Guillain Barre Syndrome

Guillain Barre Syndrome (GBS) is a rare autoimmune disorder in which our body's immune system attacks the protective covering, the myelin sheath, that surrounds the axon of many peripheral nerves resulting to axonal degeneration. The resultant inflammation and demyelination of the nerves decreases the nerve conduction velocity leading to weakness or sometimes paralysis of the affected muscles. This disorder predominantly affect the motor fibers.  GBS is known from other names: 

• Acute Inflammatory demyelinating polyneuropathy
• Landry's Ascending Paralysis

Main Types: 

1. Acute Inflammatory Demyelinating Polyneuropathy (AIDP)

• The most common type of GBS, particularly in the US. The myelin sheath of the nerves is damage preventing signals to travel to the brain causing weakness, paralysis, or numbness. It is characterized by motor weakness in a distal to proximal progression.

2. Acute Motor Axonal Neuropathy (AMAN)

• It is the same with AIDP, but purely motor and has no sensory symptoms. Usual cases occur during pediatric age. 

3. Acute Motor-Sensory Axonal Neuropathy (AMSAN)

• A severe form of GBS to include both motor and sensory symptoms. It is more prevalent in Asia and in North and South America. Typically, seen during adulthood.

4. Miller Fisher Syndrome

• This type of GBS is more common in Asia. Paralysis starts in the eyes.
• Double vision, loss of balance, and unsteady gait are some features.

Causes:

The exact etiology is unknown. It is often preceded by previous respiratory infection, stomach flu, viral infection (Influenza), vaccination, or surgery.

Symptoms:

The first symptoms is rapid onset of symmetrical muscle weakness and sometimes paralysis or an abnormal sensation in the legs that travel up the limb from fingers and toes towards the upper extremities and head. GBS symptoms usually last for a few weeks, usually peaks within 2 to 4 weeks. Severe cases involving the respiratory muscles that interfere with breathing require immediate medical attention. Other findings may include:

1. Deep muscle pain/Severe nerve pain 

2. Absence of deep tendon reflexes - Knee jerk

3. Breathing problems such shortness of breath and dyspnea on exertion

4. Heart and blood pressure problems such as paroxysmal hypertension and orthostatic hypotension.
5. Coordination and balance difficulties 
6. Blurred or double vision

7. Bowel and bladder weakness

8. Swallowing, chewing, or speaking difficulties(slurred speech)

Risk factors:

1. This disorder can occur at any age. Usually common to young adults and older adults starting at their 50's.

2. It can affect both sexes, but slightly greater in male population. 

3. Conditions associated with GBS include:

• Viral infection - HIV, Influenza
• Bacterial infection - Campylobacter
• Mycoplasma Pneumonia
• Cytomegalovirus
• Epstein-Barr Syndrome
• Surgery
• Vaccination

Diagnosis:

1. Lumbar puncture - Cerebrospinal fluids contain high protein with little or no lymphocytes.

2. Electromyography measures muscle response or electrical activity in response to stimulation of the nerves of the muscles to determine neuromuscular problems.

3. Nerve Conduction Studies measures the speed of conduction of an electrical impulse through a nerve. 

4. Physical and Neurologic examination

5.  Muscle Strength Testing

6. Medical History/Surgery

Treatment:

There is no known cure for GBS, but still there are several treatment to lessen the effects of symptoms and to help speed up recovery. Most patient may recover from GBS, while some of them may still experience weakness and fatigue. Immediate hospitalization is highly recommended to receive appropriate treatment and a good chance of recovery.

A. Pharmacologic Approach

1. Plasma Exchange (Plasmapheresis)

• A blood-cleansing procedure in which the whole blood is withdrawn and the liquid portion, the plasma, is separated. The affected plasma is replaced with a new plasma, or a  plasma substitute and then transfused back to the person.

2. Intravenous immunoglobulin (IVIGs) help improve immune system.

3. Analgesics, such as NSAIDs or Acetaminophen with added narcotics, is usually recommended for pain relief. Corticosteroids are not recommended in GBS.

