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DIAGNOSTIC APPROACH TO ACUTE ENCEPHALOPATHYIN CHILDREN 
Dr HengHock Sin 
PaediatricNeurologist 
Sabah Women & Children’s H...
Encephalopathy 
A syndrome of global brain dysfunction 
Definition [International Pediatric MS study Group 2007]: 
Beha...
Encephalopathy 
Full consciousness Death 
Restless 
Agitated 
Confused 
Delirious 
Lethargic 
Drowsy 
Stuporous ...
Causes of Acute EncephalopathyDavies E et.al. Encephalopathy in children: an approach to assessment and management. Arch D...
Causes of Acute Encephalopathy 
CNS infection /Para- infection 
Autoimmune 
Metabolic / Toxins 
Seizure related 
Hype...
Acute Encephalopathy in Children 
An important paediatric emergency 
Involves children of any age 
Previously normal ch...
Acute Encephalopathy in Children 
Associated with significant mortality & long- term morbidity in survivors 
Good assess...
Acute Encephalopathy 
Wide range of differential diagnoses 
long list of possible investigations
Clinical Assessment 
History 
Physical examination 
Investigations
History 
Timing & nature of the encepahlopathy 
Associated symptoms 
Fever, vomiting, loss of appetite 
Headache, seiz...
History 
Pre-existing medical / neurological condition 
Developmental history 
Travel, contact with animals / insects 
...
Examination 
Opportunistic examination & observation 
Vital signs: HR, BP, RR, SpO2, temperature 
Mental state, communi...
Examination 
Neurological examination: 
Focal neurological deficit 
Motor & sensory 
Cranial nerves & limbs 
Eyes: ny...
Investigations 
Initial investigations 
Blood glucose 
Blood gases 
Urea & electrolytes 
LFT 
Ammnonia 
FBC & blood...
Investigations 
Further tests should be tailored to the differential diagnoses 
Lumbar puncture: CNS infections 
Neuro-...
CNS infections / Para-infection 
Suggestive features: 
Fever , headache 
Meningism 
Focal neurological deficits 
Seiz...
CNS infection: Investigations 
FBC, CRP, ESR 
Blood culture 
Viral study (blood, throat, urine, stool) 
TB work-up 
C...
CNS infection: Neuro-imagingCT with contrast: Bacterial meningitis: Subdural effusion, meningealenhancement, abscess forma...
Neuro-imaging: TB meningitisPlain CT: Hydrocephalus 
CT with contrast: Basal enhancement
Neuro-imaging: Herpes Encephalitis 
MRI (T2): Bilateral asymmetric temporal, insular & basifrontalhyper-intensity
Neuro-imaging: Acute Disseminated Encephalomyelitis (ADEM) MRI, T2 (Lt), FLAIR (Rt): Multiple hyper-intense foci involving...
Neuro-imaging: Acute NecrotisingEncephalopathy of Childhood (ANEC) MRI (T2, FLAIR, DWI): Bilaterally symmetric signal chan...
Neuro-imaging: Infantile Bilateral StriatalNecrosis (IBSN) Plain CT: Bilaterally symmetric hypodensityof the caudate nucle...
Autoimmune Encephalitis & Immune Related Encephalopathy 
NMDA-receptor antibody encephalitis, limbic encephalitis, Hashim...
Autoimmune Encephalitis & Immune Related Encephalopathy 
Investigations: 
Work-up for vasculitic disorders 
Blood or CS...
Intracranial Haemorrhage 
Traumatic 
Accidental 
Non-accidental: Child abuse (Shaken baby syndrome) 
Spontaneous 
Vas...
Trauma / Intracranial Haemorrhage 
Suggestive features: 
History of head trauma 
Sudden onset of encephalopathy ( +seiz...
Trauma / Intracranial Haemorrhage 
Blocod count (platelet), coagulation profile 
Neuro-imaging
Metabolic Disorders 
Broad category of conditions 
Suggestive features 
History of development delay / regression, grow...
Metabolic Disorders 
Investigations 
*Initial investigations 
Metabolic work-up 
Neuro-imaging, MR spectoscopy 
MRI. L...
Neuro-imaging: MELAS syndrome(A) CT: Basal ganglia calcification. (B & C) MRI T2: Hyperintenselesion in the left temporo-p...
Tumour/ CNS Malignancy 
Suggestive features 
Signs & symptoms of raised ICP 
Focal neurological deficit 
Seizures 
Ex...
Medulloblastoma 
Gliablastoma multiforme 
Diffuse Intrinsic Brainstem Glioma
Acute Encephalopathy in ChildrenCase Illustration
Case 1 
7 year old boy, previously well 
Headache & lethargic for 3 days blurred vision, confusion, followed by status e...
Case 1 
On arrival, sedated; pupils-equal & reactive; fundus-N; no focal neuro deficit 
Noted hypertension but no bradyc...
