Term
Wallerian Degeneration
Describe, including times which landmark events occur |
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Definition
Injury -- weakness and snesory loss is immediate
Excitability is lost in 1 week
Denervation potentials occur around 2 weeks
But regeneration begins as early as 24 hours following injury
Axon distal to the injury site degenerates, and the associated target muscle tissue is denervated --> neurolemma doesnt degenerate, but remains as a hollow tube -->
24-96 hrs after injury, proximal end of axon sprouts out toward the tube, attracted by GF's produced by schwann cells assoc. w/ the neurolemma |
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Term
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Definition
When there is injury to an axon and the distal portion degenerates, the longest fibers are always affected first |
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Term
Neuronopathy/Gangliopathy
What is it?
What are 2 causes of sensory neuropathy?
1 cause of Motor neuropathy? |
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Definition
Its the loss of nerve cell bodies
Sensory neuropathy: via toxins or paraneoplasic mech
Motor neuropathy: poliomyelitis |
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Term
What is Segmental Demyelination?
3 causes?
How may secondary demyelination occur? |
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Definition
Def: myelin sheath or schwann cells are damaged
Causes could be:
Immune
Metabolic
Hereditary
Secondary myelination eg: viral infection causes immune activation, potentially resulting in cross-reactivity and/or inflam that ultimately damages and destroys the myelin
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Term
Diagnostic Clues of Neuropathy in Patient History
Positive Symptoms: Define these terms:
1. Dysesthesia
2. Paresthesia
3. Allodynia
4. Neuropathic Pain
Name 2 diagnostic negative symptoms
3 other diagnostic aspects obtained from clinical interview? |
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Definition
Positive symptoms:
1. unpleasant abnormal sense of touch
2. tingling/numbness
3. pain due to a stim that does not normally provoke pain
4. chronic pain from NS injury
Neg:
Motor weakness
Sensory loss
Other Diagnostic clues:
Family Hx
Systemic Symptoms
Acute vs. Chronic onset |
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Term
Diagnostic Clues on Examination
Describe 5 clues |
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Definition
1. Anatomical Pattern of localization
mononeuropathy
mononeuropathy multiplex - simultaneous or sequential involvement of individual noncontiguous nerve trunks, evolving over time, presents with acute or subacute loss of sensory/motor function of individual peripheral nerves
polyneuropathy - neurological disorder where many peripheral nerves throughout the body malfunction simultaneously
2. Motor loss vs. sensory loss vs. both
3. Proximal weakness vs. distal vs. both
4. Sensory Distribution
Glove-Stocking = sharply demarcated loss of sensory function (seen in distal diabetic neuropathy)
vs. more patchy loss
5. Small fiber affected vs. large fiber vs. both |
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Term
What is Charcot-Marie-Tooth disease?
(prevalence in the US?
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Definition
- Charcot-Marie-Tooth disease (CMT) is one of the most common inherited neurological disorders, affecting approximately 1 in 2,500 people in the United States.
- CMT, also known as hereditary motor and sensory neuropathy (HMSN) or peroneal muscular atrophy, comprises a group of disorders that affect peripheral nerves.
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Term
What are the symptoms of Charcot-Marie-Tooth disease?
When is typical onset of symptoms?
How variable is the severity?
Progression of the disease is rapid/gradual?
Describe the range of pain and motor function
Life expectancy?
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Definition
- Weakness of the foot and lower leg muscles, which may result in foot drop and a high-stepped gait with frequent tripping or falls.
- Foot deformities, such as high arches and hammertoes (a condition in which the middle joint of a toe bends upwards) are also characteristic due to weakness of the small muscles in the feet.
- Lower legs may take on an "inverted champagne bottle" appearance due to the loss of muscle bulk.
- Later in the disease, weakness and muscle atrophy may occur in the hands, resulting in difficulty with fine motor skills.
Onset of symtoms:
- most often in adolescence or early adulthood, but may be delayed until mid-adulthood.
- Severity of symptoms is quite variable in different patients and even among family members with the disease.
- Progression of symptoms is gradual.
- Pain can range from mild to severe, and some patients may need to rely on foot or leg braces or other orthopedic devices to maintain mobility.
- Although in rare cases patients may have respiratory muscle weakness, CMT is not considered a fatal disease and people with most forms of CMT have a normal life expectancy.
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Term
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Definition
CMT is caused by mutations in genes that produce proteins involved in the structure and function of either the peripheral nerve axon or the myelin sheath. Although different proteins are abnormal in different forms of CMT disease, all of the mutations affect the normal function of the peripheral nerves. Consequently, these nerves slowly degenerate and lose the ability to communicate with their distant targets.
The gene mutations in CMT disease are usually inherited, can be AD, AR, or X-linked. |
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Term
How is Charcot-Marie-Tooth disease diagnosed?
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Definition
Patient history, Family history, Neurological examination
Neurological examination:
- look for evidence of muscle weakness in the arms, legs, hands, and feet
- decreased muscle bulk,
- reduced tendon reflexes
- sensory loss.
- Look for evidence of foot deformities, such as high arches, hammertoes, inverted heel, or flat feet.
- (A specific sign that may be found in patients with CMT1 is hypertrophic nerves that may be felt or even seen through the skin)
Other Diagnostics:
- Nerve conduction studies and electromyography (EMG)
- Peripheral Nerve Biopsy - (CMT1 shows abnormal, onion bulb myelination; CMT2 shows axon degeneration)
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Term
What is Guillain-Barré Syndrome?
(Whats another name for it?)
Describe its symptoms and progression
What % of cases display facial paralysis?
Prognosis?
