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Conversion disorder presenting as hemiplegia and hemianesthesia with loss of neurologic reflexes: a case report.

Conversion disorders can present with a variety of sensorimotor signs and symptoms. We present the case of a young woman who presented with sudden onset hemiplegia and hemianesthesia including unilateral loss of deep tendon reflexes, Babinski reflex, and loss of rectal tone with bladder incontinence. The loss of rectal tone, loss of deep tendon reflexes, and a flaccid Babinski reflex are unusual signs in conversion disorders. A thorough medical workup including x-rays, CT scans, and magnetic resonance imaging revealed no organic causes for the neurologic deficits. Before the onset of symptoms, the patient was emotionally upset during an argument, which may have provided the psychologic stressor necessary for a conversion disorder. The young woman gradually regained full neurologic function over the next 14 hours. Conversion disorders should be considered when the neurologic findings do not correspond to known anatomic or physiologic pathways, although a thorough medical investigation must be performed to search for organic causes.

Adult↗

[Experimental studies on the biomechanical reaction of collagenous fibres during impulse mechanical strain working conditions (author's transl)].

A wide spectrum of stress-strain factors of varying directions and magnitudes are acting on collagenous fibres. Bundles of parallel collagen fibres in tendons and ligaments are mainly subject to tension forces. Single twitches and unfused tetanus of muscles fibres are giving rise to impulse strains on tendon fibres. Tendon reflexes are causing contractions of the muscle and sudden extensions of the corresponding collagenous fibres. In vitro studies were performed on tendon fibres. Tendon reflexes are causing contractions of the muscle and sudden extensions of the corresponding collagenous fibers. In vitro studies were performed on tendon fibre bundles. Systematic impulse extensions were applied as input functions. The force resp. stress impulses were recorded as output functions. When linear extensions with superimposed impulse strains were applied, the force-elongation curve showed a stepwise increase of the force impulse initially. Under consecutive isometric conditions an impulse relaxation curve arises. After partial deloading and subsequently isometric conditions an impulse force recovery function emerges. The output force impulses are dependent on the frequency of the input strain impulses. The effects are called frequency dependent force impulse relaxation and recovery. The findings are briefly discussed in correlation with functional anatomy and muscle physiology.

Animals↗

Maintenance of specificity by sprouting and regenerating peripheral nerves. II. Variability after lesions.

In previous studies we showed that collateral sprouting in cat tibialis anterior (TA) muscle was elicited by selective peripheral spinal nerve section sparing L7. After chronic (3 week) section of L5, L6, S1 and S2 spinal nerves in the present study, two different reflex patterns were observed. In some cats, with presumed prefixation of the lumbosacral plexus, the TA tendon reflex was weakened initially and became stronger beginning 2-3 days postoperative. In other cats, with presumed postfixation of the plexus, the TA tendon reflex was abolished for 7-9 days and then returned. The TA muscles were injected with HRP and labeled motor neurons plotted. In the 'prefixed' first group, the number, location and size of motor neurons projecting to TA through the spared L7 nerve were symmetrical when acute and chronic sides were compared. In the 'postfixed' group (reflex abolished then returned) the acute and chronic sides were asymmetrical: the chronic side displayed a significant increase in number of labeled cells and an increase in the rostocaudal extent of the cell column within the L7 segment. These results are consistent with two types of collateral sprouting: homonymous, in which the sprouts arise from nerves within the muscle, and heteronymous, in which the sprouts arise from nerves in adjacent muscles. In animals with very chronic (up to 2 years) spinal nerve section (L5, L6, S1 and S2) regeneration of the cut nerves was superimposed on the spared L7 innervation. Topography was completely disrupted except in the L7 segment. Thus, there appears to be a difference in specificity of motor neurons for target sites depending upon degree and location of denervation. Homonymous sprouting displays strict specificity, regeneration does not and heteronymous sprouting represents an intermediate form in which cells are recruited from adjacent motor neuron pools in the segment of the spared innervation.

Animals↗

[Polymyositis with rimmed vacuoles in a case of chronic thyroiditis].

