June 09-41-2.indd - Kma.org.kw

More documents

Recommendations

Info

June 2009KUWAIT MEDICAL JOURNAL 159with other autoimmune disorders [23] . There isclearly class I evidence for IVIg as standardtherapy for CIDP but most studies have only beenmade for induction therapy rather than at longterm therapy. Factors in favor of the use of IVIgover steroids includes safer long term profile andease of use (it can be given at home but not in thiscountry) while factors in favor of steroids includethe lower cost, availability worldwide and theease of administration whether intravenous ororal.The main drawbacks of IVIg are higher costand sometimes availability. Minor side effectssuch as rash, headache, fever and chills may beminimized by slowing infusion rate and using antiinflammatorypremedication. Serious side effectsincluding allergic reactions, aseptic meningitis andthromboembolic events occur in less than 5% ofinfusions [1] .There are different therapeutic regimens as todosage and duration of IVIg [24] . One gram/kg/day for two days may induce faster improvementthan 0.4 gram/kg/day for five consecutive days [3] .A dose of 0.4 g/kg body weight was superior to0.2 g/kg when given over five days. It can begiven as 2 g/kg over 2-5 days or 0.4 g/kg weeklyor every other week or every 28 days. The routinecycle of IVIg given over five days results in clinicalimprovement within 7-10 days in 56-79% of adultpatients. The mean half life of IVIg is 18 - 32 days.Therefore, periodic maintenance infusions atintervals of four weeks are needed [22,23,24] . Morethan 40% of patients need at least 1 g/kg every2-8 weeks to prevent further relapses. After 3-6months consider beginning gradual wean off bydecreasing frequency of treatment and loweringthe dose and later trail off the IVIG.Prednisolone is an effective alternative toIVIg as two thirds respond to either treatment.Family must be made aware of potential acuteand chronic risks of corticosteroids. The dose of1-2.5 mg /kg per day is usually used for at leastfour weeks followed by slow tapering. Relapseswere common when dose was lowered. There aremany potential adverse effects for corticosteroidstherapy (both acute and chronic) including weightgain, osteopenia, altered growth (occurred in our2 nd case), hypertension, susceptibity to infectionsand cataracts. These side effects may be minimizedby administering the medication on an alternateday basis or in pulse treatments.Children like adults demonstrate initialimprovement in two thirds of cases [1,7] afterinitiating treatment with prednisolone but childrenrequire continued high dose therapy as on taperingrelapses are frequent. This was evident in our2 nd case. If steroids are needed for more than oneyear, complete withdrawal is often very difficult.Attempts to reduce the dose without addition ofan immunosuppressive medication often lead tounnecessary relapses.The use of high dose pulse methylprednisolonehas been suggested with similar efficacy to IVIgand oral steroids [6,24] but physicians should beaware of possible significant clinical deteriorationafter high dose intravenous methylprednisoloneas reported by Rostasy et al [25,26] .PE is considered as a third option [22] andtrials comparing IVIg with PE did not findsignificant difference in short term effect of thesetwo treatments [1,17,23] . Although 60% of patientsrespond to PE, it is less often used in childrenbecause of technical difficulties with installationof central catheters which may account for itslimited use in young children [1] . In addition totechnical difficulties, there are potential infectious,thrombotic and hemodynamic complications ofthe treatment itself. The effect lasts only few