Focal dystonia: current theories.

Dystonia is a syndrome characterised by abnormal involuntary sustained muscle contractions that often result in twisted and abnormal positions. Focal dystonia affects only a single body part with symptoms varying from permanent (e.g., torticollis) to task-specific (e.g., musician's cramp). The exact causes of focal dystonia have yet to be determined. Possible causative factors have been identified at all levels along the sensorimotor pathway, including anatomical constraints of the hand (musicians), abnormal co-contractions of the muscles due to reciprocal inhibition in the spinal cord, subcortical and cortical remapping, deficiencies in sensorimotor integration and perceptual deficits. A review of the current literature on these topics is provided with a special focus on musicians with focal dystonia. Also reviewed are current treatments of focal dystonia in musicians. On the basis of the currently available evidence, certain risk factors are identified for the development of task-specific focal dystonia, including number of practice hours, personality, genetic predisposition, performance factors and sensory effects. In addition, it is highlighted that dystonic movements occur predominantly in the context of perceptual-motor tasks involving emotions. When emotional and motor traces have become associated, they are difficult to change; it is suggested that this mechanism plays an important role in the preservation of dystonic symptoms.     

Stuttering: a dynamic motor control disorder

The purpose of this review is to determine what neural mechanisms may be dysfunctional in stuttering. Three sources of evidence are reviewed. First, studies of dynamic inter-relationships among brain regions during normal speech and in persons who stutter (PWS) suggest that the timing of neural activity in different regions may be abnormal in PWS. Second, the brain lesions associated with acquired stuttering are reviewed. These indicate that in a high percentage of cases, the primary speech and language regions are not affected but lesions involve other structures, such as the basal ganglia, which may modulate the primary speech and language regions. Third, to characterize the motor control disorder in stuttering, similarities and differences from focal dystonias such as spasmodic dysphonia (SD) and Tourette’s syndrome (TS) are reviewed. This review indicates that the central control abnormalities in stuttering are not due to disturbance in one particular brain region but rather a system dysfunction that interferes with rapid and dynamic speech processing for production.

Educational objectives: The reader will be able to describe: (1) the similarities and differences between stuttering and other speech motor control disorders, (2) which brain lesions are most likely to produce acquired stuttering in adults, and (3) what type of brain abnormality most likely underlies stuttering.     

Increased variability in spiral drawing in patients with functional (psychogenic) tremor

Increased variability is a characteristic clinical and physiologic feature of functional (psychogenic) tremor. In this study, we use computerized spiral analysis to show that the variability of a motor task is a quantifiable characteristic of functional tremor. We compare functional tremor patients to phenomenologically similar dystonic tremor patients and to normal controls. We used the spiral severity score, a measure that does not incorporate spiral tightness, as a marker of spiral drawing performance, and inter-spiral tightness variability (based on the 25–75%^ile range in tightness across ten spirals) to evaluate the effects of functional tremor on drawing spirals. The spirals of 74 participants: 22 functional tremor, 21 dystonic tremor, and 31 normal controls were analyzed. Spiral severity was higher in both tremor groups compared to controls, but did not differentiate them. Inter-spiral variability, however, was higher in the functional tremor group compared to both other groups. Thus, spiral analysis captures variability of a motor task and may be used as an objective test for functional tremor. The effect of functional tremor in other motor tasks should be investigated.