We report a 37-year-old woman who developed chronic thyroiditis, and polymyositis with rimmed vacuoles (RVs). She developed paresthesia of glove and stocking type, muscle weakness of the lower extremities, and slowness in motion. On examination, her lower legs were atrophic and weak, more prominent on the right, while proximal muscles in the upper and lower extremities were slightly affected. Deep tendon reflexes of the upper and lower extremities were normal except for the absent Achilles tendon reflexes. Serum CK was high (1,809 IU/l), and laboratory data indicated chronic thyroiditis and hypothyroidism. Muscle CT showed severe fatty changes in the gastrocnemius and soleus muscles, more marked on the right, and slight atrophy of the thigh muscles. Electromyography and nerve conduction velocity study revealed peripheral neuropathy. Muscle biopsy revealed numerous myofibers with RVs, together with myofiber necrosis, phagocytosis and lymphocyte infiltration, consistent with myositis. Nerve biopsy was indicative of slight axonal degeneration. A diagnosis of polymyositis with RVs in combination with chronic thyroiditis was made. Prednisolone combined with levothyroxine markedly improved muscle weakness though atrophy and weakness of the lower legs were barely improved. Distal-dominant asymmetric affection of the leg muscles and many myofibers with RV on the biopsied muscle are atypical and rare findings of polymyositis. Associated chronic thyroiditis and neuropathy may be related to the formation of marked rimmed-vacuolar degeneration of myofibers in the present case.

Adult↗

Risk factors for abruptio placentae and eclampsia: analysis of 445 consecutively managed women with severe preeclampsia and eclampsia.

OBJECTIVE: Our purpose was to characterize the clinical presentation or laboratory variables predictive of either abruptio placentae or eclampsia in women with severe preeclampsia. STUDY DESIGN: Prospective collection of perinatal data from 445 consecutively managed women with severe preeclampsia and eclampsia. Univariate analysis was used to determine which of the independent variables were significantly different between the groups (abruptio placentae vs no abruptio placentae; eclampsia vs no eclampsia). Those with significant differences were then entered into multiple logistic regression analysis to determine those characteristics that were independently related to the outcome variable (abruptio placentae or eclampsia). Before multivariate analysis, the independent variables with an interval scale of measurement were converted to a dichotomous scale, with the receiver-operator characteristic curve used to determine a cutoff level. RESULTS: Univariate analysis revealed statistical significance for the following variables associated with eclampsia: uric acid concentration, > 8.1 mg/dL; proteinuria (>3+); headache; visual symptoms; deep tendon reflexes >3+; serum albumin concentration, <3 mg/dL; and serum creatinine concentration, >1.3 mg/dL. However, with subsequent multivariate analysis, only headache and deep tendon reflexes >3+ remained significant. Univariate analysis for variables associated with abruptio placentae revealed an association between bleeding and platelet count <60,000/mm3. There was no association between abruptio placentae and eclampsia and systolic, diastolic, or mean arterial pressure, quantitative proteinuria, epigastric pain, bleeding, gestational age at delivery, history of preeclampsia, or chronic hypertension. CONCLUSION: Quantitative proteinuria and degree of blood pressure elevation were not predictive of either abruptio placentae or eclampsia, as has previously been suggested. The greatest morbidity associated with eclampsia occurred in women with preterm gestations not receiving medical attention.

Abruptio Placentae↗

Asymmetry of hindlimb muscle activity and cutaneous reflexes after tendon transfers in kittens.

The mechanical actions of various ankle muscles were changed by surgically crossing or transferring the tendons in kittens. After the kittens grew to adults, both hindlimbs were implanted with multiple electromyogram (EMG) recording and cutaneous nerve stimulation electrodes to compare the activity of altered and normal muscles. The tendon transfers showed a remarkable tendency to regrow toward normal or only slightly altered mechanical action. In these animals and in the sham-operation controls, the patterns of muscle activity and reflexes were symmetrical in corresponding muscles of the two legs, although they could differ substantially between animals, particularly for the cutaneous reflexes. Eleven animals had at least some persistent alterations in muscle action. Their cutaneous reflex patterns tended to be asymmetric, in some cases quite markedly. EMG activity during unperturbed locomotion and paw-shaking was more symmetrical, but there were some changes in altered muscles and their synergists. The central pattern generators for locomotion and paw-shaking and particularly for cutaneous reflexes during locomotion appear to be at least partially malleable rather than entirely hardwired. This may provide a tool for studying their development and spinal plasticity in general.

