Respiratory muscle weakness in the Lambert-Eaton myasthenic syndrome

• Published in: Thorax Vol. 44; no. 11; pp. 913 - 918
• Main Authors: Laroche, C M, Mier, A K, Spiro, S G, Newsom-Davis, J, Moxham, J, Green, M
• Format: Journal Article
• Language: English
• Published: London BMJ Publishing Group Ltd and British Thoracic Society 01.11.1989; BMJ; BMJ Publishing Group LTD
• Subjects: Autoimmune Diseases - physiopathology; Biological and medical sciences; Diaphragm - physiopathology; Female; Humans; Lambert-Eaton Myasthenic Syndrome - physiopathology; Male; Medical sciences; Neurology; Phrenic Nerve - physiopathology; Respiratory Function Tests; Respiratory Muscles - physiopathology; Tumors of the nervous system. Phacomatoses; Human; Striated muscle disease; Nervous system diseases; Respiratory muscle; Lambert Eaton syndrome; Respiratory disease; Tumor; Paraneoplastic syndrome; Oat cell carcinoma; Bronchopulmonary; Strength
• ISSN: 0040-6376; 1468-3296
• DOI: 10.1136/thx.44.11.913

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.