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[Progressive dysautonomia in hemangioblastoma in the region of the fourth ventricle].

Tumors of the posterior fossa presenting orthostatic hypotension are rare and only nine cases have been reported so far. The locations of almost all these tumors were near the fourth ventricle and three of them were hemangioblastoma. A case of a tumor of the fourth ventricle showing autonomic disturbances mainly composed of orthostatic hypotension is reported. A 42-year-old male was admitted to the Department of Neurology of Chiba University Hospital on June 25th, 1981 because of three years' history of autonomic disturbances including orthostatic syncope, impotence, urinary disturbance and bowel dysfunction such as vomiting, diarrhea and constipation. He also complained of weight loss and staggering of gait to the left side. On admission, the patient was emaciated being 50 kg in weight and 172 cm in height. Neurological examination revealed hippus of bilateral pupils in light reflex, saccadic eye movement, slightly hypoactive deep tendon reflexes, mild terminal oscillations in bilateral finger-to-nose test, oscillation in the left heel-to-knee test, staggering tendency of gait to the left, slightly impaired tactile and thermal sensations in distal parts of the legs. Autonomic disturbances were showed by orthostatic hypotension (BP 104-50 in supine and 70-40 in sitting position), impotence, weight loss, anorexia, decrease of sweating, spontaneous yawning and loss of sensation of bladder fullness. About 5 weeks after admission, he began to complain of temporal headache and showed impairment of memory, drowsiness, paroxysmal apnea and papilledema.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Abdominal reflexes.

Examination of the superficial abdominal reflexes in patients thought to have idiopathic scoliosis has been considered possibly beneficial for deciding who should have magnetic resonance imaging to rule out syringomyelia. The purpose of this study was to determine what is normal for this examination. Thirty normal adolescents and 35 normal young adults underwent testing of the superficial abdominal reflexes and the patellar and Achilles deep tendon reflexes. Each test was repeated two times. Thirty-nine (60%) subjects had bilaterally equal abdominal reflexes. Nine (14%) subjects had asymmetric reflexes, and seven (11%) subjects had no reflex in at least one quadrant. No subjects had reflexes present on one side and absent on the other. Ten (15%) subjects had absence of the abdominal reflexes in all quadrants. Sixteen (25%) subjects had extinguishing of the reflex in at least one quadrant as the test was repeated. Eleven of these had asymmetric or partially absent reflexes initially. In contrast, the patellar and Achilles reflexes were more consistent. The patellar reflexes were bilaterally equal in 52 (85%), asymmetric in eight (13%), and absent in one (2%). The Achilles reflexes were bilaterally equal in 59 (97%), asymmetric in one (2%), and absent in one (2%). The finding of abdominal reflexes consistently present on one side and consistently absent on the other side did not occur in our normal subjects. This finding might warrant further workup if found in a patient with scoliosis. Other variations in abdominal reflex testing such as asymmetries, absent in some quadrants, and absent in all quadrants are fairly common in normal subjects.

Adolescent↗

The differential effects of fatigue on reflex response timing and amplitude in males and females.

We examined the effects of fatigue on patellar tendon reflex responses in males and females. A spring-loaded reflex hammer elicited a standardized tendon tap with the knee positioned in an isokinetic dynamometer and flexed to 85 degrees. We recorded vastus lateralis activity (SEMG) and knee extension force production at the distal tibia (force transducer). Reflex trials were performed before and after (immediate, 2, 4, and 6 min) an isokinetic fatigue protocol to 50% MVC (90 degrees /s). For each event, pre-motor time (PMT), electromechanical delay (EMD), and total motor time (TMT) were obtained, as well as EMG amplitude (EMG(amp)), time to peak EMG (EMG(tpk)), peak force amplitude (F(amp)), time to peak force (F(tpk)), EMG:force ratio (E:F), and rate of force production (F(rate)=N/ms). TMT increased significantly in females following fatigue, while males showed no change. The increased TMT was due to an increased EMD with fatigue, while PMT was unaffected. EMG(amp) and F(amp) were somewhat diminished in females yet significantly augmented in males following fatigue, likely accounting for the differential changes in EMD noted. Results suggest males and females may respond differently to isokinetic fatigue, with males having a greater capacity to compensate for contraction force failure when responding to mechanical perturbations.

