Cases reported "Reflex, Abnormal"

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1/23. Recurrent miller fisher syndrome: clinical and laboratory features and hla antigens.

    In rare cases, miller fisher syndrome (MFS) has been known to recur. However, clinical features of recurrent MFS have not been well analyzed, and the precipitating factors relating to recurrence remain unknown. From 1981 to 1996, we examined four patients with recurrent MFS among 28 Japanese MFS patients. In the four patients, the recurrent episodes occurred after long asymptomatic intervals, ranging from 2.5 to 12.5 years. The clinical and laboratory features of recurrent episodes were similar either to those of the initial episodes or to those of the 24 non-recurrent patients. Of the two patients tested for serum IgG anti-GQ1b antibody, both were positive. Serological HLA typing showed that all recurrent patients were both HLA-Cw3 and -DR2 positive. However, out of 13 non-recurrent patients examined, six had HLA-Cw3, and four had HLA-DR2. The frequency of HLA-DR2 among the recurrent patients was significantly higher than among healthy controls (corrected P = 0.038), and was also higher than among the non-recurrent patients but not significantly. These findings suggest that recurrent MFS is clinically the same as typical MFS and that HLA-DR2 is possibly associated with recurrence.
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2/23. Postural and action myoclonus in patients with parkinsonian type multiple system atrophy.

    patients with a parkinsonian syndrome and features of multisystem atrophy (pMSA) may exhibit abnormal movements of the hands and fingers, which are reported in the literature either as "jerky" tremor or myoclonus. We studied clinically and electrophysiologically these movements in 11 consecutive patients with pMSA. No abnormal movements were observed when the patients were at complete rest, except for a characteristic parkinsonian "pill-rolling" tremor in one patient. Abnormal small-amplitude, nonrhythmic movements involving just one or a few fingers, or more rarely the whole hand, were observed in nine patients when holding a posture or at the beginning of an action. Accelerometric recordings showed small-amplitude irregular oscillations which, contrary to those of patients with tremor, had no predominant peak in the Fast Fourier frequency spectrum analysis. Electromyographic recordings in the forearm and hand muscles showed brief jerks of less than 100 ms duration which were synchronous in antagonist muscles of the forearm and alternated with brief periods of silence. Electrical stimulation of the digital nerves evoked consistent reflex responses in the wrist flexor and extensor muscles at a latency of 55.3 /-4.1 ms (range, 50-63 ms). Routine electroencephalographic (EEG) and somatosensory evoked potentials to median nerve stimulation were normal. back-averaging of the EEG activity time-locked to the jerks was performed in two patients with no evidence of abnormal cortical activity. Two patients had episodes of transient respiratory failure related to pneumonia. This caused a long-lasting enhancement of the abnormal hand and finger movements, which became larger and more widespread, with features of posthypoxic myoclonus. We conclude that the abnormal hand and finger movements of patients with pMSA are a form of postural and action myoclonus, and can be described as mini-polymyoclonus.
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3/23. Bezold-Jarisch-like reflex during Brockenbrough's procedure for radiofrequency catheter ablation of focal left atrial fibrillation: report of two cases.

    Brockenbrough's puncture technique has been widely conducted in the electrophysiologic laboratory. We report here two cases exhibiting a rare complication of this procedure, which arose during the conduct of catheter ablation using radiofrequency energy delivered to the pulmonary vein for the treatment of focal left atrial fibrillation. These cases exhibited marked sinus bradycardia and profound hypotension, suggestive of a Bezold-Jarisch-like reflex, observed immediately after Brockenbrough's procedure but before radiofrequency application. ST elevation in the inferior leads was also observed in spite of normal coronary angiograms. This unanticipated transient complication was treated by intravenous administration of atropine, which had no influence on the ablation procedure or prognosis. This is speculated to be attributable to the elevation of vagal tone caused by the mechanical effects of transseptal puncture on the interatrial vagal network.
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4/23. nifedipine pretreatment for prevention of autonomic hyperreflexia during anesthesia-free extracorporeal shock wave lithotripsy.

    nifedipine, a calcium channel blocker, has been used to treat autonomic hyperreflexia in quadriplegics undergoing invasive urological procedures. We present a case of autonomic hyperreflexia triggered by extracorporeal shock wave lithotripsy in which nifedipine was used successfully to treat the hypertensive crisis on 1 occasion and subsequently it was used prophylactically to prevent the crisis in the same patient.
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keywords = wave
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5/23. Intradural disc herniation and epidural gas: something more than a casual association?

