Cases reported "Ataxia"

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1/9. Conservative facial nerve management in jugular foramen schwannomas.

    OBJECTIVE: Although transposition of the facial nerve is crucial in infiltrative vascular lesions involving the jugular foramen, the objective was to show that a conservative approach to management of the facial nerve is sufficient with jugular foramen neuromas because of their noninfiltrative, less vascular nature and medial location in the jugular foramen. STUDY DESIGN: Retrospective case review. SETTING: Tertiary, private, multiphysician, otologic practice. patients: Sixteen patients with jugular foramen schwannoma (18 procedures) treated between January 1975 and October 1995. The 8 male and 8 female patients ranged in age from 13 to 66 years (mean age 47.7 years). INTERVENTION: One-stage, total jugular foramen neuroma removal without transposition of the facial nerve, using a variety of surgical approaches. MAIN OUTCOME MEASURES: facial nerve transposition (yes or no), House-Brackmann facial nerve grade, lower cranial nerve status, complications. RESULTS: One-stage total tumor removal was accomplished in all the cases. In 13 (72%) of the neuromas, removal was accomplished without facial nerve transposition. Transposition was performed in 2 revision cases in which scar tissue from a previous operation prevented complete control of the carotid artery and safe removal, 2 cases with large tumor extension anteriorly to the petrous apex, and 1 case with extensive involvement of the middle ear. A House-Brackmann facial nerve Grade I or II was obtained in 16 of the 18 procedures, with 1 Grade III and 1 case that remained Grade V, as it was preoperatively. CONCLUSIONS: One-stage, total tumor removal can be achieved with excellent control of the important vascular structures and without transposition of the facial nerve in a majority of jugular foramen schwannomas.
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2/9. thiamine deficiency: a cause of childhood ataxia not to be ignored.

    We report four cases of childhood ataxia with and without encephalopathies which were successfully managed with thiamine supplementation and diagnosed in retrospect as being caused by thiamine deficiency. The clinical presentations were similar to those previously reported amongst adolescents and adults who ingested the larvae of Anaphe venata, a local delicacy among the Ijeshas of south-western nigeria, during the rainy season. This has been called seasonal ataxia syndrome. The cases were of interest because the disorder was not described in children in the previous report, which might indicate a worsening nutritional status in these communities. This report is to alert child health physicians in the developing world to the possibility of a pre-existing thiamine deficiency progressing into ataxic disorders when certain foods are ingested.
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3/9. Temporal arteritis presenting as ataxia and dementia.

    The case reported here illustrates some of the protean manifestations of temporal arteritis. Perhaps, as more cases with atypical manifestations are described, physicians will become more alert to the possibility of this diagnosis. It is now our policy to include temporal arteritis high on the list of differential diagnoses for any neuropsychiatric, visual, or systemic complaint in an elderly patient, even in the absence of typical manifestations. A temporal artery biopsy done relatively early in undiagnosed illness may reveal a very treatable cause. We wish to emphasize the need for early consideration of temporal arteritis in the elderly patient with an elevated ESR and any unexplained neuropsychiatric problem.
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4/9. Leukocytoclastic vasculitis following staphylococcal protein a column immunoadsorption therapy. Two cases and a review of the literature.

    BACKGROUND: Protein A immunoadsorption is a novel therapy for the treatment of diseases mediated by pathogenic autoantibodies. This procedure consists of circulating patients' plasma through a column containing staphylococcal protein a, which binds to the Fc portion of IgG, enabling removal of IgG. Presently, protein A immunoadsorption is used in the treatment of idiopathic thrombocytopenic purpura, but may be more widely used as an immunomodulator in human immunodeficiency virus infection and metastatic carcinoma. OBSERVATIONS: We present two histologically documented cases of leukocytoclastic vasculitis in the setting of protein A immunoadsorption. This potentially severe adverse effect is probably more common than the literature reflects and should be recognized by physicians who are treating patients with protein A column pheresis. CONCLUSIONS: The pathogenesis of protein A therapy-associated leukocytoclastic vasculitis remains unclear. Further study of vasculitis in the setting of protein A column pheresis may lead to modifications of this therapy, resulting in fewer adverse effects. Protein A-associated leukocytoclastic vasculitis may serve as a useful model of the relation of immune complexes and vasculitis.
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5/9. Paraneoplastic sensorimotor neuropathy associated with breast cancer.

