Spectrum and Outcome of Acute Disseminated Encephalomyelitis in Infancy and Childhood

Abstract

Background: Acute disseminated encephalomyelitis (ADEM) is an immune mediated disease of the brain.  It occurs following a viral infection but may appear following vaccination, bacterial or parasitic infection. It occurs in all ages, with average age around 5 to 8 years old. Full recovery is seen in 50 to 75% of cases. The estimated mortality rate may be as high as 5%.

Aim of work: To presents the clinical manifestations of spectrum of acute disseminated encephalomyelitis (ADEM), lines of management and outcome 

Materials and methods:  Sixty ADEM cases with acute onset of irritability, seizures, disturbed consciousness, inability to walk and coma were enrolled. Investigations were fundus examination, CSF analysis and MRI brain. CSF oligoclonalbands , herpes simplex PCR(2cases each) and VLCFA and adrenal profile  ((2cases).Treatment included IV corticosteroid and IVIG (early: 2weeks from onset :35 cases ) andplasmapheresis ( 10 cases)  . Cases were followed up for 6 months 

Results : Fundus showed papilloedema in 20%, CSF : pleocytosis : 53.3% , increased protein : 33.3% .MRI  T2W  showed  multifocal increased signal (52 cases),symmetric multifocal lesions involving thalami , brain stem (2cases) , hemorrhagic areas (2cases)  and bilateral ,symmetric periventricular bright signal (4 cases). Accordingly, acute necrotizing encephalopathy (ANC) and acute hemorrhagic encephalopathy (AHE) were 2cases each, negative herpes PCR .Within 2 months, 2 cases developed second flare up, multiphasic ADEM, absent oligoclonal bands. Two males showed progressive deterioration, hyperpigmentation, adrenal hypofunction (one) with increased VLCFA: Adrenoleukodystrophy (ALD) .Follow up: complete recovery in 70% (83.3% early treatment). residualsequelae in 22.4%:  blindness (6.4%),irritability and seizures (9.6%),spastic quadriparesis and cognitive deficit (16%) and hemiparesis (6.4%).Death in 6.4%(ADEM,ANE,AHE, ALD one case each).

Fig 1:  60 Cases with acute encephalopathy and  MRI white matter changes

Fig 2 : MRI Bain for some cases in our series

Fig 3: Follow up MRI brain showed regressive course

Cases Presentation

Case no 1

• Eight y old male presented with gradual and progressive deterioration of speech,gait disturbance and defective vision of 9 months duration ,recurrent seizures
• Encephalopathy
• MRI Brain
• Adrenal Profile
• VLCFA

 VLCFA

Case no 2

• Male child 5y old presented with def. hearing and disturbed gait of 3 months duration
• Family history: Consanguineous. First order, his male sib.7y  history of  acute Encephalopathy followed by progressive deterioration 1y., MRI showed multifocal white matter abnormality. Suggestive of ADEM
• Ataxia ,def. hearing
• Being second child, his sibs with loss of vision, def. hearing. E.,ryle feeding,seizures
• No possibilities of CNS infect. Or post infectious .
• X linked disorder
• XALD

MRI, VLCFA , Adrenal profile were done

Conclusion: ADEM constituted, mutADEM and multiphasic ADEM showed the best outcome especially early treatment. ANE and AHE showed the worst outcome. MRI of some cases (6.4% ) of ADEM showed bilateral and symmetric periventricular abnormal signal. ALD should be considered among male cases with acute progressive encephalopathy and multifocal MRI abnormalities.