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Effect of head-out water immersion on neuromuscular function of the plantarflexor muscles.

INTRODUCTION: Electromyograms (EMGs) recorded during head-out immersion have demonstrated reduced signal amplitudes and decreased EMG/force ratios for both maximal and submaximal isometric contractions compared with measurements on dry land. Similar EMG changes have been found in spaceflight. HYPOTHESIS: This study was designed to examine neuromuscular function in the legs during immersion with special reference to reflex sensitivity. METHODS: Recordings were made during plantarflexion in air and water. EMGs were recorded from surface and/or internal electrodes on the soleus (SOL) and medial gastrocnemius (MG) muscles during maximal voluntary contraction (MVC). Hoffman and Achilles tendon reflexes were measured during submaximal plantarflexion (50% MVC) in air and water. RESULTS: During immersion, MVC decreased 13% while EMG amplitude of the plantarflexor muscles decreased by 29% and 35% for SOL and MG, respectively. A similar trend was observed in measurements of Hofman and Achilles tendon reflexes. CONCLUSIONS: Head-out immersion induced a deterioration of neuromuscular function, perhaps by triggering inhibitory mechanisms. The origin of these mechanisms seems to be related mainly to effects of partial weightlessness, but hydrostatic pressure should also be considered.

Adult↗

Peripheral neurotoxicity following high-dose cisplatin with glutathione: clinical and neurophysiological assessment.

The use of high-dose cisplatin is limited by development of severe peripheral neurotoxicity and gradual worsening of renal function. In an ongoing study of high-dose cisplatin glutathione has been employed with the aim of preventing major cisplatin-induced toxicities. Neurotoxicity was examined in detail in 32 patients with ovarian cancer treated with cisplatin (160 mg/m2) and cyclophosphamide (600 mg/m2) every 3-4 weeks for five courses. In addition to serial complete neurological examination, sensory action potentials (SAPs) and motor conduction velocities (MCVs) were also assessed. We confirmed the development of a predominant sensory involvement, characterized by mild distal paresthesias and decrease in vibratory sensibility and in deep tendon reflexes, with a slight reduction of SAPs, observed after three courses of treatment. After five courses, distal paresthesias and disesthesias, decreased proprioception and loss of vibratory sensibility with ataxic signs, absence of deep tendon reflexes, unobtainable SAPs and only moderately reduced MCVs were seen. We did not observe any case of disabling neuropathy. There was a tendency to a more severe involvement of peripheral nerves in patients aged more than fifty. The 3 patients presenting the most serious neuropathy were the oldest in the whole group. Low degree of neurotoxicity observed in this study supports a glutathione protection against cisplatin-induced neurotoxicity. As the urinary excretion of platinum indicated no changes in the renal clearance of cisplatin following repeated courses, the lack of drug accumulation and high plasma peak due to preserved renal function might explain the reduced neurotoxicity observed.

Adult↗

Lower-extremity amputations in NIDDM. 12-yr follow-up study in Pima Indians.

The incidence of lower-extremity amputations was estimated in the Pima Indians of the Gila River Indian Community in Arizona, a population with a high prevalence of non-insulin-dependent diabetes mellitus (NIDDM). Between 1972 and 1984, from a study population of 4399 subjects, lower-extremity amputations were performed on 84 patients, 80 (95%) of whom had NIDDM. Among diabetic subjects, the incidence rate of first lower-extremity amputations was higher in men than in women. Rates increased significantly with increasing duration of diabetes. Presence of medial arterial calcification, retinopathy, or nephropathy; absence of patellar tendon reflexes; impaired great toe vibration-perception threshold; and degree of fasting and 2-h postload hyperglycemia were significant risk factors for amputations. Serum cholesterol concentration, blood pressure, age, and absence of Achilles tendon reflexes were not predictive of amputations. The death rate was greater in diabetic amputees than in diabetic nonamputees of similar age, sex, and duration of diabetes, and a significant increase in cardiovascular deaths was observed in diabetic subjects with amputations. The incidence rate of lower-extremity amputations in diabetic Pima Indians is higher than that reported in other diabetic populations. This may reflect differences in risk or a more complete case ascertainment than was possible in previous studies. If the latter is true, the rate of amputations in diabetic individuals may be higher than has been previously appreciated.

