GBS.pptx
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Guillain-Barré syndrome is an acute, autoimmune polyradiculoneuropathy that causes rapid progressive ascending paralysis. It is usually preceded by a respiratory or gastrointestinal infection. Clinical features include rapidly evolving areflexic motor paralysis that starts in the legs and may involve the face. Treatment involves intravenous immunoglobulin or plasmapheresis to prevent progression of symptoms. Most patients recover fully within months, but some have recurring or chronic symptoms.
GBS.pptx
- 1. Guillain-Barré syndrome Dr.Manoj Aryal MBBS,MD(KU) Physician/Internist Department of Internal medicine LMCTH Palpa
- 2. INTRODUCTION • An acute, frequently severe, and fulminant polyradiculoneuropathy that is autoimmune in nature. • Rapid progressive ascending paralysis. • An acute monophasic paralyzing illness, usually provoked by a preceding infection. • Heterogeneous condition with several variant forms.
- 3. PATHOGENESIS
- 4. EPIDEMIOLOGY • Occurs world-wide with an overall incidence of 1 to 2 cases per 100,000 per year. • All age groups are affected but is slightly greater in males than in females. • In Western countries, adults are more frequently affected than children.
- 5. ANTECEDENT EVENTS • Approximately 70% of cases occurs after1–3 weeks of an acute infectious process, usually respiratory or gastrointestinal. • 20–30% of all cases are preceded by infection or reinfection with Campylobacter jejuni. • Other human herpes virus infection, often CMV or Epstein-Barr virus. Other viruses (e.g., HIV, hepatitis E, Zika) and also Mycoplasma pneumonia. • C. jejuni has also been implicated in summer outbreaks of AMAN among children and young adults exposed to chickens in rural China. • Other triggering events, develop after immunization, surgery, trauma, and bone- marrow transplantation, linked to systemic processes, including Hodgkin lymphoma, systemic lupus erythematosus, and sarcoidosis
- 6. CLINICAL FEATURES • Rapidly evolving areflexic motor paralysis, with or without sensory disturbance. • An ascending paralysis that may be first noticed as rubbery legs. • Absent or depressed deep tendon reflexes. • The legs are usually more affected than the arms, and facial diparesis is present in 50% of affected individuals. • Cutaneous sensory deficits (e.g., loss of pain and temperature sensation) are usually relatively mild. • Autonomic involvement is common. • Pain is another common symptoms.
- 7. CLINICAL FEATURES • No cranial nerve, bladder and bowel involvement • The syndrome of inappropriate antidiuretic hormone secretion (SIADH), which may be due to autonomic involvement, is another complication of GBS. • Unusual features of GBS include papilledema, facial myokymia, hearing loss, meningeal signs, vocal cord paralysis, and mental status changes, reversible posterior leukoencephalopathy syndrome has been associated with GBS. • Once clinical worsening stops and the patient reaches a plateau (almost always within 4 weeks of onset), further progression is unlikely.
- 9. GBS VARIANTS
- 10. Acute inflammatory demyelinating polyneuropathy • most common form in the United States and Europe. • Peripheral nerve myelin is the target of immune attack in AIDP. • Studies from the U.S and Europe, reflecting that in AIDP, shows the following clinical features: • The weakness usually starts in the legs, but begins in the arms or facial muscles in about 10%. • Severe respiratory muscle weakness need of ventilatory support develops in 10 to 30 %. • Facial nerve palsies occur in more than 50 %, and oropharyngeal weakness eventually occurs in 50 %.
- 11. Acute inflammatory demyelinating polyneuropathy • Oculomotor weakness occurs in about 15 %. • Decreased or absent reflexes in affected arms or legs are about 90 % of presentation and with disease progression. • Paresthesia's in the hands and feet accompany the weakness in more than 80 %, but sensory abnormalities on examination are frequently mild. • Pain due to nerve root inflammation, typically located in the back and extremities, can be a presenting feature. • Dysautonomia occurs in 70 %.
- 12. Acute motor axonal neuropathy • Known as AMAN, was first recognized in 1986. • Preceded by Campylobacter jejuni infection, so has a seasonal incidence, being more frequent in the summer. • Occurs frequently in Asia, particularly in young people. • Deep tendon reflexes are occasionally preserved & Sensory nerves are not affected. • The presenting clinical features and recovery of AMAN are otherwise similar to those of AIDP.
