Successful intravenous rt-PA thrombolysis for a childhood cardioembolic stroke with Emery-Dreifuss muscular dystrophy.

نویسندگان

  • Koji Tanaka
  • Toshiyuki Uehara
  • Kazuaki Sato
  • Tatsuo Amano
  • Kazuo Minematsu
  • Kazunori Toyoda
چکیده

Case Report A right-handed 12-year-old boy had suffered from pathologically proven EDMD for 7 years and required a wheelchair in daily life due to limb weakness. He had been given warfarin for one year because of chronic atrial fibrillation and suddenly developed leftsided motor weakness and was admitted to our hospital. On admission, he was alert and had a conjugate gaze deviation to the right, dysarthria, and complete left hemiparesis including the face. His National Institute of Health Stroke Scale (NIHSS) score was 15. International Normalized Ratio (INR) was 1.49. Head CT showed indistinctness of the right lenticular nuclei and a right hyIntroduction Patients and carriers of Emery-Dreifuss muscular dystrophy (EDMD), a known inherited muscular disorder [1] , are at high risk of sudden death due to arrhythmias, heart failure, and embolism Published online: December 13, 2011

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Anaesthetic management of a patient with Emery-Dreifuss muscular dystrophy.

Emery-Dreifuss muscular dystrophy is a rare form of muscular dystrophy associated with cardiac implications such as cardiomyopathy and arrhythmias leading to sudden death. We describe the anesthetic management of a patient with Emery-Dreifuss muscular dystrophy who presented for orthopaedic surgery and discuss the disorder and its potential anaesthetic implications.

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Emery dreifuss muscular dystrophy: a clinico-pathological study.

Emery-Dreifuss muscular dystrophy (EDMD) is a rare and genetically heterogeneous disorder. We report two patients with emerin deficient X-linked EDMD and two probable patients with EDMD with typical early contractures, progressive muscle weakness and cardiac involvement. Family history was noted in one case. Muscle biopsy revealed features of dystrophy in all.

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عنوان ژورنال:
  • Cerebrovascular diseases

دوره 33 1  شماره 

صفحات  -

تاریخ انتشار 2012