Adult↗

[A case of very slowly progressive, high-cervical spondylotic myelopathy presenting with symmetric deep sensory deficits in the palms].

We report a case of very slowly progressive, high-cervical spondylotic myelopathy with symmetrical deep sensory deficits in the palms. A 76-year-old man began to feel tingling sensation in the second fingers of the bilateral hands 30 years prior to admission. The abnormal sensation spread from the first to the third fingers, and subsequently all over the palms. He noticed intermittent sharp pain in the dermatomes of C4 and 5 bilaterally from his late sixties, and later he developed clumsiness of fine finger movements. In recent years he experienced stiffness in the thighs while walking. On neurological examination, there was a mild sensory deficits in light touch over the bilateral palms, while perception of temperature and pain was normal. Vibration sense was severely and position sense mildly impaired. Discriminative sensation, including graphesthesia, stereognosis, two-point discrimination and texture recognition, was severely impaired over the bilateral palms. On the other hand, all modalities of sensation were normal in the lower limbs. Gross motor dysfunction, such as weakness of limbs, amyotrophy or gait disturbance, was not present. He did not show limb ataxia, but the dexterity of his fingers was severely impaired. Deep tendon reflexes were mildly increased except for the Achilles tendons that were hyporeactive. Plantar responses were flexor bilaterally. Nerve conduction study revealed giant F waves and H reflexes by stimulations of the median and ulnar nerves bilaterally.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗

Changes in excitability of tendon tap and Hoffmann reflexes following voluntary contractions.

The effects on the Hoffmann reflex and tendon tap responses in the human soleus and medial gastrocnemius muscles of a brief conditioning volitional contraction have been compared with the objective of distinguishing contributions from the central nervous system and the periphery. The H-wave on successive trials over a 50 sec period following contraction for the most part demonstrated a depression, especially upon the initial trial. The T-wave responses in the first few trials were greater than those in the control period. Using the assumption that responses to tendon taps had been reduced by the amounts reflected in the excitability curve for H-response, it was deducted that a marked enhancement in T-wave excitability had been present at the initial tendon tap and more moderate facilitation thereafter. This is consistent with the post-contraction discharge and increase in stretch sensitivity of spindle receptors seen after contraction of a muscle in experimental animals. It is concluded that such effects on spindles may occur in man, maybe of such force as to detectably influence dynamic movements.

Adult↗

Electromyography in disorders of muscle tone.

No single clinical electrophysiological test can evaluate disorders of muscle tone. These disorders, symptomatic of a variety of diseases have a multifactorial physiological basis. The several tests used are complimentary each aiming to study different aspects of spinal and supraspinal reflexes which become deranged. The H reflex and F wave (H max/M max and F max/M max ratios) measure motoneuron pool excitability in general. The tendon reflex includes spindle mechanisms bypassed by the H reflex and, with limitations, comparison of H max/M max and T max/M max yields information about the gamma system. Tonic vibration of a tendon inhibits the H reflex from the same muscle. The TVR measures autogenous presynaptic inhibition exerted by the Ia afferents of the muscle. Recurrent inhibition via Renshaw cells is evaluated by studying the effect of collision on the H reflex. Reciprocal inhibition of the Ia afferents can be assessed by measuring H reflex change induced by stimulating Ia afferents from antagonists. Changes in the H reflex recovery cycle measure polysynaptic influences on spinal motoneuron excitability. Cutaneo-muscular (flexor) reflexes measure poly- and oligosynaptic excitatory drive to spinal motoneurons and the blink reflex evaluates the excitatory drive to brainstem motoneurons. Long loop (segmental) responses can be evaluated by limb pertubation using a torque motor or electrical stimulation applied during voluntary muscle contraction. Finally needle electromyography is a more relevant test in several disorders of muscle tone such as the stiff-man syndrome and Isaacs' syndrome.

