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[Characteristic neurological signs in patients with cervical disc disease].

In cervical radiculopathy, neurological findings for the upper extremities and the responsible level of the radices have been precisely detailed. However, in cases of cervical spondylotic myelopathy, it is not always easy to determine the responsible level of the cervical spine solely based on neurological findings. To disclose characteristic neurological lesions of the upper extremities, if any, for different locations of disc disease, 49 patients with cervical disc protrusion at single levels, confirmed by magnetic resonance imaging, were analyzed. Thirty-six patients demonstrated long tract signs (LTS) concomitant with segmental signs in the upper extremities, and 13 patients had no LTS. Except for deep tendon reflexes and the extent of dysesthesia in the upper extremities, there were no characteristic neurological findings, such as muscle atrophy, loss of muscle strength, extent of hypalgesia, or hypesthesia, pointing to the responsible compression site. In the 13 patients without LTS, the extent of dysesthesia and diminution of deep tendon reflexes proved to be completely in agreement with results for radiculopathy: 9 had protruded discs at the C56 level and described dysesthesia in the thumb and the index finger with deep tendon reflexes in the biceps and brachioradialis being diminished. The remaining 4 had protruded discs at the C67 level and described dysesthesia in the middle finger. Their deep tendon reflexes in the triceps were diminished. A clear contrast was observed for the patients with LTS. Four out of 36 patients had C34 protruded discs and did not complain of dysesthesia in any digits. Their deep tendon reflexes were exaggerated in all muscles in the upper extremities.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

[Physiopathology of vasovagal syncope: review of the most endorsed theories and recent findings].

Vasovagal syncope is associated with an abnormal reflex and the physiopathological mechanisms of the phenomenon overall are only partially known. Experimental and clinical studies suggest that the main factor which triggers the syncope is the brusque interruption of the alpha-adrenergic tone with marked, sudden peripheral vasodilation. Although documented, vagal hypertony, with consequent bradycardia and asystolia, is only occasional and is almost always a secondary phenomenon. The most commonly suggested cause of vasovagal syncope is a Bezold-Jarish reflex starting from the cardiac receptors in the walls of the ventricle, mediated by the paradoxical activation of afferent vagal fibres. However, recent studies are suggesting that there may be other pathogenetic mechanisms such as the paradoxical activation of the venous-atrial baroceptors and other "extracardiac" vascular receptors. The neuro-endocrine aspect of the vasovagal reaction is very complex and in spite of the many studies carried out on the catecholamine, renal-angiotensive system, arginine-vasopressin, and b-endorphine trends, there are still many points awaiting clarification. The response of the autonomous nervous system linked to age also require further research.

Age Factors↗

[Electrostimulation-induced stapedius reflex. Presentation of a standardized procedure and results of clinical studies].

The electro-tactile elicitation of the stapedius muscle reflex is well known but not used in clinical practice. Despite a complex reflex pathway in the brainstem, correlations between reflex parameters and neurological impairments are still possible. We have developed a method for eliciting the electro-tactile stapedius muscle reflex and automatic analysis of different reflex parameters by using an analog-digital converter and a personal computer-integrated signal analysis system. Clinical investigations were carried out with a group of 55 healthy persons (control group), 13 patients with central facial palsies, 51 patients with multiple sclerosis (MS group) and 24 patients with absent acoustic-stapedius muscle reflexes. Findings demonstrated that the latency between stimulus and beginning of the reflex (L1) was significantly longer in the MS group compared with the control group. The best parameter for dividing the MS group into groups with different disability scores was S, which was the parameter for the increase in the L1 growth curve at different stimulus levels. Reproduction of the parameter S in repeated tests with different locations of the stimulus-electrode was excellent.

Brain Damage, Chronic↗

["Skip lesions" in neurological deficits caused by tethered cord syndrome].

