EFFICACY OF PHYSICAL THERAPY MANAGEMENT FOR GUILLAIN BARRÉ SYNDROME by regan rose

Efficacy of Physical Therapy Management for Guillain Barré Syndrome

Chapter 1 INTRODUCTION INTRODUCTION Guillain-Barré syndrome is a disorder in which the body's immune system attacks part of the peripheral nervous system. The first symptoms of this disorder include varying degrees of weakness or tingling sensations in the legs. In many instances, the weakness and abnormal sensations spread to the arms and upper body. These symptoms can increase in intensity until the muscles cannot be used at all and the patient is almost totally paralyzed. In these cases, the disorder is life-threatening and is considered a medical emergency. The patient is often put on a respirator to assist with breathing. Most patients, however, recover from even the most severe cases of Guillain-Barré syndrome, although some continue to have some degree of weakness. Guillain-Barré syndrome is rare. Usually Guillain-Barré occurs a few days or weeks after the patient has had symptoms of a respiratory or gastrointestinal viral infection. Occasionally, surgery or vaccinations will trigger the syndrome. The disorder can develop over the course of hours or days, or it may take up to 3 to 4 weeks. No one yet knows why Guillain-Barré strikes some people and not others or what sets the disease in motion. What scientists do know is that the body's immune system begins to attack the body itself, causing what is known as an autoimmune disease. Guillain-Barré is called a syndrome rather than a disease because it is not clear that a specific disease-causing agent is involved. Reflexes such as knee jerks are usually lost. Because the signals traveling along the nerve are slower, a nerve conduction velocity (NCV) test can give a doctor clue to aid the diagnosis. The cerebrospinal fluid that bathes the spinal cord and brain contains more protein than usual, so a physician may decide to perform a spinal tap. Guillain-Barre syndrome is a serious disorder that occurs when the body's defense (immune) system mistakenly attacks part of the nervous system. This leads to nerve inflammation that causes muscle weakness.Guillain-Barre syndrome is an autoimmune disorder (the body's immune system attacks itself). Exactly what triggers Guillain-Barre syndrome is unknown. The syndrome may occur at any age, but is most common in people of both sexes between ages 30 and 50.It often follows a minor infection, such as a lung infection or gastrointestinal infection. Most of the time, signs of the original infection have disappeared before the symptoms of Guillain-Barre begin.

The swine flu vaccination in 1976 may have caused rare cases of Guillain-Barre syndrome. However, the swine flu and the regular flu vaccines used today have not resulted in more cases of the illness.

Chapter 2 ANATOMY ANATOMY The anatomy of the brain is complex due its intricate structure and function. This amazing organ acts as a control center by receiving, interpreting, and directing sensory information throughout the body. There are three major divisions of the brain. They are the forebrain, the midbrain, and the hindbrain.

Brain Divisions The forebrain is responsible for a variety of functions including receiving and processing sensory information, thinking, perceiving, producing and understanding language, and controlling motor function. There are two major divisions of forebrain: the diencephalon and the telencephalon. The diencephalon contains structures such as the thalamus and hypothalamus which are responsible for such functions as motor control, relaying sensory information, and controlling autonomic functions. The telencephalon contains the largest part of the brain, the cerebral cortex. Most of the actual information processing in the brain takes place in the cerebral cortex. The midbrain and the hindbrain together make up the brainstem. The midbrain is the portion of the brainstem that connects the hindbrain and the forebrain. This region of the brain is involved in auditory and visual responses as well as motor function. The hindbrain extends from the spinal cord and is composed of the metencephalon and myelencephalon. The metencephalon contains structures such as the pons and cerebellum. This region assists in maintaining balance and equilibrium, movement coordination, and the conduction of

sensory information. The myelencephalon is composed of the medulla oblongata which is responsible for controlling such autonomic functions as breathing, heart rate, and digestion.

• • • • • • •

Fig.2.1 Cross section of Brain Prosencephalon - Forebrain Mesencephalon - Midbrain Diencephalon Telencephalon Rhombencephalon - Hindbrain Metencephalon Myelencephalon

Structures The brain contains various structures that have a multitude of functions. Below is a list of major structures of the brain and some of their functions. Basal Ganglia - Involved in cognition and voluntary movement - Diseases related to damages of this area are Parkinson's and Huntington's. Brainstem - Relays information between the peripheral nerves and spinal cord to the upper parts of the brain - Consists of the midbrain, medulla oblongata, and the pons

Broca's Area - Speech production - Understanding language Central Sulcus (Fissure of Rolando) - Deep grove that separates the parietal and frontal lobes Cerebellum - Controls movement coordination - Maintains balance and equilibrium Cerebral Cortex - Outer portion (1.5mm to 5mm) of the cerebrum - Receives and processes sensory information - Divided into cerebral cortex lobes Cerebral Cortex Lobes - Frontal Lobes -involved with decision-making, problem solving, and planning. -

Occipital Lobes-involved with vision and color recognition

Parietal Lobes - receives and processes sensory information

Temporal Lobes - involved with emotional responses, memory, and speech

Cerebrum - Largest portion of the brain -

Consists of folded bulges called gyri that create deep furrows

Corpus Callosum - Thick band of fibers that connects the left and right brain hemispheres Cranial Nerves -

Twelve pairs of nerves that originate in the brain, exit the skull, and lead to the head, neck and torso

Fissure of Sylvius (Lateral Sulcus) - Deep grove that separates the parietal and temporal lobes Limbic System Structures -

Amygdala - involved in emotional responses, hormonal secretions, and memory

Cingulate Gyrus - a fold in the brain involved with sensory input concerning emotions and the regulation of aggressive behavior

Fornix - an arching, fibrous band of nerve fibers that connect the hippocampus to the hypothalamus

Hippocampus - sends memories out to the appropriate part of the cerebral hemisphere for long-term storage and retrievs them when necessary

Hypothalamus - directs a multitude of important functions such as body temperature, hunger, and homeostasis

Olfactory Cortex - receives sensory information from the olfactory bulb and is involved in the identification of odors

Thalamus - mass of grey matter cells that relay sensory signals to and from the spinal cord and the cerebrum

Medulla Oblongata - Lower part of the brainstem that helps to control autonomic functions Meninges -

Membranes that cover and protect the brain and spinal cord Olfactory Bulb Bulb-shaped end of the olfactory lobe Involved in the sense of smell

Pineal Gland - Endocrine gland involved in biological rhythms - Secretes the hormone melatonin Pituitary Gland - Endocrine gland involved in homeostasis - Regulates other endocrine glands Pons - Relays sensory information between the cerebrum and cerebellum Reticular Formation - Nerve fibers located inside the brainstem - Regulates awareness and sleep Substantia Nigra - Helps to control voluntary movement and regulates mood Tectum - The dorsal region of the mesencephalon (mid brain) Tegmentum - The ventral region of the mesencephalon (mid brain). Ventricular System - connecting system of internal brain cavities filled with cerebrospinal fluid - Aqueduct of Sylvius - canal that is located between the third ventricle and the fourth ventricle - Choroid Plexus - produces cerebrospinal fluid

- Fourth Ventricle - canal that runs between the pons, medulla oblongata, and the cerebellum - Lateral Ventricle - largest of the ventricles and located in both brain hemispheres - Third Ventricle - provides a pathway for cerebrospinal fluid to flow Wernicke's area - Region of the brain where spoken language is understood

Chapter 3 PHYSIOLOGY PHYSIOLOGY

Immune system The immune system is a complex system that is responsible for protecting us against infections and foreign substances. There are three lines of defense: the first is to keep invaders out (through skin, mucus membranes, etc), the second line of defense consists of non-specific ways to defend against pathogens that have broken through the first line of defense (such as with inflammatory response and fever). The third line of defense is mounted against specific pathogens that are causing disease (B cells produce antibodies against bacteria or viruses in the extracellular fluid, while T cells kill cells that have become infected). The immune system is closely tied to the lymphatic system, with B and T lymphocytes being found primarily within lymph nodes. Tonsils and the thymus gland are also considered lymph organs and are involved in immunity. The lymphatic system and the immune system are terms that are used interchangeably to refer to the body's ability to defend against pathogens. The lymphatic system is comprised of three interrelated functions: (1) Removal of excess fluids, lymph, from body tissues, (2) Absorption of fatty acids and subsequent transport of fat, chyle, to the circulatory system and (3) Formation of white blood cells (WBCs), and initiation of immunity through the formation of antibodies, lending specific resistance to pathogens. Lymphatic Pathways

The lymphatic system acts as a secondary circulatory system, except it collaborates with white blood cells in lymph nodes to protect the body from being infected by cancer cells, fungi, viruses or bacteria. Unlike the circulatory system, the lymphatic system is not closed and has no central pump; the lymph moves slowly and under low pressure due to peristalsis, the operation of semilunar valves in the lymph veins, and the milking action of skeletal muscles. Like veins, lymph vessels have one-way, semilunar valves and depend mainly on the movement of skeletal muscles to squeeze fluid through them. Rhythmic contraction of the vessel walls may also help draw fluid into the lymphatic capillaries. This fluid is then transported to progressively larger lymphatic vessels culminating in the right lymphatic duct (for lymph from the right upper body) and the thoracic duct (for the rest of the body); these ducts drain into the circulatory system at the right and left subclavian veins.

