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Transcutaneous electrical nerve stimulation for treatment of spinal spasticity.

Thirty-two patients with spinally originated muscle spasticity were treated with a transcutaneous electrical nerve stimulator, the Han's acupoint nerve stimulator (HANS) via skin electrodes placed over the acupoints on the hand and leg. High frequency (100 Hz), but not the low frequency (2 Hz), stimulation was effective in ameliorating muscle spasticity. While the therapeutic effect lasted for only 10 minutes in the first treatment, it became consolidated after consecutive daily treatment for 3 months. The anti-spastic effect induced by high frequency electrical stimulation can be partially reversed by a high dose of naloxone. The results suggest that the anti-spastic effect elicited by peripheral electrical stimulation is mediated, at least in part, by the endogenous opioid ligand interacting with the kappa opiate receptors, most probably dynorphin, in the central nervous system.

Acupuncture Points↗

Botulinum toxin for the treatment of spasticity in multiple sclerosis. New observations.

Potential advantages of intramuscular botulinum toxin for the treatment of spasticity include the lack of sensory effects, ability to target specific muscle groups, ability to weaken muscles in a graded fashion and absence of caustic chemicals such as phenol. We describe the use of botulinum toxin for the treatment of severe lower extremity spasticity in two subjects with multiple sclerosis. Both subjects showed an improvement in spasticity, as measured by the modified Ashworth scale, and in functional status. Both subjects exhibited reductions in muscle tone not only in injected muscles, but also in noninjected muscles in the region. These more distant clinical effects have not been emphasized in previous studies after therapeutic injections of botulinum toxin. Further research is needed to clarify the cause and prevalence of these regional motor effects, as well as to further examine the safety and efficacy of botulinum toxin for spasticity treatment.

Activities of Daily Living↗

Intrathecal baclofen for treatment of intractable spinal spasticity.

This study assessed the safety and efficacy of intrathecal baclofen in the treatment of intractable spasticity caused by spinal cord injury or multiple sclerosis. Twenty-three patients with severe chronic spasticity underwent bolus test dosing with 50, 75, or 100 micrograms of intrathecal baclofen administered by lumbar puncture. All patients were either refractory to oral baclofen at a dose of 120 mg/d or side effects were unacceptable at a lower dose. There was a significant decrease in tone and spasticity in all 23 patients. Nineteen patients underwent implantation of a programmable pump and intrathecal catheter designed to deliver baclofen directly to the spinal cord. Rigidity (tone) was decreased from a mean prebolus Ashworth score of 3.8 to a mean postbolus Ashworth score of 1.5 and spasms from a mean prebolus score of 3.5 to a mean postbolus score of 1.2 for a minimum of 4 hours. Patients have been observed for a mean of 16 months (range 2 to 34 months). Ashworth scores have remained reduced to an acceptable level (< or = 2 with periodic adjustment in dosage in all but three patients. There has been one pump malfunction and four catheter malfunctions; few serious medication and postoperative complications have occurred. There was one death caused by underlying disease, one patient voluntarily withdrew, and three patients developed tolerance to the extent that optimal control of spasticity tone could not be maintained. Although intrathecal baclofen is safe and effective in the majority of patients, three patients required > 1,000 micrograms/d with increasingly higher doses over time and exhibited a poor response.

Adult↗

Attenuation of facial muscle spasticity with intramuscular phenol neurolysis.

Intramuscular phenol neurolysis is a well-known procedure used to decrease spasticity and improve function in patients who have failed to respond to more conservative forms of intervention. Traditionally, this approach has been limited to spasticity reduction in limb muscles, and its use in managing spasticity of the facial muscles has not been described in the literature. This case report describes a new and previously unreported application of intramuscular neurolysis for managing severe unrelenting facial muscle spasticity in a postanoxic encephalopathic patient. Prior to the procedure, hypertonicity in the orbicularis oris muscle was so profound that it limited speech and affected cosmetic, hygienic, and nutritional status. After intramuscular phenol neurolysis of the orbicularis oris muscle, the patient's level of functioning improved.

