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IMAGE OF THE WEEK



           THELENGANA A
           PG 1STYR
           FROM IMCU WARD
• 15 Yrs old female presented with h/o
               Fever 2 days

              Asymptomatic 10 days

              Headache,vomiting

              Altered sensorium for 1 week
No h/o seizures/visual disturbance
No h/o vaccination /exanthematous illness
O/E
    vitals were stable
    CNS examn :Pt was drowsy , arousable with
    painful stimulus
    PERL , DEM preserved
    exaggerated DTR
    B/L plantar extensor
    fundus examination – B/L disc edema
Other systemic examination was unremarkable
OPEN RING SIGN
MULTIPLE SCLEROSIS
DAWSONS FINGERS
CNS TUBERCULOSIS
CNS TOXOPLASMOSIS
PML
ACUTE DISSEMINATED
          ENCEPHALOMYELITIS
   Inflammatory, nonvasculitic, demyeli
    nating, immune
    mediated, monophasic and
    polysymptomatic disease of the
    central nervous system



    Post infectious encephalomyelitis,
    Post vaccination encephalomyelitis
PATHOGENESIS
• Molecular mimickery: brain vaccines
  – Th2 lymphocytes have increased reactivity to
    myelin basic protein

• Inflammatory cascade concept:
   – CNS infections triggering immune response,
     damage to BBB, brain specific antigens spills into
     systemic circulation and initiates immunologic
     process
ADEM                MULTIPLE SCLEROSIS

PRODROMAL PHASE                NO PRODROMAL PHASE

ALTERED SENSORIUM              PRESERVED AWARENESS

MENINGISMUS                    NO MENINGISMUS

NEUROSYCHIATRIC DISORDER       NO NEUROPSYCHIATRIC

B/L OPTIC NEURITIS             UNILATERAL OPTIC NEURITIS
COMPLETE TRANSVERSE MYELITIS   INCOMPLETE
SEIZURES                       DIPLOPIA

ATAXIA                         RELAPSING

MONOPHASIC                     POLYPHASIC

POLYSYMPTOMATIC                MONOSYMPTOMATIC
INVESTIGATIONS
CSF ANALYSIS
CT BRAIN
MRI – T2 , FLAIR, CONTRAST
    – MTR
EEG,VEP
NEUROIMAGING
• MRI: extensive, multifocal, subcortical
  white matter abnormalities
• MRI: subcortical white matter, may be grey
  matter also,
• CT may be normal in 50% cases
• Convalescent MRI helpful in diffrentating with
  MS, new lesions in MS
MRI Features
                    ADEM
• Patchy, poorly marginated areas of increased signal
  intensity; large, asymmetric, multiple

• Four patterns:
   – ADEM with less than 5 mm lesions
   – Large, confluent lesions with edema and mass
     effect
   – ADEM with additional symmetric bithalamic
     involvement
   – Acute hemorrhagic encephalomyelitis (worst
     prognosis)
RECURRENCE
    OF ADEM


RDEM       MDEM
TREATMENT

• Broad spectrum antibiotics and acyclovir until
  an Infectious etiology is excluded.
• Methylprednisolone in a dose of 30 mg/kg per
  day intravenously up to a maximum dose of
  1000 mg per day X 5 days
• Plasmapharesis
• Intravenous immunoglobulin
• Cyclosporin , cyclophosphamide
• Methylpred + IVIG
PROGNOSIS
• Mortality: 10% in older studies, Now <2%
• Morbidity: visual, motor, autonomic, and intellectual
  deficits and epilepsy.


