GUILLAIN-BARRÉ SYNDROME.pptx akvshsje soehsb

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1. GUILLAIN- BARRÉ SYNDROME

2. GBS is also known as Acute idiopathic polyradiculoneuritis Acute idiopathic polyneuritis French polio Landry's ascending paralysis a Landry Guillain Barré syndrome.

3. DEFINITION Guillain- Barré syndrome is an autoimmune attack of the peripheral nerve myelin. The result is acute, rapid segmental demyelination of peripheral nerves and some cranial nerves, producing ascending weakness with dyskinesia (inability to execute voluntary movements), hyporeflexia, and paresthesias (numbness). Guillain–Barré syndrome (GBS) or Landry's paralysis , is a disorder in which the body’s immune system attack part of the peripheral nervous system. It is an acute, progressive, autoimmune, inflammatory demyelination of polyneuropathy of the peripheral sensory and motor nerves and nerve roots.

4. ETIOLOGY Preceding vaccination Bacterial infection Viral infection Protozoan infection Surgeries Blood Transfusion & Transplantation Anesthesia & Analgesia Preceding heat stroke Several drugs

5. Predisposing factors Respiratory or gastrointestinal infection, Vaccination, Pregnancy Infection with Campylobacter jejuni precedes Guillain- Barré syndrome

6. Clinical manifestation The antecedent event usually occurs 2 weeks before symptoms begin. Weakness usually begins in the legs and progresses upward for about 1 month. Maximum weakness varies but usually includes neuromuscular respiratory failure and bulbar weakness. The duration of the symptoms is variable: complete functional recovery may take up to 2 years

7. PATHOPHYSIOLOGY Guillain- Barré is the result of a cell- mediated immune attack on peripheral nerve myelin proteins . The best- accepted theory is that an infectious organism contains an amino acid that mimics the peripheral nerve myelin protein. The immune system cannot distinguish between the two proteins and attacks and destroys peripheral nerve myelin. Studies indicate that an exact location within the peripheral nervous system, the ganglioside GM1b, is the most likely target of the immune attack

8. PATHOPHYSIOLOGY With the autoimmune attack there is an influx of macrophages and other immune-mediated agents that attack myelin, cause inflammation and destruction, and leave the axon unable to support nerve conduction. The cell that produces myelin in the peripheral nervous system is the Schwann cell. In Guillain- Barré the Schwann cell is spared, allowing for remyelination in the recovery phase of the disease.

9. Clinical Manifestations Classic Guillain- Barré begins with muscle weakness and diminished reflexes of the lower extremities . Hyporeflexia and weakness progress and may result in quadriplegia. Demyelination of the nerves that innervate the diaphragm and intercostal muscles results in neuromuscular respiratory failure Sensory symptoms include paresthesias of the hands and feet and pain related to the demyelination of sensory fibers.

10. Cranial nerve demyelination can result in a variety of clinical manifestations. Optic nerve demyelination may result in blindness. Bulbar muscle weakness related to demyelination of the glossopharyngeal and vagus nerves results in an inability to swallow or clear secretions. Vagus nerve demyelination results in autonomic dysfunction, manifested by instability of the cardiovascular system. The presentation is variable and may include tachycardia, bradycardia, hypertension, or orthostatic hypotension. Guillain- Barré does not affect cognitive function or level of consciousness. Clinical Manifestations

11. The 1st symptoms of Guillain–Barsré syndrome is… Pain. Progressive muscle weakness. Diminished reflexes of lower extremities. Prickly, tingling sensations. Low blood pressure. Paresthesis. Tenderness and muscle pain. Dysthesias & muscle spasms. Palpitation. Hearth rate change. Sweating abnormalities. Cardiac arrhythmia. Neuromuscular respiratory failure. Difficulty in eye movement, facial movement, speaking, chewing, or swallowing. Clinical Manifestations

12. Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) Acute motor axonal neuropathy (AMAN) Acute motor and sensory axonal neuropathy (AMSAN) Miller fisher syndrome (MFS) subtypes

13. Acute inflammatory demyelinating polyradiculoneuropathy AIDP is the most common form and accounts for around 85– 90% of cases. Prevalent in western countries Adults are affected more than the children First attack appears directed against a component of Schwann cell The clinical features are of symmetrical ascending motor weakness with hypo- or areflexia. The underlying pathological process involves inflammation and destruction of the myelin sheaths surrounding peripheral nerve axons by activated macrophages. This leads to slowing and blockage of conduction within peripheral nerves causing muscle weakness. Severe cases may develop secondary axonal damage Recovery is most often rapid as remyelination occurs.