B. Cardiac Monitoring for arrhythmias, heart rate, postural hypotension.

C. Mechanical Ventilation during the acute stage to assist breathing problems.

D. Physical Therapy

Rehabilitation will help to:
1. Regain independence and quality of life.

2. Improve balance, posture, and coordination

3. Promote relaxation

4. Promote recovery

5. Increase muscle strength and endurance

6. Increase joint mobility and flexibility.

The rehabilitation is a team effort among physical, occupational and speech therapists, nurse/caregiver, and family. Physical therapy management include:

• Respiratory care to include regular breathing exercise and airway clearance.
• Passive range of motion exercises to prevent joint stiffness.
• Light exercises may be allowed but should avoid overexertion and fatigue to avoid exacerbation of symptoms.
• Resting splints to support the wrist/hands and ankles.
• As the patient condition improves and is out of bed, approach on increasing the circulation is important. Strengthening and endurance exercises may be given as tolerated.
• Swelling is likely to develop in the ankles and hands if it remained immobile.
• Monitor the blood pressure as well as the possibilities of having orthostatic hypotension.
• Compression stocking helps with swelling, blood clots, and achy leg.
• Abdominal binder help support weak abdominal muscles.
• A shoulder joint support will also help prevent shoulder dislocation or subluxation.
• A wheelchair for functional mobility is also prescribed to help patient to move around.
• Gait training to regain independence in walking.
• Education and home program.

Gouty Arthritis

Gout is an inflammatory form of arthritis that develop from excess build up of uric acid in the tissues and blood because of too much production and/or the inability of the kidneys to remove them. These needle-like uric acid crystals deposited in the joints which in turn leads to a sudden, severe episodes of pain, tenderness, swelling, redness, warmth and stiffness of joints. In addition, it may also lead to kidney stone formation, tophi deposits in the skin and other tissues, or urate nephropathy.

The most common affectation is the joint at the base of the big toe. It will also affect many other joints together with or without big toe involvement. Gout typically occurs in the middle age, usually over the age of 40. It is more common in men and in persons with diet problem due high standards of living. Women are usually affected after menopause.





I. Stages of Gout

1. Asymptomatic Hyperuricemia

• This stage appears with higher uric acid level in the blood, but does not manifest  any symptoms. Treatment is usually not required.

2. Acute Gout

• With existing hyperuricemia, a gout attack  is triggered after a series of alcohol or drug intake, eating too much high-purine food such as liver, dried beans and peas. Pain and inflammation usually occurs at night and may last for a few days up to 10 days.

3. Interval Gout

• This stage shows no more symptoms, but gout is still there. This is the ideal time to manage gout to prevent future attacks.

4. Chronic Gout

• The uric acid remained at high level over a long time. Gout attack is more frequent and pain to last longer compared to earlier attacks.

II. Causes:

Uric acid are normally formed by our body and stays in our bloodstream in a dissolved form and end up being flushed out by the kidneys. Having too much uric acid in our blood (hyperuricemia) create a condition called gout. A high level of uric acid or the inability of our kidneys to remove them quickly, uric acid crystals begin to form and deposited in the joints. A longstanding hyperuricemia create tophi (tophus) formation. Tophi are large deposits of uric acid crystals, in the form of monosodium urate crystals, at the surface of joints, or in skin or cartilage.

III. Risk Factors:

1. Beverages, such as tea, coffee, may increase the level of uric acid. Drinking too much alcoholic drinks and fructose-rich sodas increases the risk.
2. Diet of high-purine content, such as red meat, internal organs, seafood, oily fish, dried pea and beans, lentils, or spinach, produces high level of uric acid.
3. Obesity or being overweight increases the risk of developing gout even at younger age.
4. Starvation and dehydration.
5. Certain medical conditions or illness, such as kidney failure, rapid weight loss, polycythemia, high blood pressure, and lead poisoning.
6. Regular intake of aspirin or diuretics.
7. Bypass surgery.
8. Family history of gout.
9. Age and gender. Most common in men over the age of 40. Women are usually affected after menopausal.

