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PubMed1997

Symptomatic treatment of neurolathyrism with tolperisone HCL (Mydocalm): a randomized double blind and placebo controlled drug trial.

The efficacy and safety of oral Tolperisone HCL was evaluated in double blind, placebo-controlled, randomized trial in 72 patients with neurolathyrism in stages I, II, and III of the disease at Kolla Duba Health Centre of Dembia District of North Gondar between January and April 1995. Taken orally daily for 12 weeks, tolperisone HCL (Mydocalm) in a dose of 150 milligrams (mgs) twice daily significantly improved subjective complaints such as muscle cramps, heaviness of the legs, startle attacks, flexor spasms and repeated falls. An overall subjective improvement was observed in 75% of the patients on tolperisone HCL and 39% of the placebo group (P = 0.002). When objectively assessed spastic muscle tone in the abductors, stiffness of Achilles and spontaneous ankle clonus were significantly reduced in tolperisone HCL group (P values = 0.001, 0.04, and 0.0001, respectively). Walking ability and speed of walking was also significantly improved. The drug is most effective in relieving symptoms of stage I and stage II disease. Some adverse effects like muscle pain, generalized body weakness and dizziness were recorded in patients taking the drug but all were minor and self limited, none requiring discontinuation of treatment. It is concluded that tolperisone is a well tolerated and efficacious drug for symptomatic treatment of neurolathyrism.

Adolescent↗
PubMed2006

Hereditary spastic paraplegia.

The hereditary spastic paraplegias (HSPs) comprise a large group of inherited neurologic disorders. HSP is classified according to the mode of inheritance, the HSP locus when known, and whether the spastic paraplegia syndrome occurs alone or is accompanied by additional neurologic or systemic abnormalities. Analysis of 11 recently discovered HSP genes provides insight into HSP pathogenesis. Hereditary spastic paraplegia is a clinical diagnosis for which laboratory confirmation is sometimes possible, and careful exclusion of alternate and co-existing disorders is an important element in HSP diagnosis. Treatment for HSP is presently limited to symptomatic reduction of muscle spasticity, reduction in urinary urgency, and strength and gait improvement through physical therapy. Prenatal genetic testing in HSP is possible for some individuals with the increasing availability of HSP gene analysis.

Diagnosis, Differential↗
PubMed2006

Portosystemic myelopathy: spastic paraparesis after portosystemic shunting.

Portosystemic encephalopathy (PSE) is a well-known, common complication of portal hypertension. It is thought to be caused by nitrogenous substances such as ammonia, which are normally cleared from the blood stream by the liver. In cirrhosis and other hepatic disorders with portosystemic shunting (PSS)-- either surgical portosystemic anastomoses (PSA) or spontaneous PSS-- the collateral vessels bypass the liver allowing the accumulation of toxic, ammoniacal substances in the blood and tissues. PSE is characterized by encephalopathy; portosystemic myelopathy (PSM) is characterized by paresis of the extremities, Babinski signs and muscle spasticity in patients with cirrhosis and/or PSS. Usually only the lower extremities are involved. This report presents the first case of this syndrome observed 5 years after a transjugular intrahepatic portosystemic shunt. The 31 year old man with chronic Hepatitis B developed complete spastic paraparesis within 4 weeks after onset of clinical/neurological symptoms, accompanied by an episode of severe hepatic encephalopathy. The transcortical magnetic stimulation showed normal motoric stimulation times to the abductor digiti minimi muscles but no stimulation to the tibialis muscles was seen. Lumbar stimulation to the tibialis muscles, however, was normal. This indicates loss of motor neurons in the spinal cord, a characteristic finding in patients with portosystemic myelopathy. We performed a search of the literature for all reported cases of cirrhosis and/or PSS that developed PSM. However, the intervals between the construction of a shunt and the diagnosis of portosystemic myelopathy were shorter in total portacaval shunts (median 16 months) than in partial, non-portacaval shunts (median 60 months, p < 0.01). This suggests that not only the shunt itself but also the shunted volume contributes to the development of the syndrome Sixty-one patients with PSM have been reported in the literature since 1944. PSE had developed before PSM in almost all cases. PSM occurred from 1 month to 10 years after the creation of portacaval anastomoses (PCA) or splenorenal shunts (SRS) or in cirrhotic patients without shunts. No one type of liver disease or type of shunt appears to predispose to PSM. The mechanisms of PSE and PSM are thought to be similar and of nitrogenous origin, but their pathogenesis remains unknown. Lathyrism, a toxic syndrome with similar symptoms and signs, is caused by the ingestion of a legume, Lathyrus sativa, which contains beta-N-oxalo-L amino-L-alanine (BOAA). This animal model with or without BOAA appears to offer a reliable way of studying PSM experimentally.

