Emergencies in Neuromuscular Disorders

Maxwell Damian trained in Germany and the UK, and specialises in genetic muscle disease and in neurocritical care. He was consultant and director of the neurological ICU in the universities of Giessen and Dresden, Germany, before continuing work in the UK, specialising in critical care aspects of neuromuscular disease and in research on hypoxic brain injury. He has been co-chair of the Neurocritical Care scientific panel of the European Academy of Neurology, 2015-2020; chair of the specialist group in Neurocritical Care of the Association of British Neurologists; chair of the accreditation committee of the Neurocritical Care Society, USA. He retired from Cambridge University Hospitals in 2020 but continues research work at the University Department of Anaesthetics, Cambridge University and at the Essex Cardiothoracic Centre, UK.  Marianne de Visser is an adult neurologist at the Amsterdam University Medical Centers,  location AcademicMedical Center in Amsterdam, The Netherlands and emeritus Professor of Neuromuscular Diseases. She was trained at the University Hospital of Amsterdam. In 1988 she was a visiting scientist at Dr. Andrew Engel’s lab at Mayo Clinic, Rochester, Minnesota where she performed ultrastructural studies on skeletal muscle in dermatomyositis. She obtained a position at the Department of Neurology of the Academic Medical Center in Amsterdam and was appointed as Professor of Neuromuscular Diseases at the University of Amsterdam in 1993.Her research interests are rare neuromuscular disorders such as myositis, hereditary neuropathies, postpolio syndrome and motor neuron disorders. Her work on ALS kindled interest in palliative care. She was a member of the Governing Board of the European Academy of Neurology (EAN), first as Treasurer and subsequently as Secretary-General (2014-2020). She also served on the Management Committee of the World Federation of Neurology (Elected Trustee (2002-2008), Co-opted Trustee (2020-2021)). She was co-chair of the EAN Scientific Panel on Rare Neurological Diseases (2020-2022) and now member of the Management Group.

• Diagnostic assessment in acute neuromuscular disease.- The differential diagnosis of the patient with acute neuromuscular disease and acute flaccid paralysis.- The clinical assessment of the neuromuscular emergency.- Clinical assessment in the child with an acute neuromuscular illness.- Antibody testing and immunological assessment in acute neuromuscular disorders.- Testing of the patient with exercise intolerance and acute rhabdomyolysis.- Electrophysiological assessment of acute neuromuscular disorders and EMG in the ICU.- Assessment of autonomic function.- Nerve and muscle biopsies in the acutely ill patient and histological assessment.- Imaging of the acutely weak patient.- Molecular and genetic testing algorithms in the acutely ill neuromuscular patient.- The neurology of acutely failing respiratory mechanics and its assessment.- Categories of acute neuromuscular disorders.- Poliomyelitis and acute infectious disorders of the motor neurone.- The emergency presentation of Amyotrophic lateral sclerosis and atypical motor neurone disorders (text; tables/flow charts).- Acute presentation of inherited and metabolic neuropathies.- Acute inflammatory neuropathy – Guillain Barre syndrome and its differential diagnoses.- Myasthenia Gravis and myasthenic emergencies.- Botulism, and the Lambert-Eaton myasthenic syndrome.- Congenital myasthenic syndromes and their emergency presentation- Channelopathies and disorders of skeletal muscle membrane excitability.- Rhabdomyolysis and myoglobinuria.- Acute emergencies in childhood muscular dystrophies.- Emergencies in advanced muscular dystrophy.- Acute cardiac failure, cardiomyopathy, and cardiac dysrhythmia in muscle disease.- Congenital myopathies and acute emergencies.- Metabolic myopathies and their acute presentation.- Mitochondrial myopathies: acute presentation and emergency management.- Endocrine and toxic myopathies.- Acute inflammatory and paraneoplastic myopathies.- Infectious and granulomatous myopathies.- C. Management problems in acute and emergency neuromuscular disorders.- 1. ICU-acquired weakness and neuromuscular complications of critical care- 2. Weaning from the ventilator and liberation from tracheotomy in the neuromuscular patient .- 3. Palliative care and advanced decisions in neuromuscular disease.- 4. Acute neuromuscular disorders in challenging environments.- D. ESM (media links; algorithms; self-assessment tools; MCQs).