Effects of repetitive peripheral magnetic stimulation on upper-limb spasticity and impairment in patients with spastic hemiparesis: a randomized, double-blind, sham-controlled study.

OBJECTIVE: To investigate short-term and long-term effects of repetitive peripheral magnetic stimulation (rpMS) on spasticity and motor function. DESIGN: Monocentric, randomized, double-blind, sham-controlled trial. SETTING: Neurologic rehabilitation hospital. PARTICIPANTS: Patients (N=66) with severe hemiparesis and mild to moderate spasticity resulting from a stroke or a traumatic brain injury. The average time ± SD since injury for the intervention groups was 26 ± 71 weeks or 37 ± 82 weeks. INTERVENTIONS: rpMS for 20 minutes or sham stimulation with subsequent occupational therapy for 20 minutes, 2 times a day, over a 2-week period. MAIN OUTCOME MEASURES: Modified Tardieu Scale and Fugl-Meyer Assessment (arm score), assessed before therapy, at the end of the 2-week treatment period, and 2 weeks after study treatment. Additionally, the Tardieu Scale was assessed after the first and before the third therapy session to determine any short-term effects. RESULTS: Spasticity (Tardieu >0) was present in 83% of wrist flexors, 62% of elbow flexors, 44% of elbow extensors, and 10% of wrist extensors. Compared with the sham stimulation group, the rpMS group showed short-term effects on spasticity for wrist flexors (P=.048), and long-term effects for elbow extensors (P<.045). Arm motor function (rpMS group: median 5 [4-27]; sham group: median 4 [4-9]) did not significantly change over the study period in either group, whereas rpMS had a positive effect on sensory function. CONCLUSIONS: Therapy with rpMS increases sensory function in patients with severe limb paresis. The magnetic stimulation, however, has limited effect on spasticity and no effect on motor function.

Adrenomyeloneuropathy.

A 29-year-old male patient presented with progressive spastic paraparesis of three years duration. He also had gait ataxia which led to recurrent falls. In addition, there was pigmentation of the skin creases, tongue and buccal mucosa. His clinical course was remarkable by recurrent episodes of diarrhea, pulmonary tuberculosis. The investigatory work up showed a normal MRI scan of the brain, spinal cord and normal abdominal structures. The basal serum cortisol levels were low. Adrenomyeloneuropathy was diagnosed and he was started on corticosteroid supplementation. Mineralocorticoid supplementation also is planned in the follow up. The case is being presented for its rarity.

Neurological disorders associated with cassava diet: a review of putative etiological mechanisms.

Tropical ataxic neuropathy (TAN) and epidemic spastic paraparesis (konzo) are two neurological disorders associated with the consumption of cassava (Manihot esculenta) in several African countries. TAN is characterized by sensory polyneuropathy, sensory ataxia, bilateral optic atrophy and bilateral sensori-neural deafness. It occurs in elderly individuals subsisting on a monotonous cassava diet with minimal protein supplementation. Konzo is a syndrome of symmetrical spastic paraparesis with a predilection for children and young women and invariably associated with consumption of inadequately processed bitter cassava roots with minimal protein supplementation. Despite numerous epidemiological, clinical and biochemical studies aimed at elucidating the etiological mechanisms of these disorders, their etiologies remain unknown, and there is no known treatment. The diseases continue to be prevalent in endemic areas, causing significant disability and increased mortality. A fresh appraisal of the putative etiologic mechanisms proposed for these intriguing and enigmatic syndromes is presented in this paper. Evidences against a causal role for cyanide intoxication are discussed, and evidences implicating thiamine deficiency as a unifying etiological mechanism for these neurological syndromes are presented. It is concluded that urgent research is needed to evaluate thiamine status and implement a therapeutic trial of thiamine in these debilitating neurological disorders.

Etiology of Konzo, epidemic spastic paraparesis associated with cyanogenic glycosides in cassava: role of thiamine deficiency?

Konzo is a syndrome of symmetrical, non-progressive, non-remitting spastic paraparesis occurring in epidemic and endemic forms in several countries in Africa, invariably associated with monotonous consumption of inadequately processed bitter cassava roots (Manihot esculenta) with very minimal protein supplementation. Despite numerous epidemiological, clinical and biochemical studies by authors in several countries aimed at elucidating the etiological mechanisms of Konzo, the etiology remains unknown. High cyanide consumption with low dietary sulfur intake due to almost exclusive consumption of insufficiently processed bitter cassava roots was proposed as the cause of Konzo, but there has been no evidence of a causal association between cyanide consumption and Konzo. In this paper a new etiological mechanism of thiamine deficiency is presented, based on detailed review of the epidemiological, clinical and biochemical features of Konzo. It is postulated that in Konzo patients, a severe exacerbation of thiamine deficiency results from the inactivation of thiamine that occurs when, in the absence of dietary sulfur-containing amino acids; the sulfur in thiamine is utilized for the detoxification of cyanide consumed in improperly processed bitter cassava. Thiamine is known to be rendered inactive when the sulfur in its thiazole moiety is combined with hydrogen cyanide. This hypothesis may stimulate studies examining the role of thiamine in the etiology of Konzo, and may lead to the formulation of strategies for the prevention and treatment of this debilitating disease.

