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.