    STUDY DESIGN: The case of a patient with an intradural disc herniation associated with gas in the spinal canal is presented. OBJECTIVE: To alert spine surgeons to this potential association. SUMMARY OF BACKGROUND DATA: This association was described previously in five patients, a fact that seems surprising given the relative rarity of both intraspinal gas and intradural herniations. methods: The case is presented of a female patient with lumbosciatic pain who developed an incomplete cauda equina syndrome. An asymmetric discopathy of the L2-L3 space and a gas bubble with disc material within the spinal canal was noticed in the radiologic explorations. The literature and the authors' experience are reviewed with the aim of confirming the frequency of intradural herniation in association with gas in the spinal canal. RESULTS: A laminoarthrectomy of the involved space was performed followed by direct intradural examination, which revealed a disc fragment that was excised. An instrumented L2-L3 arthrodesis was performed. Postoperative evolution was satisfactory. To date, the authors have found this association in 2% of the patients with intraspinal gas. CONCLUSION: The potential presence of an intradural disc herniation must always be considered when performing an open discectomy on a patient whose CT scan study shows the presence of epidural gas. This association is particularly striking given the relative rarity of intradural herniations and intraspinal gas. In the event that no clear disc herniation was found, an intradural examination may be indicated to justify clinical signs and symptoms or previous radiologic studies.
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6/23. Periodic nocturnal myoclonus in a patient with hyperexplexia (startle disease).

    The periodic nocturnal myoclonus of a patient with hyperexplexia has been studied. Evidence has been given that the jerks are spontaneous arousal reactions. The temporal characteristics of the jerks have been analysed. The jerks appear to be correlated with the respiratory rhythm and the data suggest a correlation of the jerks with circulatory and respiratory higher order waves. The results are discussed with relation to the literature concerning spontaneous sleep jerks.
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7/23. Long-term follow-up of percutaneous radiofrequency sacral rhizotomy.

    A series of 7 patients undergoing percutaneous radiofrequency sacral rhizotomy to change a hyperreflexic to an areflexic bladder was reported in 1978. A patient from this original series was seen for treatment nine years later with successful preservation of good vesical capacity enabling him to use Crede maneuver and intermittent catheterization to evacuate his urine. In addition, there has been no recurrence of symptoms of autonomic dysreflexia during that period.
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8/23. The localization of the lesion in patients with acute ophthalmoplegia, ataxia and areflexia (miller fisher syndrome). A serial multimodal neurophysiological study.

    Results of comprehensive serial neurophysiological tests from onset to full recovery in 3 patients with the miller fisher syndrome (acute ophthalmoplegia, ataxia and areflexia) are presented. These included EMG and nerve conduction, late response (H and F wave) and direct facial motor and blink reflex studies, computerized motor unit number estimation, automated quantitative sensory threshold measurements, quantitative pupillometric and pupillopharmacological studies and multimodality evoked potential (VEP, SEP and BAEP) and EEG recordings. The results provided unequivocal evidence of peripheral nerve dysfunction. Improvement of the peripheral neurophysiological parameters accompanied or followed clinical recovery in all 3 patients. No abnormality in the CNS pathways investigated by these tests was found. The findings support the conclusion that this syndrome is to be included within the spectrum of acute inflammatory polyneuropathy. The value of serial measurements in detecting milder peripheral nerve lesions is emphasized.
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ranking = 7.0858741825428
keywords = wave
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9/23. The EEG and clinical evolution in Creutzfeldt-Jacob disease.

    A total of nine EEGs were recorded serially from a typical, pathologically verified case of Creutzfeldt-Jacob disease (CJD). Typical periodic sharp waves (PSW) showed a progressive increase in duration, but the interval of PSW stayed fairly constant during the course of illness. Early in the illness there were PSW in approximately one-quarter of the records and this reached one hundred percent when clinical CJD was fully developed. diagnosis of CJD on the basis of the EEG alone is difficult in the early phase of the disease due to the short duration of the PSW.
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ranking = 7.0858741825428
keywords = wave
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10/23. Urge incontinence can be a disease of bladder sensors.

    In many cases of urge incontinence the pathophysiology is unclear. Detrusor instability, detrusor hyperreflexia, urethral instability and poor sensory perception all have been suggested as requirements for urge incontinence. Sensory perception, as proposed recently, may be mediated by 2 bladder sensors. The first sensor, located at the trigone and posterior urethra, is sensitive to small changes in pressure and may function as an early warning system of bladder filling. In the diagnostic evaluation of urge incontinence 5 patients had a triad of absence of the first sensor, an intact second sensor, that is the urge to void when the bladder is stretched with fluid, and normal spontaneous detrusor contraction. Based upon these findings it is suggested that some instances of urge incontinence result when the early warning system fails and detrusor contraction occurs just shortly after the second sensor is stimulated. Detrusor instability was identified in 3 of the 5 patients with urge incontinence, and when present it was associated with urinary frequency. Thus, 2 forms of urge incontinence may be associated with loss of the first sensor: 1 with and the other without associated frequency, with the difference being the presence or absence of detrusor instability.
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