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[H-reflex in the extensor digitorum brevis muscle: study in the normal subject and in latent alcoholic neuropathies].

A comparative electromyographic study was carried out in normal subjects (group I) and in alcoholics without clinical evidence of polyneuropathy (group II). -The H reflex of the extensor digitorum brevis muscle (E.D.B.), the electromyogram of EDB, the conduction velocity of the fasted motor fibers of the peroneal nerve (PN), the sensory conduction velocity and amplitude of the evoked potential of the cutaneous fibers of PN, the H reflex of the soleus muscle. -Two kinds of changes were observed in group II: --a significant increase in the latent period of H respones in EDB, in particular the latent period of responses provoked by distal stimulation of PN (+25 PER CENT); --A SIGNIFICANT DECREASE IN THE AMPLITUDe of the sensory potential of the PN(--46 per cent). The other parameters studied in group II did not show any significant difference in comparison with the control group. These results indicate that PN is involved early in a complex fashion in latent alcoholic neuropathies. They confirm that the distinction between axonal neuropathy and segmental demyelination is rarely absolute.

Adult↗

[Adie's syndrome].

The author has investigated patients suffering from disorders of the pupillary reflexes and of the tendon reflexes of the lower limbs which come under the heading of tonic pupil. Forty-five patients were followed up over a period of 15 years. The tendon reflexes of the lower limbs were recorded graphically and showed a very characteristic retardation of the second phase of the patellar reflex. The aetiopathogenesis of Adie's syndrome is discussed. The pathogenesis of Adie's syndrome has so far not been definitely established. We have merely tried to point out a few of the features which permit its early diagnosis and have drawn attention to the basic problem of the pathogenesis of certain neuro-muscular disorders on the basis of secondary considerations involved in this uncommon syndrome.

Adie Syndrome↗

[Clinico-electrophysiologic studies of the spastic syndrome and its neurosurgical treatment in patients with spinal cord lesions].

The authors analyse the results of surgical treatment of spastics in 15 patients with spinal cord lesions. The results of a dynamic, neurological and electrophysiological examinations permit to draw several conclusions on the mechanisms of spasticity and its elimination by surgery. A restoration of reflex activity in the absence of the spastic syndrome is considered by the authors as a favourable result of the operation.

Electromyography↗

[Subclinical forms of spinal disorders in discogenic lumbar radiculitis].

In patients with discogenic lumbar radiculitis with a unilateral affection of L5 or S1 root motor and central responses of the medial group of plantar muscles were recorded. In S1 root pathology the central response consisted in 25% of the cases in repeated antidromal discharges, which testified to an increased excitability of the corresponding alpha-motoneurons. In patients with L5-radiculitis a predominance of the reflex component in the response was typical. Since this phenomenon was lacking in combined L5 and S1 root lesions, it was interpreted as a sign of a decreasing function of the segmental inhibitory interneurons.

Adult↗

[Nine primitive reflexes in patients with acquired immunodeficiency syndrome (AIDS). Results of a case-control study].

This study estimated the frequency and assessed the clinical value of nine primitive reflexes (PR) in 78 AIDS cases, comparing them with 81 matched, seronegative controls. All subjects were evaluated with a standardized neurologic examination that included a Mini-Mental State Exam (MMSE). Fifty-six percent had cognitive impairment and PR. Overall, PR were 2-36 times more frequent in cases. Such association was univariately stronger for the glabellar, snout, and Rossolimo signs. Ninety-two percent of cases had > or = 2 PR vs. 8% of controls, who had up to 2 PR (p < 0.0001; 95 CI: 68% to 100%). We were able isolate or show opportunistic pathogens in CSF of 4 out of 43 cases. This study supports the association of PR to cognitive decline in patients with AIDS. Larger, long term follow-up studies with multivariate analysis in Latin America are needed to identify the PR that can serve as reliable indicators of human immunodeficiency virus type 1 (HIV-1)-associated cognitive/motor complex.

Acquired Immunodeficiency Syndrome↗

[Response to high dose corticosteroids in a girl with bilateral optic neuritis].

INTRODUCTION: Optic neuritis is rare in childhood. Frequently (35-52% of all cases depending on the series) they have, during their clinical course, foci of demyelination leading to the clinical picture of multiple sclerosis (MS). Since 1993, the optic neuritis study group has recommended treatment with high doses of corticosteroids, since this seemed to stop progression, improve long-term results and delay the appearance of MS. The course of our patient was better than we expected. CLINICAL CASE: A 10 year old prepubertal girl complained of progressive loss of vision and slight pain in the right eye for 26 days before admission to hospital. On examination there was obvious papillitis of the right ocular fundus with total loss of the pupillary light reflex, together with consensual hyporeflexia of the left eye. Study of the visual evoked potentials (VEP) showed that there was marked delay of the P-100 wave, and a lower amplitude in the right eye. Magnetic resonance imaging did not show any demyelinated focus. Serological testing for neurotropic viruses was negative. CONCLUSIONS: After the initial phase of intravenous treatment (third day) there was subjective recovery of vision and the pupillary light reflex returned. VEP studies showed marked recovery. Thirty days after treatment was started there was almost complete subjective and VEP recovery. This rapid progress, as compared to that of other paediatric cases published, suggests a mechanism involving decompression of the optic nerve.

Anti-Inflammatory Agents↗

[Miller-Fisher syndrome and cavernous angioma].

