A 3-year-old girl was admitted with typical hemolytic uremic syndrome (HUS) and conservative treatments were initiated. During hospitalization, she had seizures, right hemiparesis, and loss of consciousness. Initial MR of the brain showed changes of acute disseminated encephalomyelitis (ADEM). She was treated with intravenous methylprednisolone and immunoglobulin. Upon improvement of her clinical condition, she was discharged with oral prednisolone which was tapered after two months. After one year fallow-up, the child now has a normal renal function and normal neurodevelopment

Eshaghi, Hamid

Mehrkash, Mehryar

Moghtaderi, Mastaneh

Rahimzadeh, Nahid

Shahrokhi, Amin

English

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A  Rare case of HUS and ADEM- Moghtaderi M et alJournal of pediatric nephrology | Volume 2 | Number 1| 2014 43Case ReportJ Ped. Nephrology 2014;2(1):43-45http://journals.sbmu.ac.ir/jpnA  Rare case of HUS and ADEMHow to Cite This Article: Moghtaderi M, Rahimzadeh N, Eshaghi H, Shahrokhi A, Mehrkash M. A  Rare case of HUS and ADEM. J Ped.Nephrology 2014;2(1):43-45.Mastaneh Moghtaderi,*Nahid Rahimzadeh,Hamid Eshaghi,Amin Shahrokhi,Mehryar Mehrkash*Corresponding AuthorMastaneh Moghtaderidrmoghtaderi@gmail.comA 3-year-old girl was admitted with typical hemolytic uremic syndrome (HUS) andconservative treatments were initiated. During hospitalization, she had seizures,right hemiparesis, and loss of consciousness. Initial MR of the brain showedchanges of acute disseminated encephalomyelitis (ADEM). She was treated withintravenous methylprednisolone and immunoglobulin. Upon improvement of herclinical condition, she was discharged with oral prednisolone which was taperedafter two months. After one year fallow-up, the child now has a normal renalfunction and normal neurodevelopment.Keywords: Hemolytic-Uremic syndrome; Hematuria; Acute DisseminatedEncephalomyelitis; Child.Received: Sep-2013Revised: Nov-2013Accepted: Dec-2013 Running Title: A  Rare case of HUS and ADEMIntroductionA decreased platelet count is found in all types ofHUS but it is temporary and variable.Microangiopathic hemolytic anemia (MAHA) is aCoombs negative hemolytic anemia. Fragmentederythrocytes (schistocytes) are seen on theperipheral blood smear. The plasma level oflactate dehydrogenase is elevated but haptoglobinis reduced. Assessment of serum haptoglobin is avery sensitive test of intravascular hemolysis. It isthe last parameter to retune to normal whenMAHA resolves. There is no correlation betweenthe severity of MAHA and clinical outcome. WhenHUS occurs after an infection, the renal failure willappear suddenly and is typically oligoanuric. Alltypes of HUS have proteinuria. If there iscomplement disregulation, blood pressureelevation is usually severe at onset and difficult tocontrol. Patients with infection induced HUSusually have normal or only mildly elevated bloodpressure at first but during the recovery period ofoliguric renal failure, they experience a transienthypertension. Most of the cases of childhood HUS(over 90%) occur following intestinal E Coliinfection due to its Shiga toxin. There is typicallybloody diarrhea followed by HUS in children over6 months of age. Other cases of HUS occurfollowing severe streptococcal pneumoniainfection as empyema, pneumonia, meningitis, orrarely septicemia [1]. If there is hereditarypredisposition to HUS, the disease presents withan insidious onset, pallor, lethargy, and feedingdiscomfort but the prodormal diarrhea may beabsent. There may be severe hypertension andfamily history of HUS. In these cases, there may bemutations in complement regulators and, lessoften, an inherited deficiency of von Willebrandfactor or a hereditary error of cobalaminmetabolism. There is a small group of HUS cases inwhom the cause is not detected in spite of fullinvestigations, even suspected drug effects,cancer, or bone marrow transplant. They are lessthan 5% of all childhood HUS cases and most ofthem have only one attack with usually goodA  Rare case of HUS and ADEM- Moghtaderi M et alJournal of pediatric nephrology | Volume 2 | Number 1| 2014 44recovery of the renal function, and the prognosisappears to be favorable [2]. One of the rareneurological diseases is acute disseminatedencephalomyelitis (ADEM), which occurs inchildren more than adults. It seems that ADEM is amonophasic inflammatory demyelinatingcondition with clinical manifestations includingencephalopathy, and focal or multifocal neurologicsyndromes due to a central nervous system (CNS)inflammatory disorder. In this condition,especially in children, prolonged and severeheadaches occur along with fever. The clinicalpresentation of ADEM may be similar to otherinflammatory demyelinating disorders, especiallymultiple sclerosis (MS) and neuromyelitis optica;therefore, it is important to distinct it fordetermining the prognosis and treatmentplanning [3]. Over 50% of the patients have ahistory of an illness, usually an infection, two tofour weeks before developing ADEM. Most ofthese illnesses are very mild and brief viral orbacterial infections like an upper respiratory tractinfection. The neurologic symptoms includeconfusion, drowsiness, coma, disequilibrium,unsteadiness, and even falling down, visualblurring or diplopia (occasionally), swallowingdifficulties, and weakness of the upper or lowerlimbs. Few reports indicate that ADEM occursthree months or less following measles, mumps,and rubella vaccination. There is a markedincrease in signal intensity throughout the whitematter in T2-weighted MRI of ADEM patients,which affects corpus callosum and periventricularregion commonly [4]. However, these lesions mayresolve soon and often in the weeks that follow.The CSF is frequently normal but abnormalitieslike mild lymphocytic pleocytosis and increasedprotein concentrations may be detected. High-dose IV methylprednisolone therapy is helpful inapproximately 