Encephalopathy

2.  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.  Full consiousness death
› Restless
› Agitated
› Confused
› Delirious
› Lethargic
› Drowsy
› Stuporous
› Comatose
 Glasgow Coma Scale

4. Davies 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.  CNS infection/ Para-
infection
 Autoimmune
 Metabolic/ Toxins
 Seizure related
 Hypertensive
 Trauma/
hemorrhage
 Hypoxic-ischemic
 Tumour/ Malignancy
 Hydrocephalus/
Other causes of
raised ICP

6.  An important paediatric emergency
 Involves children of any age
 Previously normal children, or children
with pre-existing neurological impairment

7.  Associated with significant mortality and
long term morbidity in survivors
 Good assessment with appropriate
investigations identify treatable
causes minimize neurological
impairment

8.  Wide range of differential diagnoses
long list of possible investigations

9.  History
 Physical examination
 Investigations

10.  Timing and nature of the encephalopathy
 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.  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.  Opportunistic examination and
observation
 Vital signs: HR, BP, RR, Spo2, temperature
 Mental state, communication,
behaviour, orientation, memory etc.

13.  Neurological examination:
› Focal neurological deficit
 Motor & sensory
 Cranial nerves & limbs
› Eyes: nystagmus, ophthalmoplegia, pupils,
fundoscopy
› Abnormal movement
 Examination of other systems

14.  Initial investigations
› Blood glucose
› Blood gases
› Urea & electrolytes
› LFT
› Ammonia
› FBC & blood picture
› Urine FEME
 Prompt identification of treatable cause

15.  Further tests should be tailored to the
differential diagnoses
 Lumbar puncture: CNS infections
 Neuro-imaging (Ultrasound, CT, MRI)

16.  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.  FBC, CRP, ESR
 Blood culture
 Viral study (blood, throat, urine, stool)
 TB work-up
 CSF: ME, sugar, protein, C&S, virology, TB,
fungus

18. CT with contrast: Bacterial
meningitis: Subdural effusion,
meningeal enhancement,
abscess formation
CT with contrast: Brain abscess
with ring enhancement

19. Plain CT: Hydrocephalus CT with contrast: Basal
enhancement

20. MRI (T2): Bilateral asymmetric temporal, insular & basifrontal
hyper-intensity

21. MRI, T2 (Lt), FLAIR (Rt): Multiple hyper-intense foci involving the
white matter & deep grey matter

22. MRI (T2, FLAIR, DWI): Bilaterally symmetric
signal change in the thalami

23. Plain CT: Bilaterally symmetric hypodensity of the caudate nuclei &
putamen with mass effect

24.  NMDA-receptor antibody encephalitis,
limbic encephalitis, Hashimoto’s
encephalopathy, CNS lupus etc.
 Suggestive features:
› Prolonged course & fluctuating symptoms
› Unresponsive to anti-microbial drugs
› No infectious agent identified
› Specific movement disorders
› Underlying immune disorder

25.  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.  Traumatic
› Accidental
› Non-accidental: Child abuse (Shaken baby
syndrome)
 Spontaneous
› Vascular malformation
› Bleeding disorder

27.  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.  Blood count (platelet), coagulation
profile
 Neuro-imaging

29.  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.  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. (A) CT: Basal ganglia calcification. (B & C) MRI T2: Hyperintense lesion in
the left temporo-parieto-occipital regions. (D) MRS: High lactate peak

32.  Suggestive features
› Signs & symptoms of raised ICP
› Focal neurological deficit
› Seizures
› Extra-cranial primary malignancy
 Neuro-imaging: 1st line investigation

33. Gliablastoma multiformeMedulloblastoma

35.  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.  On arrival, sedated; pupils-equal &
reactive; fundus-N; no focal neurological
deficit
 Noted hypertension but no bradycardia
 Brain CT: Mild cerebral oedema
 Wean off sedation but the child remained
encephalopathic; Persistent hypertension

37.  Urine ME: RBC 5+
 ASOT 800
Diagnosis:
Hypertensive encephalopathy secondary to
post-streptococcus acute gromerulo-nephritis
(AGN)

38.  Brain MRI
Posterior Reversible Encephalopathy Syndrome

39. 11 yr old girl
 Learning disability with history of
recurrent stroke-like episodes & epilepsy
 Diagnosed Mitochondrial
Encephalomyopathy, Lactic Acidosis,
Stroke-like episodes (MELAS) syndrome at
9 yr old, confirmed by gene mutation
study

40.  Able to talk & walk independently
 Activities of daily living: need supervision
with some assistance
 On anti-epileptic drug, occasional
breakthrough seizures

41.  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.  Video EEG: Non-convulsive status epilepticus

43.  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