Adult↗
PubMed1989

[Critical observation of selected exercises of power training in relation to prevention of postural damage and physical handicaps].

Prevention of and compensation for muscular weaknesses in postural disorders and physical disability are priority tasks of physiotherapy. A range of typical exercises are therefore being used to strengthen weakened groups of muscles, some of them traditionally already included in school sports. Most therapy concepts primarily focus on muscle strengthening, without taking into consideration that it is crucial to redress muscular imbalances by stretching contracted muscle groups as well. Based on this experience, the effectiveness of several specifically modified exercises was tested, derived from the usual range of physiotherapy activities in strengthening very commonly weakened muscle groups, i.e. the abdominal, gluteal, sacrospinalis and shoulder-blade affixing musculature. To increase exercise effectiveness, training machines were used in addition. Stretching exercises were carried out initially, in terms of passive stretching with isometric pre-tension. The following muscle groups were stretched: rectus femoris m., iliopsoas m., pectoralis m., and lumbar sacrospinalis m. Altogether, 64 patients between age 16 and 27 were examined following training therapy, 58 patients presented pure postural disorders, 6 had slight cerebral movement disorders. Two almost equal study groups were formed, one (31 patients) receiving only gymnastic exercises, the other (33 patients) using training machines. The muscular situation was tested after 6 months by means of Janda's semiquantitative muscle-testing. For further objective assessment of therapy outcome, Neugebauer's 'back-index' was determined. Muscular harmony and spinal balance were found to have significantly improved in the second group who had used the training machines. A slight reduction of muscle spasticity in addition was found in the patients with cerebral movement disorders.

Adult↗
PubMed2005

[New concepts in reconstruction of arm and hand function in tetraplegia--basic research and clinical application].

Reconstructive hand surgery improves the ability to meet the needs of daily life and the independence of patients who have lost their upper extremity function due to cervical spinal cord injury. Tendon transfer procedures provide the potential to restore key functions, such as elbow and wrist extension or hand grip control, ameliorate joint balance, reduce pain in spasticity and prevent joint contractures. The choice of the optimal donor muscle should be based on a thorough understanding of the biomechanical principles of the muscle-tendon unit. Intraoperative sarcomere length measurements allow to predict and set the optimal muscle-tendon unit length during reconstructive upper extremity surgery in order to prevent overstretch which may lead to insufficient active force generation. Macro-proteins and the extracellular matrix are in charge of the muscle ultrastructure, elasticity and thus passive muscle tension. Spastic muscles are characterized by greater stiffness, a shorter sarcomere length and an extracellular matrix with inferior mechanical properties. Basic science research and clinical studies in cooperation with international centers are of great importance to promote the development of refined techniques of surgical reconstruction and postoperative rehabilitation of upper extremity function in tetraplegic patients.

Arm↗
PubMed2002

Muscle MRI findings of HTLV-1-associated myelopathy.

We report here the muscle MRI findings in two patients with human T-cell lymphotropic virus type I-associated myelopathy (HAM). It is known that thigh muscles are vulnerable in HAM patients, but detailed information about the affected muscles has not been available. Muscle MRI findings of these patients showed that thigh muscles, especially adductor magnus, and semimembranosus muscles were severely affected, but lower leg muscles were comparatively preserved. In these affected muscles, neurogenic changes were observed by EMG. We concluded that muscle MRI is very useful to estimate the affected muscles in HAM patients.

Aged↗
PubMed2006

Hereditary spastic paraplegia: clinical genomics and pharmacogenetic perspectives.

Hereditary spastic paraplegias (HSPs) are a group of single-gene disorders characterised by degeneration of the corticospinal tract axons, leading to bilateral, symmetrical, slowly-progressive spastic paraparesis, predominantly of the lower extremities. So far, ~ 30 different chromosomal HSP loci have been identified by genetic linkage analysis. Defects in intracellular trafficking and transport in myelination and abnormalities of mitochondrial proteins have been involved in HSP pathogenesis. At present, treatment of the HSPs is primarily directed symptomatically toward reducing muscle spasticity. Yet, recent progresses in the identification of HSP mutations are providing formidable tools to pharmacogenetic approaches of drug discovery, validation and prediction of individual response.

