Cases reported "Reflex, Abnormal"

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1/69. Hyperactive rhizopathy of the vagus nerve and microvascular decompression. Case report.

    A 37-year-old woman underwent microvascular decompression of the superior vestibular nerve for disabling positional vertigo. Immediately following the operation, she noted severe and spontaneous gagging and dysphagia. Multiple magnetic resonance images were obtained but failed to demonstrate a brainstem lesion and attempts at medical management failed. Two years later she underwent exploration of the posterior fossa. At the second operation, the vertebral artery as well as the posterior inferior cerebellar artery were noted to be compressing the vagus nerve. The vessels were mobilized and held away from the nerve with Teflon felt. The patient's symptoms resolved immediately after the second operation and she has remained symptom free. The authors hypothesize that at least one artery was shifted at the time of her first operation, or immediately thereafter, which resulted in vascular compression of the vagus nerve. To the authors' knowledge, this is the first reported case of a hyperactive gagging response treated with microvascular decompression. The case also illustrates the occurrence of a possibly iatrogenic neurovascular compression syndrome.
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2/69. 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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3/69. Hemimasticatory spasm associated with localized scleroderma and facial hemiatrophy.

    OBJECTIVES: To report a case and discuss the mechanism of hemimasticatory spasm. DESIGN: Case report. PATIENT: A 37-year-old woman had a 3-year history of involuntary spasms of the right masseter muscle in association with localized scleroderma and facial hemiatrophy. Electrophysiological studies revealed a normal blink reflex. However, the masseter reflex and silent period were absent on the affected side. Distal latency and compound muscle action potential of the masseter nerve were normal. Needle electromyography demonstrated irregular bursts of motor unit potentials similar to those described in hemifacial spasm. A magnetic resonance imaging scan of the head showed mild hypertrophy of the masseter muscle and atrophy of subcutaneous fatty tissues on the affected side. Local injection of botulinum toxin A into the masseter muscle resolved the patient's symptoms. CONCLUSION: On the basis of clinical and electrophysiological findings, focal demyelination of motor branches of the trigeminal nerve owing to deep tissue changes is suggested as the cause of abnormal excitatory electrical activities resulting in involuntary masticatory movement.
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4/69. Hyperreflexia in a patient with motor axonal guillain-barre syndrome.

    We report a patient who presented after an episode of diarrhoea with ascending, symmetrical weakness without sensory loss, and without sphincter or other autonomic dysfunction. On clinical examination there were no cranial nerve deficits. Hyperreflexia of tendon jerks without other upper motor neurone signs was found. Electrophysiological examination demonstrated acute distal symmetrical motor axonal polyneuropathy. No electrophysiological signs of peripheral nerve demyelination or central nervous system involvement were found. Albuminocytologic dissociation was present in the cerebrospinal fluid. Stool culture and serological tests were inconclusive. Our patient's clinical picture was, apart from hyperreflexia of tendon jerks throughout the disease, characteristic of guillain-barre syndrome. This is the first such patient reported in europe. The aetiology remained unclear. We suggest that selective axonal motor fibre affection, with possible mild pyramidal involvement, caused tendon jerk hyperreflexia.
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5/69. Clonus: an unusual delayed neurological complication in electrical burn injury.

    patients surviving high-voltage electrical injury may have early and delayed sequelae. The most apparent neurological complications are known to be cerebral injury, spinal cord lesions, peripheral-nerve injuries and motor neuropathies. In this study, clonus, which is an unusual late neurological sequela in an electrical burn patient and presented as series of rhythmic, monophasic contractions and relaxations of a group of muscles, is presented. Possible mechanisms of this unusual late sequela and the clinical outcome of the patient are discussed. ankle and patellar clonus was observed in 4 patients and uvular clonus in 1 patient. Clonus started 3 weeks following the injury in our patients and disappeared over a period of 1 yr in 2 patients, and did not disappear in the remaining 2 patients. In the current literature, this is the first report, which presents an unusual sequela following electrical injury. Clonus should also be considered a specific type of neurological sequela following high- or super-voltage electric injury. This may help to inform the patients in the postinjury period and to improve the efficacy of the rehabilitation of the victims.
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6/69. Leptomeningeal signet-ring cell carcinomatosis presenting with ophthalmoplegia, areflexia and ataxia.

