Peripheral Nervous System Disorders
Neuropathies, Guillain-Barre Syndrome & Amyotrophic Lateral Sclerosis (ALS)
Neuropathies
- Disorder of the nerves
- Symptoms can vary because of the type of nerves affected & the location of the nerves
- Clinical Manifestations: can be weakness, numbness, pins & needles, burning pain (sensory nerve damage), muscle weakness, cramps & spasms (motor nerve damage)
- May be acute or chronic.
- Causes of acute neuropathies are: drugs, infections, autoimmune reactions & toxins
- Chronic neuropathies can be caused by Diabetes Mellitus, ETOH abuse & connective tissue diseases
Generalized symmetric polyneuropathies
Neuropathy that is in the same areas on both sides of the body
Guillain-Barre Syndrome
- Also known as Acute idiopathic polyneuropathy
- An inflammatory demyelinating disease of the peripheral nervous system
- Acute onset with ascending motor paralysis (starts in lower extremities and goes up- it is a lower motor neuron disorder)
- Some causes are: viral infections & influenza vaccine
- Causes demyelination of the peripheral nerves
- Damages the myelin sheath
Clinical Manifestations
- progressive ascending weakness or paralysis in legs (usually begins in legs then goes up to arms & face), can not stand or walk.
- Some patients become bedridden, Respiratory failure (some patients may have to be intubated)
Diagnosis
Diagnosis is made by: patient history, nerve conduction tests & physical examination
Treatment
- Treatment supportive care. Most patients will recover from this in the first year
Amyotrophic Lateral Sclerosis (ALS) “Lou Gehrig Disease”
- Affects the upper & lower motor neurons of the cerebral cortex, brain stem & spinal cord (corticospinal tracts & anterior roots- these neurons start to die)
- Progressive disease that leads to respiratory failure & death
- Higher incidence in men between the ages 40-60
- Patients have normal intellectual and sensory function
Clinical Manifestations
- Early symptoms may be muscle twitching, cramping & stiffness in the hands or upper extremities
- The patient will develop severe weakness with loss of voluntary movement
- This progresses into respiratory failure than death.
Diagnosis
- Electromyography (EMG), nerve conduction studies
- MRI & laboratory testing
- At this time, Riluzole (Rilutek), a glutamate inhibitor, is the only FDA approved treatment and may only prolong life several months & delay the need for ventilator assistance
- Patient’s signs & symptoms
References
McCance, K. L., Huether, S. E., Brashers, V. L., & Rote, N. S. (2010). Pathophysiology: The biologic basis for disease in adults and children (6th ed.).
Dunphy, L.M., Winland-Brown, J. E. (2011). Primary Care: The Art and Science of Advanced Practice Nursing. Philadelphia, PA. F.A. Davis.
Uphold, C.R., & Graham, M.V. (2013). Clinical guidelines in family practice. (5th ed.) Gainesville, Fl.: Barmarrae Books, Inc.