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PubMedDate not supplied

Musculoskeletal deformities in Behr syndrome.

SUMMARY: Seventeen children with Behr syndrome were investigated, focusing on the musculoskeletal deformities and long-term outcome. Behr syndrome is characterized by optic atrophy beginning in early childhood associated with ataxia, spasticity, mental retardation, and posterior column sensory loss. The ataxia, spasticity, and muscle contractures, mainly of the hip adductors, hamstrings, and soleus, are progressive and become more prominent in the second decade. In 70% of the patients, contractures developed in the lower limbs, requiring surgery mainly for the Achilles tendon, hamstrings, and adductor longus. At last follow-up at an average age of 21.7 years (range, 8-31 years), 13 of the patients are housebound walkers, 2 are nonfunctional walkers, and 2 are nonwalkers.

Achilles Tendon↗
PubMed1975

Bladder training: its role in evaluating the effect of an antispasticity drug on voiding in patients with neurogenic bladder.

Fourteen patients with spinal cord damage were treated with Ba-34647 (Lioresal, Ciba-Geigy), a new antispasticity drug. The treatment was initiated for excessive skeletal muscle spasticity and voiding difficulty. Seven of the patients had been wearing indwelling catheters and seven were catheter-free. The former were given trials at voiding after removal of catheters; the usual assistive methods common to most bladder training regimens were administered. Despite this, the trials were unsuccessful in reducing residual urine to acceptable levels. With addition of therapeutic doses of the drug without the training regimen, voiding trials were also unsuccessful excepting the response of one patient. The drug plus the training regimen was effective in reducing residual urine to acceptable levels in all patients. On discontinuing or decreasing the dosages of the drug, there was gradual but rapid build-up of residual urine despite the active training regimen. Restoration of effective dosage again led to satisfactory voiding function in all patients. The catheter-free group suffered from frequency, nocturia, and bed-wetting owing to excessive residual urine despite the employment of active training regimens. With addition of optimal dosages of Ba-34647, these problems were markedly reduced. They increased with drug discontinuation or dosage decrease and again improved upon restoration of effective doses. Bladder training, including active assistance to the expulsion of urine, is essential to the evaluation of antispasticity drugs for their effect on voiding.

Adult↗
PubMed1976

Levodopa-enhanced recovery from paralysis induced by air embolism.

In this study levodopa was chosen as an agent that might ameliorate the clinical manifestations of paralysis induced by air embolism because of reported effects of this drug on muscle spasticity and locomotion. In order to induce air embolism, the aorta of rats was cannulated chronically. Fifteen days later, after full recovery of the animals, air was administered through the chronic cannula into the descending aorta of each rat (0.35 ml. of air per 100 Gm. during 4 seconds). The paralysis of both hind legs was observed one to 10 minutes later. Only animals that had total paralysis of both hind legs, without any sensation, were used in the experiments. levodopa was administered 2 minutes after experimentally induced paralysis. The levodopa treatment was repeated every day for 6 days. After 6 days, six levodopa-treated (intraperitoneally) animals in a group of eight and ten levodopa-treated (intra-arterially) animals in a group of 12 recovered fully from the paralysis. Only three animals in a group of 13 (untreated) or two animals in 12 (solvent administration) recovered without the levodopa treatment.

Animals↗
PubMed2004

[A novel analgesics made from Cannabis].

Bayer AG has recently announced that it acquired exclusive rights for the marketing of GW Pharmaceuticals' new medicine Sativex in Europe and in other regions. Sativex is a sublingual spray on Cannabis extract basis, and is equipped with an electronic tool to facilitate accurate dosing and to prevent misuses. It is standardized for the THC and CBD. The new analgesic is proposed for the treatment of muscle spasticity and pains accompanying multiple sclerosis and as an efficient analgetic for neurogenic pain not responding well to opioids and to other therapies available. The entirely new mechanism of action through the recently discovered cannabinoid receptor system may offer a real therapeutic potential to the drug. Although the Government of Netherlands has authorized the sale of pharmaceutical grade Cannabis herb by pharmacies in the Netherlands, the availability on the pharmaceutical market of the registered preparation may render requests for the authorization of the smoking of Cannabis herb (marihuana) by individuals suffering of multiple sclerosis, neurogenic pain, AIDS wasting syndrome unnecessary. Nevertheless, the "old chameleon" plant Cannabis appears to gradually regain its previous status in mainstream therapy and pharmacy. As long as the plant Cannabis and its products continue to be classified as narcotic drugs, medical use of the new preparation will need close supervision.