Figure 3.1: lymph node of cell

Lymph

Lymph originates as blood plasma that leaks from the capillaries of the circulatory system, becoming interstitial fluid, filling the space between individual cells of tissue. Plasma is forced out of the capillaries by hydrostatic pressure, and as it mixes with the interstitial fluid, the volume of fluid accumulates slowly. Most of the fluid is returned to the capillaries by osmosis. The proportion of interstitial fluid that is returned to the circulatory system by osmosis is about 90% of the former plasma, with about 10% accumulating as overfill. The excess interstitial fluid is collected by the lymphatic system by diffusion into lymph capillaries, and is processed by lymph nodes prior to being returned to the circulatory system. Once within the lymphatic system the fluid is called lymph, and has almost the same composition as the original interstitial fluid. Oedema

Oedema is the swelling that forms when too much tissue fluid forms or not enough taken away. It can be caused by a variety of conditions such as allergic responses (too much vasodilation), starvation (lack of albumin in blood lowers osmotic pressure and decreases amount of fluid returning to capillaries), and lymphatic disorders (e.g. blockage due to parasite in elephantiasis, or removal of lymph nodes due to a radical mastectomy). Edema is common in the lower extremities when people spend a lot of time sitting, because the fluid return is based largely on the massaging action of skeletal muscles.

Lymphatic Vessels and Ducts

The lymphatic vessels are similar in structure to the cardiovascular veins, meaning they also have valves. They are dependent upon the contraction of skeletal muscle, respiratory movements and valves that do not allow backward flow. The vessels merge before entering one of two ducts. • •

Thoracic Duct: This duct is much larger than the lymphatic duct. It serves the abdomen, lower extremities and the left side of the upper body (head, neck, and arm) Right Lymphatic Duct: This duct serves all of the right side of the upper body and thoracic area (head, neck).

Organs, Tissues and Cells of the Immune System The immune system consists of a network of lymphatic organs, tissues, and cells. These structures are supported by the reticuloendothelial system: loose connective tissue with a network of reticular fibers. Phagocytic cells, including monocytes and macrophages, are located in the reticular connective tissue. When micro-organisms invade the body, or the body encounters antigens (such as pollen), antigens are transported to the lymph. Lymph is carried through the lymph vessels to regional lymph nodes. In the lymph nodes, the macrophages and dendritic cells phagocytose the antigens, process them, and present the antigens to lymphocytes, which can then start producing antibodies or serve as memory cells. The function of memory cells is to recognize specific antigens in the future. Primary Lymphatic Organs - The primary lymphatic organs are the red bone marrow and the thymus. They and are the site of production and maturation of lymphocytes, the type of white blood cell that carries out the most important work of the immune system. •

Red Bone Marrow Red bone marrow, the soft, spongy, nutrient rich tissue in the cavities of certain long bones, is the organ that is the site of blood cell production.

Some of the white blood cells produced in the marrow are: neutrophils, basophils, eosinophils, monocytes, and lymphocytes. Lymphocytes differentiate into B lymphocytes and T lymphocytes. Red bone marrow is also the site of maturation of B lymphocytes. T lymphocytes mature in the thymus.

Figure 3.2: primary lymphatic organ Side of thorax, showing surface markings for bones, lungs (purple), pleura (blue), and spleen (green) Thymus Gland - The thymus gland is located in the upper thoracic cavity posterior to the sternum and anterior to the ascending aorta. The thymus is an organ that is more active in children, and shrinks as we get older. Connective tissue separates the thymus into lobules, which contain lymphocytes. Thymic hormones such as thymosin are produced in the thymus gland. Thymosin is thought to aid in the maturation of T lymphocytes. The Thymus is critical to the immune system. Without a thymus, a person has no ability to reject foreign substances, blood lymphocyte level is very poor, and the body’s response to most antigens is either absent or very weak • Immature T lymphocytes travel from the bone marrow through the bloodstream to reach the thymus. Here they mature and for the most part, stay in the thymus. Only 5% of T lymphocytes ever leave the thymus. They only leave if they are able to pass the test: if they react with “self” cells, they die. If they have the potential to attack a foreign cell, they leave the thymus. Secondary Lymphatic Organs--The secondary lymphatic organs also play an important role in the immune system as they are places where lymphocytes find and bind with antigens This is followed by the proliferation and activation of lymphocytes. The secondary organs include the spleen, lymph nodes, tonsils, Preyer’s patches, and the appendix. holding a reservoir of blood.located in the upper left region of the abdominal, i

Figure 3.3: Brain cell structure .

Fig3.4: Structure of the lymph node. 1. Efferent lymphatic vessel 2. Sinus 3. Nodule 4. Capsule 5. Medulla 6. Valve to prevent backflow 7. Afferent lymphatic vessel. • Lymph Nodes - are small oval shaped structures located along the lymphatic vessels. They are about 1-25 mm in diameter. Lymph nodes act as filters, with an internal honeycomb of connective tissue filled with lymphocytes that collect and destroy bacteria and viruses. They are divided into compartments, each packed with B lymphocytes and a sinus. As lymph flows through the sinuses, it is filtered by macrophages whose function is to engulf pathogens and debris. Also present in the sinuses are T lymphocytes, whose functions are to fight infections and attack cancer cells. Lymph nodes are in each cavity of the body except the dorsal cavity. Physicians can often detect the body’s reaction to infection by feeling for swollen, tender lymph nodes under the arm pits and in the neck, because when the body is fighting an infection, these lymphocytes multiply rapidly and produce a characteristic swelling of the lymph nodes. •

Tonsils - are often the first organs to encounter pathogens and antigens that come into the body by mouth or nose. There are 3 pairs of tonsils in a ring about the pharynx.

•

Peyer’s patches - located in the wall of the intestine and the appendix, attached to the cecum of the large intestine, intercept pathogens that come into the body through the intestinal tract.

The nervous system is divided into two main parts: The central nervous system (CNS) is made of the brain and the spinal cord. The brain is enclosed inside the skull and the spinal cord is enclosed inside the vertebral column for protection. The peripheral nervous system (PNS) is made of nerves that branch from the CNS. The cranial nerves branch from the brain and supply areas in the head such as the eyes, the facial muscles, the ears, and the nose. The CNS functions mostly in gathering sensory information from nerves of the PNS, processing

this information, and then transmitting signals, again by the nerves of the PNS, to effectors, or target organs or tissues, to react to this sensory input All cells have membrance potential, but only certain kinds of cells-like neurons and muscle cells- have the ability to change their membrane potential. Collectively these cells are called excitable cells. The membrance potential of an excitable cell in a resting (unexcited) state is called resting potential. Cells can change their membrane potential in response to stimuli that can be received via gated ion channels. Stimuli can cause an electrical gradient to be conducted across the membrane, called hyperpolarization. Depolarization is a reduction in the electricial gradient across the membrane. In an excitable cell, such as a neuron, the response to a depolarizing stimulus is graded with stimulus intensity only up to a particular level of depolarization, called the threshold position. If a depolarization reaches the threshold, a different type of response called an action potential is triggered. It is important to note, however, that hyperpolarizing stimuli do not produce action potentials, but make it even more likely that an action potential will be triggered by making it more difficult for a depolarizing stimulus to reach threshold. The action potential, then, is the actual nerve impulse the cell transmits. The magnitude of the action potential is independent of the strength of depolarizing stimuli that triggers it. This whole sequence of events occurs in mere milliseconds.