14. Acute motor axonal neuropathy AMAN is more common in Japan and China, with seasonal prevalence (in the summer months). Children and young are affected more than adults. It has an association with precedent infection with Campylobacter jejuni. First attack appears directed against the axolemma & Nodes of Ranvier. Axonal damage is the prominent pathological alteration. Clinical features are similar to AIDP but tendon reflexes may be preserved. Electrophysiological testing may distinguish from other variants as selective motor nerve and axonal involvement is demonstrated. Recovery takes place when axon regeneration is complete and it is rapid when lesion is localized .

15. Acute motor and sensory axonal neuropathy AMSAN is a variant of GBS in which both motor and sensory fibers are involved and which can be demonstrated on electrophysiological studies. Adults are mostly affected It is more severe and associated with prolonged or even partial recovery. First attack is directed at motor nodes of Ranvier ,but also affects Sensory nerve and roots

16. The underlying pathological process is similar to that for AMAN (i.e. antibody mediated axonal damage). Clinical features are similar to AMAN but also involve sensory symptoms. Acute motor and sensory axonal neuropathy

17. Miller Fisher syndrome Adults ,young and children are affected. Involves PNS & CNS structures. Pathological features resemble that of AIDP. MFS presents with ataxia, areflexia and ophthalmoplegia. 25% of patients may develop limb weakness. Electrophysiological studies show primarily sensory conduction failure.

18. There have been limited pathological studies in MFS but demyelination of nerve roots has been demonstrated. Recovery can be rapid Miller Fisher syndrome

19. DIAGNOSTIC EVALUATION Medical history- A history of a viral illness in the previous few weeks suggests the diagnosis. Physical examination Blood examination- Antiganglioside antibodies Nerve conduction studies (NCS)- a progressive loss of nerve conduction velocity CSF examination- elevated protein levels are detected in CSF evaluation, without an increase in other cells. Electromyography (EMG)- Electrocardiogram (ECG) Pulmonary Function Test (PFT)- Changes in vital capacity and negative inspiratory force are assessed to identify impending neuromuscular respiratory failure.

20. Because of the possibility of rapid progression and neuromuscular respiratory failure, Guillain- Barré is a medical emergency Plasmapharesis High dose immunoglobin therapy Mechanical ventilator Pain control Supportive care Medical Management

21. Supportive Airway and respiratory Cardiovascular Gastrointestinal Neurological Psychological Rehabilitation

22. Medical Management RESPIRATORY CARE Respiratory therapy or mechanical ventilation may be necessary to support pulmonary function and adequate oxygenation. Mechanical ventilation may be required for an extended period. The patient is weaned from mechanical ventilation when the respiratory muscles can again support spontaneous respiration and maintain adequate tissue oxygenation. TO PREVENT THE COMPLICATIONS OF IMMOBILITY. These may include the use of anticoagulant agents and thigh- high elastic compression stockings or sequential compression boots to prevent thrombosis and pulmonary emboli.

23. Medical Management The cardiovascular risks posed by autonomic dysfunction require continuous ECG monitoring. Tachycardia and hypertension are treated with short-acting medications such as alpha- adrenergic blocking agents. Hypotension is managed by increasing the amount of IV fluid administered

24. NURSING MANAGEMENT Ineffective breathing pattern and impaired gas exchange related to rapidly progressive weakness and impending respiratory failure Impaired physical mobility related to paralysis Imbalanced nutrition, less than body requirements, related to inability to swallow Impaired verbal communication related to cranial nerve dysfunction Fear and anxiety related to loss of control and paralysis

25. MAINTAINING RESPIRATORY FUNCTION Respiratory function can be maximized with incentive spirometry and chest physiotherapy. Monitoring for changes in vital capacity Mechanical ventilation is required if the vital capacity falls, making spontaneous breathing impossible and tissue oxygenation inadequate. Suctioning may be needed to maintain a clear airway.