Animals↗

[Multifocal axonal motor neuropathy associated with anti-ganglioside antibodies].

We report a patient with asymmetrical patchy weakness of the limbs, and with autoantibodies against gangliosides GM1, GD1b, asialo GM1. Although electrophysiological studies did not reveal conduction block, treatment with prednisolone resulted in clinical improvement. A 52-year-old man was admitted to Kyoto University Hospital, because of gait disturbance. Neurological examination revealed a patchy distribution of weakness in the limbs. Deep tendon reflex was normal at the right knee, and was depressed at the right biceps. Other deep tendon reflexes were absent. There was a slight decrease in vibratory sensation in the distal portions of the lower extremities. Routine laboratory studies, heavy metal screen, vitamin, cryoglobulin, coproporphyrin and delta-amino levulinic acid in urine, and the protein value of the cerebrospinal fluid were normal. Head and neck MRI, and myelography were normal. Immunofixation electrophoresis showed IgM lambda M-protein in serum. Thin-layer chromatography with immunostaining showed his serum IgM reacted with GM1, GD1b, and asialo GM1. ELISA (Enzyme Linked Immunosorbent Assay) demonstrated high titers of anti GM1, GD1b and low titer of anti asialo GM1. Motor conduction studies showed no demonstrable conduction block, normal conduction velocities and the low amplitudes of CMAP. Sensory conduction studies showed no abnormalities except for slightly decreased amplitude of SNAP in sural nerve. Electromyography showed active denervation in extensor digitorum communis muscle, tibialis anterior muscle and left biceps brachii muscle. Muscle biopsy specimen revealed large and small group atrophy and there was perivascular mononuclear infiltration at one point.(ABSTRACT TRUNCATED AT 250 WORDS)

Autoantibodies↗

Postexercise potentiation of the H-reflex in humans.

UNLABELLED: Post-muscle activation effects on segmental reflexes reveal divergent results dependent upon the manner in which the muscle is activated. Electrically activating triceps surae invokes a potentiation of the Achilles' tendon reflex and the soleus (S) H-reflex termed posttetanic potentiation. In contrast, brief volitional activation produces a subsequent potentiation of tendon reflexes, whereas H-reflexes become depressed. PURPOSE: The present investigation explored the effect of an intense bout of volitional resistance exercise on the S and lateral gastrocnemius (LG) H-reflexes to determine if a potentiation of the H-reflex could be induced with physiological stimuli. METHODS: LG and S H-reflexes were obtained from 10 college age men and women before and after a vigorous bout (eight sets of 10 repetitions) of concentric-eccentric triceps surae exercise. RESULTS: Every subject displayed an initial depression of the LG (P < 0.01) and S H-reflex (P < 0.05) immediately postexercise, consistent with postactivation depression. As a group, there was a significant (P > 0.01) potentiation of the LG H/M ratio following the depression. Five of 10 subjects demonstrated this potentiation, which often lasted 10 min postexercise. The other five subjects displayed a longer and more profound early depression followed by a return to control levels. CONCLUSION: The data suggest that at least two overlapping processes are occurring, a brief depression followed by or superimposed over a longer lasting potentiation. Possible neural mechanisms and implications to strength training are discussed.

Adult↗

Sensory neuropathy in autosomal recessive juvenile parkinsonism (PARK2).

Autosomal recessive juvenile parkinsonism (ARJP/PARK2) is a distinct clinical and genetic entity characterized by early-onset levodopa-responsive parkinsonism, foot dystonia, sleep benefit, and hyperactive tendon reflexes. We report a patient with genetically confirmed ARJP, who showed mild sensory disturbance and diminished deep tendon reflexes in the advanced stage. Nerve conduction studies revealed a sensory dominant neuropathy, which has not been described in ARJP. We suggest that peripheral neuropathy may occur in patients with advanced ARJP due to the loss of parkin protein function, although the function of parkin in the peripheral nervous system remains to be clarified.

