Neuromuscular Disease

Posted by e-Medical PPT Thursday, July 19, 2012
Disorders of the Motor Unit
Motor neuron disease
Peripheral nerve disorders
Neuromuscular junction disease
Muscle disease

Motor Neuron Disease
Diseases that can involve Betz cells of the motor cortex, the lower CN motor nuclei, the CST, and/or the anterior horn cells
 Amyotrophic Lateral Sclerosis (ALS)
 Progressive bulbar palsy (PBP)
 Progressive muscular atrophy (PMA), spinal muscular atrophy (SMA)
 Primary lateral sclerosis

Peripheral Nerve Disorders
Mononeuropathy
 Pattern of weakness and sensory loss conforms to the distribution of a single nerve
 Carpal tunnel syndrome
 Peroneal palsy at the fibular head
Mononeuritis multiplex
 Multiple nerves affected in a random pattern
 Acute onset, frequently painful
 Diabetes mellitus, vasculitis
Polyneuropathy (peripheral neuropathy)
 Distal, symmetric

Disorders of the Neuromuscular Junction
Pre-synaptic
 Lambert Eaton myasthenic syndrome
 Botulism
Post-synaptic
 Myasthenia Gravis

Lambert Eaton Myasthenic Syndrome (LEMS)
Presynaptic disorder of the NMJ
Voltage gates calcium channel antibodies impede release of acetylcholine
Weakness -more of LE than UE
bulbar and ocular muscles less often involved
decreased reflexes - post tetanic potentiation?
ANS involvement

Myopathies
Clinical Manifestations of Myopathies
Proximal muscle weakness
Waddling gait
Difficulty climbing stairs
Trouble lifting arms over head
Cramps with the metabolic myopathies
Myalgias with the inflammatory myopathies
Swallowing and breathing difficulties, when present, are usually late
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