Amyotrophic Lateral Sclerosis (ALS) is a degenerative disease of unknown cause that presents motor, bulbar and respiratory dysfunctions, the last of which is the main cause of death [3]. It is a part of a spectrum of disorders that target motor neurons in the cerebral cortex, brainstem, and spinal cord [1].

There is a slight male predominance, with peak incidence between 60 and 75 years. Its onset is often subtle and its course insidious, the typical time to diagnosis is 10–16 months from symptom onset [1].

Patients with ALS ultimately experience a progressive, usually rapid deterioration of these motor neurons, resulting in variable degrees of weakness, spasticity, and muscle atrophy, affecting key functions including limb use/ambulation, speech, swallowing, and breathing. Dysphagia occurs in most ALS patients at some point in their disease course due to weakness and/or spasticity of bulbar musculature. Up to half of patients with ALS will exhibit some degree of frontotemporal cognitive dysfunction, while a much smaller proportion manifests dementia of the frontotemporal type [1].

ALS is largely a clinical diagnosis, defined via the El Escorial and subsequently revised El Escorial/Airlie House Criteria by the following three factors: 1) evidence of Lower Motor Neuron (LMN) degeneration (either on exam or through specialized testing), 2) evidence of upper motor neuron (UMN) disease on exam, and 3) the presence of either of the above in more than one region of the body. In addition, the course must be progressive with no evidence of a reasonable alternative diagnosis [1,4].

NIV prolongs survival and maintains overall quality of life [2]. Because most of ALS patients die from respiratory failure (including complications of aspiration) that occurs within three years of the first symptom onset, early diagnosis and subsequent referral to an appropriate tertiary center are crucial for timely multidisciplinary care and improved quality of life [1].

Within clinical evaluations that detail mortality, pulmonary function, muscular strength and incapacity, there is the Amyotrophic Lateral Sclerosis Functional Rating Scale (ALS-FRS) [3]. The ALS-FRS is a validated rating instrument for monitoring the progression of disability in patients with ALS. The Revised ALSFRS (ALSFRS-R) retains the properties of the original scale and shows strong internal consistency and construct validity. ALSFRS-R scores correlate significantly with quality of life as measured by the Sickness Impact Profile, indicating that the quality of function is a strong determinant of quality of life in ALS [5].

In this case, the authors present a symptomatic man with progressive muscle degeneration within his first presentation on emergency department and the urgent need for respiratory support. During hospitalization it was attempted to manage for NIV to assure the best quality of his life. Diagnosis was achieved after 12 months of progressive symptoms. NIV became his main respiratory support. The patient fulfilled the three El Escorial / Arlie House Criteria.