Guillain Barre Syndrome

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Functional Independence in daily activities is essential to health and well-being. Rehabilitation aim at improving the quality of life of clients by enhancing functional performance capabilities and promoting independence in activities of daily living skills. There are various neurological diseases that can hamper or restrict us from being functionally independent in performance areas of ADL, work and lesiure. One of the most disabiling condition in current world scenario is Guillain Barre Syndrome (GBS).

Guillain Barre Syndrome (GBS) also called as Acute Inflammatory Demyelinating Polyneuropathy (AIDP) which affects 2 per 1,00,000 population per year. Guillain Barre Syndrome (GBS) is an acute autoimmune mediated disorder of the peripheral nervous system with a wide range of clinical symptoms. It is the most common form of neuromuscular paralysis, affecting people of any age leading to long term residual disability in some cases.

Characteristically, it occurs 1 -3 weeks after a viral or other infection or immunization. The condition may follow viral infections, e.g varicella-zoster, mumps and cytomegalovirus. It is also associated with Mycoplasma, Campylobacter infections, immunization with both live and dead vaccines, antitoxins, trauma, surgery and rarely, malignant disease and immunodeficiency. Both antibody and cell-mediated reactions to peripheral nerve myelin are involved. Segmental demyelination results with secondary axonal damage if the process is severe.

SYMPTOMS of GBS :

• It can strike anyone without warning regardless of gender, age, or ethnic background. GBS is an autoimmune peripheral neuropathy that can affect motor, sensory, and autonomic nerves as well as spinal roots resulting in acute neuromuscular paralysis. 
• Sensory symptoms predominate at the beginning with paresthesias or tingling sensations of the feet, then hands. Also decreased ability to perceive deep pain sensations and loss of kinesthetic and joint position sense creates an ataxic gait thus increasing the risk of falling. 
• Pain, especially back pain, is an occasional initial symptom. 
• Weakness next develops (e.g. Difficulty climbing stairs, getting up from a chair, foot drop, slippage of chappals and cramping) - this may be generalized, proximal in distribution or commence distally and ascend. Cramping commonly occurs in calf muscles, hamstring muscles and gluteus maximus muscles. Patients experience weakness of upper limbs usually starting from distal joints (hand) followed by elbow musculature. Patients fine motor activities like buttoning, eating, tying hairs, writing are majorly affected in severe cases with axonal involvement.
• These impairments cause balance problems, postural instability, gait disturbances and increase in risk of falls of patients. Tendon reflexes are absent or diminished. In severe cases, respiratory and bulbar involvement occurs. Weakness is maximal 3 weeks after the onset. 

• Disability caused by GBS generally progresses over the course of a few days to four weeks, with weakness starting distally and ascending in a matter of hours to days. At the peak of the conditions progress, many patients experience flaccid paralysis of nearly all skeletal muscles, with talking, swallowing and breathing frequently affected. Tracheostomy/ ventilation is required in 20% of cases. Facial weakness is present is some extent in 50% of cases. Autonomic involvement – tachycardia, fluctuating blood pressure. 

GBS variants :

There are several variants presentation that will share some patterns such as monophasic time course, recovery, a probable similar immune pathogenesis and prognosis. The Clinical spectrum is composed of classical GBS (AIDP), Miller-Fisher Syndrome, acute motor axonal neuropathy (AMAN), acute sensory axonal neuropathy (AMSAN), pure sensory variant and pharyngeal-cervical-brachial variant.

Several subtypes of Guillain Barre Syndrome (GBS) are recognized:-

1. Acute inflammatory demyelinating polyneuropathy (AIDP) – adults affected more than children, recovery is rapid, anti-GM 1 antibodies (less than 50%), the first attack on the Schwann cell surface; macrophage activation and lymphocytic infiltration, variable secondary axonal damage.

2. Acute Motor Axonal Neuropathy (AMAN) - Children and young adult are more affected, recovery is rapid, anti-GD 1 antibodies are found. First attach is at motor nodes of Ranvier; macrophage activation occurs, few lymphocytes , frequent peri axonal macrophages; extent of axonal damage is highly variable.

