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[Focus on current research: improving the mobility of paraplegic patients].

Since the first paraplegic centre was established in 1945, life expectancy and life quality of paraplegics have considerably improved. However, endeavours to enhance the mobility of these patients have been less successful. The most promising approach, functional electric stimulation of paralysed muscles, is poorly accepted by patients at present because of technical problems. This study describes current approaches which may help to improve patients' mobility. A central motor lesion is perceived by the patient as a movement disorder of the legs, e.g. a gait disorder. Neurological investigation indicates, on the basis of exaggerated tendon reflexes and increased resistance of the non-activated leg muscles to stretching, that spastic paresis underlies the movement disorder. This combination of symptoms and clinical (physical) signs suggests that the exaggerated tendon tap reflexes are responsible for muscle hypertonia and the latter causes the movement disorder. However, electromyography during movement shows that the exaggerated short latency reflexes are associated with loss or attenuation of the functionally essential polysynaptic spinal reflexes. In the event of impaired supraspinal control there is loss of monosynaptic stretch reflex inhibition combined with reduced facilitation of polysynaptic spinal reflexes. Development of tension in tonically active calf muscles in patients with spastic paresis during gait occurs independently of spinal reflex activity. From electrophysiological and histological observations it can be assumed that transformation of motor units resulting in simple and less well adapted regulation of muscle tone allows movements such as gait. The reduction of muscle tone obtained with antispastic drugs is usually associated with paresis and may therefore hamper locomotion. Locomotor training represents a new attempt to improve the mobility of patients with incomplete paraplegia. It includes activation of neuronal circuits within the spinal cord below the level of the lesion. In incomplete paraplegics a coordinated leg muscle activation pattern and corresponding leg movements can be triggered and trained in patients standing on a treadmill with partial weight support. Improvement of training of the spinal cord locomotor centre can be expected from triggering of spinal cord reflexes and regeneration of spinal tract fibres, which is expected to be possible in the near future.

Humans↗

Epidural electrical stimulation of posterior structures of the human lumbosacral cord: 3. Control Of spasticity.

OBJECTIVES: The purpose of this study was to evaluate the effect of spinal cord stimulation (SCS) on severe spasticity of the lower limbs in patients with traumatic spinal cord injury (SCI) under close scrutiny of the site and parameters of stimulation. MATERIALS AND METHODS: Eight SCI patients (four women, four men) were included in the study. Levels of spasticity before and during stimulation were compared according to a clinical rating scale and by surface electrode polyelectromyography (pEMG) during passive flexion and extension of the knee, supplemented by a pendulum test with the stimulating device switched either on or off over an appropriate period. RESULTS: Both the clinical and the experimental parameters clearly demonstrated that SCS, when correctly handled, is a highly effective approach to controlling spasticity in spinal cord injury subjects. The success of this type of treatment hinges on four factors: (1) the epidural electrode must be located over the upper lumbar cord segment (L1, L2, L3); (2) the train frequency of stimulation must be in the range of 50 - 100 Hz, the amplitude within 2 - 7 V and the stimulus width of 210 micross; (3) the stimulus parameters must be optimized by clinically assessing the effect of arbitrary combinations of the four contacts of the quadripolar electrode; and (4) amplitudes of stimulation must be adjusted to different body positions. CONCLUSIONS: Severe muscle hypertonia affecting the lower extremities of patients with chronic spinal cord injuries can be effectively suppressed via stimulation of the upper lumbar cord segment.

Adolescent↗

Relationship between plasma concentration and effect of dantrolene sodium in man.

Dantrolen sodium is a muscle relaxant, which is used in the treatment of spasticity. Although it is given chronically, little is known about its pharmacokinetic behaviour. The relationship between the effect of a single oral dose of dantrolene sodium and its plasma concentration in healthy volunteers was studied by measuring the effect on the twitch tension, and in spastic patients on the decrease in muscle hypertonia. On the twitch tension dantrolene gave a depression of 49.1 +/- 9.4% (+/- DS) within 1.15 and 3.45 h after ingestion of 100 mg. The mean maximal plasma concentration was 1.24 +/- 0.32 microgram/ml (+/- SD). The effect and the plasma concentration were correlated. No relationship between the plasma concentration of dantrolene sodium and its effect could be established in patients, although definite activity in 6 out of 7 patients was observed after a single oral dose of 100 mg, and plasma concentration of dantrolene sodium greater than 0.3 microgram/ml were consistenly associated with better results than placebo treatment in 6 out of 7 patients.

Adult↗

Autosomal recessive disorder with muscle contractions resembling neonatal tetanus, characteristic face, camptodactyly, hyperthermia, and sudden death: a new syndrome?

