Syringomyelic dystonia and athetosis

Hill, Michael D.; Kumar, Rajeev; Lozano, Andrés M.; Tator, Charles H.; Ashby, Peter; Lang, Anthony E.

doi:10.1002/1531-8257(199907)14:4<684::aid-mds1021>3.0.co;2-g

Cited by 23 publications

(12 citation statements)

References 21 publications

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“…The case highlights the wide spectrum of involuntary movements reported in patients with syringomyelia, i.e. those which are spontaneous and stimulus-dependant [1,2,4], spontaneous and stimulus-independent [3] or, as in our case, exclusively stimulus-dependent [2]. Interestingly, in spite the clinical heterogeneity a similar pathogenic mechanism is postulated, which is increased excitability of spinal motor neurons of probably different intensity due to altered processing at the inhibitory spinal interneuron circuits [1].…”

Section: Sollberger P Fuhrsupporting

confidence: 52%

Flexor myoclonus of the arm due to posttraumatic cervico-thoracic syringomyelia

Sollberger

Fuhr

2008

J Neurol

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Section: Sollberger P Fuhrsupporting

confidence: 52%

Flexor myoclonus of the arm due to posttraumatic cervico-thoracic syringomyelia

Sollberger

Fuhr

2008

J Neurol

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“…Syringomyelic dystonia has been rarely reported and included cases with torcicollis 1,2 , dystonia of the legs 3 , dystonia of the hands and arms 4 , cervical dystonia 4 , and blefarospasm 1 . The mechanisms of involuntary movements secondary to spinal cord lesions have yet to be fully understood.…”

Section: Discussionmentioning

confidence: 99%

Cervical and axial dystonia in a patient with syringomyelia

Silva‐Júnior

Santos

Sekeff-Sallem

et al. 2012

Arq. Neuro-Psiquiatr.

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Abnormal electromyographic activity is commonly found in patients with syringomyelia, but involuntary movements have only rarely been reported 1 .We have reported the case of a patient with large syringomyelia, who developed cervical and axial dystonia. CASE REPORTA 40-year-old woman presented with progressive sensory loss and weakness of the lower limbs, followed by urinary retention. Concomitantly, she developed repetitive

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“…[1][2][3][4] Cervical radiculopathy caused by disc herniation or cervical spondylosis is common and important to recognize because it may improve after decompression surgery. [1][2][3][4] Cervical radiculopathy caused by disc herniation or cervical spondylosis is common and important to recognize because it may improve after decompression surgery.…”

mentioning

confidence: 99%

“…[1][2][3][4] Syrinx formation accompanying these lesions is not uncommon. Dystonia and choreoathetosis are rare associations of cervical cord lesions such as ependymoma, glioma, myxoma, demyelination, and trauma.…”

mentioning

confidence: 99%

“…[1][2][3][4] These include altered sensory input (in particular proprioceptive pathways), abnormal processing of both input and output signals in the spinal inter-neurones, and increased excitability of the spinal motor neurones. [1][2][3][4] These include altered sensory input (in particular proprioceptive pathways), abnormal processing of both input and output signals in the spinal inter-neurones, and increased excitability of the spinal motor neurones.…”

mentioning

confidence: 99%

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Cervical disc prolapse with cord compression presenting with choreoathetosis and dystonia

Tan¹,

Lo²,

Chan³

et al. 2002

Neurology

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Movement disorders from spinal cord disease are rare and can be caused by underlying neoplasm, inflammation, demyelination, or trauma. [1][2][3][4] Cervical radiculopathy caused by disc herniation or cervical spondylosis is common and important to recognize because it may improve after decompression surgery. We describe a patient with cervical disc prolapse presenting with choreoathetosis and dystonia.Case report. A previously well 62-year-old Chinese woman presented with involuntary movements of her upper extremities of 3-month duration. She observed that her fingers would wriggle on their own and her arms would move involuntarily. These movements were much worse during action than rest. She reported no premonition or any urge before these movements, and did not feel any relief afterwards. The movements were aggravated by stress, agitation, or anxiety, and were relieved by rest and sleep. She also noted that her fingers (particularly the index and middle fingers) would intermittently go into spasms, with involuntary flexion at the metacarpal-phalangeal joints; these spasms were relieved by putting her fingers in contact with other body parts. In addition, she had concurrent "tingling" sensations of the fingertips of both hands (right more severely affected). There were no abnormal movements involving the oro-facial-buccal region or lower extremities. She had no urinary or bowel complaints. There was no history of exposure to neuroleptic medications, severe head trauma, or dementia. No relatives had similar problems. She was initially evaluated by an orthopaedic surgeon who suspected underlying carpal tunnel syndrome (CTS). This was confirmed on the nerve conduction test, which revealed a mild to moderately severe right CTS. However, she was subsequently referred to the Movement Disorders Clinic for further evaluation.Neurologic examination demonstrated dystonic posturing of her fingers when upper arms were outstretched and in wingbeating position. Her index and middle fingers were flexed at the metacarpal-phalangeal joints and proximal inter-phalangeal joints, together with mild pronation at the radio-ulnar joints. Stereotypic finger movements were observed when she tried using her hands. On walking, choreoathetoid movements in both of her upper extremities were present. Power in all of her extremities was full. There was increased tone in both her lower extremities, generalized hyper-reflexia in her four extremities with no clonus. Her jaw jerk was not brisk. Plantar response was flexor bilaterally. There was no loss of sensation to pinprick, light touch, or presence of a sensory level, and proprioception and vibration sense were intact. There were no signs of cerebellar dysfunction and Romberg test was negative. She had no clinical evidence of dementia and no Kayser-Fleischer ring on ophthalmic examination.MRI of cervical spine revealed severe cord compression at C3-C4 level by a prolapsed disc ( figure, A and B). Brain MRI scan did not show any abnormalities. Transcranial magnetic stimulation study demonstrat...

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Syringomyelic dystonia and athetosis

Cited by 23 publications

References 21 publications

Flexor myoclonus of the arm due to posttraumatic cervico-thoracic syringomyelia

Flexor myoclonus of the arm due to posttraumatic cervico-thoracic syringomyelia

Cervical and axial dystonia in a patient with syringomyelia

Cervical disc prolapse with cord compression presenting with choreoathetosis and dystonia

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