Spinal arteriovenous fistulae with a concomitant cauda equina schwannoma.

BACKGROUND: Perimedullary spinal arteriovenous malformations or direct spinal arteriovenous fistulaes (AVFs) may be associated with other vascular abnormalities, such as arteriovenous malformations, venous ectasis, and aneurysms, but rarely have been reported with intraspinal intradural tumors. PURPOSE: The authors present an interesting case of type IV-A spinal AVF concomitant with a cauda equina schwannoma. STUDY DESIGN: The diagnostic procedures and surgical outcome were described. METHODS: The patient underwent surgery, the vessel feeding the AVFs was identified and cauterized, and the spinal tumor was removed. The fistula was small and located inside the tumor. The pathology revealed AVF and schwannoma, respectively. RESULTS: After surgery, the patient's symptoms began to improve and subside. Two years after surgery, follow-up magnetic resonance imaging showed no vascular lesion and tumor in the spinal canal. CONCLUSIONS: The association of spinal AVFs and cauda equina schwannoma has not been reported previously in any literature. The patient presents the symptoms of myelopathy associated with a spinal vascular lesion; it has to be noted that a concomitant and related intradural spinal tumor may exist.

Effects of neuromuscular electrical stimulation treatment of cerebral palsy on potential impairment mechanisms: a pilot study.

PURPOSE: This pilot study examined the effects of neuromuscular electrical stimulation (NMES) therapy on upper limb impairment in children with cerebral palsy, specifically addressing spasticity, heightened passive resistance to wrist rotation, coactivation, and weakness. METHODS: Eight subjects, aged five to 15 years, with spastic hemiparesis subsequent to brain injury, participated in three months of NMES therapy, targeting the wrist flexor and extensor muscles. Maximum voluntary wrist extension range of motion against gravity, spasticity, passive torque, maximum voluntary isometric torque, and coactivation were recorded prior to, during, and at the conclusion of the therapy. RESULTS: Seven of the eight subjects demonstrated a significant (>15 degrees) improvement in wrist extension range of motion against gravity following the NMES treatment, with an average gain of 38 degrees. Differences in spasticity (0.01 +/- 0.14 N-m, p = 0.80) and passive torque (0.03 +/- 0.11 N-m, p = 0.52) were not significant for these subjects. Isometric wrist extension torque, however, did increase significantly (p < 0.01), accompanied by a reduction in flexor coactivation (p < 0.01). CONCLUSIONS: Evidence suggests that the NMES treatment protocol affected wrist extension by improving the strength of the wrist extensor muscles, possibly through decreased flexor coactivation. Further studies are required, however, to determine whether electrical stimulation itself or other facets of the therapy paradigm played the key role in improvement.

Electrically induced recovery of gait components for older patients with chronic stroke.

OBJECTIVE: To compare the gains for chronic stroke patients in volitional gait pattern attained from treatment with functional neuromuscular stimulation (FNS) and intramuscular electrodes (IM) with gains attained using conventional therapy, including treatment with FNS using surface electrodes (surface-stim). DESIGN: This single-subject research design consisted of a series of two subjects. Three months of conventional therapy and surface-stim were followed by treatment using the FNS-IM system. Two stroke patients had cerebrovascular accident 1 or 4 yr before the study and ambulated with a cane. Interventions consisted of 3 months of conventional exercise and gait training including surface-stim, followed by 7-14 months of treatment with the FNS-IM system. Treatments occurred up to 3 times/wk for 1-hr sessions and a home program. Outcome measures consisted of six kinematic gait components, as measured by a six-camera video-based data-acquisition system. Coordination of isolated joint movement was measured according to the Fugl-Meyer scale. RESULTS: Both subjects improved during conventional therapy to some degree. During FNS-IM treatment, gains were made beyond those attained during conventional therapy. Statistically significant differences were found between conventional and FNS-IM therapy. CONCLUSIONS: For these two subjects, gains in volitional control of gait were made during conventional treatment (including surface-stim); for these two subjects during FNS-IM treatment, additional gains were made in volitional gait pattern, beyond those attained during conventional therapy.