Baclofen↗
PubMed1999

Muscle carnitine acetyltransferase and carnitine deficiency in a case of mitochondrial encephalomyopathy.

Profound decrease of the carnitine acetyltransferase activity (0.08 U/g wet weight; 1.67% of control) and carnitine deficiency (total carnitine was 230 nmol/g wet weight in the patient vs 2730 in the controls) was detected in the skeletal muscle of a female paediatric patient. She died of her illness, which included cerebellar symptoms and slight muscle spasticity affecting mainly the lower extremities, at 1 year of age. Histological examination of the autopsy specimens revealed a selective Purkinje cell degeneration in the cerebellum: the cells had abnormal position, were shrunken and decreased in number, and displayed abnormal dendritic trees and fragmented, disorganized axons. Electron microscopy revealed mitochondrial abnormalities in skeletal and cardiac muscle and also in the Purkinje cells. Deletions of the mitochondrial DNA were detected in the muscle in heteroplasmic form (up to 7%). Mainly the ND4-ND4L region was affected, as evidenced by the PCR; however, other regions of the mitochondrial genome also showed deletions of varying size and extent, suggesting multiple deletions of the mitochondrial DNA.

Brain↗
PubMed1993

H-reflexes are less depressed following muscle stretch in spastic spinal cord injured patients than in healthy subjects.

The size of the soleus H-reflex was measured after a slow (17 deg/s) passive stretch of ankle plantarflexors and compared to its control size without muscle stretch in ten neurologically healthy subjects and in six spastic spinal-cord-injured patients. Two seconds after the end of the stretch, the size of the H-reflex was reduced to about 30% of its pre-stretch size in the healthy subjects. The depression remained for 10-15 s. In the spastic, spinal-cord-injured patients, stretch caused significantly less reduction in the size of the H-reflex. The H-reflex also regained its pre-stretch size much faster than in healthy subjects. We suggest that the smaller depression of the H-reflex observed in spastic patients may be involved in the pathophysiology of spasticity.

Adult↗
PubMed2001

Treatment of functional limitations at the knee in ambulatory children with cerebral palsy.

The gait of ambulatory children with cerebral palsy frequently involves abnormal knee motion. Spasticity, muscle contracture formation, impairments of motor control, weakness, balance deficits, and extrapyramidal motions can all contribute to the functional limitations imposed at the knee. Careful clinical evaluation of the child and their gait must be performed in order to determine the best individual course of treatment. Often, three-dimensional motion analysis with assessment of muscle activity and force is necessary to completely assess the complexities of gait. Several typical gait patterns have been described involving the knee, including 'jump knee', 'crouch', 'true equinus', 'apparent equinus', 'recurvatum' and 'stiff knee' gait. Each of these gait patterns is defined here and discussed using case examples. These typical gait patterns are usually accompanied by involvement at the hip and ankle and may be combined with transverse plane rotational abnormalities. Treatment options such as rehabilitation (physiotherapy, casting, strengthening, and/or orthoses), spasticity management (intramuscular injections of phenol, alcohol, and botulinum toxin type A) and orthopaedic approaches are discussed for each entity.

Biomechanical Phenomena↗
PubMed1990

Correlation of knee extensor muscle torque and spasticity with gait speed in patients with stroke.

The purpose of this investigation was to determine in 17 stroke patients the correlation between two independent variables (knee extensor muscle torque [KET] and spasticity on the paretic side) and one dependent variable (gait speed). The patients had a mean age of 59 years; time since onset of first stroke was 51 days. Each variable was measured twice on each of two consecutive days. Both KET and spasticity were measured on a Cybex II dynamometer. Peak torque was measured during five-second maximal voluntary isometric knee extension efforts. Spasticity was defined by the relative angle of reversal (RAR) of the Cybex electrogoniometer curve obtained during pendulum tests. Gait speed was measured as patients walked 8.0 meters at their most comfortable speed while using their usual devices. The average of each day's KETs, RARs, and gait speeds was used in the calculation of Pearson product moment correlations. The correlations between KET and gait speed were significant (p less than .05) and were .574 (day 1) and .571 (day 2). The correlations between the RAR and gait speed were not significant (-.204 day 1 and -.262 day 2). All measurements had intraday and/or interday reliability coefficients (ICC 3,1) exceeding .930. The results of this study confirm that gait performance is correlated with measures of KET but not spasticity in stroke patients. Thus, at the knee, extensor muscle torque, unlike spasticity, appears to be justified as a practical and objective clinical measure.

