Ashmita Yadav

and 4 more

ABSTRACTAcute disseminated encephalomyelitis (ADEM) is rare autoimmune neurological multifocal disorder characterised by inflammatory demyelination of brain and spinal cord usually in response to infection or sequelae of immunisation, most commonly affecting the children.Though a rare one it is estimated that 1 in 125,000-250,000 population is affected by ADEM each year. And such a case of 15 years old girl presented with acute onset of fever and headache and single episode of seizure followed by unconsciousness with GCS 6(E2V2M2).signs of meningeal irritation like neck rigidity, Kernig’s sign and Brudzinski’s sign were positive. investigation report showed Hb-11.4 g/dl ,neutrophlic leukocytosis , normal range of serum electrolytes ,ICT for malaria was negative and MRI of brains showed multiple abnormal T2 hyperintense signal in both parieto-frontal white matter. so she was diagnosed a case of ADEM and treated with intravenous methylprednisolone for 5 days followed by oral corticosteroid in tapering dose for 6 weeks.IntroductionADEM also known as post -infectious encephalomyelitis is a rare autoimmune inflammatory neurological disorder of nervous system mainly central which is characterised by demylination of the brain and spinal cord as a result of infection or sequelae of immunisation. Children are the most affected one but adult case of ADEM occurs following vaccination against Mumps,Measles and rubella(1)(2) . Though rare one estimated of 1 in 125,000-250,000 individuals are affected by ADEM each year(1). More commonly male are the most affected one with a male and female ratio of 1.3 to 1.The risk factors for ADEM include genetics, exposure to infectious organisms,immunization exposure and lighter skin pigmentation. All the ethnic groups are affected globally. The majority of cases follow either viral or bacterial infection and in majority of cases causative pathogen is unidentified(1). The pathogenesis is not fully understood but antigentic exposure in genetically susceptible individuals may cause ADEM either through molecular mimicry and or due to T cell mediated immune mechanism. It has also been suggested that ADEM may occur due to CNS injury secondary to generalised inflammation of brain and spinal cord(4).ADEM patients usually have presentation like rapid onset of fever, headache ,nausea or vomiting confusion, fatigue and neurological features like numbness, difficulty swallowing, vision, seizure, difficult in coordinating voluntary movements such as walking and some degree of impairment of consciousness(1)(3). Though there is no diagnostic criteria for ADEM but presence of signs and symptoms and radiological findings can help in diagnosis of ADEM. In patients with ADEM T2-Weighted and FLAIR magnetic resonance imaging ( MRI) of brain shows bilateral,hyperintense lesion that are diffuse and poorly demarcated(4).ADEM being a autoimmune disease treatment includes I/v Methyl prednisolone at dosage of 20 to 30 mg/kg (max 1g/day) for 3 to 5 days followed by oral corticosteroids in a tapering dose for 4 to 6 weeks.(5). Full recovery is seen in 50 to 70% of cases and ranging of 70 to 90% with some minor residual disability. In some case severity ranges to 8 to 30% with ataxia and hemiparesis.(6)Case report;We hereby present a case of 15 years old girl brought to the Mymensingh Medical College by her parents with the complaints of fever and headache for 3 days. Fever was high grade ,acute in onset without any chills and rigor with no evening rise of temperature . Headache was severe and prolonged for 3 days following which she suddenly developed single episode of seizure followed by unconsciousness for which she was brought to the hospital.On examination she had altered consciousness with GCS 6(E2V2M2),Temperature was raised, blood pressure was 100/80 mm of Hg and signs of meningeal irritation like neck rigidity, Kernig’s sign and Brudzinski’s sign were positive and bilateral planter extensor was found.Other systematic examination findings were normal. As per the clinical findings she was treated for meningoencephalitis with Inj.Ceftriaxone 1g I/V BD, inj. Acyclovir 250mg 1 vial I/v BD ,inj.dexamethasone 1 amp i/v TDS and investigation showedHb-11.4 g/dl with increased sedimentation, neutrophilic leukocytosis with WBC Count 25000/mm^3 and 87% of neutrophils, Serum electrolytes and blood glucose were within the normal limit.ICT for malaria was negative .MRI OF BRAIN revealed. -Multiple abnormal T2 hyperintense signal in both parieto-frontal white matter suggestive of acute disseminated encephalomyelitis(ADEM)