Humans↗

[Clinico-electromyographic characteristics of spinal cord and peripheral nerve function in diabetic children].

By means of stimulating electromyography it was shown that children suffering from diabetes had a decrease in segmental motoneurone stimulation. It was manifested in the in the diminishing ratio of maximum reflex responses of the musculus soleus to the motor ones, in the delay of stimulation recovery, an inhibition of the Achilles tendon reflex. The changes mentioned were less pronounced in children with a history of hypoglycemic comas than in the rest children suffering from diabetes. It is suggested that some of EMG readings (the curve of motoneurone stimulation recovery, duration and asymmetry of the Achilles tendon reflex, rate of nervous impulse transmission may be of value for earlier revealing spinal motoneurone and peripheral nerve injuries in diabetes mellitus.

Action Potentials↗

[A case of Hopkins syndrome with onset at puberty].

The patient was a 15-year-old man who developed weakness of left leg 6 days after an acute asthmatic attack. Neurological examination revealed severe muscle weakness and atrophy at L5-S1 level and mild muscle weakness and atrophy at L2-4 level in the left leg. Deep tendon reflexes were normal in the upper limbs and slightly brisk in the lower limbs except for absence of Achilles tendon reflex on the left. His sensation was normal. Needle EMG revealed neurogenic changes in both the left (L2-S1) and right (L2-4) leg muscles. Motor nerve conduction study of the left tibial and peroneal nerves revealed a marked reduction of amplitude and mild reduction of MCV. F-wave was not evoked in either nerves. Sensory nerve conduction study of the left sural nerve was normal. The titers of anti-viral antibodies in the paired sera showed no significant changes in any viruses examined including echovirus, enterovirus, coxsackievirus and poliovirus type 1, 2 and 3. The serum IgE was elevated (1,300 IU/ml) and mite antigen-specific IgE was strongly positive. Spinal cord MRI revealed no abnormality in either thoracic or lumbar spinal cord. This patient was diagnosed as a rare case of Hopkins syndrome with onset at puberty.

Adolescent↗

[Valproic acid relieved marked rigidity in three patients with end-stage parkinsonism].

We applied valproic acid (VPA) on the rigidity of three parkinsonian patients, two with Parkinson disease and one with striatonigral degeneration. They were all at Hoehn and Yahr's stage V and showed marked rigidity. In these patients, effect of L-DOPA had become limited or increasing the dosage of L-DOPA was difficult because of its side effects. Parkinsonian symptoms were assessed by using motor score of Unified Parkinson's Disease Rating Scale. The degree of rigidity in these three patients was markedly decreased with 300-600 mg/day of VPA. The blood level of VPA ranged from 24.8 to 66.5 micrograms/ml, which was relatively low compared with the effective blood level as an anti-epileptic agent. Parkinsonian symptoms other than rigidity, and the increased deep tendon reflexes which were present in the patient with striatonigral degeneration were not affected by VPA. Reduction of L-DOPA intensified rigidity again which had been under control. Trials of VPA on parkinsonism have been reported from two groups (Price PA, et al. 1978; Nutt J, et al. 1979), neither of which has observed any benefit of VPA. The difference of their results and ours seems to depend on the stage of patients; their patients had mild to moderate symptoms, whereas ours were in the end stage with marked rigidity. Since the effect of VPA upon parkinsonism is limited to rigidity, the end-stage patients whose care is difficult due to severe rigidity may obtain the best benefit of VPA. VPA is considered to take effect by activating gamma-aminobutyric acid (GABA) system. Because GABA is a common inhibitory neurotransmitter distributed in the wide areas of the central nervous system, it is difficult to locate the action site of VPA with regard to the amelioration of rigidity. The stretch reflex loop in the spinal cord does not seem to be the action site because no change was noted in deep tendon reflex. GABAergic striatal efferent neurons do not seem to be the sole action site either, because parkinsonian symptoms were not affected except for rigidity. The vestibular nucleus which receives strong GABAergic afferents from cerebellar Purkinje cells is an efficient tonus regulator. Since suppression of the function of the nucleus is known to reduce rigidity, it is at least a candidate for the action site of VPA. But there is no direct evidence for this matter. The exact action site of VPA remains to be elucidated.