Figure 3.5: Structure of the neuron Each muscle cell contains very thin fibers called myofibrils. Each myofibril is surrounded by a special type of endoplasmic reticulum called the sarcoplasmic reticulum. Each myofibril is divided alongside its length into units called sacromeres. These sacromeres contract and relax. They are made of alternating actin and myosin filaments. Actin is made of thin protein filaments and myosin of thick ones. Contraction of muscles results from the sliding of these two fibers past one another. Extending from the myosin to the actin filaments are tiny movable arms. When induced by ATP, these arms move forward slightly. When they do, they drag the actin fibers past the myosin fibers, thus causing muscle contraction. Sensory system A sensory system is a part of the nervous system responsible for processing sensory information. A sensory system consists of sensory receptors, neural pathways, and parts of the brain involved in sensory perception. Commonly recognized sensory systems are those for vision, hearing, somatic sensation (touch), taste and olfaction (smell). In short, senses are transducers from the physical world to the realm of the mind. The receptive field is the specific part of the world to which a receptor organ and receptor cells respond. For instance, the part of the world an eye can see, is its receptive field; the light that each rod or cone can see, is its receptive field.[1] Receptive fields have been identified for the visual system, auditory system and somatosensory system, so far. Stimulus Sensory systems code for four aspects of a stimulus; type (modality), intensity, location, and duration. Arrival time of a sound pulse and phase differences of continuous sound are used for localization of sound sources. Certain receptors are sensitive to certain types of stimuli (for example, different mechanoreceptors respond best to different kinds of touch stimuli, like sharp or blunt objects). Receptors send impulses in certain patterns to send information about the intensity of a stimulus (for example, how loud a sound is). The location

of the receptor that is stimulated gives the brain information about the location of the stimulus (for example, stimulating a mechanoreceptor in a finger will send information to the brain about that finger). The duration of the stimulus (how long it lasts) is conveyed by firing patterns of receptors.

Figure 3.6: Sensory system of brain Modality A stimulus modality (sensory modality) is a type of physical phenomenon that can be sensed. Examples are temperature, taste, sound, and pressure. The type of sensory receptor activated by a stimulus plays the primary role in coding the stimulus modality. In the memory-prediction framework, Jeff Hawkins mentions a correspondence between the six layers of the cerebral cortex and the six layers of the optic tract of the visual system. The visual cortex has areas labelled V1, V2, V3, V4, V5, MT, IT, etc. Thus Area V1 mentioned below, is meant to signify only one class of cells in the brain, for which there can be many other cells which are also engaged in vision. Hawkins lays out a scheme for the analogous modalities of the sensory system. Note that there can be many types of senses, some not mentioned here. In particular, for humans, there will be cells which can be labelled as belonging to V1, V2 A1, A2, etc.:

V1 (vision)

The human eye is the first element of a sensory system: in this case, vision, for the visual system. Visual Area 1, or V1, is used for vision, via the visual system to the primary visual cortex.

Ear A1 (auditory - hearing) Auditory Area 1, or A1, is for hearing, via the auditory system, the primary auditory cortex. Somatosensory Area 1, or S1, is for touch and proprioception in the somatosensory system. The somatosensory system feeds the Brodmann Areas 3, 1 and 2 of the primary somatosensory cortex. But there are also pathways for proprioception (via the cerebellum), and motor control (via Brodmann area 4).

Tongue

G1 (gustatory - taste) Gustatory Area 1, or G1, is used for taste. O1. (olfactory - smell) Olfactory Area 1, or O1, is used for smell. In contrast to vision and hearing, the olfactory bulbs are not cross-hemispheric; the right bulb connects to the right hemisphere and the left bulb connects to the left hemisphere.

Figure 3.7: Cranial nerve of brain

Sensory and Motor Both Origin Nuclei Function Cranial nerve zero (CN0 is not traditionally recognized.) Sensoryolfactory trigone, medial olfactory gyrus, and lamina terminalis New research indicates CN0 may play a role in the detection of pheromones Linked to olfactory system in human embryos[4] I Olfactory nerve

Purely SensoryTelencephalonAnterior olfactory nucleusTransmits the sense of smell; Located in olfactory foramina in the Cribriform plate of ethmoid II Optic Nerve Purely SensoryDiencephalonGanglion cells of retinaTransmits visual information to the brain; Located in optic canal III Oculomotor nerve Mainly MotorMidbrainOculomotor nucleus, Edinger-Westphal nucleusInnervates levator palpebrae superioris, superior rectus, medial rectus, inferior rectus, and inferior oblique, which collectively perform most eye movements; Also innervates m. sphincter pupillae, as well as the muscles of the ciliary body. Located in superior orbital fissure IV Trochlear nerve Mainly MotorMidbrainTrochlear nucleusInnervates the superior oblique muscle, which depresses, rotates laterally (around the optic axis), and intorts the eyeball; Located in superior orbital fissureVTrigeminal nerveBoth Sensory and MotorPonsPrincipal sensory trigeminal nucleus, Spinal trigeminal nucleus, Mesencephalic trigeminal nucleus, Trigeminal motor nucleusReceives sensation from the face and innervates the muscles of mastication; Located in superior orbital fissure (ophthalmic nerve - V1), foramen rotundum (maxillary nerve - V2), and foramen ovale (mandibular nerve - V3) VI Abducens nerve Mainly MotorPosterior margin of PonsAbducens nucleusInnervates the lateral rectus, which abducts the eye; Located in superior orbital fissure VII Facial nerve Both Sensory and MotorPons (cerebellopontine angle) above oliveFacial nucleus, Solitary nucleus, Superior salivary nucleusProvides motor innervation to the muscles of facial expression, posterior belly of the digastric muscle, and stapedius muscle, receives the special sense of taste from the anterior 2/3 of the tongue, and provides secretomotor innervation to the salivary glands (except parotid) and the lacrimal gland; Located and runs through internal acoustic canal to facial canal and exits at stylomastoid foramen VIII Vestibulocochlear nerve (or auditory-vestibular nerve or statoacoustic nerve) Mostly sensoryLateral to CN VII (cerebellopontine angle)Vestibular nuclei, Cochlear nucleiSenses sound, rotation and gravity (essential for balance & movement). More specifically. the vestibular branch carries impulses for equilibrium and the cochlear branch carries impulses for hearing.; Located in internal acoustic canal IX Glossopharyngeal nerve Both Sensory and MotorMedullaNucleus ambiguus, Inferior salivary nucleus, Solitary nucleusReceives taste from the posterior 1/3 of the tongue, provides secretomotor innervation to the parotid gland, and provides motor innervation to the stylopharyngeus. Some sensation is also relayed to the brain from the palatine tonsils. Sensation is relayed to opposite thalamus and some hypothalamic nuclei. Located in jugular foramen X Vagus nerve Both Sensory and MotorPosterolateral sulcus of MedullaNucleus ambiguus, Dorsal motor vagal nucleus, Solitary nucleusSupplies branchiomotor innervation to most laryngeal and all

pharyngeal muscles (except the stylopharyngeus, which is innervated by the glossopharyngeal); provides parasympathetic fibers to nearly all thoracic and abdominal viscera down to the splenic flexure; and receives the special sense of taste from the epiglottis. A major function: controls muscles for voice and resonance and the soft palate. Symptoms of damage: dysphagia (swallowing problems), velopharyngeal insufficiency. Located in jugular foramen XI Accessory nerve (or cranial accessory nerve or spinal accessory nerve) Mainly MotorCranial and Spinal RootsNucleus ambiguus, Spinal accessory nucleusControls sternocleidomastoid and trapezius muscles, overlaps with functions of the vagus. Examples of symptoms of damage: inability to shrug, weak head movement; Located in jugular foramen XII Hypoglossal nerve Mainly MotorMedullaHypoglossal nucleusProvides motor innervation to the muscles of the tongue (except for the palatoglossus, which is innervated by the vagus) and other glossal muscles. Important for swallowing (bolus formation) and speech articulation. Located in hypoglossal canal

Chapter 4 DEFINITION Definition AIDP (acute idiopathic demyelinating polyneuropathy), AIP(acute infective polyneuropathy), LGBSS(Landry-Guillain-Barre-Strohl syndrome),AIP(acute idiopathic polyneuropathy). Predisposing factor: Although there is no definite aetiology of GBS there are certain factors which have been found to predispose to the occurance of GBS.

1. Age: Common between 15 to 25 years of age. 2.Sex: Common in females. 3.Infection: Viral in the form of Epstein Barr Virus,bacterial in the form of mycoplasma pneumonia.

4.Drugs:

Prolonged use of antidepressant drugs like zimelidine gold therapy which are neurotoxins are found to cause GBS. 5.Autoimmune: Due to the presence of an antigen CD(+ve) T cells. 6.Ideopathic Whithout any known causes.