weekswith over two thirds having a relapse or end ofdose effect after two weeks making it unsuitablefor long term treatment. It is usually administered2-3 times per week for 6-10 treatments and maybe tapered slowly. Improvement may beginwithin days but it is temporary and relapsesand even rebound phenomenon are commonwhen treatment is terminated. PE is an effectivetemporary or adjuvant therapy for CIDP butimmunosuppressive drug treatments are requiredfor long term management.The short term effects of initial treatment withIVIg and PE, in addition to the serious side effectsof prolonged use of steroids necessitates the use ofseveral immunosuppressive agents as adjunctivetherapy to reduce the dose of steroids or the frequencyof IVIg and PE courses [23,27] . In these treatment resistantcases, different immunosuppressant includingazathioprine, methotrexate [27] , cyclosporine A [28] ,cyclophosphamide [6] , mycophenolate [29] , interferon-αand β-Ia, can be used but the evidence is insufficientto recommend one over the other [1,14] .PrognosisThe prognosis in children is generally morefavorable than adults with complete remissionor minimal weakness in 70-100% cases [1,2,3,9] . Incomparison, adults exhibit moderate to severesequelae in 30-40% of cases. A small number ofchildren respond poorly to all treatment modalitiesor develop significant side effects to protractedimmunosuppression resulting in moderate tosevere neurological disability. Pain at onset andinfectious prodromal illness predicts a betteroutcome while axonal loss on electrodiagnosticstudies predicts a poor prognosis [2] .
160CONCLUSIONChildhood CIDP is rare but potentially treatableand should be considered in any child withsubacute neuropathy, where hereditary causes areexcluded. Treatment should be initiated early inorder to minimize demyelination and secondaryaxonal loss.REFERENCESChronic Inflammatory Demyelinating Polyradiculoneuropathy in Two Children1. Rossignol E, D’Anjou G, Lapointe N, Haddad E,Vanasse M. Evolution and treatment of childhoodchronic inflammatory polyneuropathy. Pediatr Neurol2007; 36:88-94.2. Connolly AM. Chronic inflammatory demyelinatingpolyneuropathy in childhood. Pediatr Neurol 2001;24:177-182.3. Nevo Y, Topalogu H. 88 th ENMC International workshop:Childhood chronic inflammatory demyelinatingpolyneuropathy (including revised diagnostic criteria),Naarden, The Netherlands, December 8-10, 2000.Neuromuscul Disord 2002; 12:195-200.4. Rodriguez-Casero MV, Shield LK, Kornberg AJ.Subacute inflammatory demyelinating polyneuropathyin children. Neurology 2005; 64:1786-1788.5. Hattori N, Ichimura M, Aoki S, et al. Clinicopathologicalfeatures of chronic inflammatory demyelinatingpolyradiculoneuropathy in childhood. J Neurol Sci1998; 154:66-71.6. Rayan MM, Grattan-Smith PJ, Procopis PG, MorganG, Ouvrier RA. Childhood chronic inflammatorydemyelinating polyneuropathy: clinical course and longtermoutcome. Neuromuscul Disord 2000; 10:398-406.7. Korinthenberg R. Chronic inflammatory demyelinatingpolyradiculoneuropathy in children and their responseto treatment. Neuropediatrics 1999; 30:190-196.8. Cai F, Zhang J. Study of nerve conduction and lateresponses in normal Chinese infants, children andadults. J Child Neurol 1997; 12:13-18.9. Simmons Z, Wald JJ, Albers JW. Chronic inflammatorydemyelinating polyradiculoneuropathy in children:Presentation, electrodiagnostic studies and initialclinical course, with comparison to adults. MuscleNerve 1997; 20:1008-1015.10. Polat M, Tekgul H, Kilincer A, et al. Electrodiagnosticpattern approach for childhood polyneruopathies.Pediatr Neruol 2006; 35:11-17.11. Koul R, Chacko A, Javed H, et al. A profile of childhoodneuropathies