Aged↗

The meaning of distal sensory loss and absent ankle reflexes in relation to age: a meta-analysis.

CONTEXT: Polyneuropathy is a common disease and is more prevalent (at least 3 %) in elderly people. However, routine neurological examination of healthy elderly people may show distal sensory loss and absent tendon reflexes, which can obscure the distinction from polyneuropathy. OBJECTIVE: To investigate the relation between age and the prevalence of distal sensory loss, absent tendon reflexes, or muscle weakness, and to ascertain above which age these neurological signs could be considered as normal in ageing. DATA SOURCES: PubMed, Embase, the Cochrane Library, and Current Contents from 1960 until 2004. Reference lists of relevant studies were searched for additional studies, reviews or textbooks. STUDY SELECTION: Studies reporting on neurological signs upon routine neurological examination in generally healthy adult persons were considered for inclusion. Two reviewers independently assessed study eligibility and performed study inclusion. Of 629 studies initially identified, 50 (8 %) met the inclusion criteria. DATA EXTRACTION: Two reviewers independently performed data extraction and assessed study quality based on study design and the rigour by which confounding co-morbidity was excluded. DATA SYNTHESIS: The 50 included studies comprised a total of 9,996 adult persons. Assuming heterogeneity between studies, the prevalence data from different studies were pooled for separate age groups with a random-effects model. In healthy persons older than 60 years the prevalence of absent vibration sense at the big toes (29 % [95 % CI 18 % to 38%]) or ankles (15 % [95 % CI 11 % to 20%]), and absent ankle reflexes (23 % [95 % CI 16 % to 30 %]) was increased. CONCLUSIONS: Self-declared healthy adult persons younger than 60 years do not have neurological signs. After the age of 60 absent vibration sense at the big toes or ankles, and absent ankle reflexes are more prevalent, although the majority does not have these neurological signs. It seems more appropriate to apply different diagnostic criteria for polyneuropathy in adult persons younger and older than 60 years.

Adolescent↗

Linkage to chromosome 13q11-12 of an autosomal recessive cerebellar ataxia in a Tunisian family.

OBJECTIVE: To report the clinical findings and the genetic linkage mapping of an autosomal recessive cerebellar ataxia associated to peripheral neuropathy, showing an early onset cerebellar ataxia with retained tendon reflexes (EOCA) phenotype. BACKGROUND: EOCA is a clinical syndrome delimited by Harding distinguished from Friedreich's ataxia (FA) mainly by the preservation of tendon reflexes. Molecular genetic study of patients with EOCA has demonstrated genetic heterogeneity. A form of autosomal recessive spastic ataxia has been described in Charlevoix Saguenay area in Quebec (ARSACS); the gene responsible has been mapped to chromosome 13q. METHODS: Genetic linkage analysis was performed on 18 members of a large family including 8 of 9 members with EOCA. After exclusion of FA and ataxia with vitamin E deficiency loci as well as loci of autosomal dominant cerebellar ataxias, we performed a linkage analysis to markers of 13q11-12 region. RESULTS: The 9 affected members of this family showed stereotyped clinical features with cerebellar ataxia, pyramidal syndrome, and a variable degree of axonal peripheral neuropathy. Linkage was detected between the disease locus and the microsatellite marker D13S232. Surrounding markers to D13S232 confirmed the linkage and showed the homozygosity of the affected members. CONCLUSION: The family reported here showed the same locus as autosomal recessive spastic ataxia Charlevoix Saguenay disease.

Adolescent↗

Reflex effects of muscle afferents on antagonist studied on single firing motor units in man.