Adolescent↗

A longitudinal study of gait and balance dysfunction in normal older people.

OBJECTIVE: To identify the causes of deteriorating gait and balance in normal older people. METHODS: We measured visual acuity, vestibulo-ocular responses, pure-tone hearing levels, vibration sense, deep tendon reflexes, and Tinetti gait and balance scores in 59 normal older subjects (mean +/- SD age on entry, 78.5 +/- 3.7 years) followed up at yearly examinations (range, 8-10 years). White matter hyperintensities on magnetic resonance imaging taken in mid follow-up were graded qualitatively and quantitatively. RESULTS: For each variable except white matter hyperintensities, we calculated a normalized change per year. There was a significant (P<.05) age-related decrease in vestibulo-ocular reflex gain at 0.05 and 0.20 Hz but not at 0.80 Hz, an increase in pure-tone hearing thresholds (at 1, 2, 4, and 8 kHz), a decrease in vibration sense and deep tendon reflexes in the feet, and a decrease in total Tinetti score. However, only changes in vibration sense in the feet and hearing at 1 kHz were significantly correlated (Spearman rank correlation) with the change in Tinetti score. White matter hyperintensities on magnetic resonance imaging had a higher correlation with the yearly change in Tinetti scores. CONCLUSIONS: This longitudinal study showed age-related decreases in vestibular, visual, auditory, and somatosensation in normal older people, but these changes were only weakly correlated with changes in gait and balance. White matter hyperintensities on magnetic resonance imaging were more highly correlated with changes in gait and balance, but all variables together accounted for only about 29% of the measured change in gait and balance.

Aged↗

[A case of acute disseminated encephalomyelitis (ADEM) associated with demyelinating peripheral neuropathy].

A 41-year-old man had common cold on April 10, 1991. Five days after this illness he developed fever, dysarthria, visual field impairment, gait disturbance and consciousness disturbance followed. On admission in another hospital, cerebrospinal fluid showed 341/mm3 cells (303 mononuclear cells, 33 polynuclear cells, 5 red cells), protein of 238 mg/dl, and sugar of 59 mg/dl. One month later, the neurological examinations revealed flaccid paraparesis, decreased deep tendon reflexes in the lower extremities, bilateral positive Babinski and Chaddock reflexes, positive Beevor sign, stocking type superficial sensory disturbance, diminished vibration sense in the lower extremities and neurogenic bladder. Eight months later, he improved to be able to walk by himself, but decreased Achilles tendon reflexes, bilateral positive Babinski and Chaddock reflexes and paresthesia over both feet were noted neurologically. Nerve conduction study revealed reduced conduction velocities at tibialis anterior nerves, sural nerves and no response on both deep peroneal nerves. A 1 micron thick epon section of a biopsied sural nerve with toluidine blue stain showed a decreased number of myelinated fibers (6394/mm2) with many thinly myelinated fibers and Renaut bodies. There was no edema nor cell infiltration. Electronmicroscopical findings of their ultrathin sections showed many collagen pockets, denervated Schwann cell clustering and a few onion bulb formations. Teased fiber preparations suggested segmental demyelination and remyelination in many fibers. This case could be regarded as a case of ADEM associated with demyelinating peripheral neuropathy, and the possibility of the simultaneous demyelinating process in the central and peripheral nervous system was discussed.

Adult↗

The effects of caloric stimulation of the labyrinth on the soleus motor pool in man.

