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Internal and Emergency Medicine

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

Emergencies in motoneuron disease

  • Josef FinstererEmail author
  • Claudia Stöllberger
EM - REVIEW
  • 283 Downloads

Abstract

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.

Keywords

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

Abbreviations

ACP

Advance care planning

AD

Advance directive

AEDs

Antiepileptic drugs

ALD

Adrenoleucodystrophy

ALS

Amyotrophic lateral sclerosis

AV-block

Atrio-ventricular block

BSMA

Bulbospinal muscular atrophy Kennedy

BVLS

Brown–Vialetto–Van Laere syndrome

CPR

Cardio-pulmonary resuscitation

EEG

Electroencephalography

fALS

Familial amyotrophic lateral sclerosis

FVC

Forced vital capacity

HSPs

Hereditary spastic paraplegias

ICD

Implantable cardioverter defibrillator

ILAE

International League Against Epilepsy

MAC

Mechanically assisted coughing

MEP

Maximal expiratory pressure

MIDs

Mitochondrial disorders

MIP

Maximal inspiratory pressure

MND

Motor neuron disease

NIPPV

Non-invasive positive pressure ventilation

NMDs

Neuromuscular disorders

PEG

Percutaneous, endoscopic gastrostomy

RSI

Rapid sequence induction

sALS

Sporadic amyotrophic lateral sclerosis

SMA

Spinal muscular atrophy

TTS

Takotsubo syndrome

Notes

Acknowledgements

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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Copyright information

© 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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