Case 1 
Urine ME: RBC 5+ 
ASOT 800 
Diagnosis: 
Hypertensive encephalopathy secondary to 
post-streptococcus acute grome...
Case 1 
Brain MRI 
Posterior Reversible Encephalopathy Syndrome
Case 2 
11 yr old girl 
Learning disability with history of recurrent stroke-like episodes & epilepsy 
Diagnosed Mitocho...
Case 2 
Able to talk & walk independently 
Activities of daily living: need supervision with some assistance 
On anti-e...
Case 2 
Presented with: 
More frequent seizures, 1-2 episodes / day, for 3 days 
Lost her verbal skills, not interactiv...
Case 3 
Video EEG: Non-convulsive status epilepticus
Conclusions 
Acute encephalopathy in children is an emergency with wide range of differential diagnoses; significant morb...
Thank You
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Diagnostic approach to acute encephalopathy

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Presentation at PNU 2014

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Diagnostic approach to acute encephalopathy

  1. 1. DIAGNOSTIC APPROACH TO ACUTE ENCEPHALOPATHYIN CHILDREN Dr HengHock Sin PaediatricNeurologist Sabah Women & Children’s Hospital Paediatric Neurology Update 2014
  2. 2. Encephalopathy A syndrome of global brain dysfunction Definition [International Pediatric MS study Group 2007]: Behavioral change: confusion, excessive irritability Alteration in consciousness: lethargy, coma Acute or insidious onset
  3. 3. Encephalopathy Full consciousness Death Restless Agitated Confused Delirious Lethargic Drowsy Stuporous Comatose *Glasgow Coma Scale
  4. 4. Causes of Acute EncephalopathyDavies E et.al. Encephalopathy in children: an approach to assessment and management. Arch DisChild. 2012 May;97(5):452-8. doi: 10.1136/adc.2011.300998. Epub 2011 Dec 27
  5. 5. Causes of Acute Encephalopathy CNS infection /Para- infection Autoimmune Metabolic / Toxins Seizure related Hypertensive Trauma / Haemorrhage Hypoxic-ischaemic Tumour / Malignancy Hydrocephalus / Other causes of raised ICP
  6. 6. Acute Encephalopathy in Children An important paediatric emergency Involves children of any age Previously normal children, or children with pre-existing neurological impairment
  7. 7. Acute Encephalopathy in Children Associated with significant mortality & long- term morbidity in survivors Good assessment with appropriate investigations identify treatable causes minimize neurological impairment
  8. 8. Acute Encephalopathy Wide range of differential diagnoses long list of possible investigations
  9. 9. Clinical Assessment History Physical examination Investigations
  10. 10. History Timing & nature of the encepahlopathy Associated symptoms Fever, vomiting, loss of appetite Headache, seizures Current / recent febrile illness In some cases, the cause is obvious e.g. Acute renal / liver failure, DM, following head trauma or hypoxic event
  11. 11. History Pre-existing medical / neurological condition Developmental history Travel, contact with animals / insects Drug / toxin ingestion Family history Neurological / metabolic disorder; vascular / bleeding disorder Parental consanguinity Early / unexplained childhood deaths Social history: Non-accidental injury
  12. 12. Examination Opportunistic examination & observation Vital signs: HR, BP, RR, SpO2, temperature Mental state, communication, behaviour, orientation, memory e.t.c.
  13. 13. Examination Neurological examination: Focal neurological deficit Motor & sensory Cranial nerves & limbs Eyes: nystagmus, ophthalmoplegia, pupils, fundoscopy Abnormal movement Examination of other systems
  14. 14. Investigations Initial investigations Blood glucose Blood gases Urea & electrolytes LFT Ammnonia FBC & blood picture Urine FEME Prompt identification of treatable cause
  15. 15. Investigations Further tests should be tailored to the differential diagnoses Lumbar puncture: CNS infections Neuro-imaging (Ultrasound, CT, MRI)
  16. 16. CNS infections / Para-infection Suggestive features: Fever , headache Meningism Focal neurological deficits Seizures Primary source of infection Pneumonia (bacteria, mycoplasma, TB), purpuric rash (meningococcemia), mucosal herpetic lesions, cyanotic heart dis. (brain abscess)
  17. 17. CNS infection: Investigations FBC, CRP, ESR Blood culture Viral study (blood, throat, urine, stool) TB work-up CSF: ME, sugar, protein, C&S, virology, TB, fungus
  18. 18. CNS infection: Neuro-imagingCT with contrast: Bacterial meningitis: Subdural effusion, meningealenhancement, abscess formation CT with contrast: Brain abscess with ring enhancement