Eating↗

[Simple examination of spastic movement disorders of the lower limbs in the infant age].

In 334 children active dorsiflexion of the feet was tested in order to screen for spasticity of the lower limbs. The conditions were: 1. The child lying supine. 2. The head turned actively and if this is not possible passively to the side of the examined foot. 3. Passive extension of the child's hip and knee of the examined side by the examinator. 4. Stimulation of the skin at the lateral and lower part of the shin, or the dorsum pedis or the anterior part of the toes and the planta pedis to provoke active dorsiflexion of the foot. Stimulation is done by the fingers of the examinator or by a slight touch with an ordinary handbrush. Dorsiflexion of the foot is graded "normal" if it surpasses 90--100 degrees, "suspicious" if it only reaches 90 degrees and "pathologic" if 90 degrees cannot be reached or if this can be done only on the medial side or if there is constant clawing of the toes even with sufficient dorsiflexion. We have examined 334 children; 260 of these were younger than one year. The results of this kind of stimulated dorsiflexion of the foot were compared with the results of the usual extensive neurological examination with regard to spasticity of the lower limbs (see literature) and with both of VOJTA's most sensible postural reactions. The following conclusions are drawn: 1. Spasticity of the lower limbs can be excluded with a high degree of probability when dorsiflexion of the feet is graded "normal". 2. If the dorsiflexion is graded "suspicious" or "pathologic" a more thorough neurological examination is warranted and if necessary short-term controls. 3. The results of this examination are in good agreement with the one of VOJTA's most sensitive postural reactions to indicate spasticity of the lower limbs.

Age Factors↗

[Treatment of spasticity with botulinum toxin and muscle afferent block].

Spasticity is partly caused by disinhibition of spinal stretch reflex activity and blocking of the group Ia afferents may improve its clinical symptoms. Since muscle afferent block has been successfully used for treating dystonia, this method may become useful for treating spasticity as well. We injected diluted lidocaine (0.5%, 50 ml/session) and ethanol (5 ml) into thigh adductors (for leg crossing), medial hamstrings (for medial rotation of the leg), quadriceps femoris (for hyperextension of the knee), tibialis posterior and triceps surae muscles (pes equinovarus deformity), biceps brachii and forearm flexors (for flexion deformities of the upper extremity) in 12 patients with spasticity. Although the duration of action was short (< 1 day) at first, it was gradually prolonged to several weeks by repeating the injection every 3-4 days. The final outcome was comparable to that obtained by botulinum toxin injection in the same group of patients. This method may prove its promise as a means of treating spasticity.

Adult↗

[Treatment of spasticity with botulinum toxin].

Spasticity is a velocity-dependent pathologic increase in muscle resistance to stretch, and occurs in a variety of neurologic disorders. We report our controlled open study using botulinum toxin A for treatment of adductor spasticity in five patients with advanced multiple sclerosis. Clinical evaluation of spasticity and stiffness of joints is based on the Ashworth Scale and grade of passive abduction. Three patients showed no response; the two others experienced an excellent and longlasting effect. We also describe briefly the different spastic conditions where this treatment has been used successfully.

Adult↗

[Botulinum toxin in spasticity treatment].

The author reviews the current opinions on the treatment of spasticity with special consideration given to the new method of treatment with local injections of botulinum toxin A into the spastic muscles. Botulinum toxin is the treatment of choice in focal dystonias and hemifacial spasm. The mechanism of action of the toxin is unique and is a result of dose-dependent and partial chemical denervation of the muscles, with preservation of tonus and thus its function. Recent reports have confirmed the safety and effectiveness of the method in spasticity, especially when it is focal, not diffuse or severe and without concomitant severe paresis. The author describes also the basic data of the pathophysiology of spasticity and reviews other therapeutic options and practical problems concerning the injections of botulinum toxin.

Botulinum Toxins, Type A↗

[A case of autosomal dominant, pure form spastic paraplegia with thinning of the corpus callosum].

