Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0013362 (dysarthria)
 3,768 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Central alveolar hypoventilation syndrome (CAH), or Ondine's curse, is a very rare disease characterized by dysfunction of respiratory center in the brain stem. Here, we report a case of CAH associated with cerebral infarction. A 59-year-old man developed right facial sensory deficit at age 56. Then, the facial sensory deficit spread to the left side and dysarthria and dysphagia also developed. Since age 58, he often developed respiratory failure and consciousness disturbance. Arterial blood gas analysis revealed alveolar hypoventilation and respiratory acidosis. Disorders of peripheral organs such as lung, airway, thorax and neuromuscular diseases were ruled out. Brain MRI showed cerebral infarction in the brain stem. We diagnosed him as CAH associated with brain stem infarction.
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PMID:[A case of central alveolar hypoventilation syndrome associated with cerebral infarction]. 832 22

A 70-year-old man presented with unilateral lateral medullary infarction, and then died of rapidly progressive respiratory failure within a day. The clinical manifestations were hiccups, hoarseness, dysarthria, nystagmus, left central facial paralysis, paralysis of the left soft palate, dysphagia, decreased superficial sensation over the right face and upper limb, and cerebellar ataxia in the left upper and lower limbs. The arterial blood gas analysis revealed mild hypoventilation. Soon thereafter, an apneic episode occurred during a sleep and advanced to ataxic respiration, and the patient died. Pathologically, there were fresh ischemic infarction localized to the left dorsolateral area of the upper medulla, caused by atherothrombotic occlusion of the left vertebral artery. These foci were in the areas including the medullary reticular formation, the solitary nucleus, the intramedullary fibers of the vagus nerve, and the nucleus ambiguus on the left side. We attributed the fatal acute progressive respiratory impairment in the present case to impairment of the automatic respiratory system (Ondine's curse) rather than the voluntary respiratory system.
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PMID:Rapidly progressive fatal respiratory failure (Ondine's curse) in the lateral medullary syndrome. 1790 48