3. Acute Motor Sensory Axonal Neuropathy (AMSAN) – mostly adults are affected, recovery is slow , is uncommon, often incomplete; is closely related to AMAN.

4. Miller Fischer syndrome (MFS) - adults and children most affected, is uncommon, ophthalmoplegia, ataxia, and areflexia present, anti-GQ 1 b antibodies (90%) present.

PATHOPHYSIOLOGY of GBS :

Inflammatory neuropathies are characterized by inflammatory cell infiltrates in peripheral nerves, roots and sensory and autonomic ganglia. In some, an infectious agent elicits the inflammatory responses; in others, immune mechanism is presumed to be the primary cause of inflammation. Numerous inherited and acquired pathological processes may affect the nerve roots (radiculopathy), the nerve plexuses (plexopathy) and /or the individual nerve (neuropathy). 

Immune – mediated neuropathies as occur in Guillain Barre Syndrome (GBS) also called as Acute inflammatory demyelinating polyneuropathy (AIDP) leads to inflammation and demyelination peripheral nerves (neuropathy). Following are the events seen on histopathology finding in case of inflammatory neuropathies as seen in Guillain Barre Syndrome (AIDP) - there is inflammation of peripheral nerves, manifested as perivenular and endoneurial infiltration by lymphocytes, macrophages and a few plasma cells. The invading inflammatory cells vary in number from a spense seeding of the perivenous spaces to large collection of mononuclear cells, disseminated throughout the entire nerve, segmented demyelination affecting peripheral nerves is the primary lesion, but damage to the axons is also characteristic, particularly when the disease is severe.

DIAGNOSIS and TREATMENT : 

Diagnosis is based on clinical history supported by CSF (CSF proteins are elevated, the cells are usually absent, but at 20% up to 50 cells/mm3 may be found), neurophysiological investigations (findings of Multifocal demyelination soon develop with slowing of motor conduction, conduction block and prolonged distal motor latencies). Consequently, most newly diagnosed patients are placed in an ICU for monitoring. Plasma exchange (PE) or a high dose of intravenous immune globulins (IVIG) often hastens recovery. Corticosteroids are not helpful for GBS, but frequently are used to treat CIDP. 

Fortunately, GBS has typically been self-limiting with improvement usually beginning spontaneously after weakness maximizes. The death rate is approximately 3 percent, and the recurrence rate is less than 5 percent. Most patients reach a full or nearly full recovery. Many patients will walk without aids after three months and experience only minor residual symptoms by the end of the first year following onset. Nevertheless, recovery can be extremely slow (stretching over the course of 6 months to 2 years or longer) and 5 to 20 percent of patients are left with significant residual symptoms that lead to long term disability and prevent a successful return to their prior lifestyle or occupation. Along with these impairments, pain and negative psychological thinking during admission phase can act as a demotivating factor for functional performance in GBS patients. 

REHABILITATION :

Therapeutic management of the movement deficits associated with Guillain Barre Syndrome (GBS) includes supportive management during acute phase, prevention of long term medical comorbidities during the acute through early recovery stages and rehabilitation throughout recovery. GBS patients faces various problems including gross and fine motor physical impairments which impact their basic and instrumental ADL activities and causing mental distress. 

Patients experience lower limb symptoms like weakness, loss of balance while walking or climbing stairs, difficulty in performing transition from sit to stand and vice versa, decrease in speed of walking, fear of fall present etc. Patients experience weakness of upper limbs usually starting from distal joints (hand) followed by elbow musculature. Patients fine motor activities like buttoning, eating, tying hairs, writing are majorly affected in severe cases with axonal involvement.

