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IVIG has replaced PE in most centers as the treatment of choice of AIDP IVIG was shown to be at least as effective as PE in nonambulatory adults treated within the rst 2 weeks in a prospective trial (Table 11 4)27,66,69 Importantly, there is no added bene t of IVIG following PE, and it certainly makes to sense to give IVIG and then perform PE27 The dose of IVIG is 20 g/kg body weight infused over 2 5 days Randomized trials are needed to decide the effect of IVIG in children, in adults with mild disease, and in adults who start treatment after more than 2 weeks67 IVIG may inhibit the binding of ganglioside antibodies to their respective antigens, thereby preventing complement activation and subsequent pathophysiological effects70 Treatment with IVIG or PE should begin within the rst 7 10 days of symptoms Improvement with PE and IVIG is often not immediate, with the mean time to improvement of one clinical grade in the various controlled, randomized PE and IVIG studies ranging from 6 days to as long as 27 days63,64,66 There is no evidence that PE beyond 250 mL/kg63,71 73 or IVIG greater than 2 g/kg is of any added bene t in patients with AIDP and stable de cits Further, as noted above, there is no indication for PE followed by IVIG or vice versa However, as many as 10% of patients treated with either PE63,73 or IVIG71,72 develop a relapse following initial improvement In patients who suffer such relapses, we give additional courses of PE or IVIG

1 Make sure you are working in the same file from the last exercise 2 Select the first keyframe of the actions layer and open the Actions panel by

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Unlike chronic in ammatory demyelinating polyneuropathy (CIDP), corticosteroids do not appear bene cial in the treatment of GBS, and, in fact, some patients have done worse A small study of 25 patients treated with IVIG and intravenous methylprednisolone74 did better than a historical control group treated with IVIG alone66 However, a much larger British study of 142 patients treated with methylprednisolone or placebo (approximately half the patients in each group were also treated with PE) failed to demonstrate the ef cacy of corticosteroids75 A double-blind, placebo-controlled randomized study of IVIG plus intravenous methylprednisolone compared to IVIG plus placebo in 233 patients with GBS revealed no signi cant difference between treatment with methylprednisolone and IVIG versus IVIG alone76 Thus, there is indication for supplemental corticosteroid use in patients with GBS Also, a small double-blind, randomized, placebo-controlled safety trial of interferon beta 1a in 19 patients with GBS also treated with IVIG revealed no added bene t of interferon beta 1a

Children with AIDP have clinical, laboratory, and electrophysiologic ndings similar to affected adults8,51,68,77 80 An antecedent infection within 2 months of the attack is appreciated in approximately

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Figure 11 3 Immune mechanisms in (A) AIDP and (B) AMAN, AMSAN, and Miller MFS (A) A bacterial protein epitope is presented by a macrophage to a T cell The T cell is activated, penetrates the endothelium, recognizes a cross-reactive antigen, and, in the lower section, releases cytokines that activate endoneurial macrophages These release enzymes and toxic nitric oxide (NO) radicals and so ultimately invade compact myelin In the upper section, the activated T cell releases cytokines, which help the B cell to produce antibodies that cross a damaged blood nerve barrier and engage unidenti ed cross-reactive epitopes on the abaxonal Schwann cell surface, x complement, damage the Schwann cell, and so produce vesicular dissolution of myelin (B) A bacterial ganglioside-like epitope stimulates B cells to induce antibodies that opsonize cross-reactive axolemmal antigens, x complement and target macrophages to invade the periaxonal space, and block conduction or cause axonal degeneration In Miller MFS the perisynaptic Schwann cell at the motor nerve terminal is also targeted (With permission from Hughes RA, Cornblath DR Guillain-Barre syndrome Lancet 2005;366(9497):1653 1666, Fig 3, p 1568)

In either case, set gTailPtr to point to the new last element in the list. Finally, make sure the next field of the last element in the list is NULL. You ll see why we did this in the next function, ListDVDs().

Plasmapheresis Group North American Trial Number of patients Time to improve one clinical grade (days) Time to walk unaided (all patients) (days) Time to walk unaided (ventilator patients) (days) Time on ventilator (days) % improved at 1 month % improved at 6 months French trial Number of patients % of patients on ventilator after study (days) Time to wean from ventilator (days) Time to walk unaided (days) Time in hospital Dutch IVIG trial Number of patients % of patients improving one clinical grade after 4 weeks Time to improve one clinical grade (days) Time to clinical Grade 2 (days) Ventilator dependent by week 2 (%) Number of multiple complications PE/Sandglobulin Trial Group Number of patients Mean change in clinical grade after 4 weeks Time to wean from ventilator (days) Time to walk unaided (days) Number of patients unable to walk after 48 weeks

122 19 53 97 9 59 97 109 18 70 28 21 73 34 41 69 42 16 121 09 29 49 19 (167%)

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