Analgesics↗
PubMed2005

[Neurotomy of the tibial nerve for treatment of the talipes equinovarus].

OBJECTIVE: To explore indication and approaches of neurotomy of tibial nerve for the treatment of the talipes equinovarus and spastic paralysis. METHOD: A total of 25 patients including 15 patients with cerebral paralysis and other 10 patients were diagnosed with clear etiology, spastic muscles with supplied nerves were analyzed in detail, neurotomy of the tibial nerve was performed under microscope, during operation, motor nerve was lovated with electronic stimuli and monitored with induced electromyography. The surgical results were analyzed. RESULTS: A total of 36 of 37 (97%) neurotomy of the tibial nerve were improved with dorsum flex of ankle at 10 - 45 degree, and 22 of 24 (85%) talipes equinovarus improved clinically. Of 37 feet with surgery, 24 feet (65%) were in normal position and could touch ground completely, 9 feet (24%) had 10 degree with ground or could contact ground with external one third of the feet, 4 feet (11%) were abnormal. Of 25 patents, 21 (84%) had improvement of motor activity at discretion, 5 patients (20%) improved dramatically. All the 4 patients failed after the operation were cerebral paralysis, and were presumably related to disorders of spinal function, abnormal reflex and the disappearance of inhibitory reflex. CONCLUSION: Neurotomy is an effective method to treat talipes equinovarus, and can improve the motor activity of low extremity.

Adolescent↗
PubMed2001

Cannabinoids - from plant to patient. 5 April 2001, London, UK.

The active constituents of cannabis (predominantly cannabinoids and possibly flavonoids) are more effective than a single cannabinoid in reducing muscle spasticity in a multiple sclerosis animal model. Possible interactions include both pharmacokinetic and pharmacodynamic effects. Synthetic cannabinoids with agonist or antagonist/inverse agonist effects are available with high affinity for cannabinoids receptors. Pharmaceutical products that block their uptake and metabolism may enhance the endocannabinoid system. Government- and charity-funded clinical trials of cannabis are proceeding in pain, both acute (MRC multicenter trial) and chronic, and multiple sclerosis (MRC and Multiple Sclerosis Society multicenter trials). The design of these trials should enable evidence to be presented to regulatory bodies documenting the medicinal uses of standardized cannabis plant material.

Journal Article↗
PubMedDate not supplied

[Combined regional anesthesia of the lower extremities in children with cerebral paralysis].

The authors share their first experience of combined regional anesthesia during surgical interventions into the lower extremities in children with infantile cerebral paralysis (ICP). A hundred and twenty-five blocks were performed in patients aged 5 to 20 years. Lidocaine, marcaine, naropine, or a mixture of lidocaine and marcaine mixture, were used. The paper describes the specific features of techniques for femoral-sciatic nerve blocks in these patients, shows the adequacy of anesthesia at surgery and the very good course of the postoperative period due to continuous analgesia and the alleviation of muscle spasticity in the operated limb.

Adolescent↗
PubMed1991

[Pathological tonic reflexes in adolescents with infantile cerebral palsy].

A study was made of pathological tonic reflexes (PTR) in adolescents afflicted with infantile cerebral paralysis (ICP) by means of assaying the tone of thigh muscles before and after treatment and in the presence of L-DOPA (nacom, 62.5 mg/day) intake. The drug minimized the manifestations of PTR because of changes in the function of brain structures involved in tonic reactions. It is recommended that during establishment of indications for +orthopedic-surgical treatment of ICP patients with marked PTR determining the formation of reflex contractures and deformities of limb joints, conservative treatment with L-DOPA drugs be preliminarily carried out.

Adolescent↗
PubMed2006

Is there any relationship between the Modified Ashworth Scale scores and alpha motoneuron excitability indicators?