50% of the cases but in refractorycases, intravenous immunoglobulin and plasmaexchange may be beneficial. However, there is nostandard treatment protocol yet.Case ReportOur case was a 3-year-old girl with fever andgastroenteritis. At first, she was conscious butseverely ill and dehydrated. Laboratory findingsshowed anemia, thrombocytopenia, an elevatedreticulocyte count, and a high lacticdehydrogenase (LDH) level, as well asschistocytosis and spherocytosis in the peripheralblood smear. The Coombs test was negative. Shewas oliguric at first but became anuricsubsequently. The stool examination wasinsignificant and the stool culture wasunremarkable. She was managed as a case of HUSsyndrome. The primary medical care consisted ofreplacement of fluid and electrolytes, peritonealdialysis, and antihypertensive drugs for her mildhypertension. Afterwards, her condition becamebetter with an acceptable urine output per hour.Blood pressure and serum BUN, Cr, reticulocytecount and LDH levels returned to normal soon.However, in the course of hospitalization, bloodydiarrhea, high grade fever and severehypertension were added to her clinicalsymptoms and she experienced status epilepticus.Then, she lost her full consciousness anddeveloped right hemiparesis and aphasia and losther insight to people around her. Serumcomplements, factor H, I, F, ADAMTS 13 andantibodies were normal. Brain MRI demonstratedperiventricular demyelination (compatible withADEM). She received intravenous immunoglobulin(IVIG) and methylprednisolone plus othertherapies and at the same time, serumcomplements and factors H, I, F and serumADAMTS 13 antibody levels were checkedrepeatedly. As she improved and initial problemsdisappeared, she was discharged with oralprednisolone and followed up as an outpatient.Then, the oral corticosteroid dose was taperedafter two months of therapy in conjunction withneurological function improvement. After 18months of outpatient therapy, she had normalurine flow and renal function and no otherepisodes of seizure or any other neurologicaldisruption occurred. All drugs were thendiscontinued.DiscussionWe describe a 3-year-old girl with typical HUS thatwas followed by CNS involvement presenting asA  Rare case of HUS and ADEM- Moghtaderi M et alJournal of pediatric nephrology | Volume 2 | Number 1| 2014 45fever, seizure, and hemiparesia. Brain MRIfindings and CNS involvement suggested ADEMsyndrome.There are no accepted or pathologically verifiedclinical diagnostic criteria for ADEM. Theoccurrence of acute symptoms (more than oneneurological deficit) in a previously healthy child,any change in the mental state, or anycombination of alterations seen on MRI providingthat they included white matter lesions, wereconsidered as ADEM syndrome [3]. More than halfof the patients have an illness, usually an infection,two to four weeks before developing ADEM.However, there is no report of ADEM after HUS inthe literature. After the manifestation ofneurologic deficits, seizure, and fever in ourpatient, we suspected atypical HUS and evaluatedcomplements and factors H, I, F, and Adams 13antibodies, which were all normal.It seems that ADEM is an immune reactionfollowing an infectious attack which targets thecentral nervous system instead of infection andcauses inflammatory changes in it. There isdemyelination of neuroid tissues in ADEM, too [5].The cause-and-effect relationship between viralinfection and many post infection syndromes isimpossible to establish, especially when there is a30-day latency period between the viral infectionand the onset of neurological dysfunction. There isat least four to six viral illnesses each year in aschool age child, so approximately half or more ofthe children have a viral illness 30 days before theonset of any life event. This probability is higherfor preschool-aged children in day care centers.Lethargy, headache, and vomiting are commonpresentations of encephalopathy but thesesystemic features may be symptoms of a viralillness as well. Neurological symptoms oftenpresent abruptly and are characterize by focalmotor signs or altered states of consciousness, orboth. In some children, focal neurological signs donot appear while in others, the initial presentationsuggests a focal mass effect. In the first week, therisk of mortality is the highest and a favorableoutcome is not the rule. Many children with ADEMsyndrome have repeated episodes, a coursesimilar to that of multiple sclerosis [6]. Our casedeveloped ADEM secondary to probably anintestinal bacterial infection but unfortunately, wecould not detect any microorganisms in her stoolsample. However, CNS vasculitis due to HUS itselfcould be a cause of ADEM in our patient.Conflict of InterestNone declaredFinancial SupportNone declaredReferences1. Johnson S. Mark Taylor C. Hemolytic UremicSyndrome. In: Ellis D. Avner, William E. Harmon,Patrick Niaudet. Pediatric Nephrology. 2009. 6thEdition. P. 1155-1167.2. Waters AM, Kerecuk L, Luk D, et al. Hemolyticuremic syndrome associated with invasivepneumococcal disease. J Pediatr 2007;151 (2):140–144.3. Nathan P. Young, D.O. Brian G. et al. AcuteDisseminated Encephalomyelitis: CurrentUnderstanding and Controversies. Semin Neurol.2008;28(1):84-94. (Homepage at www.thieme.com)4. Hynson JL, Kornberg AJ, Coleman LT, et al: Clinicaland radiological features of acute disseminatedencephalo- myelitis in children, Neurology2001;56:1308–1312.5. Khurana DR, Melvin JM, Kothare SV, et al: Acutedissemi- nated encephalomyelitis inchildren: discordant neuro- logic and neuroimagingabnormalities and response to plasmapheresis.Pediatrics 2005;116:431–436.6. National Institute of Neurological Disorders andStroke. Acute Disseminated Encephalomyelitis.www.ninds.nih.gov/ Accessed 11/22/2011

A  Rare case of HUS and ADEM

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A Rare case of HUS and ADEM

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