Adult↗
PubMed1999

Upper extremity surgical treatment of cerebral palsy.

The purpose of this report is to review the surgical treatment of the upper extremity involved with cerebral palsy over a 25-year period and present our results with regard to changes in upper limb function. Surgical results were assessed by comparison of preoperative and postoperative levels of upper extremity functional use using a previously described 9-level scale. The effect of the following cofactors on surgical outcome were examined: type of cerebral palsy, age, voluntary control, mental impairment, sensibility, and type of surgical treatment. One hundred eighty operations representing 718 procedures in 134 patients were reviewed. Surgical treatment was based on the following principles: soft tissue releases of deforming spastic muscles, tendon transfers to augment antagonistic activity, and joint stabilization. Surgical planning was tailored to each child's particular needs. Comparison of the preoperative and postoperative 9-level functional use scores showed an average improvement of 2.6 functional levels for all patients. Patients with fair and good voluntary control had significantly greater improvement in functional use scores than those with poor voluntary control. No other statistically significant predictive cofactor was found. In selected patients with upper extremity dysfunction secondary to spastic cerebral palsy, surgical intervention improves function, as measured by the upper extremity functional use scale.

Adolescent↗
PubMed2005

Effects of surface electrical stimulation on the muscle-tendon junction of spastic gastrocnemius in stroke patients.

PURPOSE: The purpose of this study was to explore the effects of spasticity suppression by surface electrical stimulation (ES) o the muscle-tendon junction of spastic gastrocnemius muscles in stroke. METHODS: Twenty-four neurologically stable stroke patients (aged 41-69 years, 12-35 months post-stroke), with spasticity graded 2 or 3 on the modified Ashworth scale, were recruited and divided into two groups. In the ES group, each patient received 20 min of surface ES once daily, 6 days per week for 1 month. In the control groups, ES was used with stimulation intensity kept at zero. To evaluate the therapeutic effect, the modified Ashworth scale, Fmax/Mmax ratio, H-reflex latency, H-reflex recovery curve, and the 10-m walking time were tested before and after the 1-month of treatment. RESULTS: In the ES group, the modified Ashworth scale showed a trend toward reduced spasticity after 1 month of treatment. The Fmax/Mmax ratio decreased from 8.10% +/- 4.84% to 4.00% +/- 1.36%; the H-reflex latency increased from 28.87 +/- 2.45 ms to 29.40 +/- 2.57 ms; the H-reflex recovery curves indicated a downward shift; and the 10-metre walking time significantly decreased after ES. In the control group, none of the measures showed a statistically significant change. CONCLUSIONS: In this study, we demonstrated a way to suppress spasticity at a metameric site and to increase walking speed effectively by applying surface ES on the muscle-tendon junction of spastic gastrocnemius muscles.

Adult↗
PubMedDate not supplied

Progressive bone and joint abnormalities of the spine and lower extremities in cerebral palsy.

Bone and joint changes in cerebral palsy result from muscle spasticity and contracture. The spine and the joints of the lower extremity are most commonly affected. Scoliosis may progress rapidly and may continue after skeletal maturity. Increased thoracic kyphosis and lumbar lordosis, spondylolisthesis, spondylolysis, and pelvic obliquity may accompany the scoliosis. Progressive hip flexion and adduction lead to windswept deformity, increased femoral anteversion, apparent coxa valga, subluxation, deformity of the femoral head, hip dislocation, and formation of a pseudoacetabulum. In the knee, flexion contracture, patella alta, and patellar fragmentation are the most commonly seen abnormalities. Recurvatum deformity can also develop in the knee secondary to contracture of the rectus femoris muscle. Progressive equinovalgus and equinovarus of the foot and ankle are associated with rocker-bottom deformity and subluxation of the talonavicular joint. Early recognition of progressive deformity in patients with cerebral palsy allows timely treatment and prevention of irreversible change.

Cerebral Palsy↗