  19. 19. Neuro-imaging: TB meningitisPlain CT: Hydrocephalus CT with contrast: Basal enhancement
  20. 20. Neuro-imaging: Herpes Encephalitis MRI (T2): Bilateral asymmetric temporal, insular & basifrontalhyper-intensity
  21. 21. Neuro-imaging: Acute Disseminated Encephalomyelitis (ADEM) MRI, T2 (Lt), FLAIR (Rt): Multiple hyper-intense foci involving the white matter & deep grey matter
  22. 22. Neuro-imaging: Acute NecrotisingEncephalopathy of Childhood (ANEC) MRI (T2, FLAIR, DWI): Bilaterally symmetric signal change in the thalami
  23. 23. Neuro-imaging: Infantile Bilateral StriatalNecrosis (IBSN) Plain CT: Bilaterally symmetric hypodensityof the caudate nuclei & putamenwith mass effect
  24. 24. Autoimmune Encephalitis & Immune Related Encephalopathy NMDA-receptor antibody encephalitis, limbic encephalitis, Hashimoto’s encephalopathy, CNS lupus e.t.c. Suggestive features: Prolonged course & fluctuating symptoms unresponsive to anti-microbial drugs No infectious agent identified Specific movement disorders Underlying immune disorder
  25. 25. Autoimmune Encephalitis & Immune Related Encephalopathy Investigations: Work-up for vasculitic disorders Blood or CSF for specific neuronal antibodies: Anti-NMDA receptor antibody Anti-VGKC antibody e.t.c Thyroid function, anti-thyroid antibodies
  26. 26. Intracranial Haemorrhage Traumatic Accidental Non-accidental: Child abuse (Shaken baby syndrome) Spontaneous Vascular malformation Bleeding disorder
  27. 27. Trauma / Intracranial Haemorrhage Suggestive features: History of head trauma Sudden onset of encephalopathy ( +seizure) in a well child Signs of acute blood loss: Pallor, tachycardia History or family history of bleeding disorder Non-accidental injury Inconsistent / suspicious history, other suspicious body injuries, retinal haemorrhage, e.t.c.
  28. 28. Trauma / Intracranial Haemorrhage Blocod count (platelet), coagulation profile Neuro-imaging
  29. 29. Metabolic Disorders Broad category of conditions Suggestive features History of development delay / regression, growth failure, epilepsy Relapsing acute encephalopathy / septic-like episodes Multi-organ impairment Consanguineous parents, significant family history
  30. 30. Metabolic Disorders Investigations *Initial investigations Metabolic work-up Neuro-imaging, MR spectoscopy MRI. Leigh syndrome: Bilateral symmetrical T2 high signal in caudate nuclei /putamenand white matter
  31. 31. Neuro-imaging: MELAS syndrome(A) CT: Basal ganglia calcification. (B & C) MRI T2: Hyperintenselesion in the left temporo-parieto-occipital regions. (D) MRS: High lactate peak
  32. 32. Tumour/ CNS Malignancy Suggestive features Signs & symptoms of raised ICP Focal neurological deficit Seizures Extra-cranial primary malignancy Neuro-imaging: 1stline investigation
  33. 33. Medulloblastoma Gliablastoma multiforme Diffuse Intrinsic Brainstem Glioma
  34. 34. Acute Encephalopathy in ChildrenCase Illustration
  35. 35. Case 1 7 year old boy, previously well Headache & lethargic for 3 days blurred vision, confusion, followed by status epilepticus Intubated in district hospital, seizure was aborted with iv diazepam
  36. 36. Case 1 On arrival, sedated; pupils-equal & reactive; fundus-N; no focal neuro deficit Noted hypertension but no bradycardia Brain CT: Mild cerebral oedema Wean off sedation but the child remained encephalopathic; Persistent hypertension
  37. 37. Case 1 Urine ME: RBC 5+ ASOT 800 Diagnosis: Hypertensive encephalopathy secondary to post-streptococcus acute gromerulo- nephritis (AGN)
  38. 38. Case 1 Brain MRI Posterior Reversible Encephalopathy Syndrome
  39. 39. Case 2 11 yr old girl Learning disability with history of recurrent stroke-like episodes & epilepsy Diagnosed Mitochondrial Encephalopathy Lactic Acidosis Stroke-like episodes (MELAS) syndromeat 9 yr old, confirmed by gene mutation study
  40. 40. Case 2 Able to talk & walk independently Activities of daily living: need supervision with some assistance On anti-epileptic drug, occasional breakthrough seizures
  41. 41. Case 2 Presented with: More frequent seizures, 1-2 episodes / day, for 3 days Lost her verbal skills, not interactive Poor head control, needed assistance in walking Drooling of saliva Urinary incontinence Unable to eat
  42. 42. Case 3 Video EEG: Non-convulsive status epilepticus
  43. 43. Conclusions Acute encephalopathy in children is an emergency with wide range of differential diagnoses; significant morbidity & mortality A systematic approach is essential for early & accurate diagnosis to ensure appropriate & timely treatment
  44. 44. Thank You

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