Peripheral Nervous system & Neuromuscular Junction (Diabetic…
Peripheral Nervous system & Neuromuscular Junction
Occur when multiple peripheral nerves malfunction simultaneously. sensory loss; glove and stocking pattern. Longest nerves affected first
small fibre; paresthesiae, burning pain, hyperasthesia and allodynia, worse at night
Large fibre; numbness, loss of vibration sensation, wide stance, ataxic
Motor; muscle wasting, hypo-reflexia
Autonomic dysfunction eg. Less sweating, red face, odema
Treatment: glucose control, improve lifestyle factors
Complex Regional pain syndrome
Myasthenia Gravis (MG)
Two groups; women (20-30 acute onset, eye muscles and general weakness) and men (60-75 ocularbulbar abnormalities)
Repeated use fatigues muscles. post-synaptic acetyle-choline receptor damage
positive response to Tensilon (Edrophonium) within seconds. This will not work with someone with LES
association to thymic cancer
Lambert eaton Syndrome (LES)
Neuromuscular presynaptic calcium problems. Repeated use strengthens muscles
LES is more likely to be due to underlying condition
Association with oat cell carcinoma
autoimmune condition, system attacks myelin in peripheral nerves. Typically follows infection. MS of the peripheral nervous system
progressive muscular weakness, areflexia, paresthesia in the hands and feet, motor signs worse than sensory. recovery over many months. generally affects all limbs
Diagnosis; cerebrospinal fluid and elevated protien, no significantly high white blood count, slowing of nerve conduction, EMG signs consistant with denervation
Treatment; plasmaphoresis and IV immunoglobulin therapy, Intubation and mechanical ventilation, autonomic dysfunction can be a problem. Most people have complete or near complete recovery.
Charcot Marie Tooth Disease (CMT)- Hereditary motor and sensory Neuropathies
Group of disorders genetical with similar clinical presentation.
slow progressing weakness beginning in the distal limb muscles, typically in the lower extremities before the upper extremities. onset 2nd decade of life.
type1: (adolescent onset) most common type, repeated cycles of demyelinating and remyelination. results in onion bulb appearance.
type 2; primarily neuronal disorder. axonal death and Wallerian degeneration.
fq tripping difficulty walking. clumsiness and foot dropping. steppage "slap feet on ground". pes cavus deformity. no numbness
Stork leg appearance,DTRs diminished, vibration sensation and proprioception decreased, no sensory signs/symptoms, ataxia and rombergs positive, pain and temp normal, tremor present in 30-50%