ACUTE DISSEMINATED

ENCEPHALOMYELITIS
 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.
 DETAILS CONTINUED
• PMH- nil
• PSH- nil
• Allergies- nil known
• Medications- Augmentin and Panado from
a private GP.
• Feeding- Nan, cereal, purity and some
vegetables
 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.
 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
 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
 INVESTIGATIONS
• Blood results
FBC- 9,2/13,4/301 DIFF-27/10/62
U&E- 136/5/103/22/4,2/30
Glucose- 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.
 INVESTIGATIONS
CONTINUED
• LP results
CSF-clear and colourless
protein- 0,33
glucose- 4,1
chloride-123
polys- 0
lymphs- 40
erythrocytes- 0
no bacteria grown
 ASSESSMENT AND
MANAGEMENT
• 9/12 old male infant with acute mild
bronchopneumonia and viral
meningoencephalitis.
• Treatment: cefotaxime
physiotherapy
neurological consult
 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).
 ADEM
• This is an inflammatory demyelinating
disorder of the subcortical white matter.
• Most frequently seen in children, m=f ;
often evolving from antecedent infection or
immunization.
• Typical presentation: encephalitic signs
with non specific CSF changes and
minimal or no changes on CT brain.
 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.
 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.
 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.
 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.
 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.