This work describes an autosomal recessive syndrome observed over the past 25 years in 17 newborn babies (8 males, 9 females), from 12 different families in Southern Sardinia. This disorder is evident at birth and is characterized by marked muscular contraction of the facial muscles in response to tactile stimuli or during crying, with trismus and abundant salivation simulating a tetanic spasm. The contractions slowly disappear as the infant calms. There is also neck muscle hypertonia with a tendency to opisthotonus. All patients present facial anomalies such as large face, chubby cheeks, broad nose with anteverted nostrils, and long philtrum. The hands show bilateral camptodactyly. The clinical course in all patients was characterized by marked feeding difficulties and appearance of variable fever at about 38 degrees C, with peaks of irregular hyperthermia of over 42 degrees C, with onset ranging from birth to a few weeks. In some patients these symptoms were accompanied by generalized seizures. Death occurred after a period of a few weeks to some months and coincided with fever above 42 degrees C. Laboratory investigations performed in all of these cases did not give any useful pathogenetic indications. Only patients 10 and 16 are still alive today. Patient 10 is now 14 years old. She presents slow regression of the dystonic symptomatology, while dysthermia and mild psychomotor delay persist.

Chromosome Aberrations↗

Determining consistency of elbow joint threshold angle in elbow flexor muscles with spastic hypertonia.

BACKGROUND AND PURPOSE: Threshold angle, the point in passive range of motion where a muscle response or torque change is elicited, may be a potentially valid measure of hypertonus. Because the relationship of initial muscle length to threshold angle has not been addressed previously, this preliminary study examined whether starting elbow joint position and speed of stretch to elbow flexor muscles affect threshold angle. SUBJECTS: Five subjects with stroke-induced hypertonia of the elbow flexor muscles participated. METHODS: Two starting angles and two designated stretch speeds were applied randomly by a torque motor at each of three testing sessions. RESULTS: Starting angle, subject, and session affected threshold angle. A 90-degree starting angle at a stretch speed of approximately 1.0 radian/s produced the most consistent threshold angles between sessions within subjects, and threshold angle was relatively consistent for some subjects, irrespective of speed. CONCLUSION AND DISCUSSION: If future research indicates that these data can be generalized, the use of threshold angle as a consistent measure of hypertonia will require comparison within individuals, use of a consistent starting angle, and a movement condition of a 90-degree starting angle and an approximate movement speed of 1.0 radian/s across sessions.

Adult↗

[Partial blocking of the tibial nerve with phenol as treatment of gait disorders due to pes equinus in central paralysis].

Partial chemical neurolysis by means of a peripheral nerve block induced with a phenol solution can result in decreasing local muscle hypertonia of central origin. In case of a walking disability resulting from a spastic or dystonic equinovarus position of the ankle the phenol block of the tibial nerve may be a valuable treatment that improves walking ability. The technique of the procedure is explained. A phenol block is induced only after a favourable result of a trial block with a local anaesthetic. In the group of 19 patients who underwent tibial nerve block given by the author in the period 1987-1989 there appears to exist a correlation between the functional results and the extent to which the patient had been active in walking prior to the nerve block. A group of 6 'sitters' and 'hardly walking' patients had poor to moderate results, a group of 13 'walkers' had moderate, good and excellent results. The difference in functional outcome is ascribed by the author to a better possibility to actually utilise a muscular balance improved by the phenol block. In the early--recovery--phase of stroke rehabilitation a phenol block of the tibial nerve may well be useful in cases with equinovarus position of the ankle which interferes with therapeutic exercise and functional progress.

Aged↗

Hyperekplexia.

Hyperekplexia is a hereditary neurologic disorder manifested by an exaggerated startle response, generalized muscular rigidity, and prominent nocturnal myoclonus. The distinctive features of this syndrome constitute an unusual clinical entity that is easily mistaken for other disorders. The study of a family provided additional data on various aspects of this condition. The proband was 3 months old when he was referred for persistent generalized stiffness since birth; this stiffness was associated with an exaggerated startle response and intermittent apnea. Similar symptoms in infancy and prominent nocturnal myoclonus with an excessive startle response in adulthood were described in other family members for five generations. Various features of the disorder indicate a relationship to the syndrome of the "jumping Frenchmen of Maine," latah, miryachit, and other unusual startle reactions.

Humans↗

Infantile bilateral striatal necrosis. Clinicopathological classification.

Two cases of infantile bilateral striatal necrosis (IBSN) were studied retrospectively, and the literature was reviewed. The two children had presented with progressive neurologic signs of involuntary movements or muscle hypertonia from infancy. Initial computed tomography scans showed mild atrophy of the caudate nuclei or basal ganglia, and the neuropathologic examination revealed diffuse neuronal loss with some patchy preservation and marked astrogliosis in the striatum and globus pallidus. The 27 reported cases of IBSN were divided into three groups with characteristic clinical and pathologic features: early, acute onset (four cases); early, gradual onset (16 cases); and late onset (seven cases). Although metabolic changes in the developing corpus striatum may be important in the pathogenesis of IBSN, the origin is uncertain.