Chapter 5 CLASSIFICATION

Classification Six different subtypes of Guillainâ&#x20AC;&#x201C;BarrĂŠ syndrome exist: Acute inflammatory demyelinating polyneuropathy (AIDP) is the most common form of GBS, and the term is often used synonymously with GBS. It is caused by an auto-immune response directed against Schwann cell membranes. Miller Fisher syndrome (MFS) is a rare variant of GBS and manifests as a descending paralysis, proceeding in the reverse order of the more common form of GBS. It usually affects the eye muscles first and presents with the triad of ophthalmoplegia, ataxia, and areflexia. Anti-GQ1b antibodies are present in 90% of cases. Acute motor axonal neuropathy (AMAN),[1] also known as Chinese paralytic syndrome, attacks motor nodes of Ranvier and is prevalent in China and Mexico. It is probably due to an auto-immune response directed against the axoplasm of peripheral nerves. The disease may be seasonal and recovery can be rapid. Anti-GD1a antibodies[2] are present. Anti-GD3 antibodies are found more frequently in AMAN. Acute motor sensory axonal neuropathy (AMSAN) is similar to AMAN but also affects sensory nerves with severe axonal damage. Like AMAN, it is probably due to an autoimmune response directed against the axoplasm of peripheral nerves. Recovery is slow and often incomplete.[3] Acute panautonomic neuropathy is the most rare variant of GBS, sometimes accompanied by encephalopathy. It is associated with a high mortality rate, owing to cardiovascular involvement, and associated dysrhythmias. Impaired sweating, lack of tear formation, photophobia, dryness of nasal and oral mucosa, itching and peeling of skin, nausea, dysphagia, constipation unrelieved by laxatives or alternating with diarrhea occur frequently in this patient group. Initial nonspecific symptoms of lethargy, fatigue, headache, and decreased initiative are followed by autonomic symptoms including orthostatic lightheadedness, blurring of vision, abdominal pain, diarrhea, dryness of eyes, and disturbed

micturition. The most common symptoms at onset are related to orthostatic intolerance, as well as gastrointestinal and sudomotor dysfunction (Suarez et al. 1994). Parasympathetic impairment (abdominal pain, vomiting, obstipation, ileus, urinary retention, dilated unreactive pupils, loss of accommodation) may also be observed. Bickerstaff's brainstem encephalitis (BBE), is a further variant of Guillain–Barré syndrome. It is characterized by acute onset of ophthalmoplegia, ataxia, disturbance of consciousness, hyperreflexia or Babinski's sign. The course of the disease can be monophasic or remitting-relapsing. Large, irregular hyperintense lesions located mainly in the brainstem, especially in the pons, midbrain and medulla are described in the literature. BBE despite severe initial presentation usually has a good prognosis. Magnetic resonance imaging (MRI) plays a critical role in the diagnosis of BBE. A considerable number of BBE patients have associated axonal Guillain–Barré syndrome, indicative that the two disorders are closely related and form a continuous spectrum.

Chapter 6 INCIDENCE

INCIDENCE According to an epidemiologic survey, the average annual incidence of GBS in this subcontinent is 3.0 cases per 100,000 populations. In comparing age groups, the annual mean rate of hospitalizations in this subcontinent related to GBS increases with age, being 1.5 cases per 100,000 population in persons aged less than 15 years and peaking at 8.6 cases per 100,000 population in persons aged 70-79 years. International A widespread syndrome, GBS has been reported throughout the world. Most studies show annual incidence figures that are similar to those in the United States, without geographical clustering. Mortality/Morbidity In epidemiologic surveys, the overall death rate related to GBS ranges from 2-12% of patients. GBS-associated mortality rates increase markedly with age. In the United States, the case-fatality ratio ranges from 0.7% among persons younger than 15 years to 8.6% among individuals older than 65 years. Survey data has shown that in patients aged 60 years or older, the risk of death is 6-fold that of persons aged 40-59 years and is 157-fold that of patients younger than 15 years. Although the death rate increases with age in males and females, after age 40 years males have a death rate that is 1.3 times greater than that of females. GBS-related deaths usually occur in ventilator-dependent patients, resulting from such complications as pneumonia, sepsis, adult respiratory distress syndrome, and, less frequently, and autonomic dysfunction. Underlying pulmonary disease and the need for mechanical ventilation increase the risk of death, especially in elderly patients. Length of hospital stays also increases with advancing age, because of disease severity and associated medical complications. Race GBS has been reported throughout the international community. In North America, Western Europe, and Australia, most patients with GBS meet electrophysiologic criteria for

demyelinating polyneuropathy. In northern China, up to 65% of patients with GBS have axonal pathology. Sex A slight male preponderance is seen in most studies, especially in older patients. Age GBS has been reported in all age groups, with the syndrome occurring at any time between infancy and old age. In the United States, the syndrome's age distribution seems to be bimodal, with the incidence of GBS peaking in the elderly population and reaching its second-highest level in young adults. Infants appear to have the lowest risk of developing GBS.

Chapter 7 ETIOLOGY ETIOLOGY GBS is considered to be a post infectious, immune-mediated disease targeting peripheral nerves. Up to two thirds of patients report an antecedent illness prior to the onset of neurologic symptoms. Respiratory infections are most frequently reported, followed by gastrointestinal infections. In several studies, C jejuni was the most commonly isolated pathogen. Serology studies in a Dutch GBS trial identified 32% of patients as having had a recent C jejuni infection, while studies in northern China documented infection rates as high as 60%. Gastrointestinal and upper respiratory tract symptoms can be observed with C jejuni infections. C jejuni infections can also have a subclinical course, resulting in patients with no reported infectious symptoms prior to development of GBS. Patients who develop GBS following an antecedent C jejuni infection often have a more severe course, with rapid progression and a prolonged, incomplete recovery. A strong clinical association has been noted between C jejuni infections and the pure motor and axonal forms of GBS. The virulence of C jejuni is thought to be based on the presence of specific antigens in its capsule that are shared with nerves. Immune responses directed against capsular lipopolysaccharides produce antibodies that cross-react with myelin to cause demyelination. C jejuni infections demonstrate significant association with antibodies against gangliosides GM1 and GD1b. Although GM1 antibodies can be found with demyelinating GBS, GM1 antibodies are more common in the axonal and inexcitable groups. Even in the subgroup of patients with GM1 antibodies, however, the clinical manifestations vary. Host susceptibility is probably one determinant in the development of GBS after infectious illness. Cytomegalovirus (CMV) infections are the second most commonly found infections preceding GBS; they account for the most common viral triggers of GBS. The aforementioned Dutch GBS study found CMV to be present in 13% of patients. CMV infections present as upper respiratory tract infections, pneumonias, and nonspecific, flulike illnesses. GBS patients with preceding CMV infections often have prominent involvement of

the sensory and cranial nerves. CMV infections are significantly associated with antibodies against the ganglioside GM2. Other significant, although less frequently identified, infectious agents in GBS patients include Epstein-Barr virus (EBV), Mycoplasma pneumoniae, and Varicella-Zoster virus. An association between GBS and human immunodeficiency virus (HIV) also is well recognized. Infections with Haemophilus influenzae, para-influenza virus type 1, influenza A virus, influenza B virus, adenovirus, and herpes simplex virus have been demonstrated in patients with GBS, although not more frequently than they have in controls.Various events, such as surgery, trauma, and pregnancy, have been reported as possible triggers of GBS, but these associations remain mostly anecdotal in the medical literature. Vaccinations also have been linked to GBS, by temporal association. In most cases, however, no definite causal relation has been established between vaccines and GBS .

Chapter 8 PATHOLOGY PATHOLOGY GBS is a postinfectious, immune-mediated disease. Cellular and humoral immune mechanisms probably play a role in its development. Most patients report an infectious illness in the weeks prior to the onset of GBS. Many of the identified infectious agents are thought to induce antibody production against specific gangliosides and glycolipids, such as GM1 and GD1b, distributed throughout the myelin in the peripheral nervous system.2 The pathophysiologic mechanism of an antecedent illness and of GBS can be typified by Campylobacter jejuni infections.3,4 The virulence of C jejuni is thought to be based on the presence of specific antigens in its capsule that are shared with nerves. Immune responses directed against the capsular components produce antibodies that cross-react with myelin to cause demyelination. Ganglioside GM1 appears to cross-react with C jejuni lipopolysaccharide antigens, resulting in the immunologic damage to the peripheral nervous system. This process has been termed molecular mimicry.

Figure 8.1: Campylobacter jejuni infections. Pathologic findings in GBS include lymphocytic infiltration of spinal roots and peripheral nerves, followed by macrophage-mediated, multifocal stripping of myelin. This phenomenon results in defects in the propagation of electrical nerve impulses, with eventual conduction block and flaccid paralysis. In some patients with severe disease, a secondary consequence of the severe inflammation is axonal disruption and loss. A subgroup of patients may have a primary immune attack directly against nerve axons, resulting in a similar clinical presentation. Variants Several variants of GBS are recognized. These disorders share similar patterns of evolution, recovery, symptom overlap, and probable immune-mediated pathogenesis.

