Chapter 18 AIDP and CIDP Flashcards
Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP, _________-_________ syndrome) – an acquired disease of autoimmune etiology characterized by _________ paresthesias and weakness that can progress to total body paralysis, _________ disturbances, and _________ failure. _________ _________, mycoplasm, _________, Epstein-Barr virus, and Haemophilus influenza are pathogens commonly associated with AIDP.
Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP, Guillain-Barré syndrome) – an acquired disease of autoimmune etiology characterized by ascending paresthesias and weakness that can progress to total body paralysis, autonomic disturbances, and respiratory failure. Campylobacter jejuni, mycoplasm, cytomegalovirus, Epstein-Barr virus, and Haemophilus influenza are pathogens commonly associated with AIDP.
Global incidence is about 0.4 to 1.7/100,000; a mild _________ illness precedes ≈60% of cases by 1 to 4 weeks. More than 50% of patients will complain of pain that is initially described as muscular aching and may transition to _________ pain as disease progresses. _________ muscles and _________ function are typically spared. Diagnosis is supported by _________, progressive weakness in all limbs, relative _________ of involvement, CSF cytoalbuminologic dissociation (elevated protein, 10 mononuclear cells/mm3), and electrodiagnostic findings.
Global incidence is about 0.4 to 1.7/100,000; a mild flulike illness precedes ≈60% of cases by 1 to 4 weeks. More than 50% of patients will complain of pain that is initially described as muscular aching and may transition to neuropathic pain as disease progresses. Extraocular muscles and sphincter function are typically spared. Diagnosis is supported by areflexia, progressive weakness in all limbs, relative symmetry of involvement, CSF cytoalbuminologic dissociation (elevated protein, 10 mononuclear cells/mm3), and electrodiagnostic findings.
_________ or IV Ig (400 mg/kg/day × 5 days) given during the evolution of symptoms (within _________ weeks of onset) is effective and has proven to decrease the overall recovery time. Glucocorticoids are not effective. Early rehabilitation should emphasize stretching and gradual strengthening; aggressive therapies may cause overwork weakness. A tilt table may be useful in patients with autonomic instability. Prescription of appropriate assistive mobility devices and lower limb orthotics is often indicated.
Plasmapheresis or IV Ig (400 mg/kg/day × 5 days) given during the evolution of symptoms (within 2 weeks of onset) is effective and has proven to decrease the overall recovery time. Glucocorticoids are not effective. Early rehabilitation should emphasize stretching and gradual strengthening; aggressive therapies may cause overwork weakness. A tilt table may be useful in patients with autonomic instability. Prescription of appropriate assistive mobility devices and lower limb orthotics is often indicated.
Mortality is about 3% to 5%, usually due to _________ or _________ causes from autonomic dysfunction. Most patients recover completely or nearly completely. Recovery time can be weeks to months, or up to _________ to _________ months if axonal damage has occurred. About 10% have a pronounced residual disability, most often lower leg weakness and numbness of the feet; ≈5% to 10% may suffer one or more recurrences of acute polyneuropathy and some cases may evolve into a chronic, progressive inflammatory polyneuropathy.
Mortality is about 3% to 5%, usually due to respiratory or cardiovascular causes from autonomic dysfunction. Most patients recover completely or nearly completely. Recovery time can be weeks to months, or up to 6 to 18 months if axonal damage has occurred. About 10% have a pronounced residual disability, most often lower leg weakness and numbness of the feet; ≈5% to 10% may suffer one or more recurrences of acute polyneuropathy and some cases may evolve into a chronic, progressive inflammatory polyneuropathy.
Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) – CIDP is pathologically similar to AIDP, but tends to have a slower onset of at least _________ months and may recur multiple times. Weakness is symmetrical with more distal than proximal muscle involvement. Treatment is the same as with AIDP, but CIDP patients with both sensory and motor involvement will respond to high-dose _________ (usual regimen is 80 mg _________ qd, tapered over months to the lowest effective dose). As with AIDP, diagnosis is supported by electrodiagnostics, CSF findings, nerve biopsy, and MRI abnormalities.
Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) – CIDP is pathologically similar to AIDP, but tends to have a slower onset of at least 2 months and may recur multiple times. Weakness is symmetrical with more distal than proximal muscle involvement. Treatment is the same as with AIDP, but CIDP patients with both sensory and motor involvement will respond to high-dose corticosteroids (usual regimen is 80 mg prednisone qd, tapered over months to the lowest effective dose). As with AIDP, diagnosis is supported by electrodiagnostics, CSF findings, nerve biopsy, and MRI abnormalities.