INTRODUCTION: The Miller-Fisher syndrome is considered a variant of acute inflammatory demyelinating polyradiculoneuropathy, generally is a benign disorder. CLINICAL CASE: We describe a patient who has had, acutely, ataxia, areflexia and ophthalmoplegia. It has been electrophysiologically diagnosed as Miller-Fisher syndrome. The symptoms appeared after an infectious disease, namely sinusitis. The evoked potential studies suggested lesions at the brain stem and we found a cavernous angioma at MRI. CONCLUSION: We prepared a review of the literature published so far, showing that there is still much controversy about the physiopathology of this syndrome and the importance of the immunologic diagnoses.

Adult↗

Comparative analysis between Duchenne and Becker types muscular dystrophy.

Duchenne and Becker types of muscular dystrophy are usually differentiated according to age of onset and rate of progression criteria which are not sufficient. The aim of this paper was to re-establish the clues for distinguishing Duchenne from Becker types of muscular dystrophy. According to the onset and progression of the disease, one hundred and eleven patients were subdivided into two groups. First group--Becker muscular dystrophy--consisted of 40 patients and second one of 71 patients with Duchenne type of muscular dystrophy. Clinical data confirm some well known differences between Duchenne and Becker muscular dystrophy concerning the age of onset, severity of disease and rate of progression. Electromyographic signs of myopathic changes and spontaneous activity were found in both diseases. Spontaneous activity--bizarre and fibrillation potentials, as well as sharp waves are more common for Duchenne type. The differences between the Becker from Duchenne type of muscular dystrophy can be described on the basis of complex investigations (clinical, electromyographical, histological and biochemical).

Action Potentials↗

[Functional posterior rhizotomy as a surgical treatment for spastic cerebral palsy in childhood].

The author reported a case of spastic cerebral palsy in a 4-year-old boy who underwent functional posterior rhizotomy and were followed up for more than one and a half years after surgery to evaluate the degree of spasticity. The patient's preoperative ADL was highly restricted due to severe spasticity. In the surgery, the bilateral rootlets from L2 to S1 were selectively cut if an abnormal reflex activity was demonstrated by neurophysiological methods. Spasticity markedly decreased postoperatively and alleviated the family's burden for daily care. During the period of follow up, residual spasticity has subsided and the effect in controlling spasticity was long-standing. Functional posterior rhizotomy has been recognized as an established neurosurgical treatment for spastic cerebral palsy in childhood in the North America. However, the procedure is uncommon in Japan. The author outlined here the procedure and its history. Functional posterior rhizotomy is a strong armament for treating spasticity in cerebral palsy. The procedure would greatly benefit patients with spastic cerebral palsy in combination with current treatments.

Cerebral Palsy↗

Vestibular disorders in primary thrombocytosis.

OBJECTIVE: Balance disturbances are some of the most common symptoms among the clinical manifestations of chronic myeloproliferative disorders (MPDs) with a high platelet count, such as essential thrombocythaemia (ET) and polycythaemia vera (PV). In this study, we evaluated the vestibulo-oculomotor and vestibulospinal reflexes in a group of patients suffering from these diseases. DESIGN: Evaluation of balance disturbances. SETTING: Department of Neurosciences, ENT Unit, University of Pisa, Italy. METHOD: In this study, we evaluated 43 patients suffering from ET and PV who underwent otoneurologic examination, based on a study of the vestibulo-oculomotor and vestibulospinal reflexes. RESULTS: There was exclusive central vestibular involvement in 26 cases (60.4%), peripheral and central signs were associated in 8 cases (18.6%), and the involvement was purely peripheral in 1 patient. In six patients (14%), the otoneurologic examination revealed no alterations. CONCLUSIONS: The high percentage of balance disorders in cases of ET and PV probably depends upon disorders of the microcirculation due to platelet dysfunction. We also postulate a full explanation of the involvement of the central vestibular system on the basis of a greater availability of central activated serotonin acting as neuromediator.

Adult↗

Eye movements during torso rotations in labyrinthine-defective subjects.

The aim of this study was to examine whether the chronic loss of vestibular function modifies perceptual and oculomotor responses during torso rotations in darkness. Subjects (4 patients with complete vestibular loss and 7 healthy volunteers) were seated on a rotating chair. Stimuli consisted of sinusoidal chair rotations (+/-30 degrees, 0.1 Hz and 0.011 Hz). We used 2 conditions: space stationary head (neck stimulation) and space stationary head and shoulders (torso stimulation). Horizontal eye deviations and slow component of eye movements were analysed. The results showed that eye movements and perception of head motion in space during neck stimulation were similar to those during torso stimulation both in normal and labyrinthine-defective (LD) subjects. During low-frequency chair rotations (0.011 Hz) all subjects perceived illusory head or head and shoulder rotation in space (as if the lower part of the body was stationary relative to the room) and shifted their gaze in the direction of illusory head rotation. In these conditions there was no significant difference in eye movements between normal and LD subjects. During higher frequency chair rotations (0.1 Hz), LD subjects had significantly larger eye deviations as well as increases in the gain of the slow component of eye movements relative to normals. In these conditions patients mostly perceived illusory head or head and shoulder rotation in space while normal subjects mainly perceived the head as stationary in space. The results indicate that 1) neck and torso rotations can evoke similar ocular responses in LD subjects, 2) the chronic loss of vestibular function modifies the representation of axial body segment motion relative to space.

Aged↗

The significance of the Babinski sign in the newborn-a reappraisal.

A study of the clinical significance and the stimulus methods used to elicit the Babinski sign in normal newborns has been carried out. Although the most frequent response to cutaneous nociceptive stimulation of the lateral sole in neonates was extension of the great toe, a small but significant number of flexor responses were obtained. Consequently, the Babinski sign has limited clinical usefulness as a reflex indicating the presence or absence of neurological disease in the newborn. In reviewing previous studies in the literature, it was apparent that the widely differing percentages of extensor plantars reported in newborns was related directly to differences in the technique of examination.

Humans↗