    Paraneoplastic sensorimotor neuropathy occurs in association with many different types of cancer. The clinical findings are heterogeneous, and the pathogenesis is unknown. We have encountered 9 women with breast cancer and shared neurological features that suggest a distinct paraneoplastic syndrome. The syndrome is characterized by upper and lower extremity paresthesias and numbness, itching, muscle weakness and cramps, and in some, radicular symptoms and signs. serum and CSF inflammatory changes suggested an immune pathogenesis but none had detectable antibodies directed at nervous system elements. Six patients presented with neuropathy 2 months to 8 years before the discovery of the breast cancer. In 7 the neoplastic disease was localized to the breast and axillary lymph nodes. The neurologic course was chronic in all, and while symptoms were annoying, disability was minimal until late. One improved transiently with plasmapheresis, and three had mild transient improvement with treatment of the cancer. Recognition of this paraneoplastic syndrome may forewarn the physician of an underlying breast malignancy.
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6/9. Reversible neurological deficits in a chronic alcohol abuser: a case report of Wernicke's encephalopathy.

    The abuse of alcohol presents daily challenges for the emergency medicine physician. Wernicke's encephalopathy represents one of the metabolic complications associated with alcoholism. A classic presentation of Wernicke's encephalopathy is reported. The patient presented to the emergency department with the chief complaint of confusion, difficulty ambulating, and visual disturbances. Following administration of intravenous thiamine, the patient's symptoms spontaneously resolved. The pathophysiology, clinical presentation and therapy of this classic disorder are discussed.
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7/9. melaleuca oil poisoning in a 17-month-old.

    Ingestion of significant quantities of melaleuca oil or Australian tea tree oil has been described only once in the medical literature. This report describes a 17-mo-old male who ingested less than 10 ml of the oil and developed ataxia and drowsiness. Emergency physicians, poison control personnel and pediatricians should be aware of potential toxicity from this product.
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8/9. vomiting, ataxia, and altered mental status in an adolescent: late-onset ornithine transcarbamylase deficiency.

    A case of a 13-year-old boy with protracted vomiting, ataxia, and altered mental status, ultimately diagnosed with late-onset ornithine transcarbamylase (OTC) deficiency, is presented. OTC deficiency is the most common urea cycle defect and typically is diagnosed in male infants with irritability, poor feeding, vomiting, lethargy, and often death caused by the effects of hyperammonemia. Late-onset OTC deficiency has been described in patients of all ages. The presenting symptoms in these patients vary but the most common are vomiting and lethargy. The pathophysiology, clinical features, differential diagnosis, and treatment of this disorder are discussed. Because of the risks of serious consequences, early detection and treatment of OTC deficiency and hyperammonemic episodes are mandatory. Emergency physicians caring for children and adults need to be aware of the spectrum of clinical presentations of OTC deficiency, including late-onset disease.
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9/9. Pediatric benzodiazepine ingestion resulting in hospitalization.

    OBJECTIVE: To characterize the clinical findings in children hospitalized for benzodiazepine ingestion. METHOD: Retrospective case series in two urban children's hospitals, with no intervention. suicide attempts and polypharmacy ingestions were excluded. RESULTS: Forty-six children (67% male) with a mean age of 36 months (range 14-127 months) were hospitalized from January 1987 through September 1994. lorazepam was most frequently ingested (13/41 identified drugs, 32%). The most prevalent symptoms were ataxia (87%), lethargy (57%), coma (Glasgow coma score < 15, 35%; Reed coma score > 0, 22%), and respiratory depression (9%). Duration of symptoms was less than 24 hours in 88% of patients. Isolated ataxia occurred in eight patients; in five of these patients, benzodiazepine ingestion was unsuspected by the physicians. Three parents intentionally administered the benzodiazepine to their child. Only 50% of 32 toxicology screens were positive for benzodiazepines. One child required endotracheal intubation. flumazenil administration preceded clinical improvement in two other children. The remaining patients received activated charcoal administration and supportive care. CONCLUSION: Children hospitalized for benzodiazepine overdose occasionally had life-threatening symptoms. ataxia was the most common clinical finding following benzodiazepine ingestion in this series. flumazenil appeared beneficial for the treatment of severe benzodiazepine toxicity in only two patients. Most children recovered from their overdose uneventfully after receiving activated charcoal and supportive care.
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