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hospital for observation of respiratory, autonomic, and motor function The comments that follow are applicable to most other forms of acute and subacute neuromuscular respiratory failure such as myasthenia gravis Measurement of maximal inspiratory force and expiratory vital capacity suf ces for the bedside estimation of the adequacy of diaphragmatic strength and respiratory function The trend of these measurements are a guide to the likelihood of respiratory failure As in poliomyelitis, the strength of the neck muscles and trapezii, which share the same segmental innervation as the diaphragm, tends to parallel diaphragmatic strength A rough estimate of breathing capacity may be obtained by having the patient count quickly on one deep breath The ability to reach 20 generally corresponds to a vital capacity of greater than 15 L If a downward trend in these measurements is recognized and the vital capacity diminishes to below about 10 mL/kg, endotracheal intubation and mechanical ventilation are considered It should be noted that a fairly severe degree of impaired ventilation may occur before the rst sign of dyspnea appears and even before there is any elevation of arterial carbon dioxide content Incipient respiratory failure generally coincides with tachypnea and a slight decrease in arterial oxygen tension (PO2 less than 85 mmHg) re ecting pulmonary atelectasis If respiratory failure arises gradually, over days or longer, there is slight tachycardia, diaphoresis, restlessness, and tachypnea Attempts to forestall the use of a positive-pressure ventilator by negative-pressure cuirass-type devices have been unsatisfactory in our experience Patients with oropharyngeal weakness require intubation even earlier in order to prevent aspiration, but mechanical ventilation is not always necessary at the same time These treatments are so demanding that the patient should be admitted to an intensive care unit staffed by personnel skilled in maintaining ventilation and airway patency The other major aspects of the treatment in severely affected patients involve the management of cardiovascular autonomic instability and the prevention of the many general medical problems that attend any immobilizing critical illness Hypotension from dysautonomia, which occurs in about 10 percent of severely affected patients, is treated by intravenous volume infusion and by the use of vasopressor agents for brief periods Extremes of hypertension are managed by short-acting and titratable antihypertensive medications, such as intravenous labetalol The choice and dosing of an antihypertensive drug is important, since these episodes may be rapidly succeeded by precipitous declines in pressure It is not clear if severe autonomic changes can be anticipated but various provocative maneuvers such as ocular pressure to elicit heart block are used in some units; we generally omit them Prevention of electrolyte imbalance, gastrointestinal hemorrhage, and particularly pulmonary embolism in patients who are bedbound (by the use of subcutaneous heparin or pneumatic compression boots), all require careful attention Adynamic ileus is a problem in some cases, manifest rst by abdominal pain coincident with nasogastric tube feeding and by bloating; it may lead to bowel perforation even if feeding is discontinued As mentioned, a number of patients become hyponatremic, usually from SIADH but occasionally from a natriuresis, and the drop in sodium may be produced or exaggerated by positive-pressure mechanical ventilation The distinction between the two conditions that cause hyponatremia determines the course of treatment: uid restriction in the case of SIADH or salt replacement in the case of sodium loss Many patients have bizarre waking dreams or hallucinations.

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