The purpose of this study was to investigate the relationship between the modified Ashworth scale (MAS) scores and alpha motoneuron excitability indicators. Thirty-one post-stroke patients were assessed for this object. The main outcome measures were the MAS and electro physiologic assessments. The latter was performed using both conventional (Hmax/Mmax) and new (Hslope/Mslope) measures of spinal excitability. Data on thirty-one adult subjects with hemiplegia (twenty-five men and six women) were analysed. The soleus Hmax/Mmax ratio appeared to correlate directly with the MAS scores (r = 0.36; P < 0.05). Correlation between the MAS scores and either Hslope/Mslope ratio or H-reflex latency was not significant (P > 0.05). In seventeen patients whose H-reflex could be evoked bilaterally, spinal excitability indicators showed significant difference between the affected and non-affected sides (P < 0.05). Based on the results of this study, there is no relationship between the MAS scores and the preferred measure of alpha motoneuron excitability. This research suggests that the MAS could not distinguish between the reflexive and non-reflexive components of the hypertonicity in ankle plantar flexors.

Adult↗
PubMed1991

[A family with autosomal dominant hereditary myoedema, muscular irritability, stiffness and hypertrophy].

A familial case with autosomal dominantly transmitted myoedema, muscular irritability, stiffness and hypertrophy was reported. The patient is 54 years old and his father, two sisters and niece had suffered from the similar symptoms. He had noticed a feeling of stiffness and pain in the femoral muscles after several minutes of erect position from the age of 5-6 years. He had observed that light tapping of muscles caused a bulge which persisted for several seconds in the whole body. These symptoms were not progressive. The patients had an athletic appearance, hypertrophy of gastrocnemius muscles, pes cavus and hammer toes. Mild muscular weakness and wasting were noted in the intrinsic hand muscles and the anterior tibial muscles. Myoedema was seen in the muscles of the whole body. His thyroid functions were normal. EMG studies showed no myotonic discharges. Light microscopy of biopsy specimens from the biceps brachii muscle showed unspecific myopathic changes. Electron microscopy showed many vacuoles between myofibrils. The symptoms and signs of this case are very like to those of the cases which Torbergsen described in 1975 and only four families were reported after that. The family presented here is the first report in Japan.

Edema↗
PubMed1991

Motor variables correlated with the hand-to-mouth maneuver in stroke patients.

This study investigated the relationship between each of three independent variables and the completion of the hand-to-mouth maneuver in 23 hemiparetic stroke patients. The independent variables were elbow-extensor muscle tone (graded using the Modified Ashworth Scale); active elbow-flexion range-of-motion deficit measured without the influence of gravity; and elbow-flexion strength measured without the influence of gravity with a hand-held dynamometer. The hand-to-mouth maneuver was graded according to the degree of completion using a three-level ordinal scale. Spearman (rs) correlations demonstrated a significant relationship between the extent of completion of the hand-to-mouth maneuver and both the active elbow-flexion-range deficit (rs = -.853) and the elbow-flexor muscle force (rs = .829). The correlation of the maneuver with elbow-extensor muscle tone (rs = -.063) was not significant, but the relationship may have been influenced by the fact that only a minority of subjects had elevated tone, and that elevation was minimal. The result suggest that both active-movement deficits and muscle strength may be important to upper extremity function.

Arm↗
PubMed1978

[Experience using sinakten-retard in the treatment of muscular sclerosis].

The experience of the authors demonstrated that the best results in sinakten-retard treatment were attained in patients with mild and moderate degrees of severity of the disease. However, even in patients with a protracted development, treatment by sinakten-retard increased the muscular strength in the paralyzed extremities, diminished the increased muscular tone and increased the volume of movements. All these facts permit the authors to recommend sinakten-retard for the treatment of disseminated sclerosis.

17-Hydroxycorticosteroids↗
PubMed1990

[F-waves and H-reflexes in hemispastic syndrome].

Canalized F-waves and H-reflexes occurring on small hand and foot muscles are to be considered symptoms of central disinhibition. Occurrence of these waves and reflexes in patients with hemispastic syndrome is analyzed and correlated with clinical aspects.

Adolescent↗