Kinesigenic Dystonia as the First Manifestation of Multiple Sclerosis with Cervical and Brainstem Lesions

Symptomatic paroxysmal hemidystonia due to a demyelinating subthalamic lesion

“…The fact that no other episodes of neurological dysfunction have occurred, and the absence of abnormalities in the CSF and evoked potentials, precludes the diagnosis of MS in this case (Poser et al ., 1983). However, in some cases of MS paroxysmal symptoms can be the first clinical manifestation of the disease (Twomey et al ., 1980; Benito et al ., 1993; Rozza et al ., 1993; Tranchant et al ., 1995) and, furthermore, the presence of two other similar, small, periventricular white matter lesions increases the risk that this patient will develop definitive MS at some time in the future (Paty et al ., 1988). Acute post‐infectious encephalitis seems unlikely at this time, because there were no documented clinical or laboratory events to support this diagnosis.…”

“…Paroxysmal symptomatic dystonia is a well known occurrence in demyelinating diseases, and has been related to lesions in diverse structures (Tranchant et al ., 1995; Francis et al , 1996). Although initially a spinal or brainstem origin was inferred from several case reports (Osterman and Wertenberg, 1975; Matthews, 1975; Twomey and Espir, 1980; Rozza et al ., 1993; Gatto et al ., 1996), more recently a basal ganglia and internal capsule localization has been considered more in accordance with the known involvement of these structures in several movement disorders (Tranchant et al ., 1995; Burguera et al ., 1991; Lee and Marsden, 1994). These paroxysmal dystonias, also called tonic spasms or tonic seizures (Joynt and Green, 1962; Matthews, 1975; Twomey et al ., 1980), are involuntary muscle contractions that occur without warning, affect agonistic–antagonistic muscle groups, and produce uncomfortable dystonic posturings or movements of variable duration.…”

Hemidystonia secondary to cervical demyelinating lesions

“…There are a few reports of paroxysmal kinesigenic dystonia with a pure lesion of the spinal cord. Rozza et al . (1993) reported a patient with paroxysmal kinesigenic dystonia as the first manifestation of MS with cervical and brain stem lesions.…”

Tonic spasms in acute transverse myelitis

“…Usually, lesions in TS involve regions related to the somatotopic representation of segmental movements within the corticospinal tract motor pathways, at the level of the posterior limb of the internal capsule, the cerebral peduncles, the medulla oblongata, or the anterior and lateral columns of the spinal cord. 1 However, other lesions include ischemic or demyelinated lesions of sensory pathways and some cerebral structures involved in the control of movement, such as the spinal cord posterior column, 6 basal ganglia, 7 subthalamic nuclei, 8 and thalamic nuclei. [9][10][11] However, to our knowledge, no previous study has reported on TS in acute transverse myelitis (ATM).…”