RESPIRATORY-FAILURE AS THE FIRST MANIFEST ATION OF AMYOTROPHIC-LATERAL-SCLEROSIS

History and clinical finding: A 73-year-old man developed progressive respiratory failure within 24 hours, requiring emergency admission for mechanical ventilation. The cause of the dyspnoea and tachypnoea coul d not be ascertained by routine medical and neurological examination. Neuromuscular disease or a cerebral lesion was considered in the diffe rential diagnosis, together with a cardiovascular cause. Investigation s: Neither laboratory tests nor additional investigations (chest radio gram, echocardiography) gave a diagnosis. Magnetic resonance imaging a nd computed tomography excluded a focal cerebral or spinal lesion or a space-occupying lesion in the region of the phrenic nerve. An electro physiological tests (EPT) failed to establish a neuromuscular disease. However, when signs of upper motor neurone irritation appeared, more detailed EPTs, also of the muscles of breathing, provided the diagnosi s of amyotrophic lateral sclerosis (ALS). Treatment and course: Sympto matic treatment with pyri-dostimine made it possible gradually to wean the patients off the ventilator so that he could be sent home on inte rmittent mechanical ventilation. Conclusion: In case of acute respirat ory failure without cardiopulmonary cause motoneurone disease with ini tial involvement of respiratory musculature should be considered, even in the absence of clinical signs, and special electrophysiological te sts may be necessary to recognize the underlying disease.