Anesthesia & Analgesia:
TECHNOLOGY, COMPUTING, AND SIMULATION: SOCIETY FOR TECHNOLOGY IN ANESTHESIA: Case Report
Department of Neurology, Mayo Clinic, Rochester, Minnesota
August 22, 2001.
Address correspondence and reprint requests to Eelco F. M. Wijdicks, MD, Mayo Clinic, W8B, 200 First Street SW, Rochester, MN 55905. Address e-mail to firstname.lastname@example.org.
Respiratory distress is common in terminal stages of multiple sclerosis (MS) because of recurrent aspiration pneumonitis. Acute neuromuscular ventilatory failure occurs relatively infrequently in MS (1,2). Previous reports have described respiratory complications predominantly during relapses resulting either from involvement of descending respiratory pathways in the brainstem or from respiratory motor neurons in the spinal cord (1–4). We report a patient with established MS and acute respiratory failure after 2 wk of increasing difficulties with voluntary respiratory control who made a dramatic improvement after IV methylprednisolone.
A 49-yr-old man with a history of clinically definite relapsing remitting MS for 19 yr and an expanded disability status scale score of 6.0 developed increasing fatigue and increasing difficulties walking during an upper respiratory tract infection (5). During this illness he noticed that he was having increasing difficulties with his voluntary control of breathing while performing his Yoga breathing exercises, specifically he felt that his lungs “were not listening to him” and he could not coordinate the timing of inspiration, holding, and expiration of breath. He denied any shortness of breath at that time. His condition worsened over the next 2 wk, and he became progressively dyspneic in the days before admission. On arrival at his local emergency room, he had a sinus tachycardia of 115 bpm and a systolic blood pressure of 139 mm Hg. He was tachypneic and used his abdominal and accessory muscles to assist in breathing. Arterial blood gas revealed a Po2 of 50 torr, Pco2 of 37 torr, and a pH of 7.46. He was intubated and ventilated. Chest radiograph, electromyography (EMG), and cerebrospinal fluid were normal. A magnetic resonance image of brain and cervical cord revealed multiple small areas of T2 signal abnormality restricted to the periventricular and deep white matter of both hemispheres but a notably increased T2 signal intensity in the upper cervical cord (Fig. 1).
The patient was transferred to our institution. On arrival the patient’s trachea was intubated, but he was alert and communicated by pointing to letters with his finger. Eye movements were ataxic with nystagmus in all directions of gaze. He was quadriparetic with weakness more marked in the legs (Medical Research Council Scale [MRC] 3/5) than the arms (MRC 4/5) (6). He was generally hyperreflexic with bilateral Babinski signs and clonus. He had a subtle sensory level at C2 (suggesting a lesion at or above this level).
Chest computed tomography revealed minimal atelectasis in the lung bases. Pulmonary function testing (PFT) revealed a vital capacity (VC) of 0.8 L, a peak inspiratory pressure (PImax) of −22 cm H2O, peak expiratory pressure of +24 cm H2O, and a tidal volume of 300–350 mL. Sensory and motor nerve conduction studies of the left lower extremity and bilateral phrenic nerve motor conduction studies were normal. Repeat EMG of left upper and lower extremity muscles was normal except for poor activation. Needling of the left diaphragm was normal. IV methylprednisolone (1 g daily for 3 days) was administered (no antibiotic therapy was given). The patient showed dramatic improvement in both limb power and respiratory function within 24 h and was weaned off the ventilator. His PFTs steadily improved, and 7 days after cessation of steroid his oxygen saturation was 96% on room air, VC was 3.5 L, and PImax was −50 cm H2O. He was discharged 3 wk later after inpatient rehabilitation and was ambulatory with one person assisting and he was able to perform his activities of daily living independently.
Most reports of respiratory failure as a result of MS relapse are described in patients with extensive neurological involvement, although there have been reports of respiratory compromise without bulbar or limb paralysis (2,7). Previous studies of pulmonary function in MS patients have shown that respiratory dysfunction is common in MS although respiratory complaints are quite rare (8). There is a positive correlation between pulmonary dysfunction and the stage of neurological disability (8,9). Howard et al. (2) have shown that early recognition and provision of respiratory support in patients with acute respiratory failure attributable to acute bulbar or spinal cord relapse reduces risk of sudden death, may allow recovery of spontaneous ventilation with remission of relapse, and may allow survival for prolonged periods with or without respiratory support.
The localization of the putative lesion in the cervical cord is notable and resulted in a disordered central (initially voluntary and then automatic) control of breathing and quadriplegia. In addition, using pulmonary function tests, we documented neuromuscular failure (resulting from a central upper motor neuron cervical cord lesion rather than a disorder distal to the lower motor neuron) to generate adequate breaths. Improvement was confirmed with serial testing. There is little information available from the literature regarding treatment. The use of IV corticosteroids in shortening the duration of acute MS attacks is well reported. Published data regarding its role in specifically treating the respiratory failure of MS are lacking (1). We suspect that in the majority of cases corticosteroids are rarely used because bulbar dysfunction leads to aspiration pneumonitis and further immunocompromise may actually increase the risk of opportunistic superinfection.
The provision of respiratory support in MS opens difficult and ethical questions and must take into account the patient’s individual wishes. Moreover, weaning from the ventilator after steroid treatment in this patient may not be generalized to all patients with advanced MS. This case not only documents an unusual cause of respiratory distress in MS but also illustrates the potential benefit of IV corticosteroids in reversing severe respiratory failure.
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