Dear Colleague s4

Dear Colleague,

1. We are exploring the feasibility of a clinical trial on the effectiveness of IVIG in relapses of Neuromyelitis Optica (NMO) - either myelitis or optic neuritis - or any other acute longitudinally extensive transverse myelitis (LETM) e.g. post viral /idiopathic TM. MS related myelitis is excluded. Many of us currently use plasma exchange (PLEX) but not IVIG, though there are anecdotal reports of the latter’s benefit.

2. There are 2 proposals: a. Two-armed trial of any patient with LETM/NMO relapse to be randomised at the outset itself to steroids alone vs. steroids + IVIG, with the option of rescue PLEX in both groups. (Hypothesis: combination Rx at the outset improves outcomes at 3/6 months and/or reduces need for PLEX)

b. Two-armed trial of patients who have not responded to initial steroid treatment to be randomised to IVIG vs. PLEX. Both arms again would have the option of ‘rescue’ PLEX. (Hypothesis: PLEX and IVIG may have similar effects. If correct, then patients will benefit from early use of an easy safe treatment as in GBS and myasthenia; If wrong, then we should argue for ease of access to PLEX)

3. If you could return the brief questionnaire to us, it would be extremely helpful in planning a study. The study is planned to be undertaken along with Paediatric Neurologists.

4. We are also planning a retrospective review on IVIG use in the above conditions and we welcome cases and invite you to become a co –author. Thank you for your support

Anu (Jacob) and Jackie (Palace)

PS - You could return this form by email to me ([email protected] , click forward mail in outlook, open word doc, complete survey, SAVE and send!) or fax to 0151 529 5513 for the attention of Jenny Hatton, Coordinator NMO service, Liverpool

NMO IVIG Trial –Survey to assess interest AJ V.2 1.12.11 2

Your Name & Hospital

Is this the best email id to contact Yes No. Email me at: you?

1 Do you treat patients with Yes No (Ok ! we wont bother LETM/NMO you anymore but please return form) 2 How many patients NMO LETM (approximately) do you have in your center with: (put numbers in columns) 3 How many do you follow up personally? 4 How many new patients present every year ? 5 How many acute relapses attacks have you seen in last 1 year? 6 How are you treating patients during acute episodes :IVMP, prednisone, IVIG, PLEX? e.g.: 5 days of IVMP- if no response in 2 weeks 5 cycles of PLEX 7 When do you decide to start PLEX after initial steroids? (What level of neurological disability) e.g. <1/5 power in legs 8 Would you PLEX a patient with an acute episode but can walk with: -2 canes -1 cane Can walk normally but is incontinent Can walk normally but has burning pain /numbness in legs Who has only acute unilateral N/A ON 6/36 9 Who has only acute unilateral N/A ON 6/60 10 On average by what day after admission do you decide to do PLEX assuming steroids are given only after admission

NMO IVIG Trial –Survey to assess interest AJ V.2 1.12.11 3

11 How many days after the decision does PLEX actually begin? (delays are common- anesthetists not available, PLEX nurse on leave, too many cases – wait till next week etc. etc.) 12 How many PLEX have you arranged in the last 1 year? 13 What is your experience with >75% patients >75% improve PLEX? (highlight and underline improve 75-50% improve 75-50% improve <50% improve your choice) <50% improve <25% improve <25% improve 14 Have you ever used IVIG in acute episodes? If so how many patients 15 Do you think IVIG could have  Likely to have  Likely to have benefit some benefit in treatment of benefit equivalent to PLEX equivalent to  Some benefit but less acute NMO relapses or 1st PLEX than PLEX episodes of idiopathic LETM  Some benefit  Unlikely to have any (highlight and underline your but less than benefit PLEX  Don’t know choice)  Unlikely to have any benefit  Don’t know 16 Would you be interested in Yes / NO Yes / NO ( If ‘No’ please explain (Pl explain) joining a clinical trial to why not, so we can try to investigate the effectiveness of get you interested!) IV immunoglobulin or plasmapheresis in acute Non- MS LETM and NMO relapses?

17 If yes, how many patients could you randomise /year? 18 Any other thoughts?

NMO IVIG Trial –Survey to assess interest AJ V.2 1.12.11