Action Potentials↗

[A case report of Bickerstaff's brainstem encephalitis with positive anti GQ 1 b, GT 1 a, GM 1 ganglioside antibodies].

Patient was an 18-year-old female student. After she had symptoms of common cold for 3 days, she developed somnolence, diplopia, dysarthria, urinary disturbance and ataxia. On admission neurological examination revealed coma with mydriasis, ophthalmoplegia, ptosis and weakness of the upper limbs. Light reflex, corneal reflex and oculocephalic test were all negative. Deep tendon reflexes were brisk and extensor toe signs were positive bilaterally. She did not have nuchal rigidity. Laboratory test revealed normal cerebrospinal fluid with negative myelin basic protein. Brain MRI, brainstem evoked potentials presented no abnormality. EMG revealed normal conduction velocity and no conduction block. EEG had diffuse theta and delta slowing. Culture of the stool represented no Campylobacter jejuni. At the fifth day of admission consciousness level improved, and other neurological findings disappeared in about 6 weeks. She had anti GQ 1 b, GT 1 a(IgG, IgM) and anti GM 1(IgM) antibodies in the serum. We made a diagnosis of Bickerstaff's brainstem encephalitis from these neurological symptoms and clinical course. The main lesion was present in the brainstem from midbrain to medulla oblongata in the midline. High titer of anti GT 1a antibody may be related to the ophthalmoplegia as noted in Miller Fisher syndrome. As a result of EMG and stool culture, it denied the complication of Guillain-Barré syndrome. We had no proof of the reason of the presentation of anti GM 1 antibody.

Adolescent↗

Fractioned reflex and reaction time in children with developmental coordination disorder.

The patellar tendon reflex (PTR) and simple visual reaction time (RT) were fractionated and compared in 40 subjects with developmental coordination disorder (DCD) and normal coordination (NC) in two age groups. Four equal groups of subjects, 6 years DCD (6DCD), 6 years NC (6NC), 9 years DCD (9DCD), and 9 years NC (9NC) were compared using ANOVA for the main effects of coordination and age. PTR and its components of reflex latency and motor time were not significantly affected by the level of coordination; however, a significant coordination by age interaction (p < .05) revealed an increased motor time in the 6DCD group. RT, premotor time, and motor time were all significantly (p < .05) increased in children with DCD; the increased RT and premotor time support earlier findings, whereas the increased motor time has not previously been found. These findings suggest that the processing of reflexive and volitional responses by children with DCD differs from that of their NC peers.

Analysis of Variance↗

[Ataxic sensory and autonomic neuropathies associated with primary Sjögren's syndrome: a case report].

A 49-year-old woman had xerostomia and foreign body sensation of eye since 27 years old. Since the age of 30 she developed occasional fever attack released by cooling body without medication. Three years later she began to complain difficulty to maintain standing position with closed eyes. Upon admission axillary lymph node swelling, anisocoria and Adie's pupils were evident. She had reddish and atrophic dry tongue. Peripheral reflexes of upper extremities were decreased. Patellar and achilles tendon reflexes were diminished. Perception of pain and temperature were reduced on the right peripheral upper and lateral lower extremities. Deep sensation of four extremities were also decreased. Romberg's sign was positive. She had ataxic gait, orthostatic hypotension, Valsarlva abnormalities and hypohidrosis. Laboratory findings revealed elevated erythrocyte sedimentation rate, high IgG and rheumatoid factor levels, leukopenia, positive anti SS-A/Ro antibody and positive minor salivary gland biopsy. Axillary skin biopsy, CT scan cerebrospinal fluid were normal. Sensory nerve conduction velocity and amplitude were decreased, but motor nerve conduction velocity was normal. Sural nerve biopsy was characterized by loss of large and small myelinated fibers. All findings found in our patient were consistent with those of thirteen patients reported by Griffin et al in 1990 who postulated that T-cell inflammation of the dorsal root ganglion was an extraglandular site of autoimmune attack in Sjögren's syndrome.

Ataxia↗

[Lateral medullary syndrome due to cavernous malformation in the brain stem].