Autosomal dominant hereditary spastic paraplegia (HSP) is genetically classified into three types, all of which are characterized by insidiously progressive spasticity of the lower extremities. Patients with a complicated form of autosomal recessive HSP associated with hypoplasia of the corpus callosum have been reported by Iwabuchi et al. Here we report a 64-year-old patient with a pure form of autosomal dominant HSP with thinning of the corpus callosum. He had been well until 12 years of age, when spasticity and weakness of the lower extremities began to develop. His symptoms gradually worsened and he had difficulty in walking at the age of 44. When he was 56 years old, he visited our hospital. Eleven family members over five generations have been affected, and anticipation, i.e., an apparent decrease in age of onset, has been observed. On admission, he had mild cataracts, equinovarus and pes cavus, and neurological examination revealed spastic paraplegia. However, the intelligence test was normal, and nystagmus, ataxia of the extremities, involuntary movement, orthostatic hypotension or urinary disturbance was not observed. Trinucleotide repeat diseases, such as Huntington's disease, spinocerebellar ataxia type 1, spinocerebellar ataxia type 2, Machado-Joseph disease and dentatorubral-pallidoluysian atrophy, were excluded by DNA analysis. Brain MRI at the age of 64 revealed marked thinning of the corpus callosum. We considered this patient had a pure form of HSP. However, thinning of the corpus callosum has never been reported in autosomal dominant HSP.

Adolescent↗

EMG analysis of voluntary elbow movements in subjects with spasticity.

Surface electromyography (EMG) signals were measured from the biceps brachii (BIC), the brachioradialis (BRD) and the triceps brachii (TRI) of seven young adults with pure spastic quadriplegia and eight normal individuals. The signals were analyzed in both the time domain and the frequency domain. The subjects sat in an adjustable chair and were asked to perform two voluntary tasks involving the elbow joint. For task A, the subjects moved the hand from the proximal target plate to the distal target plate and then back to the proximal one along the table plane. For task B, the subject followed the sound of a metronome (0.5 Hz), perform 5 elbow flexion/extension cycles in the vertical plane. Certain quantitative parameters were identified as statistically significant in describing quantitatively the condition of spasticity. Compared to the normal subjects, the spastic subjects showed significantly higher EMG activities for both the elbow flexors and extensor. The stretch/shortening (S/S) ratios for BRD and TRI were significantly different from those of the normal subjects. The power spectra analysis showed higher mean power frequency (MPF). MPF of BIC and TRI were uncorrelated for normal subjects, but correlated for the spastic subjects. It is speculated that low motor unit discharge rate and fiber type abnormalities may account for these differences.

Adult↗

[Microsurgical drezotomy for the treatment of spasticity of the lower limbs].

Ablative functional neurosurgery can be useful in some selected patients for the treatment of harmful spasticity in the lower limbs. Microsurgical drezotomy was introduced in 1972, on the basis of anatomical studies of the human dorsal root entry zone (DREZ) showing a topographical segregation of the afferent fibers according to their size and thus functional destinations. It consists of a 3 mm deep microsurgical lesion directed at a 45 degree angle in the postero-lateral sulcus, penetrating the DREZ in its ventro-lateral aspect, at the level of all the rootlets considered as involved in spasticity (and pain). It destroys mainly the lateral (nociceptive) and central (myotatic) afferent fibers as well as the facilitatory medial part of the Lissauer tract, whilst sparing most of the medial (lemniscal) fibers and the inhibitor lateral part of the Lissauer tract. We report a series of 121 bedridden patients suffering from harmful spasticity in one (15) or both (106) lower limbs and treated with microsurgical drezotomy. Surgery was decided on because of abnormal postures in flexion in two-thirds of the patients and in hyperextension in one-third, additional pain in 75 of them, and hyperactive bladder in 38 cases. The post-operative results were evaluated after a mean follow-up time of 5 years and 6 months. Both spasticity and spasms were significantly decreased or suppressed respectively in 78% and 88% of the patients. When present, pain was relieved without abolition of sensation in 82%. These benefits resulted in either disappearance or marked reduction of the abnormal postures and articular limitation in 90% of the patients. When present preoperatively, urinary leakage disappeared in 85% of the cases. Mild to severe complications occurred in 32 patients and precipitated or were responsible for death in 6 cases (5%). This is explained by the fact that most of the patients, especially those affected by multiple sclerosis, were in very precarious general and neurological conditions. Microsurgical drezotomy has however enabled a large majority of these severely disabled patients to sit and lie comfortably, and allowed them to reach a significantly improved quality of life.

