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156KUWAIT MEDICAL JOURNAL June 2009Case ReportChronic Inflammatory DemyelinatingPolyradiculoneuropathy in Two ChildrenMaliha Askar Soud Al-Bloushi, Yousif Kassim Habeeb, Eman Sadiq Al-JumahDepartment of Pediatrics, Neurology Unit, Mubarak Al-Kabeer Hospital, KuwaitABSTRACTChronic inflammatory demyelinating polyradiculoneuropathy(CIDP) is relatively rare in children. We report two casesdiagnosed over a thirteen year period. One patient had aKuwait Medical Journal 2009; 41 (2): 156 -161KEY WORDS: CIDP, children, neuropathymonophasic course resulting in complete recovery whilethe other case had a slowly progressive relapsing coursewith significant morbidity.INTRODUCTIONChronic inflammatory demyelinatingpolyradiculoneuropathy (CIDP) is relatively rarein children [1,2] . It is a chronic, potentially treatable,paralytic disorder of the peripheral nervous systemdeveloping over at least two months [3] . Musclestretch reflexes are either absent or depressed.Cerebrospinal fluid shows cytoalbuminologicdissociation. Demyelination is shown in bothelectrophysiological and pathologic studies. Patientscan be classified into two groups; those with completerecovery after a monophasic course and those withprolonged disability and morbidity after a protractedor relapsing course [2,4-6] . Intravenous immunoglobulin(IVIg) and corticosteroids are the first line treatment [7] .In progressive or treatment resistant cases other optionsshould be considered like plasma exchange (PE) andvarious immunosuppressive drugs. We report twocases diagnosed over a thirteen year period; one withmonophasic course resulting in complete recovery andone with slowly progressive relapsing course withsignificant morbidity.CASE REPORTA previously healthy 34-month-old girlpresented with a two month history of progressivegait disturbance after achieving normal walkingat the age of one year. She had frequent falls withdifficulty in standing from sitting position and goingup the stairs. There was no associated upper limbweakness. She had difficulties with chewing andswallowing but no visual or respiratory symptoms.There was no history of skin rash or joint pain. Shehad uneventful prenatal and perinatal periods. Herfamily history was unremarkable for neurologicaldiseases.Examination on presentation revealed a wellchild with wide based waddling gait and mildlumbar lordosis. She had atrophy of quadricepsmuscle with normal sensation and tone. There wasweakness in lower limbs (4/5) but no weaknessin upper limbs, face or eye movement. Knee andankle tendon reflexes were absent. Romberg signwas positive. Sensory examination was normal.Her other systems examination was normal. Over aperiod of three months the course was progressivewith weakness of her lower limbs (3/5) and mildweakness of upper limbs (4/5).Investigations were normal including: creatinekinase, complete blood count, ESR, renal profile,liver profile, lipid profile, calcium, lead, serumimmunoglobulin, X-ray hips and spine, computedtomography of brain, magnetic resonance imaging(MRI) of brain and lower spine. Muscle biopsyshowed non-specific atrophic changes withno inflammatory changes. Cerebrospinal fluidanalysis showed increased protein (862 mg/l) withno cells.Her electrodiagnostic study showed signs ofdenervation affecting lower limbs more than upperlimbs. Motor conduction velocity (CV) in mediannerve was 36 m/s (normal range for age is 53.7 ± 4.70m/s) [8] and pernoneal motor CV was 39 m/s (normalrange for age is 48.7 ± 4.86 m/s) [8] .CIDP was considered and the child receivedher first intravenous immunoglobulin (IVIg)Address correspondence to:Dr Maliha A AL-Bloushi, Neurology Unit, Department of Pediatrics, Mubarak Al-Kabeer Hospital, Kuwait. Tel: 25318 502, E- mail: dr_askar@hotmail.com