Multiprofessional Critical Care Review: Pediatric 2024 (MCCRCP24ON)-Neuro-Musc Diseases

Neuro-Musc Diseases

Pdf Summary

The document is a comprehensive overview of various neuromuscular diseases provided by Dr. Edward E. Conway Jr. It covers disorders affecting both upper and lower motor neurons, along with their implications on respiratory function and muscle control. Key areas include:<br /><br />1. <strong>Anatomy and Symptoms of Lesions</strong>:<br />   - <em>Upper Motor Neuron</em>: Involves the cortex and spinal cord, leading to increased muscle tone (spasticity), hyperreflexia, and reemergence of primitive reflexes.<br />   - <em>Lower Motor Neuron</em>: Involves anterior horn cells, peripheral nerves, neuromuscular junctions (NMJ), and muscles, resulting in flaccidity, decreased reflexes, fasciculations, and atrophy.<br /><br />2. <strong>Respiratory Issues</strong>:<br />   - Emphasizes the serious consequence of respiratory muscle weakness, which can lead to poor cough, reduced vital capacity (VC), atelectasis, hypoxia, and increased work of breathing (WOB).<br /><br />3. <strong>Specific Neuromuscular Diseases</strong>:<br />   - <em>Guillain-Barre Syndrome</em>: Characterized by diffuse peripheral nerve demyelination, ascending paralysis, and often follows a viral illness. Diagnosis includes clinical signs, CSF analysis, and EMG studies.<br />   - <em>Botulism</em>: Caused by Clostridium botulinum, leading to symmetrical, descending muscle weakness, autonomic dysfunction, and requires prompt diagnosis and treatment.<br />   - <em>Tick Paralysis</em>: Results from neurotoxins of ticks, causing weakness and loss of reflexes. Immediate tick removal is crucial.<br />   - <em>Tetanus</em>: Involves severe muscle spasms and rigidity due to toxin interference with GABA release in the CNS.<br />   - <em>Myasthenia Gravis (MG)</em>: An autoimmune disease causing muscle fatigue, ptosis, and generalized weakness. Treatment includes cholinesterase inhibitors, steroids, and sometimes IVIG or plasmapheresis.<br />   - <em>Duchenne Muscular Dystrophy</em>: A progressive muscle-wasting disease often leading to scoliosis and respiratory failure as the disease progresses.<br /><br />4. <strong>Critical Illness Neuropathy and Myopathy</strong>:<br />   - Highlights factors in ICU-acquired weakness, such as prolonged ventilation, steroids, sepsis, and immobility, with diagnosis potentially confirmed by EMG or muscle biopsy.<br /><br />5. <strong>Management of Neuromuscular Diseases</strong>:<br />   - Recommendations for ventilatory management, including elective intubation, positive pressure ventilation, chest physical therapy, and incentive spirometry for conditions like hypoventilation and atelectasis.<br /><br />The document emphasizes the importance of anatomical and physiological understanding for accurate diagnosis and management of these conditions.

Keywords

neuromuscular diseases

upper motor neuron

lower motor neuron

respiratory muscle weakness

Guillain-Barre Syndrome

Botulism

Myasthenia Gravis

Duchenne Muscular Dystrophy

Critical Illness Neuropathy

ventilatory management

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