Brain↗

Hereditary stiff-baby syndrome.

Clinical and electrophysiologic features of an infant with stiff-baby syndrome were studied; his father, uncle, and paternal grandmother suffered from a similar disorder. They all had a similar facial appearance during infancy (an alert, frightened, tense look) and a tendency to vomit because of hiatal hernias. The condition is benign but causes anxiety and, later, embarrassment as affected adults suddenly fall when stumbling or startled. We suggest that the condition be called hereditary stiff-baby syndrome. The family history is suggestive of autosomal dominant inheritance.

Adult↗

Morphology and histochemistry of myogelosis.

Myogelosis is a common diagnosis in the case of chronic pain conditions, especially in the region of the pectoral girdle musculature, the glutei muscles, and the erector spinae muscle. Although such indurative areas continue to be palpable even on the cadaver, few studies concerning the morphological substrate of these areas have been undertaken. Selected biopsies as well as larger tissue samples were taken from 11 corpses and prepared for histological study. Following staining, the frozen sections were examined morphometrically. A histologically constant, significant morphological alteration was found in the areas of concern. The spaces between the individual muscle fibers of healthy muscle tissue appear relatively wide, the endomysium of the myogelotic area are clearly narrowed. Split fibers, ragged red fibers, Type II fiber atrophy, and fibers with a moth-eaten appearance have been detected. The morphometry shows considerable increase in thickness of the affected muscle fibers, suggestive of a pathological, local hypertrophy. The changes described may well represent a fixed condition, so that it should not be surprising that myogelosis therapy is difficult and protracted.

Aged↗

Myosin isoforms in female human detrusor.

The aim of this study was to document the relative proportions of two isoforms of myosin heavy chain in detrusor smooth muscle of women with detrusor overactivity and in asymptomatic controls. Women aged 35-65 with documented detrusor overactivity and without a history of neurologic disease, prior incontinence surgery, elevated post-void residual urine volume, or indwelling urinary catheter were eligible for the study. Full-thickness biopsies of extraperitoneal bladder dome were obtained at the time of laparotomy in six patients with documented detrusor overactivity and in a control group of eight continent patients. Biopsies were frozen in liquid nitrogen, crushed with a frozen mortar and pestle at -80 degrees C, and homogenized in buffer, and the extracts were electrophoresed on 6% polyacrylamide sodium dodecyl sulfate gels and stained with Coomassie blue. The gels were de-stained and then the protein bands were scanned with a densitometer. The mean patient age was 48 years (range, 36-59). Seven patients were Caucasian and seven patients were African American. Detrusor smooth muscle contains a mean of 34% (range, 27-43%) SM1 and 66% (range, 57-73%) SM2 isoforms. There was no difference in isoform composition when patients were compared according to urogynecologic diagnosis or according to race. In detrusor biopsies from women, approximately 34% of myosin is of the SM1 isoform and approximately 66% is of the SM2 isoform. This ratio is relatively constant in the two races studied and unchanged in women with detrusor overactivity. Animal models utilizing outlet obstruction of the bladder to provoke detrusor instability and detrusor hypertrophy are known to alter myosin isoform distribution and may not be appropriate models of detrusor instability in human females.

Adult↗

Urodynamic monitoring during percutaneous sacral nerve neurostimulation in patients with neurogenic detrusor hyperreflexia.

The purpose of this study was to assess urodynamics parameter changes induced by acute sacral neurostimulation (SNS) in spinal cord injury (SCI) patients with neurogenic detrusor hyperreflexia. Fourteen SCI patients with urge incontinence owing to a hyperreflexive bladder were prospectively evaluated. Neurostimulation was performed on the root that obtained the best motor response (10 Hz, 210 microseconds) and amplitude ranges of >0 to 10 V. The urodynamics study was done in the prone position. Four cystometries were recorded before (two), during (one), and 10 minutes after stimulation (one). Maximal bladder capacity (MBC) (volume to first leakage), bladder volume at first uninhibited contraction (BV(1stC)), and maximal detrusor pressure during uninhibited contractions (P(det)max) were compared. Left S3 foramen was the most commonly used (9/14). Mean amplitude of stimulation was 4.5 V (range, 0.5-8.5 V). Statistically significant differences (Mann-Whitney U-test) were found for MBC, BV(1stC), and P(det)max with mean variations of +206.8 mL (+107%, P < 0.001), +151.5 mL (+98%, P < 0.01), and -23.4 cm H2O (-27%, P < 0.05), respectively. Patient gender and SCI level did not reveal any difference. Patients who reached a MBC of up to 400 mL (+278.2 mL, +129%, n = 10) with SNS had statistically significant improvement of BV(1stC) (+210.2 mL, +125%, P < 0.01) and P(det)max (-33.3 cm H2O, -38%, P < 0.01). Acute temporary SNS in SCI patients has a profound effect on key urodynamics parameters. These neurologically impaired patients with detrusor hyperreflexia may be appropriate candidates for implantable SNS.