Figure 8.2: Immune-mediated pathogenesis The Miller-Fisher syndrome, a common variant of GBS, is observed in about 5% of all GBS cases. The syndrome consists of ataxia, ophthalmoplegia, and areflexia. 5 Ataxia is primarily noted during gait and in the trunk, with lesser involvement of the limbs. Motor strength is characteristically spared. The usual course is one of gradual and complete recovery over weeks or months. A close association exists between antiganglioside antibodies and the Fisher variant. Anti-GQ1b antibodies, triggered by certain C jejuni strains, have a relatively high specificity and sensitivity for the disease. 6 Dense concentrations of GQ1b ganglioside are found in the oculomotor, trochlear, and abducens nerves, which may explain the relationship between anti-GQ1b antibodies and ophthalmoplegia.

The AMAN variant is associated closely with enteric C jejuni infections and high titers of antibodies to gangliosides (ie, GM1, GD1a, GD1b). Patients with AMAN have pure motor symptoms and appear clinically to be very similar to patients with the demyelinating form of GBS with ascending, symmetric paralysis. AMAN is distinguished by electrodiagnostic study results that are consistent with a pure motor axonopathy. 7 Biopsies show wallerianlike degeneration without significant lymphocytic inflammation. Many cases have been reported in rural areas of China, especially in children and young adults during the summer months. 8 Pure axonal cases may occur more frequently outside of Europe and North America. AMAN cases may also be different from cases of axonal GBS described in the West. Prognosis is often quite favorable. Although recovery for many is rapid, severely disabled patients with AMAN may show improvement over a period of years. The axonal form of GBS, also referred to as acute motor-sensory axonal neuropathy (AMSAN), often presents with rapid and severe paralysis with delayed and poorer recovery (in comparison with recovery from electrophysiologically similar AMAN cases). Like AMAN, axonal GBS also is associated with preceding C jejuni diarrhea. Pathologic findings show severe axonal degeneration of motor and sensory nerve fibers with little demyelination.9 A pure sensory variant of GBS has been described in the medical literature, typified by a rapid onset of sensory loss and areflexia in a symmetric and widespread pattern. Lumbar puncture studies show albuminocytologic dissociation in the cerebrospinal fluid (CSF), and electromyography (EMG) results show characteristic signs of a demyelinating process in the peripheral nerves. Prognosis is generally good, but immunotherapies, such as plasma exchange and the administration of intravenous immunoglobulins (IVIGs), can be tried in patients with severe disease or slow recovery. Acute pandysautonomia without significant motor or sensory involvement is a rare presentation of GBS. Dysfunction of the sympathetic and parasympathetic systems results in severe postural hypotension, bowel and bladder retention, anhidrosis, decreased salivation and lacrimation, and pupillary abnormalities. The pharyngeal-cervical-brachial variant is distinguished by isolated facial, oropharyngeal, cervical, and upper limb weakness without lower limb

Chapter 9 CLINICAL FEATURE

CLINICAL FEATURE 1.Antecedent illness Up to two thirds of patients with GBS report an antecedent illness or event 1-3 weeks prior to the onset of weakness. Upper respiratory and gastrointestinal illnesses are the most commonly reported conditions. Symptoms generally have resolved by the time of medical presentation for the neurologic condition.

C jejuni is the major causative organism that is identified in most studies and is responsible for AIDP and AMAN cases. In one major study, previous diarrheal illness had occurred in 60% of patients with axonal GBS (by neurophysiologic testing). Vaccinations, surgical procedures, and trauma have been reported to trigger the development of GBS. Much of this information is anecdotal, although vaccination with the swine flu vaccine (administered in 1976) was shown to increase the risk of contracting GBS to a small, but definable, degree. Rabies vaccine prepared from infected brain tissue also was found to have an association with GBS. Studies of other vaccines, however, have not shown a significant relationship between these drugs and GBS. 2.Weakness The classic clinical picture of weakness is ascending and symmetrical in nature. The lower limbs are usually involved before the upper limbs. Proximal muscles may be involved earlier than the more distal ones. Trunk, bulbar, and respiratory muscles can be affected as well. Weakness develops acutely and progresses over days to weeks. Severity may range from mild weakness to complete tetraplegia with ventilatory failure. Peak deficits are reached by 4 weeks after the initial development of symptoms. The progression of symptoms beyond that point brings the diagnosis under question. Recovery usually begins 2-4 weeks after the progression ceases. 3.Sensory changes Most patients complain of paresthesias, numbness, or similar sensory changes. Sensory symptoms often precede the weakness. They are frequently ascending in nature and are more pronounced in a distal distribution. Sensory symptoms are usually mild. In most cases, objective findings of sensory loss tend to be minimal and variable.On nerve conduction studies (NCS), 58-76% of patients exhibit sensory abnormalities. 4.Cranial nerve involvement Cranial nerve involvement is observed in 45-75% of patients with GBS. Common complaints may include the following:    

Facial droop Diplopias Dysarthria Dysphagia

Facial and oropharyngeal weakness usually appears after the trunk and limbs are affected. 5.Pain In a prospective, longitudinal study of pain in patients with GBS, 89% of patients reported pain that was attributable to GBS at some time during their illness. On initial presentation, almost 50% of patients described the pain as severe and distressing.

The mechanism of pain is uncertain and may be a product of several factors. Pain can result from direct nerve injury or from the paralysis and prolonged immobilization. Most patients complain of back and leg pain, often described as aching or throbbing in nature. The mechanism of pain is thought to be inflamed nerve roots. Dysesthetic symptoms are observed in approximately 50% of patients during the course of their illness. Dysesthesias frequently are described as burning, tingling, or shocklike sensations and are often more prevalent in the lower extremities than in the upper extremities. Dysesthesias may persist indefinitely in 5-10% of patients. Other pain syndromes in GBS include the following:   

Myalgic complaints, with cramping and local muscle tenderness Visceral pain Pain associated with conditions of immobility (eg, pressure nerve palsies, decubitus ulcers)

The intensity of pain on admission correlates poorly with neurologic disability on admission and with the end outcome. 6.Autonomic changes Autonomic nervous system involvement with dysfunction in the sympathetic and parasympathetic systems can be observed in patients with GBS. Autonomic changes can include the following:      

Tachycardia Bradycardia Facial flushing Paroxysmal hypertension Orthostatic hypotension Anhidrosis and/or diaphoresis

Urinary retention and paralytic ileus also can be observed. Bowel and bladder dysfunction rarely presents as an early symptom or persists for a significant period of time. Dysautonomia is more frequent in patients with severe weakness and respiratory failure.Autonomic changes rarely persist in a patient with GBS. 7. Respiratory involvement Upon presentation, 40% of patients have respiratory or oropharyngeal weakness.Typical complaints include the following:    

Dyspnea on exertion Shortness of breath Difficulty swallowing Slurred speech

Ventilatory failure with required respiratory support is observed in up to one third of patients at some time during the course of their disease.

Physical 1.Vital signs Cardiac arrhythmias, including tachycardias and bradycardias, can be observed as a result of autonomic nervous system involvement. Tachypnea may be a sign of ongoing dyspnea and progressive respiratory failure. Blood pressure lability is another common feature with alterations between hypertension and hypotension. Cranial nerves :Facial weakness (cranial nerve VII) is observed most frequently, followed by symptoms associated with cranial nerves VI, III, XII, V, IX, and X. Involvement of facial, oropharyngeal, and ocular muscles results in facial droop, dysphagia, dysarthria, and findings associated with disorders of the eye. Ophthalmoparesis may be observed in up to 25% of patients with GBS. Limitation of eye movement most commonly results from a symmetric palsy associated with cranial nerve VI. Ptosis from cranial nerve III (oculomotor) palsy also is often associated with limited eye movements. Pupillary abnormalities, especially those accompanying ophthalmoparesis, are relatively common as well. 2.Motor changes Lower extremity weakness usually begins first and ascends symmetrically and progressively over the first several days. Upper extremity, trunk, facial, and oropharyngeal weakness is observed to a variable extent. Marked asymmetric weakness calls the diagnosis of GBS into question. 3.Sensory changes Despite frequent complaints of paresthesias, objective sensory changes are minimal. A well-demarcated sensory level should not be observed in patients with GBS; such a finding calls the diagnosis of GBS into question. 4.Reflex changes Reflexes are absent or hyporeflexic early in the disease course and represent a major clinical finding on examination of the patient with GBS. Pathologic reflexes, such as the Babinski sign, are absent. Hypotonia can be observed with significant weakness.