IV. Symptoms:

1. Sudden onset of a warmth, red, extreme pain and tenderness, swollen joints, usually the big toe joints (Podagra). Gouty attack usually happen during nighttime.
2. Usually it affect one joint at a time, but may involve other joints (heels, knees, wrist and fingers) if no treatment made.
3. First gout attack stop spontaneously within 1 to 2 weeks even without intervention.
4. With time, gout attack is more frequent and to last longer.
5. At the beginning, it will only involve one or two joints and then progress to affect multiple joints.
6. Tophi formation in skin and other tissues.

V. Diagnosis:

1. A detailed medical history and physical examination. This include:

• Diet
• Medications
• Intensity and causes of pain
• Presence of other symptoms
• Duration and description of attack
• Joints involved

2. Blood Test

• This measure the level of uric acid in the blood.

3. Joint Aspiration

• The most important diagnostic tool. This procedure is by extracting fluid from the affected joint for testing under a microscope. Presence of uric acid crystals suggest gout.

4. X-ray, Ultrasound , CT Scan, or a MRI to look at soft tissues involvement.

VI. Treatment:

Home Management:

1. Take the prescribed medicines, the antiinflamatory drugs during flare-up. Don't take aspirin or diuretics it may only aggravate the symptoms.
2. Elevate leg above the heart level.
3. Use ice packs to alleviate pain and swelling.
4. Use an assistive walking device to lessen weight-bearing on the affected joints.
5. Splinting to immobilize the affected joint(s) is helpful.
6. Stay hydrated.
7. Take a rest to relieve stress.

Medications:

Medicines are given during acute attack as well as to prevent future attack. Some of these medicines may work effectively on some people and some of them may just give problems from side effects. To stay safe, it is still best to call and see your doctor.

1. Nonsteroidal anti-inflammatory drugs (NSAIDs), such as indomethacin, ibuprofen, naproxen for pain and inflammation during acute attack.
2. Colchicine is an alternative medication. Side effects may include abdominal cramps and nausea.
3. Corticosteroids help control inflammation. Usually given if NSAIDs is not available or contraindicated. Administered either orally or directly injected to the joint.
4. Allopurinol and Probenecid both help to lower uric acid level and prevent future attack.
5. Febuxostat is another choice of medication to lower uric acid level.

Physical Therapy:

A management program formulated towards the maintenance or improvement of ROM, strength & endurance, and function. Physical therapy with medications is helpful for gouty sufferer. Begin with low-impact exercises and slowly progress to a more strenuous exercises as tolerated. Stop the exercise if it causes unusual, uncomfortable pain because this might to lead to injury or worsening of the symptoms. Avoid doing any exercises during flare-up instead do it in between flare-up.








Benefits of Regular Exercise:

1. Improve circulation.
2. Improve mobility and flexibility of joints.
3. Improve muscle strength and endurance.
4. Improve posture and balance.
5. Reduce stress and improve general well-being.
6. Alleviate pain and inflammation.
7. Maintain a healthy weight.
8. Lower the risk of gout.
9. Lower the risk of cardiac and pulmonary problems.

Management:

1. Modalities such as ice or a cold compress may help during flare-up to alleviate pain and inflammation.
2. Use a walking aid devices (a cane, walker, or crutches) to reduce weight-bearing of the painful joint and to help in maintaining a normal gait. Limping or incorrect gait may put stress on the other joints.
3. Rest and elevation of the affected joint during acute attack.
4. Range of motion and gentle stretching exercises improve flexibility and mobility of joints. A simple, light self-stretching help a lot to reduce joint stiffness and increase muscle blood flow.
5. Strengthening and endurance exercises, using a light dumbbell, will help your muscles to stay in shape. Slowly progress the exercises as tolerated.
6. Proprioception exercise keep up the joint sense of position.
7. Cardiovascular training recommended to keep a healthy lungs, heart, and circulation. Walking, swimming, and jogging are some of the activities that improve overall fitness.
8. Lifestyle change, such as diet control and avoiding other risk factors, is to consider.