26. ENHANCING PHYSICAL MOBILITY The paralyzed extremities are supported in functional positions, passive range- of- motion exercises are performed at least twice daily Range- of- motion exercises, altering positioning, anticoagulation, thigh- high elastic compression stockings or sequential compression boots, and adequate hydration will decrease the risk for DVT. Padding may be placed over bony prominences such as the elbows and heels to reduce the risk for pressure ulcers. Consistent position changes every 2 hours

27. PROVIDING ADEQUATE NUTRITION IV fluids and parenteral nutrition Monitor for the return of bowel sounds gastrostomy tube Assess the return of the gag reflex and bowel sounds before resuming oral nutrition.

28. IMPROVING COMMUNICATION Picture cards or an eye blink system Collaboration with the speech therapist

29. DECREASING FEAR AND ANXIETY Participate in physical care providing information about the condition Emphasizing a positive appraisal of coping resources Teaching relaxation exercises and distraction techniques Encouraging visitors Engaging visitors or volunteers to read to the patient Listening to music or books on tape, and watching television

30. MONITORING AND MANAGING POTENTIAL COMPLICATIONS Respiratory failure Cardiac dysrhythmias Transient hypertension, orthostatic Hypotension DVT Pulmonary embolism, Urinary retention, Other threats to any immobilized and paralyzed patient.

31. PROGNOSIS Start after the 4th week from the onset. Approximately 80% of patient have a complete recovery. Eighty- five percent of patients recover with minimal residual symptoms. Severe residual deficits occur in up to 10% of patients. Residual deficits are most likely in patients with rapid disease progression, those who require mechanical ventilation, or those 60 years of age or older. Death occurs in 3% to 8% of cases, resulting from respiratory failure, autonomic dysfunction, sepsis, or pulmonary emboli

32. AMYOTROPHIC LATERAL SCLEROSIS

33. Introduction Amyotrophic lateral sclerosis (ALS), also known as a motor neuron disease (MND), is a progressive neurodegenerative disorder that causes the degeneration of upper and lower motor neurons. It is the most common progressive and fatal neurodegenerative disease with an adult- onset, characterized by motor neuron degeneration in the primary motor cortex, brainstem, and spinal cord and progressive atrophy and weakness of the skeletal muscles

34. Motor neurons are found in both the brain and spinal cord. The voluntary muscles are the muscles we can control, such as those in the arms, face, and legs. Amyotrophic lateral sclerosis affects all voluntary muscles as it progresses. As the stages of ALS progress, the motor neurons degrade and die, thereby stopping the transmission of signals to the muscles. The muscles weaken and become functionless and the brain can no longer control voluntary movement.

35. The average period of time for a patient to survive ranges from 3 to 5 years. 20% of patients live more than 5 years and about 10% of them live more than 10 years.

36. Other names of ALS Motor neuron disease (MND) Lou Gehrig's disease Charcot's disease.

37. Definitions Amyotrophic lateral sclerosis (ALS), also called Lou Gehrig’s or, is a neurodegenerative disease characterized by progressive degeneration of upper (UMN) and lower (LMN) motor neurons, in the brain and spinal cord. They are the cells that control voluntary muscle activity such as skeletal muscle movement, breathing, speaking and swallowing. Progressive neurodegenerative disease in which the motor neurons gradually degenerates and after some time, they eventually die.

38. Definition Amyotrophic lateral sclerosis (ALS) refers to a group of progressive, neurological diseases that attacks the motor neurons responsible for the functioning of the voluntary muscles.

39. Types There are two types of amyotrophic lateral sclerosis (ALS): Sporadic ALS and Familial ALS. Sporadic ALS: It may happen sometimes without a clear cause and affect anyone, anywhere. Up to 95% of people are affected by the disease. Familial ALS: It relates to the family history of the disease and runs in families. This constitutes 5 to 10% of all cases of ALS.

40. Epidemiology The prevalence of amyotrophic lateral sclerosis ranges between 1 - 6 cases out of every 100,000 people each year, while ALS in 5% - 10% of cases is genetically predominant. Sporadic form : 95% Familial form: 5%, about 10- 20% of them are associated with mutation in the Cu Zn Superoxide dismutase 1 (chromosome 21) Age of onset: Mean age of onset of sporadic ALS is 65 years; mean age of onset of familial ALS ranges from 46- 55 years M:F 3:2 93% of patients in the database are caucasian

41. ETIOLOGY& RISK FACTORS Unknown Age: ALS is most common between the ages of forty and mid-sixties and the risk of ALS increases with age. Gender: Men are more susceptible to developing ALS than women before the age of 65 and after the age of 70, both may develop equally and the sex difference disappears Family History Chemical imbalance: It has been shown that glutamate levels are often higher in people with ALS and the presence of glutamate in large quantities is toxic to nerve cells. Genetic factors Environmental factors

42. Environmental factors Environmental factors may also play a role in the occurrence of the disease. The following environmental factors may cause ALS Exposure to environmental toxins: The exposure to lead or other substances at home or in the workplace may be invariably associated with ALS. Smoking: Smoking can also be an environmental risk factor for ALS. For women, the risk appears to be greater, especially after menopause.

