ACUTE DISSEMINATED ENCEPHALOMYELITIS

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ACUTE DISSEMINATED ENCEPHALOMYELITIS
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PATIENT DETAILS

• 9 month old male visiting Jhb with his parents
  and brother, living in Durban
• Three day history of mild gastroenteritis with a
  low grade fever and a cough.
• One day history of not moving his left arm.
• Birth history- NVD at a hospital in Natal, no
  birth complications and well after birth.

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DETAILS CONTINUED

• PMH- nil
• PSH- nil
• Allergies- nil known
• Medications- Augmentin and Panado from a private
  GP.
• Feeding- Nan, cereal, purity and some vegetables

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DETAILS CONTINUED

• Development- appropriate for his age i.e. pulling
  to stand, sitting on his own and saying a few
  words.
• Social- lives in Durban with his parents and an
  older brother, all of whom are well.
• Immunizations up to date according to mother.

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ON EXAMINATION

• Growth parameters all on 50th centiles
• Vitals essentially normal with a mild pyrexia
• No lymphadenopathy
• Not pale/jaundiced, not dehydrated
• Chest harsh breath sounds bilaterally
• CVS normal heart sounds no murmurs or
  gallop

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EXAMINATION CONTINUED

• Abdomen soft, non tender, no organomegaly
• CNS lethargic and irritable but consolable,
  tone, power and reflexes decreased in
  left upper limb other limbs NAD
  mild terminal neck stiffness
• ENT mild pharyngitis

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INVESTIGATIONS

• Blood results FBC- 9,2/13,4/301
  DIFF-27/10/62 UE- 136/5/103/22/4,2/30 Glucos
  e- 5 CRP- 2,3
• CXR- features suggestive of mild pneumonia
• In view of the apparent localising signs, we
  requested a CT brain prior to doing the LP-
  essentially normal.

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INVESTIGATIONS CONTINUED

• LP results CSF-clear and colourless prot
  ein- 0,33 glucose- 4,1 chloride-123
  polys- 0 lymphs- 40 erythrocytes-
  0 no bacteria grown

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ASSESSMENT AND MANAGEMENT

• 9/12 old male infant with acute mild
  bronchopneumonia and viral meningoencephalitis.
• Treatment cefotaxime physiotherapy neur
  ological consult

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NEUROLOGICAL REVIEW

• On review it was noted that there was mild
  weakness in the left upper limb and that the
  condition seemed to be progressing to involve the
  left lower limb.
• A suggestion was made to order an MRI scan which
  was done a few days later at Donald Gordon Centre
  with a finding of Acute Disseminated
  Encephalomyelitis (ADEM).

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ADEM

• This is an inflammatory demyelinating disorder of
  the subcortical white matter.
• Most frequently seen in children, mf often
  evolving from antecedent infection or
  immunization.
• Typical presentation encephalitic signs with non
  specific CSF changes and minimal or no changes on
  CT brain.

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ADEM CONTINUED

• Thought to be an autoimmune disease via cross
  reactivity of the antiviral antibodies with the
  myelin autoantigens.
• Viruses associated include HSV,HIV, HSV6,
  measles, hepatitis, influenza, EBV etc.
• There has also been an association post
  immunisation for MMR,Influenza, BCG.

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APPROPRIATE INVESTIGATIONS

• Lymphocytosis, raised CRP and ESR
• CSF- can have a raised protein but can
  be normal.
• CT Brain - may be normal
• MRI- gold standard for diagnosis T2 weighted
  images show areas of prolonged T2 in
  subcortical white matter, usually assymetrical.

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TREATMENT

• Empirically treated as a meningitis with
  cefotaxime /- acyclovir.
• Once diagnosis is made then steroids become the
  mainstay of management.
• Physiotherapy can also be helpful.

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DIFFERENTIAL DIAGNOSIS

• At first presentation, it is difficult to
  differentiate between ADEM and MS.
• New lesions and relapses, esp after 6/12 should
  alert to the possibility of MS.
• MS - no prodromal viral illness and no fever or
  meninigism at presentation. It presents as a
  monosymptomatic syndrome eg optic neuritis or
  myelopathy and develops a relapsing remitting
  course.

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PROGNOSIS OF ADEM

• Most make excellent progress over the following
  days, weeks and months with no subsequent
  neurological impairment.
• A minority have neurological impairment eg motor
  disability, visual/ cognitive or behavioural
  impairment.

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