A 59-year-old female was admitted with complaints of vertigo, dysarthria and dysphagia. On neurological examination, right-sided cranial nerve signs included ptosis, Bruns's nystagmus, decreased corneal sensation, diminished facial pain and temperature sensation, decreased palatal excursion and loss of gag reflex. There was no evident motor weakness, but deep tendon reflexes were slightly exaggerated on the left extremities. Coordination testing showed right cerebellar signs. Sensory examination of the remaining parts of the body was quite normal. X-ray CT scan showed multiple high density areas in the right medulla, right pons, right temporal and frontal lobes. T2 weighted MRI demonstrated these lesions as mixed signal intensity areas with marked low signal intensity rim. There were multiple black dots in the bilateral frontal and temporal lobes, cerebellar hemispheres on T2-weighted images. Carotid and vertebral angiograms showed no abnormality. This is the first report of the cavernous malformation presenting as lateral medullary syndrome.

Cavernous Sinus↗

Tendon jerks in Parkinson's disease.

Tendon reflexes were examined in 119 patients with idiopathic parkinsonism (IP) and 40 spouse controls to estimate the type and frequency of any alterations in the reflexes. Forty one of 119 patients and 2 of 40 controls had reflex ratings of 3+ at two or more sites (p < 0.001). There was no correlation of reflex score with the severity of disease or with the cardinal signs of IP. In 21 patients with asymmetric tendon jerks the side with the more active reflexes correlated with the side with greater parkinsonian signs. We conclude that an increase in tendon jerks is a feature of IP. The pathophysiology of this change in reflexes should be investigated further to establish if it is a heretofore overlooked manifestation of basal ganglia dysfunction or a link with other neurodegenerative diseases.

Adult↗

Effects of whole-body vibration on spinal reflexes in man.

Recent studies have described sensory-motor function alterations resulting from vibrations applied to various parts of the body. The present work describes the effects produced at the myotatic loop level by long-term vibration. Hoffmann and Tendon reflexes as well as tendon vibration response were substantially depressed by 18 Hz, +/- 0.25 G vibration applied to the whole body or to the legs of seated human subjects. The reflex inhibition lasted throughout the 15-min vibration period and persisted minutes after stimulus cessation. In contrast, vibration limited to the S's head and trunk showed much weaker effects. This suggests that the vibration acts mainly upon extero- and proprioceptive receptors rather than upon the vestibular organs. The results are discussed in relation to findings derived from experiments involving locally applied short-duration vibration.

Adolescent↗

[Reevaluation of physiological mechanisms generating the stretch reflex: new hypotheses on the physiopathology of spasticity].

INTRODUCTION: Spasticity has been defined as tendon reflex exaggeration and increased muscle reflex contraction in response to its stretch (Lance, 1980). It has been generally admitted that stretch reflex exaggeration was due to myotatic reflex hyperexcitability (Ia fibre-motoneurone synapses). This interpretation has been recently revisited taking into account neurophysiological data obtained in cat and recent data obtained in spastic patients. PROBLEMATICS: Myotatic reflex has been described in decerebrate cat in 1924 by Liddel and Sherrington. In 1943, Lloyd demonstrated that myotatic reflex was due to monosynaptic Ia fibre motoneurone alpha synapses. Almost all the following studies of stretch reflexes were devoted to monosynaptic reflexes both in animal and humans. In fact, the existence of group II fibres coming also from spindles and some restricted experiments performed in man have led to discuss the role of group II fibres in the static component of the stretch reflexes. RESULTS: Recent studies performed in humans have shown that group II fibres play an important role in the development of stretch reflexes in leg muscles and that in spastic hemiplegic patients group II fibres facilitatory effects was deeply increased. DISCUSSION: Results obtained both in humans and animals suggest that group II fibres play also a role in a stretch reflex and led to consider that the pathophysiological modifications of group II effects could contribute to spasticity. CONCLUSION: Modifications of network fed by group II fibres and modifications of muscular fibres intrinsic properties are likely to play a role in the developments of spasticity in humans.

Animals↗

Tone and reflex development before term.

