Neuro: Peripheral Neuropathies I and II: Dubisky

Terms in this set (25)

What are some neuropathic symptoms?
Pain
Paresthesias
Sensory loss
Weakness
Imbalance
Normal conduction velocities but reduced compound muscle action potentials
Axonal: pathological process affects the underlying axon
Results in delayed conduction
Demyelinating: pathological process that damages or destroys the myelin sheath
What is the most common cause of peripheral neuropathy in the Western world?
DM
Can you get neuropathic symtoms from DM before you are actually diagnosed with DM?
Yes, somtimes by 1 or 2 years
Pathologically characterized by extensive segmental demyelination and remyelination and by a consequential thickening of the peripheral nerves
Nerves become palpably enlarged
One of the parents will also have some degree of abnormality on nerve conduction testing
Autosomal Dominant
Charcot-Marie-Tooth Type I

or Hereditary Motor Sensory Neuropathy (HMSN)
Autosomal dominant transmission
Onset of symptoms usually in the second decade
Progression is slower than in type 1
Nerve conduction studies are usually normal, but electromyography shows evidence of denervation
CMT type II
HSMN type II
Symptoms begin in first year of life
Recessive transmission
Hypotonia
Muscular atrophy
Weakness
Ataxia
Sensory disturbances
Facial weakness and thick-lipped pouting mouth
Hereditary Motor Sensory Neuropathy (HMSN) Type III

Also known as Dejerine-Sottas Disease (Hypertrophic Interstitial Neuropathy of Infancy)
Nerve conduction velocities are very slow
Distal sensory abnormalities are noted (predominantly vibration)
Kyphoscoliosis and pes cavus deformities are common
Can be very difficult to differentiate from HMSN Type I clinically at times
Recessive transmission
Both parents will have normal nerve conductions
Hereditary Motor Sensory Neuropathy (HMSN) Type III

Also known as Dejerine-Sottas Disease (Hypertrophic Interstitial Neuropathy of Infancy)
Symptoms usually begin between the ages of 4 and 7
Peripheral neuropathy (demyelinating)
Ichthyosis
Sensorineural deafness
Cardiomyopathy
Retinitis pigmentosa
Hereditary Motor Sensory Neuropathy (HMSN) Type IV

Also known as Refsum Disease (Heredopathia Atactica Polyneuritiformis)
Nerve damage results from excess of phytanic acid
There is a block in the alpha-oxidation of phytanic to prostanoic acid
The mechanism is not known
Reduction of exogenous phytanic acid intake (from nuts, spinach, coffee) may result in improvement
Hereditary Motor Sensory Neuropathy (HMSN) Type IV

Also known as Refsum Disease (Heredopathia Atactica Polyneuritiformis)
Looks like HMSN Type I, but with pyramidal tract signs and normal nerve conductions
Hereditary Motor Sensory Neuropathy (HMSN) Type V
Looks like HMSN Type I, but with optic atrophy and deafness
Hereditary Motor Sensory Neuropathy (HMSN) Type VI
An acute autoimmune demyelinating polyneuropathy
Mortality is 3% to 5%
This is a medical/neurological emergency that must be recognized early to prevent significant morbidity and mortality
Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP)

Also known as Guillain-Barré Syndrome
Symptoms usually begin with distal paresthesias followed by ascending muscle weakness
Can affect cranial nerves
Can lead to respiratory failure
Serious complications may arise due to autonomic dysfunction
Eventual loss of muscle stretch reflexes
Guillain-Barré Syndrome

preceded by viral, bacterial, stress, or other events
A chronic autoimmune disorder characterized by slowly progressive weakness and sensory loss
-affects individuals of any age but peaks in 40-60
symmetrical
lower extremeties
Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)
Based on history and neurological examination
Lumbar puncture reveals elevated protein with no cells
Electrodiagnostic studies reveal findings consistent with demyelinating neuropathy
Nerve biopsy shows "onion-bulb formation"
Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)
What is the treatment for CIDP?
Corticosteroids can be very helpful in CIDP
Plasma exchange or IVIG can be used for exacerbations
Immunosuppressant drugs may be used for long-tem management
Early stages of HIV associated neuropathies
AIDP and CIDP
Facial nerve palsy
Intermediate stages of HIV associated neuropathies
Multiple mononeuropathy
Late stages of HIV associated neuropathies
Distal symmetrical polyneuropathy
Lumbosacral polyradiculopathy
Autonomic neuropathy
Any stages of HIV associated neuropathies
Mononeuropathies
Herpes zoster radiculitis
Potential etiologies of neurologic complications of HIV-infected individuals
Direct effect of the virus
Opportunistic infection
Direct effect of malignancy
Iatrogenic effect of drug therapy
Malnutrition and weight loss
Vitamin deficiency
Autoimmune process directed at the nerve or vasa navorum
Diabetes mellitus, renal failure, or other concurrent condition
Other/unknown
Usually affects the upper trunk of the brachial plexus
May be idiopathic or can occur after an antecedent illness, immunization, or surgery
Brachial Plexitis or Parsonage-Turner Syndrome (neuralgic amyotrophy)
Usually begins as neck, shoulder, or upper arm pain that is deep, or aching in character
Pain can evolve over hours to days and can be very severe
The patient has a tendency to hold the affected arm close to the body and flexed at the elbow
After several days, sensory loss and weakness begin to appear
Profound muscle atrophy may occur
Brachial Plexitis or Parsonage-Turner Syndrome (neuralgic amyotrophy)

pain subsides after 3 to 6 wks

sensory loss and weakness take longer--months

long term deficits may persist

Txt is supportive
;