The neurological deficits caused by tethered cord syndrome do not necessarily follow a dermatomal pattern of the affected spinal cord from caudal to cephalic fashion. The authors defined these neurological deficits as "skip lesions", and reviewed the neurological deficits in tethered cord syndrome. Among 29 cases, inconsecutive segmental motor weakness in 3 cases, inconsecutive segmental sensory impairment in 6 cases, positive Babinski reflex in 8 cases, supranuclear neurogenic bladder in 3 cases and lumbago or leg pain in 4 cases were observed and they were thought to be compatible with the neurological deficits called "skip lesions". The basic mechanism of this sign is that the cephalic portion of the spinal cord is functionally impaired during flexion of the lumbosacral spine, while the caudal spinal cord with decreased visco-elasticity dose not elongate. Inconsecutive motor-sensory disturbance, accelerated deep tendon reflexes in the lower extremities, positive Babinski or other pathological reflexes in the lower extremities, uninhibited neurogenic bladder, lumbago and pain in the legs were considered to belong to the category of "skip lesions" and they can be treated by the untethering procedure. The authors concluded that preoperative careful insight into the mechanism of "skip lesions" revealed surgical efficacy for the reversible neurological deficits by the untethering procedure, or the efficacy of decompression of the lipoma or neurolysis around the involved spinal nerve roots.

Humans↗

Blink reflex in Wilson's disease.

Blink reflex and brainstem auditory evoked potential (BAEP) were studied in 10 patients with Wilson's disease (WD) and 10 family members. In blink reflex, R1 and R2 latencies as well as R2 duration were prolonged in the patient group, but not in the family group. In BAEP, latencies of peaks III and V and interpeaks I-III, III-V and I-V were also delayed only in the patient group. These data suggest that blink reflex abnormalities in WD are different from other movement disorders and are mainly due to brainstem dysfunction.

Adolescent↗

The ankle jerk and the tibial H-reflex: a clinical and electrophysiological correlation.

In an attempt to correlate the tibial H-reflex with the ankle jerk, we evaluated 130 reflexes in 65 patients (35 men and 30 women). All the ankle jerks were graded by one of the authors (BK) without the knowledge of the H-reflex results. The maximal H-reflex amplitude, and the maximal H/maximal M amplitudes correlated in a positive fashion with the ankle jerk (r = 0.75 and 0.69 respectively). In contrast, there was no correlation between the H-reflex latency and the ankle jerk (r = -0.11). We conclude that, in most situations, the tibial H-reflex amplitude and the ankle jerk correlate well; their discordance is infrequent (11.5%).

Adult↗

GM-1 ganglioside and motor recovery following human spinal cord injury.

Neurological deficit resulting from spinal cord injury varies widely in severity, ranging from transient abnormal reflexes to lifelong complete absence of motor and sensory function. Medical treatment to aid damaged neurons to recover function has been very limited; therapeutic efforts have focused primarily on initial stabilization of fractures, hemodynamic resuscitation, and then aggressive rehabilitation to enhance the full development of any remaining neuronal activity. Pharmacological treatment to improve restoration of neurological function may be possible, however, as indicated by many animal studies and a few clinical studies with a number of agents. A recent clinical trial of GM-1 ganglioside conducted in patients with spinal cord injuries showed that GM-1 ganglioside enhanced the recovery of neurological function 1 year after major spinal cord injury. In addition to GM-1 ganglioside treatment, these patients received aggressive medical and surgical treatment, as well as methylprednisolone. Neurological recovery was assessed with the Frankel scale and the American Spinal Injury Association (ASIA) motor scale. The findings show enhanced motor recovery compared with placebo in the lower extremities, but not in the upper extremities, over time. This corresponds to improved function of axons passing through the site of injury. Analysis of individual motor groups showed that neurological recovery in the GM-1 ganglioside-treated patients increased in initially paralyzed muscles, enabling them to regain useful motor function; paretic muscles were not found to be strengthened. The study provides the basis for larger studies of GM-1 ganglioside and methylprednisolone, which are currently under way.

