Adult‐onset primary lower limb dystonia

Schneider, Susanne A.; Edwards, Mark J.; Grill, Stephen; Goldstein, Suzanne; Kanchana, Su; Quinn, Niall; Bhatia, Kailash P.; Hallett, Mark; Reich, Stephen G.

doi:10.1002/mds.20794

Cited by 62 publications

(56 citation statements)

References 33 publications

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“…Furthermore, foot onset in adults is not common in primary torsion dystonias, but may occur. 20 While leg involvement is frequent in psychogenic dystonia, 21 the clinical course and presentation in this patient speaks against a psychogenic cause. Peripherally induced dystonia is usually characterized by fixed posturing at rest and marked limitation of passive range of motion.…”

Section: Discussionmentioning

confidence: 96%

Abnormality of motor cortex excitability in peripherally induced dystonia

2007

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It is widely accepted that peripheral trauma such as soft tissue injuries can trigger dystonia, although little is known about the underlying mechanism. Because peripheral injury only rarely appears to elicit dystonia, a predisposing vulnerability in cortical motor areas might play a role. Using single and paired-pulse pulse transcranial magnetic stimulation, we evaluated motor cortex excitability of a hand muscle in a patient with peripherally induced foot dystonia, in her brother with craniocervical dystonia, and in her unaffected sister, and compared their results to those from a group of normal subjects. In the patient with peripherally induced dystonia, we found a paradoxical intracortical facilitation at short interstimulus intervals of 3 and 5 milliseconds, at which regular intracortical inhibition (ICI) occurred in healthy subjects. These findings suggest that the foot dystonia may have been precipitated as the result of a preexisting abnormality of motor cortex excitability. Furthermore, the abnormality of ICI in her brother and sister indicates that altered motor excitability may be a hereditary predisposition. The study demonstrates that the paired-pulse technique is a useful tool to assess individual vulnerability, which can be particularly relevant when the causal association between trauma and dystonia is less evident.

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Section: Discussionmentioning

confidence: 96%

Abnormality of motor cortex excitability in peripherally induced dystonia

2007

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“…The initial leg involvement was associated with high probability of dystonia generalization, although we followed only two patients. However, in patients with adult-onset primary lower limb dystonia, the prognosis with regard to progression or spread was favorable [18].…”

Section: Discussionmentioning

confidence: 99%

Spread of primary dystonia in relation to initially affected region

Svetel¹,

Pekmezovic²,

Jovic³

et al. 2007

J Neurol

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Not only childhood-onset, but also adult-onset primary dystonia may spread to multiple body parts. The relative risk of spread by site of onset of dystonia, important for clinical prognosis and approach, has not been well characterized. The aim of this study was to prospectively follow the spread of dystonia in 132 consecutive patients and to estimate the risk of spread by the site of onset of dystonia. The patients were included in the study if primary focal dystonia was the only sign of neurological disease other than tremor; i.e. in all patients a single body part could be identified as affected at the onset. At the end of the followup (mean duration 7.5 years; range 5.2-13.4 years), 96 patients (73%) remained focal, while 26 (20%) and 10 (7%) progressed to segmental and generalized dystonia, respectively. The highest likelihood for further spread was observed in patients with initial blepharospasm (10 out of 30 patients; 33.3%), followed by dystonia of upper extremities (32.3%), torticollis (19.6%), and laryngeal dystonia (6.7%). In addition to the highest risk for further spread of dystonia, blepharospasm was associated with the fastest rate of spread (the second region affected on average after 1.2 years). Our results demonstrated that the initial site of primary dystonia was relevant for the risk of spread.

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“…Task-specificity in LLD has been previously described [3][4][5][6], but young age of onset is rare [4,5]. Related cases have been described in long distance runners [7] and flamenco dancers [3].…”

Section: Dear Sirsmentioning

confidence: 89%

Drummer’s lower limb dystonia

2011

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Musician's dystonia is an occupational focal dystonia characterized by loss of motor control and coordination, generally affecting particularly demanding tasks on overused body parts. The right hand is generally involved in pianists and guitarists, left hand in violinists, and embouchure in trumpeters [1]. Drummers can develop upper extremity dystonia but there are no prior reports of lower limb dystonia (LLD) even though both feet also become engaged in repetitive, stereotyped, and skilled movements. Here we report two cases.A 23-year-old jazz percussion student presented with a 4-month coordination problem of his left foot when he played the left pedal. He started playing at age 16, and had been playing 5 h/day during the past 4 years. Six months prior to onset of symptoms he had switched to a raised heel playing technique. There were no symptoms during daily life activities. When playing, he developed involuntary tension at left toes, ankle, and knee muscles. This provoked ankle and knee blocking and extension of toes, mainly the first one, during drum playing. The left hip and right leg were not involved. The patient underwent reeducation based on Sensory Motor Retuning experiencing progressive improvement [2]. After 1 year of tailored work at our center, he has resumed conservatorium classes, and is finishing his rehabilitation process.A 22-year-old semiprofessional hard rock drummer presented with a 2-year history of playing difficulties. He had played 3 h/day since he was 17 years old. He also worked as a bulldozer driver for 10 h/day. He had been intensively practicing the double bass pedal technique increasing the kick speed progressively (both feet alternately push their own pedal to kick the bass drum). He first noticed incoordination when playing sixteenths at C160 beats/min. The problem progressively worsened and he became unable to coordinate alternating leg movement at 60 beats/min (Fig. 1). This was associated with involuntary tension at knee and ankle muscles in both legs. Alternating flexion and extension of the ankle was difficult and, as he played, flexion of the toes and rising of the heels appeared. He had no symptoms during daily activities, or driving the bulldozer. When kicking with only one of the pedals problems were of less intensity. During the follow-up, symptoms persisted for 1 year and, after that, he experienced a mild improvement secondary to modifications in practice routines during 1 year more.In both cases, ancillary tests, including brain MRI, electromyogram, and nerve conduction studies were normal.Electronic supplementary material The online version of this article (

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Adult‐onset primary lower limb dystonia

Cited by 62 publications

References 33 publications

Abnormality of motor cortex excitability in peripherally induced dystonia

Abnormality of motor cortex excitability in peripherally induced dystonia

Spread of primary dystonia in relation to initially affected region

Drummer’s lower limb dystonia

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