at a university hospital in Oman. SaudiMed J 2002; 23:450-456.12. Cocito D, Durelli L, Isoardo G. Different clinical,electrophysiological and immunological features ofCIDP associated with paraproteinaemia. Acta NeurolScand 2003; 108:274-280.13. Research criteria for diagnosis of chronic inflammatorydemyelinating polyneuropathy (CIDP). Report froman Ad Hoc Subcommittee of the American Academy ofNeurology AIDS Task Force. Neurology 1991; 41:617-618.June 200914. Odaka M, Yuki N, Hirata K. Patients with chronicinflammatory demyelinating polyneuropathyinitially diagnosed as Guillain-Barre' syndrome. JNeurol 2003; 250:913-916.15. Mori K, Hattori N, Sugiura M, et al. Chronicinflammatory demyelinating polyneuropathypresenting with features of GBS. Neurology 2002;58:979-982.16. Rodriguez-Casero MV, Shield LK, Coleman LT,Kornberg AJ. Childhood chronic inflammatorydemyelinating polyneuropathy with central nervoussystem demyelination resembling multiple sclerosis.Neuromuscul Disord 2003; 13:158-161.17. Nobile-Orazio E, Terenghi F. IVIg in idiopathicautoimmune neuropathies: analysis in the light ofthe latest results. J Neurol 2005; 252 Supp1:I7-1318- Likasitwattanakul S, Visrutaratna P. Chronicinflammatory demyelinating polyneuropathy ina child: clinical-spinal MR imaging correlation.Singapore Med J 2004; 45:536-537.19. Midroni G, de Tilly LN, Gray B, Vajsar J. MRI of thecauda equina in CIDP: clinical correlations. J NeurolSci 1999; 170:36-44.20. Molenaar DS, Vermeulen M, de Haan R. Diagnosticvalue of sural nerve biopsy in chronic inflammatorydemyelinating polyneuropathy. J Neurol NeurosurgPsychiatry 1998; 64:84-89.21. van Schaik IN, Winer JB, de Haan R,Vermeulen M. Intravenous immunoglobulinfor chronic inflammatory demylinatingpolyradiculoneuropathy: a systematic review. LancetNeurol 2002; 1:491-498.22- Hughes RA. Systematic reviews of treatment forinflammatory demyelinating neuropathy. J Anat2002; 200:331-339.23. Nobile-Orazio E. Treatment of dys-immuneneuropathies. J Neurol 2005; 252:385-395.24. Gold R, Kieseier BC. Therapy of immuneneuropathies with intravenous immunoglobulin. JNeurol 2006; 253 Suppl 5 : V59-63.25. Lopate G, Pestronk A, Al-Lozi M. Treatment of chronicinflammatory demyelinating polyneuropathywith high-dose intermittent intravenousmethylprednisolone. Arch Neurol 2005; 62:249-254.26. Rostasy KM, Diepold K, Buckard J, Brockmann K,Wilken B, Hanefeld F. Progressive muscle weaknessafter high-dose steroids in two children with CIDP.Pediatr Neurol 2003; 29:236-238.27. Fialho D, Chan YC, Allen DC, Reilly MM, Hughes RA.Treatment of chronic inflammatory demyelinatingpolyradiculoneuopathy with methotrexate. J NeurolNeurosurg Psychiatry 2006; 77:544-547.28. Visudtibhan A, Chiemchanya S, Visudhiphan P.Cyclosporine in chronic inflammatorydemyelinatingpolyradiculoneuropathy. Pediatr Neurol 2005;33:368-372.29. Umapathi T, Hughes R. Mycophenolate in treatmentresistantinflammatory neuropathies. Eur J Neurol2002; 9:683-685.
Page 2 and 3:
Vol. 41 No. 1KUWAIT MEDICAL JOURNAL
Page 4:
KUWAIT MEDICAL JOURNALKUWAIT MEDICA
Page 8 and 9:
June 2009KUWAIT MEDICAL JOURNAL 91E
Page 10 and 11:
June 2009KUWAIT MEDICAL JOURNAL 93R
Page 12 and 13:
June 2009KUWAIT MEDICAL JOURNAL 95O
Page 14 and 15:
June 2009KUWAIT MEDICAL JOURNAL 97c
Page 16:
June 2009KUWAIT MEDICAL JOURNAL 99p
Page 20 and 21:
June 2009KUWAIT MEDICAL JOURNAL 103
Page 22 and 23:
Page 24 and 25:
Page 26 and 27: June 2009KUWAIT MEDICAL JOURNAL 109
Page 74 and 75: June 2009400 mg/kg/day for five day
Page 86 and 87: March 2009KUWAIT MEDICAL JOURNAL 16
show all

June 09-41-2.indd - Kma.org.kw

You also want an ePaper? Increase the reach of your titles

Delete template?

Save as template?