The effects of caloric stimulation of the labyrinth on the soleus motor pool have been investigated using the Achilles tendon reflex, the H reflex, and the vibratory inhibition of the H reflex. The excitability of the myotatic reflex are is augmented from the beginning of irrigation and remains so until the end of nystagmus. The effects are bilateral and roughly symmetrical. The observed effects are due to simultaneous activation of non-specific, probably reticular, and specific, doubtless vestibular, , mechanisms. The Achilles tendon reflex is more facilitated than the H reflex, and the vibratory inhibition of the H reflex is not modified following irrigation of the ear canal. These results indicate a simultaneous facilitation of alpha and gamma extensor motoneurones but gamma effects clearly predominate. This interpretation is in good agreement with the findings of the animal neurophysiology. The vibratory inhibition argues against a modification of presynaptic inhibition. Before interpreting this fact as different from animal mechanisms, one must take into account the particular experimental conditions realized in man: the stimulus is complex and interactions between various nervous structures are possible.

Achilles Tendon↗

Transcutaneous electrical nerve stimulation versus baclofen in spasticity: clinical and electrophysiologic comparison.

OBJECTIVES: Clinical and electrophysiologic comparison of the efficacy of transcutaneous electrical nerve stimulation (TENS) and oral baclofen in the treatment of spasticity. DESIGN: Patients with spinal cord injury and spasticity were included in the study. Ten patients were assigned to oral baclofen and 11 to TENS groups. For the comparison of H-reflex variables, 20 healthy individuals were allocated to a control group. TENS was applied to the tibial nerve for 15 days at a frequency of 100 Hz. Clinical (spasm frequency scale, painful spasm scale, lower limb Ashworth score, clonus score, deep tendon reflex score, plantar stimulation response score) and electrophysiologic evaluations (H-reflex response at the highest amplitude, latency of maximum H-reflex, and ratio of H-reflex response at the highest amplitude to M response at maximum amplitude) of the lower limb and functional evaluations (functional disability score and FIM) were carried out in baclofen and TENS groups before and after treatment. Posttreatment evaluation was made 24 hrs after the 15th session in the TENS group. In addition, clinical spasticity scores and electrophysiologic variables were measured 15 mins after the first application and 15 mins after the 15th session. RESULTS: Significant improvement was detected in lower limb Ashworth score, spasm frequency scale, deep tendon reflex score, functional disability score, and FIM in the baclofen (P = 0.011, P = 0.014, P = 0.025, P = 0.004, and P = 0.005, respectively) and TENS (P = 0.020, P = 0.014, P = 0.025, P = 0.003, and P = 0.003, respectively) group after treatment. Decrease in H-reflex maximum amplitude was significant in the TENS group (P = 0.026). Most marked improvement was observed in the third evaluation, 15 mins after the 15th session, particularly in lower limb Ashworth score (P = 0.006) and H-reflex maximum amplitude (P = 0.006) in the TENS group. The percentage change in clinical, electrophysiologic, and functional variables caused by baclofen was not different from that caused by repeated applications of TENS in the short- and long-term evaluations (P > 0.05). CONCLUSION: TENS may be recommended as a supplement to medical treatment in the management of spasticity.

Adult↗

Small-fiber polyneuropathy in leprosy without skin changes: study of 17 cases.

Leprosy is one of the most common diseases of the peripheral nerves. In some cases there is only neural involvement without skin changes (neuritic form). The neuropathy has often a distal stocking and glove distribution with thermal and pinprick anesthesia and preservation of proprioception. There is no weakness, the tendon reflexes may be preserved and sometimes the nerves are thickened. We reported 17 patients with a predominantly small-fiber polyneuropathy due to leprosy. All patients had distal temperature and pain anesthesia with different individual variations. The tendon reflexes were normal in seven patients and in eight there was thickening of the nerves. The nerve conduction was normal in three patients. Sural nerve biopsy consisted of: 1) inflammatory infiltrates, 2) vacuolated "foamy" cells, 3) fibrosis of endoneurium, perineurium, and epineurium, 4) partial or total loss of nerve fibers, 5) large number of bacilli. We concluded that in countries where leprosy is frequent, nerve biopsy is an obligatory procedure in patients with predominantly small-fiber polyneuropathy.

