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wallerian degeneration
after injury to axons and myelin, a distal disintegration of axon and myelin occurs
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neuronal/axonal degeneration
after damage to cell body of the neuron, distal dying of the axon and subsequent loss of myelin
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symptoms associated with involvement of small nerve fibers
neuropathing pain (aching, shooting, throbbing, burning), temperature sensations, autonomic dysfunction (cardiac arrhythmias, orthostatic hypotension, impotence, incontinence, and constipation
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symptoms associated with involvement of large fibers
loss of vibration and joint position sense, weakness, fasciculations, loss of DTRs
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first-line tests when investigating PNS symptoms
CBC, ESR, rheumatoid profiles (for collagen disase, leukemia, vasculitis), renal and liver function tests, glucose, HbA1C, vitamin B12, folate, thyroid function tests (hypothyroid neuropathy), serum and urine protein electrophoresis (monoclonal gammopathy, lymphoma, amyloidosis)
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causes of peripheral neuropaties
DANG RAPIST
- diabetes
- alcohol
- nutritional
- guillain-barre
- trauma
- hereditary
- environmental (toxins/drugs)
- rheumatic (vascular)
- amyloid
- paraneoplastic
- infectious
- systemic disease
- tumors
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polyneuropathy
symmetric involvement of peripheral nerves, usually logs more than arms, distal more than proximal
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mononeuropathy
involvement of only a single nerve (ex. carpal tunnel)
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mononeuropathy multiplex
involvement of individual nerves in multifocal distribution
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radiculopathy
involvement of nerve roots
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Peripheral neuropathies...
symmetric proximal and distal weakness with sensory loss
guillain-barree, chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)
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Peripheral neuropathies...
symmetric distal weakness with sensory loss
drug-induced, metabolic neuropathies, hereditary neuropathies, amyloidosis
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Peripheral neuropathies...
asymmetric distal weakness with sensory loss
multiple nerves - vasculitis, HNPP, infections (leprosy, lyme), sarcoidosis, HIV
single nerve - compressive mononeuropathy and radiculopathy
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Peripheral neuropathies...
asymmetric distal weakness without sensory loss
motor neuron disease, multifocal motor neuropathy
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Peripheral neuropathies...
asymmetric proximal and distal muscle weakness with sensory loss
polyradiculopathy, meningeal carcinomatosis or lymphomatosis, HNPP, hereditary neuropathies
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Peripheral neuropathies...
symmetric sensory loss without weakness
cryptogenic sensory polyneuropathys, metabolic, drug induced, leprosy
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Peripheral neuropathies...
asymmetric proprioceptive sensory loss without weakness
sensory neuronopathies (ganglionopathies), paraneoplastic, sjorgen syndrome, B6 toxicity, HIV sensory neuronopathies, cis-platinum toxicity
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Peripheral neuropathies...
autonomic symptoms and signs
diabetes, amyloidosis, GBS, vincristine, porphyria, HIV-related autonomic neuropathy
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guillain-barre syndrome
often preceded by infection with campylobacter jejuni or CMV, EBV (herpes viruses)
antibodies that target gangliocides
rapidly evolving, ascending areflexic motor paralysis without w/o sensory symptoms. often starts with tingling, pins-and-needles in the feet, lower back pain, bulbar weakness and respiratory muscle paralysis may occur, automonic dysfunction (orthostatic hypotension, transient hypertension, cardiac arrythmias
reflexes disappear around day 3, worst at day 14
dx - albuminocytologic dissociation in the CSF (elevated protein but few or no cells)
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miller-fisher variant of guillain-barre
gain ataxia, areflexia, and external opthalmoplegia, usually w/o limb weakness
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chronic inflammatory demyelinating polyneuropathy (CIDP)
chromic GBS
improves with steroids
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multifocal motor neuropathy
pure motor multiple mononeuropathy
slowly progressive, asymmetric, predominantly distal limb weakness beginning in arms, develops in distribution of individual nerves (not myotomes), can be severe in muscles with relatively normal bulk
dx - conduction block in motor nerves in arease not prone to compression
tx - IVIg or immunosuppressive drug therapy (cyclophosphamide)
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uremic neuropathy
symmetric, distally predominant sensorimotor axonal polyneuropathy. foot drop and leg weakness are major manifestations
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polrphyric neuropathy
sensorimotor axonal polyneuropathy manifested by parasthesias and dysesthesias of extremities (sometimes mimicking GBS), with areflexia and abdominal pain
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Charcot-Marie-Tooth disease
presents in adolescence with symmetric, slowly progressive distal musclular atrophy of legs and feet, then hands. Hammer toes and pes cavus common
CMT2 is only axonal motor neuropathy in CMT family, the others are primarily deemyelinating
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lumbosacral polyradiculopathy
associated with CMV infection in HIV pts
rapidly progressice flaccid paraparesis with sphincter dysfunction, perineal sensory loss, and lower limb areflexia
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Neuropathy of leprosy
sensory and motor involvement. presents as mononeuropathy multiplex or mononeuropathies with predilection for cooler areas such as distal limbs, nose, ears. DTRs preserved. Palpable nerve hypertrophy.
axonal damage, myelin changes, and nerve fiber loss are cardinal features
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symptoms of autonomic neuropathies
orthostatic hypotension, diarrhea, constipation, early satiety, tachycardia, blurred vision, urinary retention, erectile dysfunction
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pandysautonomia
often follows viral infection, ANS affected.
Postural orthostatic tachycardia syndrome most commonly in women, with light-headedness and near-syncopal episodes
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meralgia paresthetica
burning sensation and variable loss of sensation over anterolateral thigh, no motor involvement.
entrapment of lateral femoral cutaneous nerve near the inguinal ligament
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femoral neuropathy
leg weakness on attempting to stand or walk, pain in anterior thigh
usually due to trauma from surgery, stretch injury (ex during birth), DM, inflammatory processes
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Peroneal neuropathy
presents with foot-drop with minimal sensory complaints
entrapment of peroneal nerve btwn neck of fibula and insertion of peroneus lungus muscle
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root syndromes...
C5
pain in lateral shoulder, sensory loss over deltoid
paresis of deltoid, supraspinatus, and biceps
impairment of biceps reflex
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root syndromes...
C6
sensation radial side of arm to thumb
paresis of biceps and brachioradialis
impairment of biceps reflex
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root syndromes...
C7
sensation btwn second and 4th finger
paralysis/weakness of triceps, wrist extensors and flexors, pectoralis major
impairment or loss of triceps reflex
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root syndromes...
L3
often no sensory loss, sometimes medial thigh and knee
paralysis of quadriceps, adductor (differentiates from femoral neuropathy)
loss of knee jerk, loss or impaired adductor reflex
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root syndromes...
L4
sensation medial leg below knee to medial malleolus
quadriceps and anterior tibial muscles
decreased knee jerk reflex
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root syndromes...
L5
sensation dorsum of foot to great toe
paralysis of extensor hallicis longus, extensor digitorum longus, inversion and eversion of foot
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root syndromes...
S1
sensation lateral border of foot
loss of plantar and toe flexion
decreased or absent ankle jerk
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neuropathies that may begin in arms rather than legs
lead toxicity, leprocy, sarcoidosis, porphyria, entrapments, diabetes, vasculitic neuropathy, Tangier disease
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neuropathy associated with cranial nerve involvement
diphtheria, sarcoidosis, DM, GBS, sjorgren syndrome, polyarteritis nodosa, lyme disease, porphyria, syphilis, arsenic
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neuropathies associated with palpable peripheral nerves
amyloidosis, leprosy, acromegaly, neurofibromatosis, charcot-marie-tooth
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