ALS with respiratory onset: Clinical features and effects of non-invasive ventilation on the prognosis

  title={ALS with respiratory onset: Clinical features and effects of non-invasive ventilation on the prognosis},
  author={Guillaume Gautier and Annie Verschueren and Armelle Monnier and Shahram Attarian and Emmanuelle Salort-Campana and Jean Pouget},
  journal={Amyotrophic Lateral Sclerosis},
  pages={379 - 382}
Abstract Respiratory muscle involvement is one of the main prognostic factors in amyotrophic lateral sclerosis (ALS). Acute respiratory failure is sometimes the first manifestation of the disease, although onset can be more insidious. In the present retrospective study, it was proposed to review the clinical features and to assess the effects of non-invasive ventilation (NIV) on the prognosis of patients with respiratory onset, which was taken to be present when the first symptoms of muscular… 

Management of respiratory symptoms in ALS

Early recognition of respiratory decline and symptomatic intervention, including non-invasive ventilation can significantly enhance both quality of life and life expectancy in ALS.

Clinical and electrophysiological characteristics of respiratory onset amyotrophic lateral sclerosis: a single-centre study.

Patients with respiratory onset ALS were characterised by male predominance, with a higher baseline ALSFRS-R, lower BMI and phrenic nerve study well discriminated respiratory onsetALS from bulbar or limb onset ALS patients.

Respiratory onset in amyotrophic lateral sclerosis: clinical features and spreading pattern.

This phenotype is typically seen in emaciated older men with predominant lower motor neuron involvement, and is associated with diaphragm paresis and central respiratory involvement.

Non‐invasive positive‐pressure ventilation in patients with amyotrophic lateral sclerosis: spinal versus bulbar form

Any respiratory abnormality should prompt the clinician to start discussing NIV treatment for all types of patients with ALS, without regard to bulbar involvement, as this treatment might improve respiratory function and prolong survival in this entire group of patients.

Amyotrophic Lateral Sclerosis Identified by Failure to Wean From Mechanical Ventilation

An 81-year-old man with ALS, diagnosed by an unexplained failure to wean from mechanical ventilation, is wediagnosed after a physical and neurologic examination and electrodiagnostic testing.

Clinical Course of Amyotrophic Lateral Sclerosis according to Initial Symptoms: An Analysis of 500 Cases

Inappropriate operations significantly delayed the diagnosis of ALS, and respiratory-dominant onset appears to show male predominance, older age at symptom onset, and poor respiratory prognosis.

Presentation of motor neuron disease in a patient with weight loss and acute-on-chronic respiratory failure

This case highlighted the importance of considering neuromuscular causes for acute respiratory failure in acute presentations and demonstrated the challenges in the diagnosis of MND in those presenting atypically with non-specific symptoms and the limitations of remote consultations in complex cases.

Diagnosis of muscle diseases presenting with early respiratory failure

Here we describe a clinical approach and differential diagnosis for chronic muscle diseases which include early respiratory failure as a prominent feature in their presentation (i.e. respiratory

Respiratory measures in amyotrophic lateral sclerosis

The most commonly used measurement, forced vital capacity (VC), has been shown to correlate with clinical milestones including survival, but also requires good motor coordination and facial strength to form a tight seal around a mouthpiece.

Long-term survival in a patient with amyotrophic lateral sclerosis and respiratory presentation

  • H. NzwaloM. de Carvalho
  • Medicine
    Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases
  • 2012
The patient presented with progressive RI, signs of upper motor neuron dysfunction in one limb and widespread neurogenic signs on EMG which made the diagnosis of ALS, a severe restrictive pulmonary disease with severe involvement of the respiratory muscles, particularly the diaphragm.



Prognosis of amyotrophic lateral sclerosis with respiratory onset

It is suggested that ALS with respiratory onset does not necessarily follow a rapidly progressive course and non-invasive positive pressure ventilation (NIPPV) significantly improved survival compared with those who did not use NIPPV.

[Amyotrophic lateral sclerosis (ALS): evaluation of respiratory function].

Respiratory involvement is an almost constant feature of als, with a usually rapid progression leading to respiratory failure. These characteristics justify a close follow up, usually at three-month

Dyspnea-fasciculation syndrome: early respiratory failure in ALS with minimal motor signs

  • S. ScelsaB. YakubovS. Salzman
  • Medicine
    Amyotrophic lateral sclerosis and other motor neuron disorders : official publication of the World Federation of Neurology, Research Group on Motor Neuron Diseases
  • 2002
ALS may present with unexplained RF, or sleep disturbance resembling sleep apnea, without significant bulbar or limb weakness, despitehypercapnia, and FVC may be relatively preserved, despite hypercapnia.

Factors Predicting Survival following Noninvasive Ventilation in Amyotrophic Lateral Sclerosis

NIV is a helpful treatment of sleep-disordered breathing, including patients with bulbar involvement, and is significantly poorer in patients with advanced age or with airway mucus accumulation, which represents poorer prognostic factors of ALS patients treated with NIV.

Respiratory failure due to bilateral diaphragm palsy as an early manifestation of ALS.

Failure of respiratory muscle function can be the first symptom of motor neuron disease, and may precede clinical manifestation in voluntary motor units in ALS, and should be taken into consideration in cases of unexplained acute respiratory failure.

Motor neuron disease presenting as acute respiratory failure: a clinical and pathological study.

Electrophysiological studies showed widespread denervation and, in particular, diaphragmatic involvement to explain the severe respiratory failure in seven patients with motor neuron disease who presented with acute respiratory failure of unknown cause and required mechanical ventilation.

Causes of death in a post‐mortem series of ALS patients

  • P. CorciaP. Pradat V. Meininger
  • Medicine
    Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases
  • 2008
A retrospective pathological study on 100 ALS patients referred for a deterioration of their clinical condition and who died in the ALS clinic of Salpétrière found that broncho‐pneumonia and pneumonia were the main causes of death, and heart failure was two times more frequent in bulbar than in spinal ALS.

Amyotrophic lateral sclerosis presenting with respiratory insufficiency as the primary complaint. Clinicopathological study of a case.

The neuropathological examination corroborated the diagnosis of amyotrophic lateral sclerosis, and showed a preponderance of lesions in the phrenic nuclei and axonal alterations associated with a fast evolution.