Respiratory muscle weakness in the Lambert-Eaton myasthenic syndrome.

Respiratory muscle function was assessed in six patients with the Lambert-Eaton myasthenic syndrome. Five had histologically proved small cell carcinoma of the lung; the sixth later developed metastases from an unknown primary site. Two patients had ventilatory failure, one without respiratory symptoms; another, who had emphysema, had dyspnoea and orthopnoea. The remaining three patients had no respiratory symptoms. Four patients had limb muscle weakness as judged by the maximal voluntary contraction of the quadriceps muscle (range for all subjects 32-100% predicted). Transdiaphragmatic pressure (Pdi) was measured during a maximal unoccluded sniff (Pdi: sniff), a maximal sustained inspiratory effort against a closed airway (Pdi: Pimax), and phrenic nerve stimulation (Pdi: twitch). Mild to moderate diaphragmatic weakness was present in all six patients in proportion to the degree of leg weakness (Pdi: sniff 30-64% predicted; r = 0.6; Pdi:Pimax 6-69% predicted, r = 0.8); this was associated with very low or absent Pdi:twitch during phrenic nerve stimulation. Four patients had weakness of the expiratory muscles. Improvement in muscle strength was documented in two patients after tumour chemotherapy and specific treatment with 3,4-diaminopyridine and prednisolone; one patient was still alive five years from first diagnosis. It is concluded that the respiratory muscles may be implicated in this condition more often than has previously been recognised. As the lack of mobility may cause respiratory symptoms to be minimised, the presence of respiratory muscle weakness may remain undiagnosed unless formal measurement of respiratory muscle function is made.

The value of sniff esophageal pressures in the assessment of global inspiratory muscle strength.

Esophageal pressure generated during a maximal sniff (sniff Pes) was compared with mouth pressure generated during a maximal inspiration against a closed airway (Pimax) as a measure of global inspiratory muscle strength in 61 patients referred for investigation of respiratory muscle function. Transdiaphragmatic pressure (Pdi) was also measured during both maneuvers to compare maximal diaphragmatic strength. Sniff Pes (males, 68 +/- 27 cm H2O; normal greater than 53; females, 66 +/- 21; normal greater than 48) was greater than Pimax (males, 45 +/- 24 cm H2O; normal greater than 42; females, 42 +/- 24; normal greater than 17) in 55 of the 61 patients, both in absolute values and as a percentage of normal. In 36 patients Pimax and sniff Pes were both normal (mean +/- 2 SD), whereas in 13 patients they were both low. In 11 patients, Pimax was low, but sniff Pes was normal. One patient had a reduced sniff Pes but a Pimax at the lower limit of normal. In the 36 patients in whom both Pimax and sniff Pes were normal, Pdi was also normal or only moderately reduced, and in the 13 patients in whom both Pimax and sniff Pes were reduced, Pdi was very low. However, in the group of 11 patients with a low Pimax but a normal sniff Pes, Pdi was normal or only moderately reduced, suggesting that Pimax was falsely low, perhaps because of difficulties with the technique. Conversely, in the single patient with a low sniff Pes but a Pimax just within the normal range, Pdi was very low. We conclude that measurement of esophageal pressure during a maximal sniff is a useful test of inspiratory muscle strength and overcomes the difficulty some patients have in carrying out the Pimax maneuver.

Diaphragm strength in patients with recent hemidiaphragm paralysis.

Eleven patients with unilateral diaphragm paralysis of recent onset were studied to investigate the effect of the paralysis on inspiratory muscle function. Nine of the patients had noticed a decrease in exercise tolerance, which was not explained by any other pathological condition. Hemidiaphragm dysfunction was confirmed by the demonstration of a greatly reduced or absent transdiaphragmatic pressure on stimulation of the phrenic nerve in the neck, by means of surface bipolar electrodes (unilateral twitch Pdi), compared with normal values on the contralateral side. Transdiaphragmatic pressure was 44.6% (9.4%) predicted during a maximal sniff and 30.3% (16.8%) predicted during a maximal static inspiration against a closed airway, confirming diaphragm weakness. Maximum static inspiratory mouth pressures were also low (61.7% (12.7%) predicted), consistent with a reduction in inspiratory muscle capacity. Phrenic nerve conduction time was prolonged on the affected side in nine patients, consistent with phrenic nerve dysfunction, whereas on the unaffected side it was normal. It is concluded that recent hemidiaphragm paralysis causes a reduction in transdiaphragmatic pressure that is associated with a reduction in maximum inspiratory mouth pressure. Phrenic nerve stimulation is a useful technique with which to confirm and quantify hemidiaphragm dysfunction. Measurement of phrenic nerve conduction time provides useful information about the underlying pathology.

Diaphragmatic myasthenia in mother and child.

A 28 year old patient with ocular myasthenia for 2 y gave birth to a baby with diaphragmatic weakness. Following delivery the mother developed severe weakness of the diaphragm and required assisted ventilation. The baby recovered spontaneously and the mother responded to treatment with plasma exchange and immunosuppression. Neither mother nor baby responded to anticholinergic drugs and in neither serum were acetyl choline receptor antibodies detected.