Adult↗

Idiopathic central diabetes insipidus followed by progressive spastic cerebral ataxia. Report of four cases.

A distinctive syndrome of diabetes insipidus developed in four boys in early childhood and progressive spastic cerebellar ataxia developed in adolescence. The boys have been observed for 12 to 19 years and are currently 19 to 25 years old. In patient 1, 12 years after the onset of diabetes insipidus and 4 years after the onset of spastic cerebellar ataxia, bone lesions that proved to be histiocytosis were detected. In patient 2, calcification that developed in the cerebellar dentate nuclei was similar to calcification described in patients with histiocytosis. In the other two patients, an etiologic diagnosis has not been established. We conclude that there is a distinctive syndrome characterized by early diabetes insipidus with subsequent progressive spastic cerebellar ataxia. While histiocytosis may not account for this complex syndrome in all cases, diabetes insipidus followed by progressive spastic cerebellar ataxia merits intensive evaluation.

Adult↗

Acoustic and perceptual comparison of chronic and incipient spastic dysphonia.

Vocal symptoms of patients with chronic and incipient spastic dysphonia were compared on a number of acoustic and perceptual parameters. Patients with incipient spastic dysphonia displayed less severe strain-strangle phonation, effort, rhythm, and stress. Harshness was the only perceptual parameter on which incipient spastic dysphonics were rated higher than chronic spastic dysphonics. Changes in the acoustic measures of laryngealization, harmonic change, mean vowel duration, and duration ratio between stressed and unstressed vowels accompanied changes in listeners' perception of strain-strangle phonation and effort. The variation in acoustic characteristics such as laryngealization and harmonic change as well as normal phonation indicated that both groups were characterized by a variety of phonatory modes.

Acoustics↗

Striking intrafamilial phenotypic variability and spastic paraplegia in the presence of similar homozygous expansions of the FRDA1 gene.

We report on a Friedreich's ataxia (FA) family with 3 affected siblings with markedly different phenotypic presentations, including one with spastic paraplegia. Molecular analysis showed midsize GAA repeat expansion sizes in all 3 individuals. Gait spasticity in FA, although rare, has been described in a few patients who are compound heterozygotes for a point mutation, or who had GAA expansions of less than 200 repeats. The occurrence of spastic paraplegia in our family, in the presence of homozygous midsize GAA repeat expansions, is an unusual finding. Spasticity can be the main feature in both sporadic and familial patients with FA, either as an isolated finding, or in addition to other neurological abnormalities, and should be included as a rare feature in the clinical spectrum of FA. This family also demonstrates that in FA, marked intrafamilial phenotypic variability can arise in the presence of similar GAA expansion sizes. Therefore, in familial FA, the disease course in relatives therefore cannot be predicted solely from repeat length. Factors such as somatic mosaicism, repeat interruptions, modifying mutations and environmental factors must also be considered.

Adult↗

[About the relief of severe spasticity in paraplegics (author's transl)].

The treatment of spasticity following spinal cord injuries demands a well coordinated team of neurologists, neuro-surgeons, surgeons and orthopedic surgeons. The causes of the beginnings of spasms, circumstances, by which the intensity of existing spasticity is supplementary advanced, will be described. The intensity of spasms in joints of the paralysed limbs can be improved by controlled "Passive Body Exercises" for a short time. But we have never seen relievings of spasticity by drugs. In certain cases a systematic rehabilitation takes only effect, if intractable spasticity--especially concerning the knee and hip joints--is either completely removed or at least recovered. By means of a posterior rhizotomy by Förster (posterior nerve rotts section) spasms following traumatic spinal cord lesions can be eliminated completely or at least changed for the better just depending largely upon the level of the injury to the cord.

