Update on the Use of Botulinum Toxin Therapy for Focal and Task-Specific Dystonias.

Focal dystonia is defined by anatomical distribution and represents a distinct entity from generalized dystonia. Task-specific dystonia occurs in the context of specific patterns of movement. Botulinum neurotoxin (BoNT) injections are the treatment of choice in most cases. Several formulations are available; the approved indications, dosing, and some administration details, differ between them. The major forms of focal and task-specific dystonia are reviewed, along with the evidence for BoNT therapy, the expected benefit and side effects, and practical points guiding the injections.

Botulinum toxin and occupational therapy for Writer's cramp.

BACKGROUND: Writer's cramp (WC) is a form of focal hand dystonia, for which focal botulinum neurotoxin (BoNT) injections are the current best therapy. Past studies have shown that some types of rehabilitative therapy can be useful. We hypothesized that BoNT together with a specific type of occupational therapy would be better than BoNT alone for treating WC patients comparing the effects with a patient-rated subjective scale. METHODS: Twelve WC patients were randomized to two groups. Six received only BoNT therapy and 6 received BoNT & occupational therapy. The occupational therapy involved specific exercises of finger movements in the direction opposite to the dystonic movements during writing. BoNT was injected by movement disorders neurologists in the affected muscles under electromyography-guidance. The primary outcome was the patient-rated subjective scale at 20 weeks. Secondary exploratory outcomes included the writer's cramp rating scale (WCRS), writer's cramp impairment scale (WCIS), the writer's cramp disability scale (WCDS), handgrip strength and kinetic parameters. RESULTS: The patient-rated subjective scale scores at 20 weeks were not significantly different between the two groups. Significant objective improvement was noted in the BoNT & occupational therapy group, as noted by the decrease (28%) in WCIS scores. CONCLUSIONS: Improvement of the primary outcome measure, the patient-rated subjective scale, was not achieved. However, significant improvement was found in the BoNT & occupational therapy group in a secondary measure of impairment. Our hypothesis-driven study results are likely limited by small sample size, and further large-scale studies of occupational therapy methods to improve the efficacy of BoNT seems worthwhile.

The Relationship between Saccades and Locomotion.

Human locomotion involves a complex interplay among multiple brain regions and depends on constant feedback from the visual system. We summarize here the current understanding of the relationship among fixations, saccades, and gait as observed in studies sampling eye movements during locomotion, through a review of the literature and a synthesis of the relevant knowledge on the topic. A significant overlap in locomotor and saccadic neural circuitry exists that may support this relationship. Several animal studies have identified potential integration nodes between these overlapping circuitries. Behavioral studies that explored the relationship of saccadic and gait-related impairments in normal conditions and in various disease states are also discussed. Eye movements and locomotion share many underlying neural circuits, and further studies can leverage this interplay for diagnostic and therapeutic purposes.

Electromyographic and Joint Kinematic Patterns in Runner's Dystonia.

Runner’s dystonia (RD) is a task-specific focal dystonia of the lower limbs that occurs when running. In this retrospective case series, we present surface electromyography (EMG) and joint kinematic data from thirteen patients with RD who underwent instrumented gait analysis (IGA) at the Functional and Biomechanics Laboratory at the National Institutes of Health. Four cases of RD are described in greater detail to demonstrate the potential utility of EMG with kinematic studies to identify dystonic muscle groups in RD. In these cases, the methodology for muscle selection for botulinum toxin therapy and the therapeutic response is discussed. Lateral heel whip, a proposed novel presentation of lower-limb dystonia, is also described.

Retinal detachment with macular holes in infants with retinopathy of prematurity.

OBJECTIVE: To describe retinal detachment with macular holes in infants with retinopathy of prematurity and the methods of surgical repair with their outcomes. METHODS: A retrospective review of 4 cases of retinopathy of prematurity and 1 case of retinopathy in a full-term infant that resembled retinopathy of prematurity, in which a macular hole and associated retinal detachment developed and surgical repair was attempted. RESULTS: The average gestational age of the 4 infants with retinopathy of prematurity was 26 weeks. All 5 patients had a history of vitreoretinal surgery prior to discovery of the macular hole. All 4 who underwent further surgical correction have partially or completely attached retinas and ambulatory vision. CONCLUSIONS: Vitrectomy combined with fluid-air exchange failed to close a macular hole associated with retinal detachment in infants with retinopathy of prematurity. Athermal scleral buckling of the macula can close macular holes and reattach the retina. Scleral buckling appears to be the method of choice for repair.

Severe retinopathy in a child with hypoplastic left heart syndrome.

PURPOSE: We present the first reported case of a condition similar to retinopathy of prematurity in a full-term patient with hypoplastic left heart syndrome. DESIGN: Interventional case report. METHODS: We describe the clinical presentation and surgical treatment of a male baby with severe retinopathy and retinal detachments. RESULTS: The clinical course of this baby was similar to retinopathy of prematurity. The patient had areas of avascular retina, significant retinal neovascularization, fibrous proliferation, and tractional detachment. One eye had a totally detached retina; the other had functional vision after the retina was reattached by several vitreoretinal surgeries, including the use of intraocular silicone oil. CONCLUSIONS: This cyanotic condition can cause a retinopathy with chronic retinal neovascularization and retinal detachments. Permanent surgical retinal reattachment is difficult in the presence of neovascular activity that leads to intravitreous cicatrization.