The effects of electrical stimulation of mixed nerves and of the evoked tendon reflex on single firing MUs of antagonists were investigated. The following muscles were studied: m. biceps fem., m. tibialis ant. and m. triceps surae. Under a weak voluntary contraction MU potentials were picked up. Post-stimulus histograms of MU potentials were plotted and the duration of interspike intervals in the post-stimulus period was compared with that in the prestimulus period. Under electrical stimulation of the nerve (Ia afferents) a distinct reciprocal inhibition was observed on MUs of all the 3 investigated muscles. In some cases a weak excitatory drive could be revealed along with the reciprocal inhibition. The tendon (knee) reflex was accompanied by a distinct excitatory effect on MUs of the antagonist. The dependence between the efficiency of the inhibitory volley and the moment within an interspike interval at which the volley arrived was found. The lengthening of an interval was observed only when an inhibitory drive occurred close to its end. The peculiarities of reciprocal inhibition in the firing motoneurone, as well as the possible mechanism of the excitatory effect on the antagonist accompanying the tendon reflex, are discussed.

Electric Stimulation↗

Neuropathy in non-insulin-dependent diabetes mellitus. The significance of symptoms.

OBJECTIVE: Clinical evaluation of significance of symptoms suggestive of neuropathy in non-insulin-dependent diabetics in general practice. DESIGN: Case control study. Interviewer-administered questionnaire. Physical examination by general practitioner researcher. SETTING: Government family practice clinic in Singapore. SUBJECTS: 55 patients with non-insulin-dependent diabetes (NIDDM) aged 35-84 years (51% males), and 53 non-diabetic controls matched for age (+/- 5 years) and gender. MAIN OUTCOME MEASURES: Proportions of subjects with presence of symptoms and physical signs. RESULTS: More patients than controls experienced symptoms suggestive of distal neuropathy (paraesthesiae: 13 vs. 4%), and lower extremity proximal myopathy (weakness climbing stairs: 42 vs. 19%, getting up from squatting position: 31 vs. 11%). No difference in proportions of patients and controls experiencing symptoms suggestive of autonomic neuropathy. More patients than controls had absent tendon reflexes (35 vs. 13%) and weaker hip muscles (24 vs. 6%). Of all who experienced symptoms indicating peripheral neuropathy, 36% of patients had absent tendon reflexes, compared with 8% of controls; and of those who experienced weakness of hip muscles, 31% of patients and 12% of controls had diminished power in the hip muscles. CONCLUSION: Symptoms suggestive of diabetic neuropathy are common and should be asked about in the routine follow-up of patients with diabetes. Up to a third of patients with symptoms will have clinical signs of diabetic neuropathy.

Adult↗

A case of spinal cord injury that occurred in utero.

Spinal cord injury is a rare occurrence that is often difficult to diagnose. This report describes a 2-year-old male with cervicothoracic spinal cord injury. The injury is thought to have occurred in the fetal period because the patient's thorax was markedly hypoplastic at birth and his mother had noticed a decrease in fetal movements for several weeks before the birth. The deep tendon reflex of the upper and lower extremities was absent at birth, as observed in other cases of spinal cord injury. It was noteworthy that deep tendon reflex of the upper and lower extremities remained decreased at 2 years of age despite the injury being located at C(7)-T(1). Based on this case, we propose that spinal cord injuries occurring in utero display different reflex responses from those occurring at birth.

Cervical Vertebrae↗

[Reflexometry: an early prognostic test for assessing postoperative hypothyroidism].

The serum content of thyroxin (T4) and the duration of Achilles reflex was studied in 23 patients with toxic goiter 1-3 months after subtotal thyroidectomy. Concentration of T4 assessed in the serum revealed 9 euthyroid subjects and 14 patients with hypothyrosis. But as the duration of Achilles tendon reflex was within the norm in all 14 hypothyrosis patients reflexometry technique turned to be inconsistent in distinguishing between hypo- and euthyroidism. However the high prognostic value of this method for differential diagnosis of transient or long-standing forms of the disease was shown. Six months after the surgery hypothyrosis disappeared spontaneously, i.e. turned to be transient, in 5 patients whose Achilles tendon reflex parameters were normal, though in all 9 hypothyrosis patients with the reflex parameters higher than 350 m/s clinical and biochemical signs of thyroid under-activity were detected. The data obtained give evidence in favour of reflexometry to be used for early prognostic assessment of post-operative hypothyrosis.

Achilles Tendon↗