Diagnostic approach to acute encephalopathy
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DIAGNOSTIC APPROACH TO ACUTE ENCEPHALOPATHY
IN CHILDREN
Dr Heng Hock SinPaediatric Neurologist
Sabah Women & Children’s Hospital
Paediatric Neurology Update 2014
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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
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Encephalopathy
Full consciousness Death
Restless
Agitated
Confused
Delirious
Lethargic
Drowsy
Stuporous
Comatose
*Glasgow Coma Scale
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Causes of Acute Encephalopathy
Davies E et.al. Encephalopathy in
children: an approach to assessment and
management. Arch Dis Child. 2012
May;97(5):452-8.
doi: 10.1136/adc.2011.300998. Epub
2011 Dec 27
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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
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Acute Encephalopathy in Children
An important paediatric emergency
Involves children of any age
Previously normal children, or children with pre-existing neurological impairment
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Acute Encephalopathy in Children
Associated with significant mortality & long-term morbidity in survivors
Good assessment with appropriate investigations identify treatable causes
minimize neurological impairment
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Acute Encephalopathy
Wide range of differential diagnoses
long list of possible investigations
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Clinical Assessment
History
Physical examination
Investigations
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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
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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
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Examination
Opportunistic examination & observation
Vital signs: HR, BP, RR, SpO2, temperature
Mental state, communication, behaviour, orientation, memory e.t.c.
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Examination
Neurological examination:
Focal neurological deficit
Motor & sensory
Cranial nerves & limbs
Eyes: nystagmus, ophthalmoplegia, pupils, fundoscopy
Abnormal movement
Examination of other systems
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Investigations
Initial investigations
Blood glucose
Blood gases
Urea & electrolytes
LFT
Ammnonia
FBC & blood picture
Urine FEME
Prompt identification of treatable cause
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Investigations
Further tests should be tailored to the differential diagnoses
Lumbar puncture: CNS infections
Neuro-imaging (Ultrasound, CT, MRI)
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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)
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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
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CNS infection: Neuro-imaging
CT with contrast: Bacterial meningitis: Subdural effusion, meningeal enhancement, abscess formation
CT with contrast: Brain abscess with ring enhancement
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Neuro-imaging: TB meningitis
Plain CT: Hydrocephalus CT with contrast: Basal enhancement
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Neuro-imaging: Herpes Encephalitis
MRI (T2): Bilateral asymmetric temporal, insular & basifrontal hyper-intensity
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Neuro-imaging: Acute Disseminated Encephalomyelitis (ADEM)
MRI, T2 (Lt), FLAIR (Rt): Multiple hyper-intense foci involving the white matter & deep grey matter
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Neuro-imaging: Acute NecrotisingEncephalopathy of Childhood (ANEC)
MRI (T2, FLAIR, DWI):Bilaterally symmetric signal change in the thalami
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Neuro-imaging: Infantile Bilateral Striatal Necrosis (IBSN)
Plain CT: Bilaterally symmetric hypodensity of the caudate nuclei & putamen with mass effect
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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
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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
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Intracranial Haemorrhage
Traumatic
Accidental
Non-accidental: Child abuse (Shaken baby syndrome)
Spontaneous
Vascular malformation
Bleeding disorder
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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.
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Trauma / Intracranial Haemorrhage
Blocod count (platelet), coagulation profile
Neuro-imaging
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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
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Metabolic Disorders
Investigations
*Initial investigations
Metabolic work-up
Neuro-imaging, MR spectoscopy
MRI. Leigh syndrome: Bilateral symmetrical T2 high signal in caudate nuclei /putamen and white matter
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Neuro-imaging: MELAS syndrome
(A) CT: Basal ganglia calcification. (B & C) MRI T2: Hyperintense lesion in the left temporo-parieto-occipital regions. (D) MRS: High lactate peak
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Tumour / CNS Malignancy
Suggestive features
Signs & symptoms of raised ICP
Focal neurological deficit
Seizures
Extra-cranial primary malignancy
Neuro-imaging: 1st line investigation
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Medulloblastoma Gliablastoma multiforme
Diffuse Intrinsic Brainstem Glioma
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Acute Encephalopathy in Children
Case Illustration
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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
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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
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Case 1
Urine ME: RBC 5+
ASOT 800
Diagnosis:
Hypertensive encephalopathy secondary to
post-streptococcus acute gromerulo-nephritis (AGN)
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Case 1
Brain MRI
Posterior Reversible Encephalopathy Syndrome
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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) syndrome at 9 yr old, confirmed by gene mutation study
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Case 2
Able to talk & walk independently
Activities of daily living: need supervision with some assistance
On anti-epileptic drug, occasional breakthrough seizures
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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
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Case 3
Video EEG: Non-convulsive status epilepticus
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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
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Thank You