Chapter 10 INVESTIGATION

INVESTIGATION  Laboratory Studies  CSF studies -

During the acute phase of GBS, characteristic findings include albuminocytologic dissociation, which is an elevation in CSF protein (>0.55 g/L) without an elevation in white blood cells. The increase in CSF protein is thought to reflect the widespread inflammatory disease of the nerve roots. Basic laboratory studies, such as complete blood counts and metabolic panels, are of limited value in the diagnosis of GBS. They are often ordered, however, to exclude other infectious or metabolic causes of the weakness. A basic peripheral neuropathy workup is recommended in cases in which the diagnosis is uncertain. These studies may include thyroid panel, rheumatology profiles, vitamin B-12, folic acid, hemoglobin A1C, erythrocyte sedimentation rate (ESR), rapid protein reagent, and immunoelectrophoresis of serum protein, as well as tests for heavy metals. The ordering of specific tests should be guided by the patient's history and presentation.

 Serologic studies are of limited value in the diagnosis of GBS. -

An increase in titers for infectious agents, such as CMV, EBV, or Mycoplasma, may help in establishing etiology for epidemiologic purposes. HIV has been reported to precede GBS, and serology should be tested in high-risk patients to establish possible infection with this agent.

 Serum auto-antibodies are not measured routinely in the workup of GBS, but results may be helpful in patients with a questionable diagnosis or a variant of GBS. -

Antibodies to glycolipids are observed in the sera of 60-70% of patients with GBS during the acute phase, with gangliosides being the major target antigens. - Antibodies to GM1 frequently are frequently found in the sera of patients with the motor axonal neuropathy or AIDP variants of GBS. Antecedent C jejuni infections are closely associated with elevated titers of anti-GM1 antibodies. - Anti-GQ1b antibodies are found in patients with GBS with ophthalmoplegia, including patients with the Miller-Fisher variant. - Other antibodies to different major and minor gangliosides also have been found in GBS patients.  Imaging Studies  Magnetic resonance imaging (MRI) -

Although nonspecific, MRI can reveal nerve root enhancement.

Imaging studies, such as MRI or computed tomography (CT) scanning of the spine, may be more helpful in excluding other diagnoses, such as mechanical causes of myelopathy, than in assisting in the diagnosis of GBS.

 Other Tests  EMG -

EMG studies can be very helpful in the diagnostic workup of patients with suspected GBS. Abnormalities in NCS that are consistent with demyelination are sensitive and represent specific findings for classic GBS. Although NCS results classically show a picture of demyelinating neuropathy in most patients, other electrophysiologic subgroups include axonal and inexcitable groups. The inexcitable studies may represent either axonopathy or severe demyelination with distal conduction block. Although most patients exhibit sensory abnormalities on NCS, these findings are much less marked than they are in motor nerves. On NCS, demyelination is characterized by nerve conduction slowing, prolongation of the distal latencies, prolongation of the F-waves, conduction block, and/or temporal dispersion. Changes on NCS should be present in at least 2 nerves in regions that are not typical for those associated with compressive mononeuropathies (preferentially in anatomically distinct areas, such as an arm and a leg or a limb and the face). The needle examination is of limited value in GBS. Reduced motor unit recruitment and absent denervation help to support the suggestion of a demyelinating mechanism, although the same changes can be observed in early axonal damage with pending wallerian degeneration. In severe cases, denervation changes may be observed later in the disease course. In the axonal variant of the disease, absent or markedly reduced distal compound muscle action potentials (CMAP) are observed on NCS. On needle examination, profuse and early denervation potentials also support the conclusion that there has been axonal injury.

 Pulmonary function tests -

Maximal inspiratory pressures and vital capacities are measurements of neuromuscular respiratory function and predict diaphragmatic strength. Maximal expiratory pressures also reflect abdominal muscle strength. Frequent evaluations of these parameters should be performed at bedside to monitor respiratory status and the need for ventilatory assistance. - Respiratory assistance should be considered when the expiratory vital capacity decreases to <18 mL/kg or there is a decrease in oxygen saturation (arterial PO2 <70 mm Hg).  Procedures  Lumbar puncture for CSF studies is recommended.  Histologic Findings

Lymphocyte and macrophage infiltration is observed on microscopic examination of peripheral nerves. Macrophage influx is believed to be responsible for the multifocal demyelination seen in GBS. A variable degree of wallerian degeneration also can be observed with severe inflammatory changes. Cellular infiltrates are scattered throughout the cranial nerves, nerve roots, dorsal root ganglions, and peripheral nerves. Differential Diagnosis Basilar artery thrombosis

Nutritional Neuropathy

Botulism

Poliomyelitis

Hypophosphatemia

Polymyositis

Leptomeningeal Carcinomatosis

Vasculitic Neuropathy

Lyme Disease Metabolic Myopathies Mononeuritis Multiplex ď&#x192;&#x2DC; Other Problems to Be Considered Toxic neuropathies (eg, arsenic, thallium, organophosphates, lead) Multifocal motor neuropathy Critical illness polyneuropathy Vasculitic neuropathies Diphtheritic polyneuritis Acute myasthenia gravis Tick paralysis Paralytic shellfish poisoning Porphyria polyneuropathy Chronic inflammatory demyelinating polyneuropathy Acute myelopathy (for example, from compression, transverse myelitis, vascular injury) Periodic paralysis Paraneoplastic neuropathy Relapsing inflammatory polyneuropathy Neoplastic meningitis Conversion disorder/hysterical paralysis The diagnosis of GBS usually depends on findings such as rapid development of muscle paralysis, areflexia, absence of fever, and a likely inciting event. Cerebrospinal fluid analysis (through a lumbar spinal puncture) and electrodiagnostic tests of nerves and muscles (such as nerve conduction studies) are common tests ordered in the diagnosis of GBS.

Cerebrospinal fluid Typical CSF findings include albumino-cytological dissociation. As opposed to infectious causes, this is an elevated protein level (100â&#x20AC;&#x201C;1000 mg/dL), without an accompanying increased cell count pleocytosis. A sustained increased white blood cell count may indicate an alternative diagnosis such as infection.

Electrodiagnostics Electromyography (EMG) and nerve conduction study (NCS) may show prolonged distal latencies, conduction slowing, conduction block, and temporal dispersion of compound action potential in demyelinating cases. In primary axonal damage, the findings include reduced amplitude of the action potentials without conduction slowing.

Diagnostic criteria Required • • • •

Progressive, relatively symmetrical weakness of two or more limbs due to neuropathy Areflexia Disorder course < 4 weeks Exclusion of other causes (see below)

Supportive • • • • • •

relatively symmetric weakness accompanied by numbness and/or tingling mild sensory involvement facial nerve or other cranial nerve involvement absence of fever typical CSF findings obtained from lumbar puncture electrophysiologic evidence of demyelination from electromyogram

Differential diagnosis • • • • • • • • • • • • • • • •

acute myelopathies with chronic back pain and sphincter dysfunction botulism with early loss of pupillary reactivity and descending paralysis diphtheria with early oropharyngeal dysfunction Lyme disease polyradiculitis and other tick-borne paralyses porphyria with abdominal pain, seizures, psychosis vasculitis neuropathy poliomyelitis with fever and meningeal signs CMV polyradiculitis in immunocompromised patients critical illness neuropathy myasthenia gravis poisonings with organophosphate, poison hemlock, thallium, or arsenic intoxication with Karwinskia humboldtiana leaves or seeds paresis caused by West Nile virus spinal astrocytoma motor neurone disease West Nile virus can cause severe, potentially fatal neurological illnesses, which include encephalitis, meningitis, Guillain-Barré syndrome, and anterior myelitis.

Chapter 11 MANAGEMENT

MANAGEMENT Good supportive care is critical in the treatment of patients with GBS. Because most deaths related to GBS are associated with complications of ventilatory failure and autonomic dysfunction, many patients with GBS need to be monitored closely in ICUs by physicians experienced in acute neuromuscular paralysis and its accompanying complications. Competent intensive care includes the following features: -

Respiratory therapy Cardiac monitoring Safe nutritional supplementation Monitoring for infectious complications, such as pneumonia, urinary tract infections, and septicemia

Approximately one third of patients with GBS require ventilatory support. Monitoring for respiratory failure, bulbar weakness, and difficulties with swallowing help to anticipate complications. Proper positioning of the patient to optimize lung expansion and secretion management for airway clearance is required to minimize respiratory complications. Close monitoring of heart rate, blood pressure, and cardiac arrhythmias allows early detection of life-threatening situations. Critically ill patients require continuous telemetry and close medical supervision in an ICU setting. Antihypertensives and vaso-active drugs should be used with caution in patients with autonomic instability. Enteral or parenteral feedings are required for patients on mechanical ventilation to ensure that adequate caloric needs are met when the metabolic demand is high. Even patients who are off the ventilator may require nutritional support if dysphagia is severe. Precautions against dysphagia and dietary manipulations should be used to prevent aspiration and subsequent pneumonias in patients at risk. The prevention of secondary complications of immobility, such as deep venous thrombosis (DVT), pressure sores, and contractures, also is required. This preventative action entails careful positioning, frequent postural changes, and daily ROM to prevent the latter 2 complications. Subcutaneous heparin and thromboguards are often used in the treatment of immobile patients to prevent lower extremity DVTs and secondary pulmonary embolisms (PE). Pain management with analgesics and adjunct medications also may be needed. Modalities such as transcutaneous electrical nerve stimulation (TENS) and heat may prove beneficial in the management of myalgia. Desensitization techniques can be used to improve the patient's tolerance for activities. Although bowel and bladder dysfunction is generally transitory, management of these functions is needed to prevent other complications. Initial management should be directed toward safe evacuation and the prevention of overdistension. Monitoring for secondary infections, such as a urinary tract infection, also is an area of concern. Hospitalized patients with GBS may experience mental status changes, including hallucinations, delusions, vivid dreams, and sleep abnormalities. These occurrences are thought to be associated with autonomic dysfunction and are more frequent in patients with severe symptoms. Such problems resolve as the patient recovers.

