What every physician needs to know: Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) are progressive myopathies that are inherited as X-linked recessive traits. Classification Not applicable. Continue Reading Are you sure the patient has Duchenne or Becker Muscular Dystrophy? What should you expect to find? Notable clinical features of DMD include symptom onset…
What every physician needs to know Lambert-Eaton myasthenia syndrome (LEMS) is an uncommon disorder characterized by skeletal muscle weakness that results from impaired release of acetylcholine from presynaptic terminals. The syndrome arises in the setting of related conditions that include small-cell lung cancer (3%), in which LEMS may be a presenting symptom; Hodgkin’s lymphoma; atypical…
What every physician needs to know Myasthenia gravis (MG) is an autoimmune disorder in which antibodies to acetylcholine receptors (AChR) or to muscle-specific receptor tyrosine kinase (MuSK) lead to weakness. Clinical forms include generalized myasthenia and ocular myasthenia; about half of patients with ocular MG will develop generalized disease within two years of onset. The…
What every physician needs to know: Guillain-Barre syndrome (GBS), or acute idiopathic polyneuritis, is characterized by ascending symmetric paralysis. Respiratory involvement may be absent, or it may be severe enough to necessitate mechanical ventilation. Seventy percent of cases are preceded by viral or bacterial infection. The incidence of GBS worldwide ranges from 1.1 per 100,000…
What every physician needs to know Diaphragm paralysis may be unilateral or bilateral and may be seen in a variety of settings. Cardiac surgery (thought to be secondary to phrenic nerve injury due to exposure to cold cardioplegic solution or nerve stretch), chest trauma, mediastinal tumors, pleural space infection, or forceful neck manipulation may be…
What every physician needs to know Twenty-five percent of cases of acute poliomyelitis are characterized by paralysis, which may be severe enough to require mechanical ventilation. Respiratory muscle function typically returns to normal, but progressive muscle weakness in the form of post-polio syndrome (PPS) may occur years later. Post-poliomyelitis syndrome (PPS) incorporates neurologic and musculoskeletal…
What every physician needs to know: Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig’s Disease, is a progressive neurodegenerative disorder of upper and lower motor neurons that results in loss of strength of skeletal muscles, including respiratory muscles. The most common presenting symptom (in 80% of patients) is progressive distal limb weakness. The next…
What every physician needs to know. After spinal cord injury, paresis or paralysis may occur below the level of injury. Classification Not applicable. Continue Reading Are you sure the patient has a spinal cord injury? What should you expect to find? Clinical findings that may be seen with acute spinal cord injury include pain at…
Upper Motor Neuron Cerebral – Stroke, spinal cord injury Continue Reading Lower Motor Neuron Anterior horn cells – poliomyelitis, amyotrophic lateral sclerosis Peripheral nerves – phrenic nerve injury, Guillain-Barré syndrome, critical illness polyneuropathy Neuromuscular junction – myasthenia gravis, Lambert-Eaton myasthenic syndrome, botulism, aminoglycosides Muscle – dystrophy, acid maltase deficiency Corticosteroids – acute intensive care myopathy…
