Acute disseminated encephalomyelitis (ADEM) .Case Report.

2. Acute disseminated encephalomyelitis
(ADEM) .Case Report.
• Azad A. Haleem1 Khaled Hussein Haleem 2
• 1.Pediatrician &Lecturer at college of Medicine, University of
Duhok/Kurdistan/Iraq.
• 2.Pediatric SHO in Hevi pediatrics teaching hospital, Duhok/Kurdistan/Iraq.

4. Case Summary
Nine years old girl ………
• Upper respiratory tract (URT) illness, which
was characterized by fever, dry cough and
neck lymph nodes.
• With the onset of this prodromal URT illness,
the patient was given Amoxicillin by the
primary physician.
08-04-2018

5. • Two days after Amoxicillin was started, she
developed a diffuse maculopapular rash
involving the face, trunk and limbs.
• This had been the first time she developed a
rash following medications although she did
receive amoxicillin in the past, this rash was
treated as an allergic reaction.

6. • The girl's URT symptoms subsided and her rash was
resolving within the first 7 days.
• She subsequently developed the neurologic symptoms:
• Headache
• Vomiting
• Fever
• Confusion
• Seizures
• Generalized body weakness and
• Alter left eye vision
• and was admitted to the unit.

7. • Her medical history & Family history was
essentially unremarkable and was up to date
on his vaccination schedule.

8. Primary investigations
• The following investigation done to the
patient:
• A CT examination of the brain showed no
appreciable parenechymal abnormality.
• Cerebrospinal fluid (CSF) analysis normal.

9. Diagnosis ???
• They insist on meningo encephalitis and put
the patient on:
• Supportive measures and
• Vancomycin , ceftriaxone & acyclovir
• But the condition did not improve

10. On further enquiry
• By complete assessment of patient regarding history,
physical examination and farther investigations .
• History: fever, dry cough and neck lymph nodes.
• With the onset of maculopapular rash after
Amoxicillin ??
• This is mostly infectious mononucleosis
• But subsequently developed the neurologic
symptoms??

11. On further enquiry
• On examination the girl was afebrile, but
appeared drowsy, had a congested throat and
a resolving lower extremity rash.
• Neurological examination;
• meningeal irritation signs and bilateral
Babinski signs were all positive with
• hypertonia and weakness and
• abnormalities in left eye (gaze).

12. On further enquiry
• Biochemical and haematological investigations were
normal except for
• mild leucocytosis,
• Liver function tests showed mildly elevated,
• Infectious workup including blood cultures was negative,
but immunological workup revealed positive IgM for EBV.
• Cerebrospinal fluid (CSF) analysis normal.
• A CT examination of the brain showed no appreciable
parenechymal abnormality
• while brain and spine MRI showed an extensive
demyelinating lesions????

13. MRI: widespread, multifocal, asymmetric and extensive white
matter lesions (typical “periventricular sparing” with partial
contrast enhancement and mainly a restriction in diffusion-
weighted sequences

14. Diagnosis…..

15. Treatment:
• Intravenous Immunoglobulin & Pulse steroid
therapy was initiated with 30 mg/kg/day of
methylprednisolone intravenously for the
5 days with close observation and follow up.
• Outcome and follow-up:
• Complete recovery after 6 weeks of follow up
and treatment.
• Now she is well …

16. Conclusion
• This case illustrates a young female with Acute
disseminated encephalomyelitis (ADEM) after
Epstein Barr virus infection.
• treated by Intravenous Immunoglobulin &
Pulse steroid with complete recovery after 6
weeks of treatment and follow up.

18. Some theory …..

19. Acute disseminated encephalomyelitis
(ADEM)
• ADEM is a monophasic, inflammatory
demyelinating disease of the CNS seen
predominantly in children.
• ADEM is an inflammatory disease of
immunologically mediated by the central
nervous system (CNS) that produces multifocal
demyelinating lesions affecting gray matter
and white of the brain and spinal cord.

20. How Common is ADEM?
• Reported annual incidences of this disease in
developed countries have ranged from 0.07 to
0.8/100 000
• ADEM can appear at any age but is more
common in children with the average age
between 6 and 9years.
• with male to female proportion of 3:1
• Prompt diagnosis of this disease entity helps
in its better management and reduces
unnecessary investigations in the patients
suffering from this disease.

21. Etiology
• Three sets of patients with ADEM have been reported :
• 1) post-vaccination,
• 2) after infection fever and
• 3) spontaneous.
 Post infectious:
• Viral ○ Measles ○ Varicella ○ Rubella ○ Herpes Zoster ○
EpsteinBarr virus
• Bacterial ○ Myoplasma ○ Gram- ve organisms ○ Salmonella
typhi
• Protozoal ○ Cerebral malaria
 Post Vaccination : Anti rabies vaccine
○ Influenza vaccine ○ measles vaccines

22. Pathogenesis
• The hallmark of pathogenesis of post infectious
encephalomyelitis is zones of perivenous
demyelination and penetration of lymphocytes
and macrophages.
• The current confirmation proposes that ADEM
comes about because of a transient immune
system reaction against myelin or different auto
antigens, potentially, by means of molecular
mimicry or by non-specific actuation of an auto
reactive T cell clone.

23. Clinical Features
• The prodromal illness may precede neurologic symptoms.
• The clinical presentation is variable and usually poly-
symptomatic:
• The clinical signs and symptoms are identified with the
place and seriousness of cerebrum injuries.
• Loss of consciousness
• Headache
• Vomiting
• Fever
• Confusion
• Meningism
• Seizures or coma may occur

24. Clinical Features
• Other clinical features include the rapid onset of
multifocal neurological disorders, such as :
• Unilateral or two-sided pyramidal signs (60-95%),
• serious hemiplegia (76%),
• loss of visual power as a result of optic neuritis (7-23%),
• spinal cord affiliation (24%),
• cranial nerve loss of motion (22-45%),
• ataxia (18-65%),
• hemiparesthesis (2-3%)
• ultimately changes in levels of mindfulness from lethargy to
unconsciousness.

25. Investigative Approach for a Child
with ADEM
• MRI: widespread, multifocal, asymmetric and extensive
white matter lesions (typical “periventricular sparing”
and lesions of similar inflammatory age) and possible
affection of grey matter, with partial contrast
enhancement and mainly a restriction in diffusion-
weighted sequences
• Follow-up MRI may reveal resolution of lesion
• The cerebrospinal liquid (CSF) May be normal or may
demonstrate a nonspecific lymphocytic pleocytosis and
height of albumin levels.

26. How are Children with ADEM
Managed?
• The disease may be fatal in the acute stages
but is otherwise self limiting
• In general treatment should be initiated as
early as possible and as aggressive as
necessary.

27. Management
• There is no standard treatment for ADEM.
• Many treatment approaches have used some form of
specific immunosuppressive therapy similar to that
used for other autoimmune diseases, including
• High -dose steroids,
• Intravenous immunoglobulin (IVIG) or
• Plasmapheresis.
• In very severe cases immuno suppression with
cyclophospamide or mitoxantrone should be
attempted

28. Prognosis
• Recovery may occur over 1- 6 months
• 60-80% cases fully recover
• Rest of them may show residual neurological
signs intellectual impairment and behavioral
abnormalities
• The mortality rate of up to 5%.

29. THANKS FOR YOUR
ATTENTION