 MEDICATION  Inpatient/Outpatient Medications -

Immunomodulatory medications have been used to hasten recovery. In wellcontrolled clinical trials, the efficacy of IVIGs in GBS patients has been shown to equal that of plasma exchange. IVIG treatment is easier to implement and potentially safer than plasma exchange, and the use of IVIGs versus plasma exchange may be a choice of availability and convenience. Attempts have been made to employ oral and IV steroids in the treatment of GBS, but they have not been found to have a clinical benefit; these drugs are not currently employed in GBS treatment. Hemodynamic changes related to autonomic dysfunction are usually transitory, and patients rarely require long-term medications to treat blood pressure or cardiac problems. Pain medications may be required in the inpatient and outpatient settings. A tiered pharmacologic approach that starts with nonsteroidal anti-inflammatory drugs or acetaminophen, with narcotic agents added as needed, is usually recommended. Most patients do not require narcotic analgesics after the first couple of months of illness. Adjunct medications for pain, such as tricyclic antidepressants and certain anticonvulsants, also may be beneficial for dysesthetic-type pains. Anticoagulants, such as heparin or low – molecular weight heparin, are recommended to prevent thromboembolic disease in the sedentary patient.

Immunomodulatory therapy, such as plasmapheresis or the administration of IVIGs, is frequently used in GBS patients.The efficacy of plasmapheresis and IVIGs appears to be about equal in shortening the average duration of disease. Combined treatment has not been shown to produce a further, statistically significant reduction in disability. The decision to use immunomodulatory therapy is based on the disease's severity and rate of progression, as well as on the length of time between the condition's first symptom and its presentation. Risks, such as thrombotic events associated with IVIGs, should be taken into consideration.Patients with severe, rapidly progressive disease are most likely to benefit from treatment, with improvements occurring in the rate of functional recovery.  Immunomodulatory agents These medications are used to improve the clinical and immunologic aspects of GBS. They may decrease auto-antibody production and increase the solubilization and removal of immune complexes. IVIG is derived from fractionated, purified human plasma collected from a large pool of multiple donors. The product is treated with solvents and detergents to inactivate any bloodborne virus. IVIG may work via several mechanisms, including the blockage of macrophage receptors, the inhibition of antibody production, the inhibition of complement binding, and the neutralization pathologic antibodies. -

Dosing Interactions Contraindications Precautions

 Adult 2 g/kg IV, generally divided over 5 d Some centers administer IVIG over 2 d at 1 g/kg/d, especially in younger patients with normal renal and cardiovascular function  Pediatric  Administer as in adults • • • • • • • •

Dosing Interactions Contraindications Precautions Dosing Interactions Contraindications Precautions Dosing Interactions Contraindications Precautions Plasma exchange or plasmapheresis The mechanism of plasmapheresis is the removal of immunoglobulins and antibodies from the serum by removing the blood from the body, separating cells from the plasma, and replacing the cells in fresh frozen plasma, albumin, or saline. -

Dosing Interactions Contraindications Precautions

 Adult 3-5 exchanges of 50 mL/kg of plasma IV over 1-2 wk via central venous catheter suggested  Pediatric  Administer as in adults • • • • • • • • • • •

Dosing Interactions Contraindications Precautions Dosing Interactions Contraindications Precautions Dosing Interactions Contraindications

Precautions

•

Chapter 12 PHYSIOYHERAPY MANAGEMENT

Physiotherapy Treatment of Guillain-Barré Syndrome Physiotherapy treatment for Guillain-Barré syndrome (GBS) should start in hospital and continue until you have reached your maximum potential. Manchester Neuro Physio are able to provide physiotherapy assessments and treatment as soon and as often as you require. Physiotherapy for Guillain-Barré syndrome: -

increase muscle strength increase mobility increase balance retrain normal patterns of movement increase energy levels assist in return to previous activity levels educate about GBS and symptoms Physiotherapy treatment can consist of: - ‘hands on’ physiotherapy - home based exercise programmes - specialist gym based rehab programmes - hydrotherapy

Physiotherapy has an important role in the management of GBS patients. It helps to maximise a patients physical potential, particularly where weakness is predominant problem. 1. Acute phase 2. Recovery or acute rehabilitation stage. 3. The long-term or ongoing rehabilitation stage Acute phase: In the acute phase, a large part of physiotherapy treatment is related to for respiratory care. When patient is under intensive care in the acute progressive stage, the main aims of physiotherapy are to maintain clear airways, prevent lung infection, maintain anatomical joint

range, support joints in a functional position to minimise damage or deformity, prevention of pressure sores and maintain peripheral circulation by using various physiotherapy techniques. Recovery stage : The recovery stage is when the patient can maintain his own airway and ventilation and there is some motor recovery. After doing a problem-oriented assessment physiotherapist make a treatment plan. Physiotherapists give emphasis on respiratory function; increase active and passive joint range of movement, increase muscle power, improve balance in various functional positions.

Figure 12.1: Postural dranage Rehabilitation stage : As patients recover at different rates it is impossible to outline a course of treatment to suit them all. However some basic principles are common to all treatment programmes. Maintenance of the airway and ventilatory capacity-patients can be taught breathing techniques and coughing together with instruction on frequency of practice.

Strengthen and re educates normal muscle function-the proximal muscles tend 0 recover first. To facilitate voluntary contraction of the muscles some of the following techniques may be useful:

Figure 12.2 : Wheel chaitr activity •

Neuromuscular facilitation techniques

•

Afferent stimulation of skin

•

Free active exercise

•

Equilibrium and righting reactions

•

Progressive resistance exercise

•

Suspension

•

Springs/pulleys

•

Hydrotherapy

•

Re education of sensory awareness.

Physical Therapy Estimates suggest that approximately 40% of patients who are hospitalized with GBS require inpatient rehabilitation. Unfortunately, no long-term rehabilitation outcome studies have been conducted, and treatment is often based on experiences with other neurologic conditions. The goals of the therapy programs are to reduce functional deficits and to target impairments and disabilities resulting from GBS

Figure 12.3: Passive stretching. Early in the acute phase of the disease course, patients may not be able to fully participate in an active therapy program. At that stage, patients benefit from daily range of motion (ROM) exercises and proper positioning to prevent muscle shortening and joint contractures. Addressing upright tolerance and endurance also may be a significant issue during the early part of rehabilitation. Active muscle strengthening can then be slowly introduced and may include isometric, isotonic, isokinetic, or progressive resistive exercises. Mobility skills, such as bed mobility, transfers, and ambulation, are targeted functions. Patients should be monitored for hemodynamic instability and cardiac arrhythmias, especially upon initiation of the rehabilitation program. The intensity of the exercise program also should be monitored, because overworking the muscles may, paradoxically, lead to increased weakness. Occupational Therapy Occupational therapy professionals should be involved early in the rehabilitation program to promote upper body strengthening, ROM, and activities that aid functional selfcare. Both restorative and compensatory strategies can be used to promote functional improvements. Energy conservation techniques and work simplification also may be helpful, especially if the patient demonstrates poor strength and endurance. Speech Therapy Speech therapy is aimed at promoting speech and safe swallowing skills for patients who have significant oropharyngeal weakness with resultant dysphagia and dysarthria. In ventilator-dependent patients, alternative communication strategies also may need to be implemented. Once weaned from the ventilator, patients with tracheostomies can learn voicing strategies and can eventually be weaned from the tracheostomy tube. Cognitive

screening also can be performed conjointly with neuropsychology to assess for deficits, since cognitive problems have been reported in some patients with GBS, especially after they have had an extended stay in the intensive care unit (ICU). Chest physiotherapy

The aims of the management in this stage consist of the following:

Postural Drainage

•

Maintain clear airways and to prevent respiratory complication

•

Continuous change of position of drain out secretion and also to facilitate proper air entry to all t lobes of the lung.

