; DYT6 *Corresponding Author: David Eidelberg, MD, Center for Neurosciences, The Feinstein Institute for Medical Research, North ShoreLong Island Jewish Health System, 350 Community Drive, Manhasset, NY 11030, Phone: 516-562-2498; Fax: 516-562-1008; Email: david1@nshs.edu. Publisher's Disclaimer: This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. NI...

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Section: Abnormal Brain-behavior Relationships In Dyt1 Carriersmentioning

confidence: 64%

Abnormal structure-function relationships in hereditary dystonia

2009

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“…Some have described abnormalities in kinematic variables recorded during reaching studies similar to the task used in this article (movement time was not matched between groups and thus some of this data is difficult to interpret [40]) and electrophysiologically, abnormalities in inhibition have been demonstrated at many levels of the nervous system concerned with the control of the arm musculature (e.g., abnormal reciprocal inhibition of forearm muscles in cervical dystonia [43]). However, other studies in including ours suggest near normal motor performance [29].…”

Section: Discussionmentioning

confidence: 99%

Normal Motor Adaptation in Cervical Dystonia: A Fundamental Cerebellar Computation is Intact

et al. 2014

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The potential role of the cerebellum in the pathophysiology of dystonia has become a focus of recent research. However, direct evidence for a cerebellar contribution in humans with dystonia is difficult to obtain. We examined motor adaptation, a test of cerebellar function, in 20 subjects with primary cervical dystonia and an equal number of aged matched controls. Adaptation to both visuomotor (distorting visual feedback by 30°) and forcefield (applying a velocity-dependent force) conditions were tested. Our hypothesis was that cerebellar abnormalities observed in dystonia research would translate into deficits of cerebellar adaptation. We also examined the relationship between adaptation and dystonic head tremor as many primary tremor models implicate the cerebellothalamocortical network which is specifically tested by this motor paradigm. Rates of adaptation (learning) in cervical dystonia were identical to healthy controls in both visuomotor and forcefield tasks. Furthermore, the ability to adapt was not clearly related to clinical features of dystonic head tremor. We have shown that a key motor control function of the cerebellum is intact in the most common form of primary dystonia. These results have important implications for current anatomical models of the pathophysiology of dystonia. It is important to attempt to progress from general statements that implicate the cerebellum to a more specific evidence-based model. The role of the cerebellum in this enigmatic disease perhaps remains to be proven.

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“…Abnormalities in cortical, 41,42 brainstem, [43][44][45] and spinal inhibition 46,47 have been found in dystonia, but these too cannot alone be responsible for the abnormal movement pattern, because they may be seen outside of the clinically involved area, and, like the 4 to 7 Hz drive, may not be limited to patients with dystonia. 48 Of interest, in our sample of dystonic patients, frequency analysis could differentiate between some aetiologies in dystonia.…”

Section: Figmentioning

confidence: 95%

Patterns of EMG–EMG coherence in limb dystonia

et al. 2004

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Dystonia of the limbs may be due to a wide range of aetiologies and may cause major functional limitation. We investigated whether the previously described pathological 4 to 7 Hz drive to muscles in cervical dystonia is present in patients with aetiologically different types of dystonia of the upper and lower limbs. To this end, we studied 12 symptomatic and 4 asymptomatic carriers of the DYT1 gene, 6 patients with symptomatic dystonia due to focal basal ganglia lesions, and 11 patients with fixed dystonia, a condition assumed to be mostly psychogenic in aetiology. We evaluated EMG-EMG coherence in the tibialis anterior (TA) of these and 15 healthy control subjects. Ten of 12 (83%) of symptomatic DYT1 patients had an excessive 4 to 7 Hz common drive to TA, evident as an inflated coherence in this band. This drive also involved the gastrocnemius, leading to co-contracting electromyographic bursts. In contrast, asymptomatic DYT1 carriers, patients with symptomatic dystonia, patients with fixed dystonia, and healthy subjects showed no evidence of such a drive or any other distinguishing electrophysiological feature. Moreover, the pathological 4 to 7 Hz drive in symptomatic DYT1 patients was much less common in the upper limb, where it was only present in 2 of 6 (33%) patients with clinical involvement of the arms. We conclude that the nature of the abnormal drive to dystonic muscles may vary according to the muscles under consideration and, particularly, with aetiology.

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Reciprocal inhibition of forearm flexor muscles in spasmodic torticollis

Cited by 70 publications

References 19 publications

Abnormal structure-function relationships in hereditary dystonia

Abnormal structure-function relationships in hereditary dystonia

Normal Motor Adaptation in Cervical Dystonia: A Fundamental Cerebellar Computation is Intact

Patterns of EMG–EMG coherence in limb dystonia

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