Peripheral Nerve Disorders Flashcards
Polyneuropathy
sensory, motor or mixed deficit affecting more than one peripheral nerve simultaneously
Mononeuropathy
sensory, motor or mixed deficit affecting a specific nerve
Mononeuropathy Multiplex
sensory and/or motor deficits affecting single nerves in different, unrelated parts of the body
Radiculopathy
disease of the spinal nerve roots
Plexopathy
sensory and/or motor deficit involving
the lumbar or brachial plexus
Amyotrophy
pathological wasting of muscles due to a disease of the nerves supplying them
Charcot-Marie-Tooth (3)
- HMSN I & II
- Usually autosomal transmission
- Short arm of chromosome 17 (or 1 or X)
Charcot-Marie-Tooth Presentation (5)
- Foot deformities or gait disturbances in childhood
- Distal weakness and wasting starts in the legs
- Variable distal sensory loss
- Depressed or absent tendon reflexes
- Tremor is sometimes present
What are the phenotypes for Charcot Marie Tooth? (4)
– High arches (pes cavus)
– Calf muscle wasting
– Barrel chest
– Distinctive walk
Outcome of Charcot Marie Tooth (2)
- EMG, NCS shows marked reduction in both sensory and motor conduction
- Despite the neuropathy, these patients can live long, productive lives (orthotics help)
Dejerine-Sottas Dz (5)
- HMSN III
- Onset in infancy or childhood
- Motor and sensory polyneuropathy
- Peripheral nerves may be palpable
- Segmental demyelination, Schwann cell hypertrophy and thin myelin sheaths
Inherited Neuropathies (5)
– Charcot-Marie-Tooth – Dejerine-Sottas – Fredrich Ataxia – Refsum disease – Porphyria
Neuropathies a/w Systemic and Metabolic Disorders (4)
– Diabetic
– Uremic
– Alcohol and nutritional deficiency
– Paraproteinemias
Infectious and Inflammatory Neuropathies (6)
- Leprosy,
- AIDS,
- Lyme disease,
- Sarcoidosis,
- Polyarteritis,
- RA
Fredreich Ataxia (6)
– Chromosome 9
– Onset in childhood
– Gait is ataxic, Hands are clumsy, Other signs of cerebellar dysfunction
– Weakness in the legs, but primarily a sensory neuropathy (dorsal root ganglia, dorsal and lateral columns)
– Depressed DTRs
– Upgoing Babinski’s
Refsum Dz (6)
- HMSV IV
- Autosomal recessive disorder
- Disturbance in phytanic acid metabolism
- Pigmentary retinal degeneration
- Progressive sensorimotor polyneuropathy, cerebellar signs, auditory dysfunction, cardiomyopathy, cutaneous manifestation
- Dietary reduction in phytanic acid, plasmapheresis
Diabetic Peripheral Neuropathy Overview
• Peripheral neuropathy – Distal symmetric polyneuropathy – Isolated peripheral neuropathy – Painful diabetic neuropathy – Diabetic neuropathic cachexia • Autonomic neuropathy
Distal Symmetric Polyneuropathy (5)
- Most common type
- Due to an axonal neuropathic process
- Longer nerves are most vulnerable (foot)
- Motor and sensory nerves
- Achilles reflexes may be absent
Sensory Distal Symmetric Polyneuropathy(4)
– decreased sensation to vibration, pain and temperature
• Painful
• Progressive in severity and distribution
• Examination with monofilament picks up patients who are at risk for unperceived neuropathic injury
Motor Distal Symmetric Polyneuropathy (3)
- denervation of the lumbricals in the feet -> clawing of the toes, anterior displacement of the submetatarsal fat pads -> increased plantar pressure
• Neuropathic injury results from decreased pain sensation, increased plantar pressures and repetitive stress (walking)
• Charcot foot
Distal Symmetric Polyneuropathy Tx (5)
• Treatment of ulcers: – Revascularization if possible ** – Unloading – Treat infection – Debride if necessary • Recurrence should be minimized by wearing appropriate footwear, checking the feet daily
Isolated Peripheral Neuropathy
- Sudden onset with later recovery of all or most of the function
- Attributed to vascular ischemia or traumatic damage
- Cranial and femoral nerves are commonly involved
- Predominantly motor abnormalities
Isolated Peripheral Neuropathy Presentation (6)
– Diplopia: involvement of CN III, IV, OR VI
• Pupil is spared
• Full recovery in 6-12 weeks
• Severe pain in the anterior thigh (unilateral)
• Within days muscle wasting and weakness
• As weakness appears, pain improves
Isolated Peripheral Neuropathy Tx (2)
- Analgesia, better control of diabetes
* Symptoms improve over 6-18 months
Painful Diabetic Neuropathy (2)
- Hypersensitivity to light touch
* Severe burning pain (nocturnal)
Painful Diabetic Neuropathy Tx (6)
– Amitriptyline – Tricyclic antidepressants (TCA) – Gabapentin • Improvement in 48-72 hours is usual • Improves sleep • Discontinue medication if no improvement in 5 days