Journal of the neurological sciences
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To investigate longitudinal changes in the survival rate of patients with amyotrophic lateral sclerosis in Wakayama Prefecture, Japan, we made a retrospective hospital-based study of 454 patients diagnosed with motor neuron disease (MND) at Wakayama Medical University (WMU) Hospital between 1966 and 2005. Of the 454 patients, 240 who were born and who lived in Wakayama Prefecture were diagnosed with definite or probable ALS during this period, according to the El Escorial criteria. The clinical data of the 240 patients, including sex, birth date, birthplace, address, age at onset, initial symptoms, date when respiratory support was applied (tracheostomy, noninvasive positive pressure ventilation, or mandatory artificial ventilation), and date of death were reviewed retrospectively. ⋯ These findings indicated that younger onset patients with ALS decreased after 1990 in Wakayama Prefecture and this might partly explain the recent decline of ALS incidence in Wakayama Prefecture. The shift of the mean age at onset to older age might be due to exogenous factors, including changes in lifestyle, food, and drinking water in this area. Bulbar palsy onset and age at onset were expected as predictors of the survival rate.
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Case Reports
Cerebrovascular events on awakening, patent foramen ovale and obstructive sleep apnea syndrome.
Patent foramen ovale is a defect in the interatrial septum associated with cryptogenic stroke. The presumed mechanism of cryptogenic stroke due to a patent foramen ovale is the migration of thrombus from the venous side of the circulation to the left atrium with subsequent systematic embolism, called paradoxical embolization. Provacative maneuvers and pre-existing cardiopulmonary disease can cause elevation in right atrial pressure leading to right-to-left shunting through a patent foramen ovale. ⋯ During nocturnal sleep, obstructive sleep apnea can cause right atrium pressure elevation resulting in right-to-left shunting through patent foramen ovale. The possibility of patent foramen ovale and an obstructive sleep apnea syndrome may be considered in patients with cryptogenic strokes-on-awakening. Further studies are needed to support our observation.
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Randomized Controlled Trial
Effect of intravenous methylprednisolone on the number, size and confluence of plaques in relapsing-remitting multiple sclerosis.
The aim of the present study was to evaluate whether intravenous methylprednisolone (IVMP) pulses affect the confluence and enlargement of T2 lesions in the long term in patients with relapsing-remitting (RR) multiple sclerosis (MS). Of 88 RR MS patients, randomly assigned to regular pulses of IVMP (1 g/day for 5 days with an oral prednisone taper) or IVMP on the same dose schedule only for relapses, and followed up without other disease-modifying drug therapy for 5 years, 81 patients completed the trial as planned. Pulsed IVMP was given every 4 months for 3 years, and then every 6 months for the subsequent 2 years. ⋯ Patients who received higher total doses of IVMP showed the smallest changes in confluent T2-LV during the study. This study suggests that treatment with pulses of IVMP may prevent the confluence of T2 lesions, which may in turn contribute to slower progression of disability in the long term. However, pulsed IVMP treatment did not significantly slow down accumulation of overall T2-LV and there were more smaller T2 lesions in the IVMP pulsed arm at the end of the study.
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The prevalence of pain and its impact on outpatients with neuromuscular disease, and their attitude towards the use of analgesics were studied. Seventy-eight outpatients at the university hospital, Tokyo, diagnosed with Parkinson's disease, spinocerebellar degeneration, amyotrophic lateral sclerosis, or multiple sclerosis were asked whether they had experienced pain in the preceding week. The Brief Pain Inventory, Japanese version was used to interview participants reporting pain, about its intensity and interference with activities, the way they dealt with it, attitudes to pain and use of analgesics, and desire for treatment. ⋯ Half of the participants wanted more information on methods for pain relief. Approximately 80% of participants were anxious about adverse reactions of analgesics. These findings suggest that medical staffs should provide appropriate information and educate their patients.
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In acute stroke patients, mild and moderate hypothermia with a body temperature (T core) target of 32 degrees C to 34 degrees C is being tested and has shown some promising results. The feasibility of MH to control of ICP increases in patients with malignant ischemic stroke has been proven, but controversy as to its effectiveness and safety still continues. The most recent results of clinical trials and possible future applications of MH in acute stroke patients are analyzed in this review. DESIGN, METHODS AND MATERIAL: A search in MEDLINE/PubMed was performed. The references of selected articles were investigated and the Cochrane Library searched. Articles including severe, massive, malignant or hemispheric ischemic stroke, induced hypothermia, and animal studies with focal cerebral or brain ischemic models were considered. ⋯ Moderate hypothermia ameliorates ischemic injury by multiple mechanisms. Treatment of acute ischemic stroke patients is feasible, and additional studies, including randomized clinical trials, are warranted.