Mycophenolate Mofetil for Chronic Inflammatory Demyelinating Polyradiculoneuropathy
Author(s) -
A.J. Radziwill,
Kathi Schweikert,
Thierry Küntzer,
Peter Fuhr,
Andreas Steck
Publication year - 2006
Publication title -
european neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.573
H-Index - 77
eISSN - 1421-9913
pISSN - 0014-3022
DOI - 10.1159/000095139
Subject(s) - medicine , polyradiculoneuropathy , prednisone , pediatrics , surgery , guillain barre syndrome
We conducted an open-label study in 7 patients with CIDP diagnosed according to common guidelines [5] . Two women and 5 men with a mean age of 57.6 years (44–67 years), a mean disease duration of 9 years (2–23 years), 4 relapsing courses and 3 progressive courses were enrolled after oral informed consent because of insufficient response to previous therapies or because of a high frequency of IVIg therapy (at least every 4 weeks). Two patients had predominantly sensory signs, 2 predominantly motor signs and 3 motor and sensory signs. Superimposed axonal loss was demonstrated by electroneuromyography in 2 out of 7 cases. One gram of MM was administered twice daily, in 3 patients in association with prednisone and/or IVIg ( table 1 ). Efficacy was defined as stability or improvement of disability after 6 months and/or reduction of IVIg dose within 12 months. The disability was measured by the disability grade (0–5 points; 0 = healthy, 1 = minor signs, 2 = able to walk without assistance but unable to run, 3 = able to walk 5 m only with help, 4 = chair/bed bound, 5 = death), the Neurological Disability Scale (NDS; 0–202 points; 0 = healthy, 202 = worst situation) [6] , the Hammersmith Motor Ability Test Dear Sir, Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), an immune-mediated heterogeneous progressive or relapsing disorder, is considered as the chronic variant of the Guillain-Barre syndrome that differs from the latter disease by its temporal evolution over at least 2 months, by its relative absence of cranial nerve implication or autonomic disorder, by its relative absence of previous infection and by treatment response to corticosteroids. There are several effective evidencebased therapies, but not all patients improve with these treatments. Around 30% of patients do not respond to immunotherapy such as intravenous immunoglobulin (IVIg), plasma exchange, corticosteroids, azathioprine, cyclophosphamide or interferon[1] . Mycophenolate mofetil (MM), a noncompetitive reversible inhibitor of inosine 5 -monophosphate dehydrogenase, is now widely used in transplantation medicine and in immune-mediated diseases. Its antiproliferative effect acts by inhibition of the de novo purine synthesis of activated T and B lymphocytes. MM can improve several neurological disorders such as myasthenia gravis, inflammatory myopathy or CIDP [2–4] . Received: December 13, 2005 Accepted: March 29, 2006 Published online: August 17, 2006
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