   – Problems persist after the first few weeks of
     illness in only about 35% of cases, and in most of
     these patients, the deficits resolve within 1 year of
     onset.
FOLLOW UP
• The long-term (10-y follow-up) risk of patients
  with ADEM for development of MS is 25%.
• Risk for MS is highest in children whose ADEM
  onset was
  – (1) afebrile,
  – (2) without mental status change,
  – (3) without prodromal viral illness or
    immunization,
  – (4) without generalized EEG slowing,
  – (5) associated with an abnormal CSF immune
    profile

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Adem

  • 1. IMAGE OF THE WEEK THELENGANA A PG 1STYR FROM IMCU WARD
  • 2. • 15 Yrs old female presented with h/o Fever 2 days Asymptomatic 10 days Headache,vomiting Altered sensorium for 1 week No h/o seizures/visual disturbance No h/o vaccination /exanthematous illness
  • 3. O/E vitals were stable CNS examn :Pt was drowsy , arousable with painful stimulus PERL , DEM preserved exaggerated DTR B/L plantar extensor fundus examination – B/L disc edema Other systemic examination was unremarkable
  • 4.
  • 5.
  • 6.
  • 12. PML
  • 13. ACUTE DISSEMINATED ENCEPHALOMYELITIS  Inflammatory, nonvasculitic, demyeli nating, immune mediated, monophasic and polysymptomatic disease of the central nervous system Post infectious encephalomyelitis, Post vaccination encephalomyelitis
  • 14. PATHOGENESIS • Molecular mimickery: brain vaccines – Th2 lymphocytes have increased reactivity to myelin basic protein • Inflammatory cascade concept: – CNS infections triggering immune response, damage to BBB, brain specific antigens spills into systemic circulation and initiates immunologic process
  • 15. ADEM MULTIPLE SCLEROSIS PRODROMAL PHASE NO PRODROMAL PHASE ALTERED SENSORIUM PRESERVED AWARENESS MENINGISMUS NO MENINGISMUS NEUROSYCHIATRIC DISORDER NO NEUROPSYCHIATRIC B/L OPTIC NEURITIS UNILATERAL OPTIC NEURITIS COMPLETE TRANSVERSE MYELITIS INCOMPLETE SEIZURES DIPLOPIA ATAXIA RELAPSING MONOPHASIC POLYPHASIC POLYSYMPTOMATIC MONOSYMPTOMATIC
  • 16. INVESTIGATIONS CSF ANALYSIS CT BRAIN MRI – T2 , FLAIR, CONTRAST – MTR EEG,VEP
  • 17. NEUROIMAGING • MRI: extensive, multifocal, subcortical white matter abnormalities • MRI: subcortical white matter, may be grey matter also, • CT may be normal in 50% cases • Convalescent MRI helpful in diffrentating with MS, new lesions in MS
  • 18. MRI Features ADEM • Patchy, poorly marginated areas of increased signal intensity; large, asymmetric, multiple • Four patterns: – ADEM with less than 5 mm lesions – Large, confluent lesions with edema and mass effect – ADEM with additional symmetric bithalamic involvement – Acute hemorrhagic encephalomyelitis (worst prognosis)
  • 19. RECURRENCE OF ADEM RDEM MDEM
  • 20. TREATMENT • Broad spectrum antibiotics and acyclovir until an Infectious etiology is excluded. • Methylprednisolone in a dose of 30 mg/kg per day intravenously up to a maximum dose of 1000 mg per day X 5 days • Plasmapharesis • Intravenous immunoglobulin • Cyclosporin , cyclophosphamide • Methylpred + IVIG
  • 21. PROGNOSIS • Mortality: 10% in older studies, Now <2% • Morbidity: visual, motor, autonomic, and intellectual deficits and epilepsy. – Problems persist after the first few weeks of illness in only about 35% of cases, and in most of these patients, the deficits resolve within 1 year of onset.
  • 22. FOLLOW UP • The long-term (10-y follow-up) risk of patients with ADEM for development of MS is 25%. • Risk for MS is highest in children whose ADEM onset was – (1) afebrile, – (2) without mental status change, – (3) without prodromal viral illness or immunization, – (4) without generalized EEG slowing, – (5) associated with an abnormal CSF immune profile