Dystonia

Herz, Ernst

doi:10.1001/archneurpsyc.1944.02290280003001

Cited by 210 publications

(14 citation statements)

References 15 publications

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“…This issue was gradually settled by the observation that torticollis and torsion spasms (initially defined as pseudo-parkinsonism) could be secondary to encephalitis lethargica (20). In 1940, the Association for Research in Nervous and Mental Diseases extensively discussed the phenomenology of dystonia, its similarity with athetosis, the related EMG reading, and the underlying pathology (21). According to Herz (21), the term dystonia musculorum deformans should be confined to the idiopathic form.…”

Section: Early Medical Descriptionsmentioning

confidence: 99%

“…In 1940, the Association for Research in Nervous and Mental Diseases extensively discussed the phenomenology of dystonia, its similarity with athetosis, the related EMG reading, and the underlying pathology (21). According to Herz (21), the term dystonia musculorum deformans should be confined to the idiopathic form. He gave the following criteria for the clinical diagnosis of idiopathic dystonia: (a) selective systemic symptoms in the form of dystonic movements and postures; (b) gradual development, without recognizable etiological factors at the onset.…”

Section: Early Medical Descriptionsmentioning

confidence: 99%

“…Applying ‘Occam’s razor’ of scientific parsimony, it is certainly the most logical conclusion that this family exhibits ‘formes frustes’ as well as full-fledged cases of dystonia. Obviously, the strict diagnostic criteria as set forth by Herz (21) were not applied to the subject cases.” It was then recognized that “idiopathic torsion dystonia” is a genetic disorder with heterogeneous phenomenology, from focal to generalized within a same family.…”

Section: Lumping Focal Dystonias Togethermentioning

confidence: 99%

“…This paper contains all the elements for considering dystonia a unique disease. He used Herz’s diagnostic criteria (21) and distinguished three types of onset: the commonest being a difficulty to use one or both arms, the second commonest was an abnormality of gait, whereas in a minority of patients, the initial abnormality was confined to the neck and trunk. In half of the patients, the disease progressed to involve all the limbs and trunk, and in the remaining half, the disease was confined to one portion of the body and never became generalized.…”

Section: Lumping Focal Dystonias Togethermentioning

confidence: 99%

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How Many Dystonias? Clinical Evidence

Albanese

2017

Front. Neurol.

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Literary reports on dystonia date back to post-Medieval times. Medical reports are instead more recent. We review here the early descriptions and the historical establishment of a consensus on the clinical phenomenology and the diagnostic features of dystonia syndromes. Lumping and splitting exercises have characterized this area of knowledge, and it remains largely unclear how many dystonia types we are to count. This review describes the history leading to recognize that focal dystonia syndromes are a coherent clinical set encompassing cranial dystonia (including blepharospasm), oromandibular dystonia, spasmodic torticollis, truncal dystonia, writer’s cramp, and other occupational dystonias. Papers describing features of dystonia and diagnostic criteria are critically analyzed and put into historical perspective. Issues and inconsistencies in this lumping effort are discussed, and the currently unmet needs are critically reviewed.

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Section: Early Medical Descriptionsmentioning

confidence: 99%

Section: Early Medical Descriptionsmentioning

confidence: 99%

Section: Lumping Focal Dystonias Togethermentioning

confidence: 99%

Section: Lumping Focal Dystonias Togethermentioning

confidence: 99%

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How Many Dystonias? Clinical Evidence

Albanese

2017

Front. Neurol.

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“…Muscle activity in dystonia typically exhibits both overflow into task-unrelated muscles as well as greater variability within task-related muscles than in healthy subjects [3–5]. Increased variability may represent an inability to suppress motor noise [6], resulting in the superposition of unwanted motion components on the desired movement.…”

Section: Introductionmentioning

confidence: 99%

Increased task-uncorrelated muscle activity in childhood dystonia

Lunardini

Maggioni

Casellato

et al. 2015

J NeuroEngineering Rehabil

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BackgroundEven if movement abnormalities in dystonia are obvious on observation-based examinations, objective measures to characterize dystonia and to gain insights into its pathophysiology are still strongly needed. We hypothesize that motor abnormalities in childhood dystonia are partially due to the inability to suppress involuntary variable muscle activity irrelevant to the achievement of the desired motor task, resulting in the superposition of unwanted motion components on the desired movement. However, it is difficult to separate and quantify appropriate and inappropriate motor signals combined in the same muscle, especially during movement.MethodsWe devise an innovative and practical method to objectively measure movement abnormalities during the performance of a continuous figure-eight writing task in 7 children with dystonia and 9 age-matched healthy controls. During the execution of a continuous writing task, muscle contractions should occur at frequencies that match the frequencies of the writing outcome. We compare the power spectra of kinematic trajectories and electromyographic signals of 8 upper limb muscles to separate muscle activity with the same frequency content of the figure-eight movement (task-correlated) from activity occurring at frequencies extraneous to the task (task-uncorrelated).ResultsChildren with dystonia present a greater magnitude of task-uncorrelated muscle components. The motor performance achieved by children with dystonia is characterized by an overall lower quality, with high spatial and temporal variability and an altered trade-off between speed and accuracy.ConclusionsFindings are consistent with the hypothesis that, in childhood dystonia, the ability to appropriately suppress variable and uncorrelated elements of movement is impaired. Here we present a proof-of-concept of a promising tool to characterize the phenomenology of movement disorders and to inform the design of neurorehabilitation therapies.

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The "Stiff-Man" Syndrome

Trethowan¹

1960

Arch Neurol

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Dystonia

Cited by 210 publications

References 15 publications

How Many Dystonias? Clinical Evidence

How Many Dystonias? Clinical Evidence

Increased task-uncorrelated muscle activity in childhood dystonia

The "Stiff-Man" Syndrome

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