43. Environmental factors Military service: The study report indicates that people who served in the military are more at risk of developing ALS. In the service, military personnel has to deal with traumatic injuries, high levels of exercise, viral infections, extreme stress, and exposure to certain metals or chemicals. One study stated that military personnel who completed their service in the Gulf region during the 1991 war were more susceptible to the development of ALS than military personnel and conscripts who were deployed elsewhere in the world.

44. PATHOPHYSIOLOGY Motor nerve degeneration is triggered by the death of the neuron cell body. Death of cell body leads to the degeneration of the axon. When axons die, the remaining must therefore innervate bigger muscle fibers, leading to the atrophy of muscle cells. Oxidative Stress: imbalance between reactive oxygen species and the body’s ability to detoxify the reactive intermediates (undergo reduction). Leads to the synthesis of free radicals, that react with cell components: proteins, lipids, DNA etc… Disrupting cell functions leading to its death

45. Clinical manifestations The symptoms of ALS vary from one person to another because they depend on which neurons are affected. The progression of the disease varies from person to person, as in the early stages, signs and symptoms of ALS may be hardly noticeable on the patient, but the weakness that accompanies the disease becomes more evident over time. Most patients (about 70%) present with limb- onset ALS, about 25% patients have bulbar- onset disease, and the rest of the patients (5%) manifest respiratory involvement or initial trunk onset.

46. Clinical manifestations Early stage Dysphagia Dysarthria Emotional liability Spasticity Fasciculation's Cramps Muscle weakness Atrophy

47. Progression Dyspnea Loose the ability to walk, or use hands and arms Loose the ability to speak, and swallow Clinical manifestations

48. Clinical manifestations Late stage Dementia Respiratory failure Aspiration pneumonia Occulo- motor nerve of extra ocular muscle is affected May resemble locked in syndrome

49. Diagnosis History & physical examination Electromyography (EMG): Nerve conduction study (NCS): Magnetic Resonance Imaging (MRI) Blood and urine tests Lumbar puncture (spinal tap): Muscle biopsy

50. Management

51. Management There is no cure for amyotrophic lateral sclerosis and treatments cannot reverse the damage of ALS, but current treatments aim to slow the progression of symptoms and prevent serious or unnecessary complications and make you more comfortable and independent.

52. Medications for ALS Rilutek (Riluzole): Rilutek is an oral tablet available to treat ALS approved by the U.S FDA Rilutek inhibits the release of glutamate, slows expectancy from three to the disease progression and extends six months. life This process may involve lowering the levels of glutamate in the body. Glutamate is an excitotoxin that has been linked to nerve cell damage

53. Tiglutik (Riluzole): Tiglutik is an oral suspension approved by the FDA in September 2018 for the treatment of ALS. Exservan (riluzole): Exservan is an oral film approved by the FDA, in November 2019. Exservan is administered without the need for water, which can be beneficial for ALS patients who have difficulty swallowing. Radicava (Edaravone): Radicava was approved by the FDA in May 2017 to treat ALS. Radicava has been shown to reduce the decline in physical activity by one third. Some side effects of Radiceva are headache, bruising, and shortness of breath. It is given daily for two weeks a month.

54. Therapies for ALS Physical therapy: Physical therapy helps people with ALS deal with pain and mobility problems and helps their muscles function at their best. Breathing therapy: Breathing therapy may be needed in a time when the respiratory muscles become weaker than before Occupational therapy: Occupational therapy can help the patient maintain their independence for a longer period despite hand and arm weakness. Speech therapy: Speech therapy is useful when ALS symptoms begin to make speaking more difficult. Other communication methods include an alphabet board or pen and paper, computer- based equipment with the synthesized speech or tablet computers with text- to- speech applications. These devices can help patients communicate with others.

55. Psychological and social support: Psychologists, social workers, and others provide emotional support for patients and their families. Nutritional support Dental hygiene

56. Emerging therapy Gene therapy

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