The evolution of tone and reflexes from 25 weeks postmenstrual age (gestational age plus chronologic age) to term in a population of 42 surviving infants is described. The infants were born in 1983 at the Johns Hopkins Hospital, had birth weights less than 1300 g, were examined weekly until neonatal intensive care unit discharge, and did not develop cerebral palsy. Lower-extremity flexor tone was first detectable at 29 weeks post-menstrual age by the popliteal angle and heel to ear maneuvers. Flexor tone, recoil, and hyperreflexia were all noted 2 to 3 weeks earlier in the lower extremities (33 to 35 weeks) than in the upper extremities (35 to 37 weeks). Hip tone (35 to 37 weeks) followed knee flexor tone, but preceded shoulder tone (37 to 38 weeks). Trunk tone on ventral suspension emerged closer to term (36 to 40 weeks), and more than half of infants evaluated at term continued to demonstrate head lag when pulled to sitting position. The emergence of the primitive and pathologic reflexes reflects (both in timing and pattern) the evolution of tone: development of the reflexes in the lower extremities precedes that of those in the upper extremities, and development of the distal reflexes precedes that of the proximal. Maturation of tone, deep tendon reflexes, pathologic reflexes, and primitive reflexes occurs in an orderly, sequential manner, with a well-defined pattern: caudocephalad (lower extremities to upper extremities) and centripetal (distal to proximal).

Child Development↗

[Dynamics of spinal cord motor neuron excitability in epileptic patients following surgical elimination of seizures].

H-reflex testing in epileptics with frequent seizures reveals both an increase and decrease of the activity of the spinal cord motoneurons. A surgical removal of the epileptogenic focus and the disappearance of seizures is followed by a significant elevation of the H-reflex induction threshold, which may be a reflection of the compensatory process. The dynamics of the stimulation EMG data pertains to the H-reflex alone, and is not true in case of the tendon reflexes. In this connection a dystony of the supraspinal regulation of the alpha- and gamma-motoneurons is suggested in epilepsy with a prevailing involvement of the alpha-cells.

Epilepsy↗

Topical anesthetics: effects on the Achilles tendon and H-reflexes. I. Able-bodied subjects.

Achilles tendon reflexes (ATR) and H-reflexes were monitored to determine changes in motor responses after the application of a benzocaine spray or an air spray to normal individuals. There was no change in ATR after application of the spray or the benzocaine, even when the sequence of reflex elicitation was reversed. The only significant change (p less than 0.05) in the H-reflex was an increase in its amplitude from baseline at all posttest times following (1) the placebo application when the H-reflex preceded the ATR (0.264 +/- 0.054V, ten minutes; 0.290 +/- 0.054V, 20 minutes; and 0.322 +/- 0.058V, 30 minutes), and (2) the benzocaine spray when the ATR preceded the H-reflex (0.277 +/- 0.084V, ten minutes; 0.309 +/- 0.152V, 20 minutes; and 0.329 +/- 0.131V, 30 minutes). Within-group comparisons revealed that a significantly greater H-reflex (p less than 0.05) was obtained at 20 and 30 minutes after air spray when H-reflex/ATR test sequence was used, whereas at 30 minutes after application of the benzocaine spray a significantly greater H-reflex was seen using the ATR/H-reflex test sequence. Changes in H-reflex amplitude appear to be as much a function of the reflex testing sequence as whether an air or benzocaine spray is used.

Achilles Tendon↗

Centronuclear myopathy: possible central nervous system origin.

The authors describe a case of myopathy characterized physically by limb weakness, eyelid ptosis, voluntary and reflex paralysis of vertical movements of gaze, and loss of deep tendon reflexes; and morphologically by the abnormal presence of centrally located nuclei in muscle fibers and type 1 fiber hypotrophy. The establishment in this case study of two particular findings--the probably nuclear or supranuclear ophthalmoplegia and the apparently impaired nuclear migration from the center of the muscle fiber toward its periphery--supports the hypothesis of a neuromuscular disorder whose level of severity depends on the degree of difficulty in the nuclear migration itself. This would be linked to a reduction in central nervous system influence.

Adolescent↗