Print version ISSN 0004-282X
Arq. Neuro-Psiquiatr. vol.70 no.9 São Paulo Sept. 2012
Distonia cervical e axial em uma paciente com siringomielia
Francisco Pereira da Silva-JúniorI; Jasper Guimarães SantosI; Flávio Augusto Sekeff-SallemI; Leandro Tavares LucatoII; Egberto Reis BarbosaI
IDepartment of Neurology of Universidade de São Paulo (USP), São Paulo SP, Brazil
IIDepartment of Radiology of USP, São Paulo SP, Brazil
Abnormal electromyographic activity is commonly found in patients with syringomyelia, but involuntary movements have only rarely been reported1.
We have reported the case of a patient with large syringomyelia, who developed cervical and axial dystonia.
A 40-year-old woman presented with progressive sensory loss and weakness of the lower limbs, followed by urinary retention. Concomitantly, she developed repetitive painful involuntary movements of the head, neck, and thoracolumbar region.
Her history was remarkable for pulmonary and meningeal tuberculosis at the age of 35 years. In that occasion, she discovered to be HIV positive.
Neurologic examination revealed reduced pinprick, touch and temperature sensation to the level of C4 on the right, and T2 on the left side of the body. Vibration and joint position senses were absent in the lower limbs, which were plegic and atrophic. Deep tendon reflexes were normal in the upper and absent in the lower extremities. Involuntary jerky movements, primarily consisting of retrocollis and thoracolumbar extension were noticed. Mental status and cranial nerves were normal.
Laboratory evaluation was unremarkable. Brain magnetic resonance imaging (MRI) was normal. Cervical and thoracic MRI disclosed a large septated syringomyelia extending from the cervicothoracic junction to the conus medullaris, with evidence of arachnoid adhesions (Figure). The electromyography showed cervical and axial dystonia.
The patient underwent a T4-6 laminectomy with syringe-subarachnoid shunt. Her neurologic condition remained unchanged two years after the surgery. She received botulinum toxin A in the splenius capitis, deep cervical paraspinal, and thoracolumbar paravertebral muscles bilaterally. She experienced marked improvement of the involuntary movements and pain.
The patient presented cervical and axial dystonia concomitantly with the onset of progressive neurological signs, which led to syringomyelia diagnosis.
Syringomyelic dystonia has been rarely reported and included cases with torcicollis1,2, dystonia of the legs3, dystonia of the hands and arms4, cervical dystonia4, and blefarospasm1.
The mechanisms of involuntary movements secondary to spinal cord lesions have yet to be fully understood. Electrophysiological findings suggest a role for damage of α-motor neuron, lesion of spinal interneurons, and damage to descending motor pathways1.
Few cases of syringomyelia as a complication of tuberculous meningitis have been described. There is usually a latent period between the evidence of inflammation and the development of syringomyelic symptoms, which varies between 7 to 28 years. In these cases, arachnoid adhesions and multifocal loculations were often present, which resulted in treatment failures and poor prognosis5.
We described a rare case of dystonia associated with syringomyelia, highlighting the involvement of spinal cord lesions in the genesis of involuntary movements and reinforcing the importance of obtaining a spinal cord MRI when investigating a case of dystonia with atypical presentation.
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2. Kiwak KJ, Deray MJ, Shields D. Torcicollis in three children with syringomyelia and spinal cord tumor. Neurology 1983;33:946-948. [ Links ]
3. Berardelli A, Thompson PD, Day BL, Rothwell JC, O'Brien MD, Marsden CD. Dystonia of the legs induced by walking or passive movement of the big toe in a patient with cerebellar ectopia and syringomyelia. Neurology 1986;36:40-44. [ Links ]
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5. Kaynar MY, Koçer N, Gençosmanoğlu BE, Hanci M. Syringomyelia-as a late complication of tuberculous meningitis. Acta Neurochir 2000;142:935-939.
Francisco Pereira da Silva-Júnior
Rua Doutor Melo Alves 55 / apto. 123
01417-010 São Paulo SP - Brasil
Received 15 April 2012
Received in final form 27 April 2012
Accepted 04 May 2012
Conflict of interest: There is no conflict of interest to declare.