Double-Blind Method↗

[Spinal stenosis. A review].

Spinal stenosis is characterized by a broad spectrum of symptoms which are often vague. Therefore, patients suffering from this condition are typically seen by various specialists over a long period of time. Among the symptoms are various degrees of low back pain, neurogenic claudication, parasthesias, muscle weakness and reflex abnormalities. When spinal stenosis is suspected, CT-scanning seems to visualise stenosis well. Because the localisation of the stenosis is often unknown, myelography is still the first choice. CT-scanning can then provide supplementary information regarding the specific location. MR-scanning is not as yet used as a routine procedure, but will in time replace both procedures. Conservative treatment consisting in analgesics and physiotherapy is sufficient in some cases. When conservative treatment is not enough, surgical intervention consisting in decompression by broad laminectomy is performed. Fusion is performed when there are signs of vertebral instability or in young patients. Operational results are often good. Failure is seen following insufficient decompression.

Humans↗

[Immunoadsorption therapy on Fisher's syndrome--removal ability of anti-ganglioside antibodies by tryptophan-linked immunoadsorbent].

There have been several reports describing that immunoadsorption therapy improves the neurologic involvement in Fisher's syndrome (FS). However, few studies have assessed the usefulness of immunoadsorption therapy in view of the removal ability of anti-GQ(1b) antibody, which may function the development of FS. We examined the ability of immunoadsorbents for the anti-GQ(1b) antibody in a patient with FS. A 28-year-old woman developed diplopia and giddiness following a cough, fever and diarrhea. On admission (day 22), neurologic examination showed bilateral moderate oculomotor paralysis and bilateral complete abducens paralysis. She had areflexia, numbness of middle and ring fingers on the left and mild ataxic gait. Her serum had IgG anti-GQ(1b) and anti-GD(1b) antibodies. We examined the absorption of anti-ganglioside antibodies onto a polyvinyl alcohol gel (PVA), a phenylalanine-linked PVA (PH-350) and a tryptophan-linked PVA (TR-350) by the batchwise adsorption method. TR-350 absorbed the autoantibodies, but the removal ability of autoantibody by PVA and PH-350 was not proved. The FS patient was treated with TR-350 (days 29, 34 and 43) and PH-350 (day 39). Anti-GQ(1b) and anti-GD(1b) antibodies were significantly removed by the TR-350, in accordance with the results of the in vivo study. There was little loss of albumin as compared with the immunoglobulins and complements. The numbness and ataxia disappeared on day 44. The diplopia disappeared on day 106. TR-350 would be better than PH-350 in the treatment of FS by immunoadsorption therapy.

Adult↗

[Functional state of the spinal cord in syringomyelia and tuberculous spondylitis with spinal disorders].

In patients with syringomyelia and tuberculous spondilitis accompanied by spinal disturbances (which are in accordance with clinical data considered as models with prevalent signs of disintegration and irritation in the spinal cord) the authors studied separate parameters of the H-reflex and 2 functionally different muscles: m. gastrocnemium and m. soleus. It was possible to show certain differences in the parameters of the H-reflex between the two groups of patients and between the indices of the 2 studied motoneuron pools within each group. The report contains some ideas in relation to the possible causes of these phenomena.

Humans↗

[Immunoadsorption therapy for Fisher's syndrome: analysis of the recovery process of external ophthalmoplegia and the removal ability of anti-GQ1b antibodies].