Adult↗

Intrafamilial phenotypic variability in Friedreich ataxia associated with a G130V mutation in the FRDA gene.

BACKGROUND: Most patients with Friedreich ataxia (FA) have a GAA trinucleotide repeat expansion in intron 1 of the FA gene (FRDA) on both arms of chromosome 9. However, some patients are compound heterozygotes and harbor a GAA expansion on one allele and a point mutation on the other. Compound heterozygous patients with FA who have a GAA expansion and a G130V mutation have been reported to have an atypical phenotype with a slow disease progression, minimal or no ataxia, or gait spasticity. OBJECTIVE: To describe intrafamilial phenotypic variability in a GAA expansion/G130V mutation compound heterozygous family with FA. SETTING: Tertiary referral university hospital setting. PATIENTS AND METHODS: A 34-year-old man presented to our hospital with a 24-year history of stiff legs and mild unsteadiness of gait. Clinical examination showed a spastic paraparesis with normal to pathologically brisk deep tendon reflexes and mild left upper limb ataxia. His 27-year-old sister presented with a slowly progressive early-onset ataxic syndrome. She had ataxia of gait, mild to severe limb ataxia, and reduced or absent deep tendon reflexes, but no evidence of spasticity on examination. RESULTS: Neurophysiologic investigations showed evidence of a sensory axonal neuropathy, and molecular genetic analysis showed that both siblings were compound heterozygotes with a GAA expansion and a G130V mutation. CONCLUSIONS: This report confirms that compound heterozygous patients with FA who have a GAA expansion and a G130V mutation may present with an ataxic phenotype and that intrafamilial phenotypic variability in these pedigrees can occur. It also emphasizes the importance of performing molecular genetic analysis for the GAA trinucleotide expansion in patients presenting with a spastic paraparesis of undetermined etiology, especially when there is neurophysiologic evidence of a sensory axonal neuropathy.

Adaptor Proteins, Signal Transducing↗

[Subclinical hypothyroidism].

Subclinical hypothyroidism was detected in 5.7% of 853 patients referred for ambulatory examination to a department in Prague. In patients referred with a mild degree of subclinical hypothyroidism with an evaluated level of thyrotropic hormone in serum (TSH) no significant deviations of serum thyroxine and triiodothyronine (T4 and T3) were found nor different values of the Achilles tendon reflex time, as compared with patients with normal thyroid function. In case of a more marked grade of subclinical hypothyroidism with an elevated TSH level without stimulation the patients have on average significantly lower T4 vales, significantly longer Achilles tendon reflex times and preejection stage of left ventricular contraction, and of Rodbard's Q-Kd interval (Q-Kd) than euthyroid patients. The values are, however, still within the reference range for normal function and in individual cases cannot be decisive for establishment of the diagnosis. The authors discuss the differential diagnosis and views on treatment of this disease.

Adult↗

A study of prognostic predictors of supratentorial haematomas.

The prognosis of supratentorial haematomas is based on clinical signs and radiological features. The role of evoked potentials has not been evaluated systematically. In a prospective study of supratentorial haemorrhage a number of clinical (17), radiological (3) and evoked potential (2) parameters were evaluated employing univariate logistic regression analysis in 69 patients and multivariate logistic regression stepdown analysis in 51 patients. The outcome was graded on the basis of the Barthel index (BI) score at 3 months as good (BI > or = 12) or poor (death or BI < 12) recovery. Employing univariate analysis the significant prognostic variables were Glasgow Coma Scale, Canadian Neurological Scale, tendon reflex, associated medical complications, urinary incontinence, ventricular extension of the haematoma and motor evoked potentials. Using multivariate logistic regression analysis the best set of parameters in relation to outcome included Glasgow Coma Scale (P < 0.05), Canadian Neurological Scale (P < 0.05), tendon reflex (P < 0.1), ventricular extent (P < 0.01) and motor evoked potentials (P < 0.05). From this study it is concluded that, in addition to clinical and radiological parameters, motor evoked potentials also have an important role in predicting outcome.