    We report a very rare case of occult leptomeningeal carcinomatosis (LC) in whom repeated cytological examination did not show malignant cells in cerebrospinal fluid (CSF) and the primary focus was not discovered by extensive survey. The patient presented with ophthalmoplegia, ataxia and areflexia mimicking miller fisher syndrome (MFS) at the initial stage, and later, the clinical profile and laboratory findings including CSF examination simulated tuberculous meningitis. Postmortem autopsy disclosed metastatic signet-ring cell carcinoma infiltrating into cranial nerves and leptomeninges. We would like to emphasize that LC sometimes shows symptoms and signs similar to MFS or tuberculous meningitis.
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7/69. Chronic sensory neuronopathy associated with human T-cell lymphotropic virus type I infection.

    We described two patients with chronic sensory neuronopathy who had anti-HTLV-I antibody in serum and cerebrospinal fluid but no signs of myelopathy. A sural nerve specimen revealed severe degeneration of myelinated and unmyelinated axons. The second patient had subclinical sjogren's syndrome suggestive of a possible link among human T-cell lymphotropic virus type I (HTLV-I), sjogren's syndrome and sensory neuronopathy, respectively. The broad spectrum of neurologic disorders associated with HTLV-I infection now would include chronic sensory neuronopathy.
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8/69. Presigmoid transpetrosal approach for the treatment of a large trochlear nerve schwannoma--case report.

    A 61-year-old man presented with a rare, large trochlear nerve schwannoma manifesting as left-sided weakness and hypesthesia, bilateral bulbar pareses, and trochlear nerve paresis persisting for 3 months. T1-weighted magnetic resonance imaging with gadolinium revealed an intensely enhanced, well-circumscribed lesion with multicystic formation occupying the prepontine and interpeduncular cisterns and compressing the pons and midbrain with greater extension to the right. The mass was completely removed through the presigmoid transpetrosal approach with preservation of the posterior cerebral, superior cerebellar, and basilar arteries and their branches. Neuroradiological examination after 3 years demonstrated no recurrence. Enlargement of a tumor in the cisternal portion is inclined to involve and/or encase the adjacent major arteries and their branches. The presigmoid transpetrosal approach is one of the best surgical routes to remove a large trochlear nerve schwannoma safely and completely.
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9/69. Treatment of gustatory hyperlacrimation (crocodile tears) with injection of botulinum toxin into the lacrimal gland.

    PURPOSE: To establish the efficacy and safety of botulinum toxin in the treatment of Crocodile Tear Syndrome and record any possible complications. methods: Four patients with unilateral aberrant VII cranial nerve regeneration following an episode of facial paralysis consented to be included in this study after a comprehensive explanation of the procedure and possible complications was given. On average, an injection of 20 units of botulinum toxin type A (Dysport) was given to the affected lacrimal gland. The effect was assessed with a Schirmer's test during taste stimulation. Careful recording of the duration of the effect and the presence of any local or systemic complications was made. RESULTS: All patients reported a partial or complete disappearance of the reflex hyperlacrimation following treatment. Schirmer's tests during taste stimulation documented a significant decrease in tear secretion. The onset of effect of the botulinum toxin was typically 24-48 h after the initial injection and lasted 4-5 months. One patient had a mild increase in his preexisting upper lid ptosis, but no other local or systemic side effects were experienced. CONCLUSIONS: The injection of botulinum toxin type A into the affected lacrimal glands of patients with gusto-lacrimal reflex is a simple, effective and safe treatment.
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10/69. A case of miller fisher syndrome: atypical findings and therapeutic considerations.

    The miller fisher syndrome (MFS) is generally considered to be a disease of the peripheral nervous system. In some cases contemporary involvement of the central nervous system has been described (CNS). We report a case in which it was possible to prove involvement of cranial nerves VII, VIII, IX and X and to exclude CNS involvement. We discuss the possible role of early plasmapheresis treatment on disease evolution.
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