Severe diaphragm weakness in multiple sclerosis.

Abstract

Respiratory failure is only rarely associated with multiple sclerosis.' 2 We describe a patient in whom a diagnosis of demyelinating disease was established and who presented in relapse with acute bilateral diaphragm weakness associated with other signs suggestive of a lesion extending up to the cervicomedullary junction. He developed respiratory failure and required mechanical ventilation.

Common mechanisms of compensatory respiratory plasticity in spinal neurological disorders

“…For example, with brainstem demyelination, patients may exhibit abnormal respiratory rhythm and pattern, and altered sensory processing of chemoafferent respiratory signals (Newsom-Davis, 1974; Rizvi et al, 1974; Mochizuki et al, 1988; Howard et al, 1992; Miller et al, 2003). Lesions in the cervical spinal cord are associated with ventilatory insufficiency, primarily inspiratory and expiratory muscle weakness (Cooper et al, 1985; Kuwahira et al, 1990; Foglio et al, 1994; Buyse et al, 1997; Mutulay et al, 2005; Fry et al, 2007; Karpatkin, 2008). Although mild at first, these respiratory deficits progress in severity, causing significant morbidity and mortality as patients succumb to respiratory or upper airway dysfunction (Redelings et al, 2006; Hirst et al, 2008; Karpatkin, 2008).…”

“…Patients with myelin disorders also exhibit inspiratory and expiratory muscle weakness (Cooper et al, 1985; Foglio et al, 1994; Buyse et al, 1997; Mutulay et al, 2005; Fry et al, 2007; Karpatkin, 2008). Although muscle weakness is likely due to impaired central synaptic drive from the demyelination of central pathways, changes at the NMJ or respiratory muscles themselves may also contribute to, or compensate for these deficits.…”

Respiratory dysfunction in multiple sclerosis: a prospective analysis of 60 patients

“…Respiratory involvement occurring earlier in the course of the disease is mostly due to reversible neuromuscular failure [1][2][3][4][5][6][7][8][9][10].…”

Eight-Year Follow-Up Study of a Patient with Central Alveolar Hypoventilation Treated with Diaphragm Pacing

“…This abnormality had remained unchanged since the patient's first admission at the age of 5 years, when a probable diagnosis of multiple sclerosis was made. Her left hemidiaphragmatic weakness might have been related to multiple sclerosis, which could lead to a generalized weakness of the respiratory muscles and subsequent respiratory failure [5,17].…”

“…However, CT scans of the brain and the brainstem revealed no abnormalities. Chest X-ray showed an elevation of the left hemidiaphragm, which could be attributed to the unilateral diaphragmatic damage caused by multiple sclerosis [5].She was hospitalized at the age of 6 years with drowsiness, cyanosis, and high fever. Arterial blood gas analysis at that time revealed severe hypoventilation (pH 7.11, PO 2 31.2 Torr, PCO 2 120.8 Torr).…”