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Definition
Aka Acute Inflammatory Demyelinating Polyridiculoplathy: Imflammation and demyelination of peripheral nerves and motor fibers of ventral roots due to autoimmune attack
- sensory effect is less severe than motor defects
- GBS causes symmetric ascending muscle weakness beginning in distal lower extremities
Progression of Symptoms:
- First - weakness & parasthesia of the legs.
- Often spreads to the arms and upper body
- Can progress to almost total paralysis
- Autonomic function may be severely affected - life threatening if it begins to affect breathing, HR, or BP (can cause hyper or hypotension)
- (resp support is critical until recovery; additional treatment may include:
- plasmapheresis -removal, treatment, and return of blood components from circulation
- IV immunoglobulins)
- 50% of cases display facial paralysis
Almost all patients survive, and the majority recover completely after weeks/months
(symptoms can progress over the course of hours, days, or weeks. Most people reach the stage of greatest weakness within the first 2 weeks after symptoms appear, and by the third week of the illness 90 percent of all patients are at their weakest)
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Term
Prevalence of Guillian-Barre' Syndrome |
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Definition
- Guillain-Barré syndrome can affect anybody
- Can strike at any age and both sexes are equally prone to the disorder.
- The syndrome is rare, however, afflicting only about one person in 100,000.
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Term
What causes Guillain-Barré syndrome?
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Definition
We don't know why this non-contagious autoimmunity disease strikes some people and not others, nor do we know what sets the disease in motion, but it is associated w/ resp/GI viral infections, and occasionally surgery or vaccinations will trigger GBS
When Guillain-Barré is preceded by a viral or bacterial infection, it is possible that the virus has changed the nature of cells in the nervous system so that the immune system treats them as foreign cells. It is also possible that the virus makes the immune system itself less discriminating about what cells it recognizes as its own, allowing some of the immune cells, such as certain kinds of lymphocytes and macrophages, to attack the myelin. Sensitized T lymphocytes cooperate with B lymphocytes to produce antibodies against components of the myelin sheath and may contribute to destruction of the myelin. |
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Term
How is Guillain-Barré syndrome diagnosed?
(provide clinical indicators of GBS)
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Definition
Guillain-Barré is called a syndrome rather than a disease because it is not clear that a specific disease-causing agent is involved. A syndrome is a medical condition characterized by a collection of symptoms (what the patient feels) and signs (what a doctor can observe or measure).
Clinical Indicators of GBS:
- Major Indicator: Increased protein in the CSF with normal cell count (this is known as albuminocytologic dissociation)
- This increased protein can lead to papilledema
- symptoms are generally symmetrical in GBS
- The quickness with which the symptoms appear is indicative of GBS over other neuro/muscular disorders
- reflexes such as knee jerks are usually lost in GBS
- Nerve conduction velocity (NCV) test will show signals are traveling slower
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Term
What is Diabetic Neuropathy? |
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Definition
A peripheral nerve disorder caused by diabetes or poor blood sugar control
(The most common types of diabetic neuropathy result in problems with sensation in the feet. It can develop slowly after many years of diabetes or may occur early in the disease. The symptoms are numbness, pain, or tingling in the feet or lower legs. The pain can be intense and require treatment to relieve the discomfort. The loss of sensation in the feet may also increase the possibility that foot injuries will go unnoticed and develop into ulcers or lesions that become infected.) |
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Term
Is there any treatment to Diabetic Neuropathy?
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Definition
The goal of treating diabetic neuropathy is to prevent further tissue damage and relieve discomfort.
- First, bring blood sugar levels under control by diet and medication.
- Taking special care of the feet by wearing proper fitting shoes and routinely checking the feet for cuts and infections.
- Analgesics, low doses of antidepressants, and some anticonvulsant medications may be prescribed for relief of pain, burning, or tingling.
- (Some individuals find that walking regularly, taking warm baths, or using elastic stockings may help relieve leg pain.)
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Term
Prognosis for Diabetic Neuropathy |
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Definition
Depends largely on how well the underlying condition of diabetes is handled.
- Treating diabetes may halt progression and improve symptoms of the neuropathy, but recovery is slow.
- The painful sensations of diabetic neuropathy may become severe enough to cause depression in some patients.
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Term
What is Vasculitis Syndromes of the Central and Peripheral Nervous Systems ?
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Definition
Vasculitis is an inflammation of the vascular system, which includes the veins, arteries, and capillaries. Researchers think that inflammation is due to a faulty immune system response. Vasculitis can cause problems in any organ system, including the central (CNS) and peripheral (PNS) nervous systems. Vasculitis disorders, or syndromes, of the CNS and PNS are characterized by the presence of inflammatory cells in and around blood vessels, and secondary narrowing or blockage of the blood vessels that nourish the brain, spinal cord, or peripheral nerves.
Symptoms include:
- Headaches, especially a headache that doesn’t go away;
- fever;
- rapid weight loss;
- confusion or forgetfulness leading to dementia;
- aches and pains in the joints and muscles;
- pain while chewing or swallowing;
- paralysis or numbness, usually in the arms or legs;
- visual disturbances, such as double vision, blurred vision, or blindness
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Term
Is there any treatment for CNS/PNS Vasculitis Syndrome?
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Definition
Treatment for a vasculitis syndrome depends upon the specific diagnosis.
- Most of the syndromes respond well to steroid drugs, such as prednisolone.
- Some may also require treatment with an immunosuppressive drug, such as cyclophosphamide.
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Term
Prognosis for CNS/PNS Vasculitis? |
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Definition
Depends on the specific syndrome, but most are fatal if left untreated |
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