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Document Details : Title: Intermittent positive airway pressure by nasal mask as a treatment for respiratoy insufficiency in a patient with Charcot-Marie-Tooth disease Author(s): DARQUENNES K, DE JONGHE P, DAEMS D, DE BACKER W, VERBRAECKEN J Journal: Acta Clinica Belgica Volume: 61 Issue: 4 Date: 2006 Pages: 176-181 DOI: 10.2143/ACB.61.4.1002690 Abstract : Charcot-Marie-Tooth disease (CMT) is a slowly progressive hereditary neuropathy characterised by degeneration of motor and sensory peripheral nerves resulting in distal muscle weakness with atrophy and sensory impairment. We report a 35-year-old woman with CMT presenting with respiratory failure due to a pneumonia, sputum impaction and insufficient cough reflex. After recovery, we diagnosed a very severe restrictive lung function disturbance caused by muscle weakness and a possible coexistent unilateral diaphragm paralysis. A very severe REM (Rapid Eye Movement Sleep) related sleep hypopnea syndrome was successfully treated with Nasal Intermittent Positive Pressure Ventilation (NIPPV). |
