Bilateral diaphragmatic paralysis

Severe diaphragm weakness producing respiratory failure in a MS patient has been reported. A 41-years old man with a three-year history of relapsing progressive MS acutely developed positional dyspnea, marked sensory loss in a C2-C5 distribution and urinary hesitancy. Esophageal and gastric pressure monitoring revealed no pressure gradient during tidal breathing, confirming the diagnosis of diaphragm weakness. Fifteen days later the patient developed increased limb paresis, tongue fasciculations and greater respiratory compromise, necessitating ventilatory support. This patient shared some features with the present case. Initially, signs indicated an incomplete high cervical cord lesion. Diaphragmatic pathways were affected, but considerable corticospinal tract functions remained.

The literature also contains two reports of central disturbances of respiration in MS referable to the brain stem. In 1977, Boor et al described reversible paralysis of automatic respiration on a background of acute bulbar dysfunction. The patient subsequently died and autopsy revealed a discrete plaque involving dorsomedial and central regions of the lower medulla. In contrast, Noda and Umezald have reported loss of voluntary breathing with preservation of automatic breathing in the setting of acute quadriplegia.

The present case differs substantially from previous reports of respiratory dysfunction in MS. The patient exhibited corticospinal dysfunction but no brain stem abnormalities. Limb paresis did not significantly interfere with ambulation or performance of ADL. The only disabling symptom was dyspnea, which developed insidiously and has now been present for over one year without increase in other neurologic signs. The MRI revealed extensive involvement of the upper cervical spinal cord, confirming the clinical impression. This is the first such case in which MRI is available to corroborate the diagnosis in vivo.

The diagnosis of bilateral diaphragmatic paralysis was made clinically at the bedside. Fluoroscopy alone may be unreliable, but the chest x-ray film findings, the patients spirometry result, inspiratory and expiratory muscle strength as well as the patients buy Viagra and Cialis clear history with the absence of other conditions that can give similar symptoms facilitated confirmation of the diagnosis.

The patients pulmonary symptoms were present for six months before the etiology was diagnosed. Diaphragmatic weakness is frequently overlooked in neurologic disease states. Careful evaluation of pulmonary dysfunction in MS patients may lead to earlier detection and intervention.

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