- 13. Acute motor and sensory axonal neuropathy • More severe form of AMAN, in which both sensory and motor fibers are affected with marked axonal degeneration, causing delayed and incomplete recovery. • Clinically, AMSAN resembles the AMAN variant but has more sensory symptoms. • The pathology is predominantly axonal lesions of both motor and sensory nerve fibers.
- 14. Miller Fisher syndrome • The typical presentation is that of ophthalmoplegia with ataxia and areflexia. • Incomplete forms include acute ophthalmoplegia without ataxia, and acute ataxic neuropathy without ophthalmoplegia. • The MFS variant accounts for ~5% of all cases and is strongly associated with antibodies to the ganglioside GQ1b
- 15. Other variants • Bickerstaff encephalitis • Pharyngeal-cervical-brachial weakness • Paraparesis • Acute pan dysautonomia • Pure sensory GBS.
- 18. Laboratory Features • Cerebrospinal fluid analysis: • an elevated CSF protein with a normal CSF white blood cell count. • known as albumin cytologic dissociation • Present in first week after the onset of symptoms
- 19. Laboratory Features • Electrodiagnostic studies: • Nerve conduction studies (NCS) and needle electromyography (EMG) are valuable for confirming & for providing information regarding prognosis. Also useful in classifying the main variants of GBS as demyelinating (eg, AIDP ) or axonal (eg, AMAN ) • Demyelinating forms: are supported by features of demyelination, including decreased motor nerve conduction velocity, prolonged distal motor latency, increased F wave latency, conduction blocks, and temporal dispersion. • Axonal forms: are supported by decreased distal motor and/or sensory amplitudes. Transient motor nerve conduction block (ie, reversible conduction failure) can be present
- 20. Laboratory Features • Antibodies: • testing for serum IgG antibodies to GQ1b is useful for the diagnosis of Miller Fisher syndrome • Antibodies to GQ1b may also be present in GBS with ophthalmoparesis, Bickerstaff encephalitis, and the pharyngeal-cervical brachial but not in disorders other than GBS • MRI: • Spinal MRI may reveal thickening and enhancement of the intrathecal spinal nerve roots and cauda equine and also enhancement of the oculomotor, abducens, and facial nerves may be seen.
- 23. TREATMENT • In the vast majority of patients with GBS, treatment should be initiated as soon after diagnosis as possible • Each day counts; -2 weeks after the first motor symptoms, it is not known whether immunotherapy is still effective. • If the plateau stage is reached , then treatment probably is no longer indicated, unless there is severe motor weakness and one cannot exclude the possibility that an immunologic attack is still ongoing • High-dose intravenous immune globulin (IVIg) or plasmapheresis can be initiated • IVIg is administered as five daily infusions for a total dose of 2 g/kg body weight • A course of plasmapheresis usually consists of ~40–50 mL/kg PE 4–5 times over 7–10 days. • Glucocorticoids have not been found to be effective in GBS.
- 24. Prognosis and Recovery • Approximately 85% achieve a full functional recovery within several months to a year, although minor findings (such as areflexia) may persist & often complain of continued symptoms, including fatigue. • The mortality rate is <5% in optimal settings; death usually results from secondary pulmonary complications. The outlook is worst in patients with severe proximal motor and sensory axonal damage. • Such axonal damage may be either primary or secondary in nature. • Between 5 and 10% of patients with typical GBS have one or more late relapses; many of these cases are then classified as chronic inflammatory demyelinating polyneuropathy (CIDP).
- 25. REFERENCES • Davidsons-Principles-and-Practice-of-Medicine-23rd-Edition • Harrisons Principle of Internal Medicine 21th edition.
Editor's Notes
- #6: Campylobacter jejuni infection is the most commonly identified precipitant of GBS. A case-control study from the United Kingdom involving 103 patients with the disease found that 26 percent of affected individuals had evidence of recent. about 60 to 70 percent of AMAN and AMSAN cases and up to 30 percent of AIDP cases are preceded by C. jejuni infection