Recovery usually starts within two to four weeks after onset and lasting for about 12 weeks in most patients and has  favourable outcomes in the majority of cases. However, GBS recovery can be prolonged and therefore, early rehabilitation is necessary following medical stability to prevent or minimize long term complications are a must. Following hospital discharge long term issues may include loss of functional independence in performance areas like basic activities of daily living, instrumental activities of daily living, return to work and resumption of role within family and community. Impairments at the initial post-discharge examination include gait deficits, impaired static and dynamic standing balance, decreased bilateral ankle range of  motion, decreased bilateral lower extremity strength, decreased activity tolerance and endurance, and impaired coordination. Disorders of balance in GBS result from symmetrical muscle weakness (ranging from mild to severe muscle strength loss), sensory disturbances (dysesthesia, tingling, numbness, loss of joint position sense and kinaesthetic sense). All  these deficits increases the patients risk of fall and thus adversely affects their activity participation.

During rehabilitation, the primary goal of treatment is to help patient regain functional independence by regaining mobility. To achieve mobility in patients with peripheral neuropathies, balance, strength and posture are important components to be worked on. A key feature of our independence is mobility. Mobility is defined as the ability to independently and safely move oneself from one place to another. Mobility includes many types of tasks like the ability to stand up from the bed or  chair, to walk or run, and to navigate through often quite complex environments. 

In GBS due to nerve inflammation and degeneration, the patient suffer from loss of muscle mass and experience muscle weakness. Voluntary control is affected in GBS due to decrease in body's muscle strength, its motor recruitment capacity (peripheral nerve affected)  and muscle mass. Generation of force produces movements effective in controlling the body position in space. So building muscle mass and improving muscle control (concentric, eccentric and isometric) is important in these cases. For recovering GBS patients, strength training should be given, which includes resistive training, body-weight training, training with theraband and weighted cuffs.

The three primary peripheral sensory inputs contributing to balance and postural control are the bilateral receptors of the somatosensory system, visual system and vestibular system. Strengthening of these peripheral sensory systems of body, which helps in maintaining balance, should also be implemented in mobility training.

• The feet, ankles, knee, hip, back, neck all furnish useful information for balance maintenance. Somatosensation is the dominant sense for the upright posture control and is responsible for triggering an automatic postural response. 
• Orientation to the wider environment, primarily from vision, allows feed-forward, or anticipatory, postural adjustments.
• Detection of the head movements by the vestibular system and cervical somatosensory systems. Detection of body sway by somatosensory and peripheral visual system provides feedback for automatic postural response.

Maintaining our balance is as simple as keeping our center of gravity over our base of support, and that is where the balance strategies come in. Various balance strategies, i.e. ankle strategy, hip strategy and stepping strategy are used for body stabilization in multiple directions, including anteroposterior, mediolateral and also multi directional plane of motion. Using all three balance strategies effectively and in the correct sequence is an integral part of a balance training and fall prevention program.

Various intervention approaches including educational approach, remedial approach, adaptation approach, disability prevention approach should be implemented for upper-limb and lower-limb impairments. It includes :

• Provide patient and caregiver with education and training for the prevention of contractures, DVT and bedsores as well as proper positioning and expected course of disease.
• For preventing contractures and supporting muscles, splintage should be provided like ankle-foot orthosis (for foot drop), functional cock-up splint (for hand muscle paralysis), knuckle bender (to prevent clawing of hand), leaf spring ankle foot orthosis (to support foot drop).
• Initially active range of motion exercises  should be performed at  low repetitions and low resistance with frequent rest breaks is order to avoid injury or strain to muscles, tendon and joints. Progressive resistive exercises by increasing weights and repetitions within patient's tolerance level. 
• PNF diagonal pattern with and without weights. 
• Core muscle strengthening in supine,sitting like abdominal curl ups, back extension and bilateral pelvic bridging .
• Use of therapeutics equipment and machines like stationary cycling, inclined sanding, shoulder wheel, hand gym, medicine ball kicking in sitting. 
• Sit-to-stand activity, mini-squats and lunges with or without support to be given . Sit to stand with assistance of walker or by therapist. Improve standing tolerance. Stand to sit with  or assistance
• Sensory re-education should be given to patients with sensory impairment. For sensory loss issues, heavy joint compression, tapping and vibrations over the joints for proprioception feedback.
• Oro-motor exercises of facial muscles and tongue to be given for patients experience facial muscles weakness ( facial cranial nerve involvement). Various oro-motor daily activities like chewing, swallowing, smiling, whistling, blowing, biting, holding air in closed mouth, gargling to be practiced.
• For hand function weakness various adaptive devices like 
• built-up handle (for decreased grip and range), 
• universal cuff (for limited grip strength and dexterity), 
• replacing buttons with velcro straps or press buttons for dressing. Using t-shirt instead of shirts.
• Using mugs with large handle size. 
• ADL simulated activities related to upper limbs namely fine motor hand activities like cutting paper, dressing and folding clothes, writing should be practiced.
• Energy conservation techniques should be explained in home exercises program. Energy conservation refers to the way activities are performed in a way which will minimize fatigue, joint stress and pain. Patient performs any activity using extra efforts or learns to compensate or begin to use different strategies to complete the activity. This makes the patient to use wrong muscles, overstrain muscles and attain wrong postures which can lead to overuse fatigue, muscle or ligament injury, muscle soreness. Implementing energy conservation techniques (ECT) in daily living tasks, by patients, proves beneficial in completing any work with easy and less efforts. 