Adolescent↗

Severe pan-sensory neuropathy in leprosy.

The sensory loss which occurs in leprosy is essentially cutaneous, resulting from centripetally ascending infection, the host cellular response and fibrosis, from dermal to certain mixed nerves. The hallmark is pain/temperature and touch/pressure loss. Muscle denervation is a byproduct of mixed nerve involvement. Leprous sensory and motor neuropathy presents a stereotyped picture, with preservation of position sense, noninvolvement of the large girdle muscles, and retained deep tendon reflexes. We report clinical and investigative details of 7 patients (3 males, 4 females) with mild-to-moderate polyneuritic leprosy who manifested severe proprioceptive loss in the upper per limbs; the lower limbs were similarly affected in 4 of them. Tendon reflexes were absent in the ataxic limbs. No other cause was found for the ataxia. Electrophysiological studies confirmed damage to large cutaneous and muscle afferents, and a normal EMG pattern in hip and shoulder muscles. Of great interest was the histology of a lumbar sensory ganglion biopsied in a severely disabled patient. There was extensive neuron loss and degeneration and reactive proliferation of capsular cells ("nodules of Nageotte"), an inflammatory focus of lymphocytes, and no bacilli. This suggests to us that the proprioceptive loss in these patients could well be the result of an unusual "leprous ganglionitis." Further clarification of the mechanism of ganglion degeneration and the frequency of inflammation could come from immunohistology of tissues from African green monkeys with experimental polyneuritic leprosy.

Adult↗

Spasticity after stroke: its occurrence and association with motor impairments and activity limitations.

BACKGROUND AND PURPOSE: There is no consensus concerning the number of patients developing spasticity or the relationship between spasticity and disabilities after acute stroke. The aim of the present study was to describe the extent to which spasticity occurs and is associated with disabilities (motor impairments and activity limitations). METHODS: Ninety-five patients with first-ever stroke were examined initially (mean, 5.4 days) and 3 months after stroke with the Modified Ashworth Scale for spasticity; self-reported muscle stiffness; tendon reflexes; Birgitta Lindmark motor performance; Nine Hole Peg Test for manual dexterity; Rivermead Mobility Index; Get-Up and Go test; and Barthel Index. RESULTS: Of the 95 patients studied, 64 were hemiparetic, 18 were spastic, 6 reported muscle stiffness, and 18 had increased tendon reflexes 3 months after stroke. Patients who were nonspastic (n=77) had statistically significantly better motor and activity scores than spastic patients (n=18). However, the correlations between muscle tone and disability scores were low, and severe disabilities were seen in almost the same number of nonspastic as spastic patients. CONCLUSIONS: Although spasticity seems to contribute to disabilities after stroke, spasticity was present in only 19% of the patients investigated 3 months after stroke. Severe disabilities were seen in almost the same number of nonspastic as spastic patients. These findings indicate that the focus on spasticity in stroke rehabilitation is out of step with its clinical importance. Careful and continual evaluation to establish the cause of the patient's disabilities is essential before a decision is made on the most proper rehabilitation approach.

Activities of Daily Living↗

Neurological manifestations of osteoid osteoma.

