Interesting Presentation of Syringomyelia

INTRODUCTIONSyringomyelia - chronic progressive degenerative or developmental disorder of spinal cord presenting with painless weakness wasting of hands and arms dissociated sensory loss trophic ulcers nystagmus ataxia bulbar weakness etcRare presentation of dystonia hands reported in our case.CASE VIGNETTE12 years old male presented with action dystonia hands- 6 months duration.Clinical examination revealed no motor weakness no sensory loss normal DTR no trophic ulcers. MRI spine and brain revealed cervical syrinxDISCUSSIONSyringomyelia -chronic progressive disorder of spinal cord degenerative developmental or due to tumors -pathological cavitation of central part of spinal cord usually cervical region extend to pons and lumbar segments associated with abnormalities of vertebral column base of skull cerebellum and brain stem. Clinically present with motor weakness dissociated segmental sensory loss in hemicape or cape distribution atrophy or wasting of hands trophic ulcers. Syringobulbia present as nystagmus analgesia thermoanaesthesia of face wasting and weakness of tongue palatal and vocal cord paralysis hoarseness of voice and dysphagia. Rare presentation of dystonia choreoathetoid movements of hand and arm have been reported. Dystonia being disorder of basal ganglia occur in spinal cord lesions. Abnormality of normal reciprocal inhibition between agonist and antagonist due to defective cortical control or changes in tonic afferent input to the interneurons from cutaneous and muscle afferents. Diffuse or focal dystonias also reported in lesions of mesencephalon cerebellum medulla peripheral nerves central pontine myelinolysis. Surgical decompression of the syrinx relieves the dystonia.