•

Periodic sunctioning in a strictly hygienic manner.

•

Percussion,shaking,manual mobilization with the help of ambubag may be necessary to loosen out the secretion.

•

Nebulization in case of any respiratory infection.

1. Maintanance of Range of motion at All Joints i.

Passive range of motion

ii.

Gradual stretching

2. Support of the Limbs Static splints are given to prevent contracture as well as to immobilize the part to prevent any unwanted damage to the muscles or joints.Splints are commonly used for the foot and hand to maintain it in neutral position. 3. Pain relief Patients with GBS in the acute stage suffer from pain and paresthesia which may interfere with the rehabilitation process hence to give patient release from this altered sensation TENS may be used. 4. Maintenance of muscle properties

Functional electrical stimulation may be used to maintain the muscle property.Even stimulation with interrupted direct current to the functionally important muscles may be given to maintain their properties. 5. Prevention and Treatment of Pressure sores Pressure sore can be prevented by constantly changing the posture,gentle message of the pressure prones area,by keeping the skin dry and soft as well as by giving mattress which help in more even distribution of the weight.In case pressure sores do develop then measures like ice cube message around the wound,UVR radiation,LASER etc. radiation etc.may be used to treat the sore. 6. Prevention of Postural Hypertension Postural hypotension is very common in this case due to the combine effect of severe hypotonicity and supine position. 7. Psycological support The patient motivation must be kept high.The main aim of the treatment in this stage involves: • Strengthen of the weak muscles •

Functional reduction

•

Gait training

•

Respiratory & cardiovascular conditioning

8. Strenthening

IG stimulation can be continued till the patient is able to generate contraction of the muscles voluntary bat is unable to perform any movement(grade 1). 9. Functional Training It is essential to incorporate to improvement in the muscle strength into various activities of daily living 10. Gait Training Gait training is initiated in the parallel bar and then progressed to walking unsupported on an even surface within the clinic. Sensory deficit is not marked in GBS patient, hence sensory reduction may not be always given FOLLOW-UP Further Inpatient Care GBS treatment requires careful and intensive care to monitor for such complications as respiratory difficulties and autonomic dysfunction. Approximately one third of patients require admission to an ICU, primarily because of respiratory failure. After medical

stabilization, patients can be treated on a general medical/neurologic floor, but continued vigilance remains important in preventing respiratory, cardiovascular, and other medical complications. Continued care also is needed to minimize problems related to immobility, neurogenic bowel and bladder, and pain. Early involvement of allied health staff is recommended. Further Outpatient Care -

Although follow-up studies generally have assessed patients 6-12 months after onset, some studies have reported continued improvements in strength even beyond 2 years. With prolonged recovery possible, GBS patients with continued neurologic deficits may benefit from ongoing physical therapy and conditioning programs.

Numerous papers have addressed the issue of persistent fatigue after recovery from GBS. Studies have suggested that a large percentage of patients continue to have fatiguerelated problems, subsequently limiting their function at home, work, and during leisure activities. Treatment suggestions range from gentle exercise to the prescription of medications traditionally used to stimulate initiation. The effectiveness of various interventions continues to be studied. GBS can produce long-lasting changes in the psychosocial status of patients and their families. Changes in work and leisure activities can be observed in just over one third of these patients, and psychosocial functional health status can be impaired even years after the GBS event. Interestingly, psychosocial performance does not seem to correlate with the severity of residual physical function. Poor conditioning and easy fatigability may be contributory factors. Therefore, providing long-term attention and support for this population group is important. Transfer -

Patients may require transfer to the ICU if serious respiratory or cardiac problems occur. Upon medical and neurologic stabilization, patients may need to be transferred to an inpatient rehabilitation unit if functional impairments persist

Figure 12.4: Walking aid Deterrence -

C jejuni is the most common cause of bacterial gastroenteritis in industrialized countries and is also the organism that is most frequently identified in association with GBS. Preventive measures to control C jejuni infections, such as vaccinations, may be the best means to prevent GBS.

Chapter 13 CONCLUSION Conclusion: GBS can herald a malignant process. Usual therapy for GBS and successful treatment of the cancer may lead to neurologic recovery. When Guillain-Barre is preceded by a viral infection, it is possible that the virus has changed the nature of the cells in the nervous system so that the immune system treats them as foreign cells, and will try to get rid of them. It is also possible that the virus makes the immune system itself less discriminating about what cells it recognizes as it own, allowing some of the immune cells, such as certain kinds of lymphocytes, to attack the myelin. There is no known cure for Guillain-Barre syndrome. However there are therapies that lessen the strength of the syndrome and acceleration. This project explains the vital role of Physical therapy and the treatment procedures for the improvement of Guillain Barre Syndrome patients.

Chapter 14 BIBLIOGRAPHY

Bibliography Books: 1. Physiotherapy in Neuro-conditions - Glady Samuel Raj 2. Cashâ&#x20AC;&#x2122;s Textbook of Neurology for Physiotherapists- PATRICA A.DOWNI 3. Neurology and Neurosurgery illustrated -Kenneth W.Lindsay &Ian Bone

Websites: 1. 2. 3. 4. 5.

http://www.mozilla.com http://www.gmail.com http://www.tue.com http://www.asahi-net.or.jp/~uy7t-wtnb/help/trouble.html http://www.yahoo.com

6. http://www.google.com 7. http://www.opera.com http://www.pis.or.jp/medical/medical.htm 8. http://www.pis.or.jp/medical/iryou/index.htm 9. http://www.healthnetworkamerica.com/ 10. http://www.patientadvocatesllc.com 11. http://www.Healthcare.com 12. http://www.Medical Marketing Tips.com 13. http://www.Medical Copywriting.com 14. http://www.Medical Clients Served.com 15. http://www.lopnor.gr.jp/data/tobu_f_organization.html.

Journals: 1. Griggs RC, J贸zefowicz RF, Aminoff MJ. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 418. 2. Barohn RJ. Muscle diseases. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 447. 3. Hughes RA, Raphael JC, Swan AV, van Doorn PA. Intravenous immunoglobulin for Guillain-Barre syndrome. Cochrane Database Syst Rev. 2009;(1):CD002063. 4. Hughes RA, Wijdicks EF, Barohn R, et al. Practice parameter: immunotherapy for Guillain-Barre syndrome: report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2003;61(6):736-740. 5. Shy ME. Peripheral neuropathies. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier;2007:chap 446 6. Autonomic Nervous System," Microsoft (R) Encarta. Copyright (c) 1994 Microsoft

Corporation. Copyright (c) 1994 Funk & Wagnall's Corporation. 7. "Brain," Microsoft (R) Encarta. Copyright (c) 1994 Microsoft Corporation. Copyright (c) 1994 Funk & Wagnall's Corporation. 8. Carlson, N. (1977). Physiology of Behavior, 5th ed. Boston, MA: Allyn and Bacon. 9. Hooper, J. & Teresi, D. The 3-Pound Universe. New York: G.P. Putnam's Sons. 10. Joseph, R., Dr. The Right Brain and the Unconscious: Discovering the Stranger Within. New York: Plenum Press. 11. "Nervous System," Microsoft (R) Encarta. Copyright (c) 1994 Microsoft Corporation. Copyright (c) 1994 Funk & Wagnall's Corporation. 12. "Neurophysiology," Microsoft (R) Encarta. Copyright (c) 1994 Microsoft Corporation. Copyright (c) 1994 Funk & Wagnall's Corporation.

APPENDIX- 1 NEUROLOGICAL ASSESSMENT

Neurological Assessment Subjective Assesment Name : Age : Sex : Occupation : Past Medical History : Present Medical history : Family history : Personal history : Social history : Vital sign :

Objective assessment Higher cerebral function Consciousness: Glasgow coma scale 1.Eye opening-4 categories 2.verbal response-5 categories 3.Motor response- 5 categories

Memory test 1.Immediate memory 2.Recent memory 3.Remote memory 4.verbal memory 5.Visual memory

Intelligent capacity 0rientation Cranial Nerve Examination: 12 pairs of cranial nerve assessment

Motor assessment o

Muscle tone assesmernt

Muscle power

Range of motion

Sensory assessment -superficial sensation -Deep sensation -Cortical sensation

Reflex assessment Bowel and bladder assessment Posture assessment Balance assessment Co-ordination assessment Gait Examination Functional assessment