The clinical and radiological features of 38 children with osteoid osteomas were analysed retrospectively. Twenty nine patients had lesions of the femur (n = 17) or tibia (n = 12). The mean duration from the onset of symptoms to diagnosis was 13.8 months. In seven patients the history of pain and abnormalities on examination suggested a possible neurological disorder. Fourteen of 29 patients (48%) with femoral or tibial osteomas had localised muscle atrophy, and 10 patients (34%) had diminished or absent deep tendon reflexes in the affected limb. Two patients had painless lesions. Six patients had normal plain radiographs. Delay in the diagnosis of osteoid osteoma may be prevented by the knowledge that pain may be referred or radicular, that the concomitant occurrence of muscle atrophy and depressed deep tendon reflexes are relatively common findings, and that the characteristic radiological features may only appear late in the course of the disease.

Adolescent↗

[Friedrich's ataxia: clinical difficulties and genetic possibilities].

Atypical Friedreich's ataxia was diagnosed by DNA-analysis in 4 patients, 2 men aged 70 and 67 and 2 women aged 32 and 37, who had features that included an onset of ataxia after the age of 25, retained tendon reflexes or hyperreflexia, absence of Babinski's sign, and/or a slowly progressive course. Friedreich's ataxia is the most frequent autosomal recessive cerebellar ataxia. Classical characteristics of the disease are a progressive cerebellar ataxia with an onset before the age of 25, loss of lower extremity tendon reflexes, and bilateral Babinski's sign. However, DNA-diagnostic testing based upon the detection of expanded GAA-repeats in the X25-gene, has shown that the clinical spectrum is broader than was previously assumed.

Adult↗

Neurological examination is possible using telemedicine.

To compare the reliability of neurological examination performed by telemedicine and face to face, a junior doctor examined 23 patients face to face, these examinations being witnessed either by one or by two telemedicine observers using a telemedicine video-link at 384 kbit/s. The gold standard was a face-to-face examination from a panel of six consultant neurologists. Power, deep tendon reflexes, plantar responses, coordination, sensation, eye movements, facial strength, tongue movements, sitting balance and gait were studied. Seventeen patients satisfied the inclusion criteria, and a total of 1,084 matched pairs of observations were made. The reliability of the telemedicine examination ranged from fair to moderate for deep tendon reflexes, coordination and eye movement, to near perfect for plantar responses. Overall, examination by telemedicine compared favourably with face-to-face examination. Telemedicine examination was more sensitive in detecting abnormalities than face-to-face examination for all the neurological tests studied and more specific for all but one (plantar responses). The study showed that neurological examination using telemedicine is at least as good as face-to-face examination performed by a junior doctor.

Humans↗

[Adie's syndrome from a neurological standpoint (author's transl)].

The findings in 22 examinations of tonic pupils, 15 of which presented Adie's syndrome, are described. The neurological and chemical as well as liquor analyses are normal except for the well known tendon reflex anomalies. Neurophysiologically, in 7 of 10 examinations mild disruptions in the sensory nerve conduction velocity were observed. The literature of the last 10 years, where it is of interest from a neurological standpoint and where it relates to the site of damage or to the origin of the tendon reflex abnormalities in Adie's syndrome, is discussed. Adie's syndrome is interpreted as damage to the peripheral nervous system in a polyneuropathic form.

Adie Syndrome↗

Effect of sojourn at 3200-m altitude on spinal reflexes in young adult males.

Studies of the H reflex and the Achilles tendon reflex (ATR) were performed in 12 healthy males under six conditions: 1) sea level control, 2) during the first 1-3 h at altitude while normoxic conditions were maintained, 3-5) at 6, 24 and 72 h of hypoxia, and 6) after Ve acclimatization at altitude in 5-14 d. After altitude acclimatization, the peak-to-peak amplitude of the H reflex and the ATR response was significantly elevated over control. A trend toward decreased amplitudes of both the ATR and the H reflex was observed at 6 h after the onset of hypoxia. There was no significant alteration in latency of the H reflex under any test condition. These findings suggest that sojourn at altitude by sea level residents induces changes in spinal motor reflexes. These changes are most probably mediated by altered high central modulation of the cord.

Acclimatization↗