Adult↗

Neonatal manifestations of Schwartz-Jampel syndrome.

Schwartz-Jampel syndrome generally presents in childhood with short stature, limited joint mobility, masklike facies with blepharophimosis, myotonia, and often muscle hypertrophy. Few cases with neonatal manifestations have been described. A newborn with severe manifestations is reported and the literature is reviewed.

Electromyography↗

Congenital stiff-man syndrome.

A second large family with a dominantly inherited benign disorder resembling the stiff-man syndrome is reported. The infants are markedly hypertonic at birth, but their tone becomes almost normal by 3 years of age. Stiffness reappears at adolescence, often precipitated by sudden movement or cold, but remains mild when compared with the sporadic disorder.

Electromyography↗

GM1 gangliosidosis in adults: clinical and molecular analysis of 16 Japanese patients.

Clinical findings were compared with the results of molecular analysis in 16 Japanese patients from 10 unrelated families with the adult/chronic form of GM1 gangliosidosis. Age of onset ranged from 3 to 30 years. Major clinical manifestations were gait and speech disturbances caused by persistent muscle hypertonia. Dystonic postures and movements, facial grimacing, and parkinsonian manifestations were commonly seen. Cerebellar signs, myoclonus, severe intellectual impairment, dysmorphism, or visceromegaly were not observed. A common single-base substitution, 51Ile(ATC)----Thr(ACC), reported in a previous study of ours, was confirmed in 14 patients by the Bsu36I restriction site analysis; one was a compound heterozygote with another mutation (457Arg[CGA]----Gln[CAA]) and the others were homozygotes of this mutation. Clinically, the compound-heterozygous patient showed more severe neurological manifestations and a more rapid clinical course than those of homozygotes. The homozygotes showed considerable variations in the age of onset and subsequent clinical course. The 51Ile----Thr mutant allele expressed a significant amount of beta-galactosidase activity, whereas the 457Arg----Gln mutant allele expressed extremely low activity in human GM1 gangliosidosis fibroblasts. We conclude that these gene mutations causing different residual enzyme activities are related to the severity of clinical manifestations, but some other genetic or environmental factors contribute to clinical heterogeneity. The Bsu36I restriction site analysis was performed in 7 families and provided clear results for the diagnosis of heterozygotes as well as homozygotes of this specific clinical form of GM1 gangliosidosis. The technique is applicable to prenatal diagnosis and genetic counseling.

Adult↗

Comparison of pharyngoesophageal segment pressure in total laryngectomy patients with and without pharyngeal neurectomy.

BACKGROUND: To compare pharyngoesophageal segment (PES) pressure values in total laryngectomy patients with and without pharyngeal neurectomy (PN) in the early postoperative period. METHODS: Forty-five previously untreated laryngeal carcinoma patients were enrolled into this prospective randomized study. Twenty of them underwent total laryngectomy with PN, and 25 underwent total laryngectomy without PN. PES pressures were measured on the tenth postoperative day with a four-channel catheter. RESULTS: Average PES pressures in patients with and without pharyngeal neurectomy were 12.82 +/- 6.11 mmHg and 17.40 +/-.72 mmHg respectively (p <.05). When compared with the critical point of 20 mmHg that is closely related to voice attainment in the group without pharyngeal neurectomy, 10 (40%) patients had pressure levels greater than 20 mmHg and in the other group only 1 (5%) patient had a pressure level greater than 20 mmHg. The difference between the groups with pressure levels greater than 20 mmHg was found to be statistically significant (p <.05). CONCLUSIONS: Pharyngeal neurectomy results in a statistically significant decrease of PES pressures in total laryngectomy patients.

Adult↗

Muscle tone abnormalities.

Rehabilitation nurses frequently encounter clients with neurological disorders that adversely affect muscle tone. By understanding the physiological etiology of abnormal muscle tone, individual practitioners can design nursing interventions for various care settings that appropriately protect clients from injury and that can help clients and caregivers learn effective techniques for managing muscle tone problems. This article explains muscle tone abnormalities in detail and offers insight into how rehabilitation nurses can play a key role in managing clients' alterations in muscle tone.

Humans↗