Exercise Therapy↗

Outcomes after selective dorsal rhizotomy for spastic cerebral palsy.

OBJECT: The purpose of this article was to review the published outcomes after selective dorsal rhizotomy (SDR) for treatment of spastic cerebral palsy. METHODS: A literature search identified all articles related to outcomes after SDR. The outcomes were reviewed according to a paradigm developed by the National Center for Medical Rehabilitation Research (NCMRR). The quality of the evidence for each outcome was assessed using Sackett's criteria and the classification system developed by the Brain Trauma Foundation and the American Association of Neurological Surgeons. RESULTS: There is very strong evidence for benefits of SDR in the impairment domain of the NCMRR classification. SDR has been shown conclusively to decrease lower limb spasticity and increase lower limb range of motion. There is strong, but not as conclusive evidence that SDR has a positive impact in the functional limitation dimension, with improvements in motor function, and in particular the Gross Motor Function Assessment (GMFM). There is a moderate degree of certainty that SDR results in improvements in the disability dimension, as evidenced particularly by improvements in the Functional Independence Measure for Children (WeeFIM) and Pediatric Evaluation of Disability Inventory (PEDI). There is a moderate degree of certainty that SDR results in positive suprasegmental effects, especially related to upper limb function and cognition. There is weak evidence that SDR may reduce the need for orthopedic procedures in patients with spastic cerebral palsy, and the impact on hip subluxation relative to the natural history of this problem is unclear. CONCLUSIONS: This information could help to define the role of SDR in the management of the child with spastic cerebral palsy, in the light of alternative therapies, such as intrathecal baclofen and botulinum toxin, which have been introduced more recently. It also reveals the need for further studies, particularly dealing with quality of life and economic impact.

Cerebral Palsy↗

Preservation of endothelium-dependent vasodilation in the spastic segment of the human epicardial coronary artery by substance P.

The objective of this study was to determine if endothelium-dependent vasodilation is preserved in the spastic segment of the epicardial coronary artery. Segmental responses of the coronary artery to substance P were examined by the use of a quantitative angiographic technique in 21 patients with variant angina. Coronary diameter at the basal state did not differ between the spastic and the nonspastic segments (2.3 +/- 0.2 mm, 2.3 +/- 0.4 mm, p greater than 0.05). Changes in coronary diameter in response to substance P did not differ between segments with ergonovine-induced spasm and nonspastic segments. Maximal dilation averaged 27.1 +/- 9.5% in the spastic segment and 24.4 +/- 9.6% in the nonspastic segment (expressed as a percent increase over the value before drug administration). It appears that both the potential of the endothelium to release endothelium-dependent relaxing factor and the dilating response of the smooth muscle to endothelium-dependent relaxing factor are preserved, even in the spastic segment.

Angina Pectoris, Variant↗

Sensibility deficiencies in the hands of children with spastic hemiplegia.

We evaluated 40 children with spastic hemiplegia due to cerebral palsy for sensory function and relative limb size in the affected and unaffected upper extremities. Sensory function of each limb was evaluated with respect to stereognosis (12 objects), two-point discrimination, and proprioception. Four size measurements of each limb were made: arm and forearm circumference and forearm and forearm-hand length. This study showed that 97% of the spastic limbs had a stereognosis deficit, 90% had a two-point discrimination deficit, and 46% had a proprioception deficit. Thus sensory deficits are the rule rather than the exception in children with spastic hemiplegia. Those children with severe stereognosis deficits had significantly smaller limbs in all four measurement parameters than the children with mild or moderate stereognosis deficits. In the preoperative evaluation of children with spastic hemiplegia, severe size discrepancy is a physical examination tool that can be used as a predictor of severe sensory deficits. This information is helpful for the hand surgeon in establishing realistic surgical goals.

Anthropometry↗