Balance training exercises should be progressed from simple to gradually increasing challenging complex activities with respect to patients improving capacity. 

• Exercises like spot marching, one leg standing, stepping in multi-direction, step-ups on stool, walking on dynamic surfaces like mattress or treadmills with and without support. 
• Facilitation of equilibrium reactions using wobble board and therapeutic ball by giving reach-out or object transfer activities and perturbations in all directions. 
• For ADL mimicking movements PNF diagonal patterns of both upper-limb and lower-limb was given followed by ADL activities related to lower limbs to be practiced like lower limb dressing, wearing shoes in standing, ball kicking, ball catching etc.

• Outdoor activities like stair-climbing or slope-walking in forward, backward and sideway direction etc. 
• Training progression or increasing difficult during the intervention period can be reached by reducing or manipulating sensory information, necessary to obtain balance and by adding movement to make the activity more dynamic. Visual information for example was disturbed by closing the eyes. Proprioceptive feedback was manipulated by standing on different unstable surfaces instead of normal over ground followed by one-leg ball balancing and rolling. 
• Challenges were further increased by performing few of above activities by visually blinding the patient, performing obstacles courses, adding of speed variation component during performance.

For more information about ' Balance systems of our body' and how it works to control our body, you can read the below blog. Click on the below link ⬇️

https://otpthealthcarerehab.blogspot.com/2020/07/balance-systems-of-human-body.html

Risk factor categories of fall due to LMN type muscle weakness includes intrinsic (person), extrinsic (environment) and behavioral (occupation). By considering the 3 risk factors, the fall prevention and intervention program will maximize the potential to impact positively on an individual’s ability to carry daily activities (occupational performance). 

Based on many studies, the improvement seen in muscle strength, mobility and functional independence in peripheral neuropathic patients after balance and strength training  might be because of :

• The positive changes or effect at the neurophysiological level on peripheral nerves, joint proprioception, muscle growth and recovery. 
• A limited number of studies on the human and animal have demonstrated that exercises stimulates endothelium dependent vasodilation and vascular endothelial growth factor expression, increasing endoneurial blood flow , oxygen and glucose to mitochondria to produce energy in a more efficient manner. 
• There has been some evidence of improvement in proprioception of the trunk, postural compensatory strategy between ankle and hip joints and improved rate of force development  through balance training. 
• Exercises with eye closed conditions aids in improving ankle strategy. 
• One more study discusses about neural adaptations within CNS i.e. adaptation in grey and white brain matter followed by decrease in excitability of spinal reflex.
• The combined effect of balance training and strengthening exercises may have stimulated the  endoneurial blood flow, due to facilitation of endothelial vascularity, and might have enhanced early recovery of neural structures which were damaged due to GBS. 
• Also, improved ankle, knee and hip strategy after balance training has led to facilitation and improvement of static and dynamic balance of these patient population.

Thankyou for reading !!!

Dr.Ashwini Sangar, Dr.Pallavi Khadse-Kolhe, Dr.Sheetal Tatar-Dhande