Hemidystonia secondary to cervical demyelinating lesions

Abstract

Hemidystonia is usually associated with a structural lesion in the contralateral basal ganglia. We report a patient with definite multiple sclerosis, according to Poser's criteria, presenting with an acute-onset sustained left hemidystonia. Cranial T2-weighted magnetic resonance imaging (MRI) showed several hyperintense lesions in the centri semiovali and in the periventricular area without basal ganglia involvement. Moreover cervical spinal cord T2-weighted MRI showed two hyperintense lesions in the left posterolateral spine at C2 and C3, and one lesion in the right posterolateral spine at C4 levels. The hemidystonia improved completely after daily treatment with 1000 mg of methylprednisolone, and cervical MRI was performed after the improvement which showed that the lesions had become smaller and less intense. Finally we consider that the hemidystonia may be caused by the cervical spinal cord lesions of multiple sclerosis.

Movement disorders in multiple sclerosis: causal or coincidental association?

“…The brain and spinal cord MRI of our patient disclosed either cerebellum and brainstem atrophy or cervical demyelinating lesions as reported in previous studies [7,8], and dystonia did not improve with MPIV.…”

The occurrence of dystonia in upper-limb multiple sclerosis tremor

“…The most well described MS related dystonia is thought to be paroxysmal dystonia (or tonic spasms) hypothesized to be caused by spreading ephaptic activation of axons within a lesion at any level in a motor pathway. 18 Other focal dystonias are thought to be rare in MS, with occasional case reports of spasmodic torticollis, 1,19 focal dystonia, 3,20 blepharospasm, 21 hemidystonia, 22 and writer's cramp, 23 mostly corresponding to acute inflammatory lesions. A few cases of generalized dystonia in MS have been published with plaques in the basal ganglia implicated.…”

Focal Hand Dystonia as a Sign of Demyelinating Attack in Multipl Sclerosis: 'Report of Three Cases’

“…The mechanism causing the movement disorder was argued to be descending pathways controlling the reciprocal innervation of motor neurons and the involvement of large diameter afferents. Yücesan et al discovered posterolateral and cervical C2-3 and C4 right posterolateral demyelination lesions in an MS patient with left hemidystonia in the absence of any basal ganglia lesions (7). It was hypothesized that the symptoms were due to cervical lesions when 1 gr pulse steroid administration caused the improvement in the cervical lesions as well as the hemidystonia in this patient.…”

“…In such patients, the most commonly seen movement disorder is tremor (25-58%) (1,2,3), followed by paroxysmal dystonia, ballism and chorea, paroxysmal kinesigenic dyskinesia, parkinsonism, myoclonus, hemifacial spasms and continuous facial myokymia, Tourette syndrome and complex hyperkinetic movement disorders (4). Dystonia types associated with MS are cervical dystonia, dystonic writer's cramp (5), hand dystonia, blepharospasm, oromandibular dystonia (6), generalized dystonia or hemidystonia (7). The lesion producing the clinical picture can be at the levels of cervical spinal cord, brainstem, cerebellum, cerebellar peduncles, thalamus, subthalamic nuclei, internal capsule or basal ganglia (8,9,10).…”