Internal and Emergency Medicine

, Volume 12, Issue 5, pp 641–650 | Cite as

Emergencies in motoneuron disease

  • Josef FinstererEmail author
  • Claudia Stöllberger


Genetic and acquired motor-neuron-disorders (MNDs) may undergo acute deterioration resulting in various emergency situations. This literature review aims at summarising and discussing current knowledge about emergencies in MNDs. Emergencies that have been reported in MND patients include: respiratory, bulbar, cardiac, septic, epileptic, psychiatric, pain-related, and traumatic emergencies. Emergencies due to respiratory insufficiency have the strongest impact on morbidity and mortality in MNDs. To optimise the management of emergencies in MNDs, it is recommended to discuss these topics with the patient prior to their occurrence. After informed consent, patients may indicate their decision by signing an advance directive as to how such emergencies should be managed in case they arise. Generally, treatment of emergencies in MNDs is not at variance from treatment of similar emergencies due to other causes, but some peculiarities need to be pointed out. It is concluded that patients with MNDs may experience various emergencies during the disease course. Management of these conditions should be discussed with the patient prior to their appearance. Management of these emergencies follows general guidelines, which widely vary between countries, and depend on the availability of a patient’s advance directive.


Anterior horn cell disease Emergency Respiratory insufficiency Motoneuron disease Seizures Sepsis Takotsubo Artificial ventilation 



Advance care planning


Advance directive


Antiepileptic drugs




Amyotrophic lateral sclerosis


Atrio-ventricular block


Bulbospinal muscular atrophy Kennedy


Brown–Vialetto–Van Laere syndrome


Cardio-pulmonary resuscitation




Familial amyotrophic lateral sclerosis


Forced vital capacity


Hereditary spastic paraplegias


Implantable cardioverter defibrillator


International League Against Epilepsy


Mechanically assisted coughing


Maximal expiratory pressure


Mitochondrial disorders


Maximal inspiratory pressure


Motor neuron disease


Non-invasive positive pressure ventilation


Neuromuscular disorders


Percutaneous, endoscopic gastrostomy


Rapid sequence induction


Sporadic amyotrophic lateral sclerosis


Spinal muscular atrophy


Takotsubo syndrome



No funding was received.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

All applicable international, national, and/or institutional guidelines for the care of patients were followed.

Statement of human and animal rights

This article does not contain any studies with human participants or animals performed by any of the authors.

Informed consent

Informed consent was obtained from all individual participants included in the study.


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© SIMI 2017

Authors and Affiliations

  1. 1.Krankenanstalt RudolfstiftungViennaAustria
  2. 2.2nd Medical Department with Cardiology and Intensive Care MedicineKrankenanstalt RudolfstiftungViennaAustria

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