The beneficial effect of plasma exchange, plasmapheresis or immunoadsorption therapy on Fisher's syndrome, suggested previously, has not been proved since no controlled studies have been conducted. In order to assess the effect of any treatment on Fisher's syndrome, simple and reasonable grading scales for evaluating the major neurological signs are needed. We tried immunoadsorption therapy in four patients with Fisher's syndrome, whose sera had anti-GQ1b antibodies. The clinical course was observed, with assessment of the severity of the major neurological signs based on grading scores; ranging from 0 to 30 for external ophthalmoplegia, from 0 to 10 for ataxia, and from 0 to 16 for areflexia. Tryptophan- or phenylalanine-linked polyvinyl alcohol gel column (TR-350, PH-350) was used as an adsorbent. In a patient who had IgG anti-GQ1b antibody and another patient who had both IgG and IgM anti-GQ1b antibodies, we compared the effectiveness of TR-350 and PH-350 to remove the anti-GQ1b antibody during the therapy. Two patients underwent immunoadsorption therapy at the height of clinical manifestations: in one patient, the therapy was discontinued because of critical hypotension and arrhythmia; the other was given only three sessions of therapy. The other two patients received six or seven sessions during the early recovering stage. All patients recovered without major neurological sequelae. Since ataxia was improved earlier than external ophthalmoplegia, the duration of hospitalization and the time of return to social life depended upon the recovery of external ophthalmoplegia. Analysis of the time course of external ophthalmoplegia score indicated that the improving period and the 50%-recovery day came earlier in the patients who were given a sufficient number of sessions than those who received an insufficient number of sessions. The treatment with TR-350 reduced the IgG anti-GQ1b antibody titer more than that with PH-350, but reduced the IgM anti-GQ1b antibody titer similarly. Immunoadsorption therapy using TR-350 has a probable beneficial effect on Fisher's syndrome even though it is carried out after the height of illness. The evaluation method for the severity of external ophthalmoplegia that we used in the present study is useful for assessing the effect of therapy on Fisher's syndrome.

Adult↗

The effect of levodopa on the habituation of the acoustic-palpebral reflex in Parkinson's disease.

The aim of the present study was to assess the habituation of the blink responses evoked by repetitive auditory stimuli in patients with Parkinson's disease in different clinical states. We studied 28 parkinsonian patients. Eighteen patients without motor fluctuations were studied off and on levodopa, on two different sessions, one week apart. The remaining 10 parkinsonians had the wearing off phenomenon and were studied while being off their medication and while ON and OFF, as determined clinically, after a single dose of levodopa. Twelve age-matched controls were also studied in a single session. Unilateral auditory 1 kHz, 105 dB stimuli were delivered at a frequency of 0.2 Hz. The number of responses (R) from the orbicularis oculi was registered. R values were significantly smaller in controls and in treated parkinsonians than in untreated patients. R was also significantly smaller when ON than at baseline and when OFF in parkinsonians with the wearing off phenomenon. These results suggest a failure of the inhibition of the acoustic-palpebral reflex in Parkinson's disease. The improvement with levodopa and the similarities between the clinical and the electrophysiological changes suggest a dopaminergic role in the inhibition of this brainstem reflex.

Acoustic Stimulation↗

[A case of relapsing Guillain-Barré syndrome following Miller Fisher syndrome].

Miller Fisher syndrome (FS) is thought to be a variant of Gullain-Barré syndrome (GBS), both of which rarely relapse. We report a rare case of GBS that followed FS. A 38-year-old woman had ophthalmoplegia, ataxia and areflexia following an upper respiratory tract infection with a diagnosis of FS. Serum anti-GQ1b IgG antibody was found to be increased, but decreased through immunoadsorption as the neurological symptoms of the patient improved. She became completely asymptomatic three months after the onset of FS. Following a common cold two months later, however, she developed weakness of all four limbs and dysesthesia of hands and feet with albuminocytologic dissociation of cerebrospinal fluid, which was consistent with the diagnosis of GBS. Moreover, serum anti-GQ1b IgG antibody had increased again. Anti-GQ1b IgG antibody frequently becomes positive not only in FS but also GBS with ophthalmoplegia. However, the antibody was positive in this particular patient with GBS, even in the absence of ophthalmoplegia. This case suggests that anti-GQ1b IgG antibody might be a common pathogenesis of both FS and GBS.

Adult↗