diagnostic approach to acute encephalopathy
TRANSCRIPT
DIAGNOSTIC APPROACH TO ACUTE ENCEPHALOPATHY
Paediatric Update 2014Hospital Tawau
Dr. Thay Wee Ying
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
ENCEPHALOPATHY Full consiousness death
› Restless› Agitated› Confused› Delirious› Lethargic› Drowsy› Stuporous› Comatose
Glasgow Coma Scale
CAUSES OF ACUTE ENCEPHALOPATHY
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
CAUSES OF ACUTE ENCEPHALOPATHY CNS infection/ Para-
infection
Autoimmune
Metabolic/ Toxins
Seizure related
Hypertensive
Trauma/ hemorrhage
Hypoxic-ischemic
Tumour/ Malignancy
Hydrocephalus/ Other causes of raised ICP
ACUTE ENCEPHALOPATHY IN CHILDREN
An important paediatric emergency
Involves children of any age
Previously normal children, or children with pre-existing neurological impairment
ACUTE ENCEPHALOPATHY IN CHILDREN
Associated with significant mortality and long term morbidity in survivors
Good assessment with appropriate investigations identify treatable causes minimize neurological impairment
ACUTE ENCEPHALOPATHY
Wide range of differential diagnoses
long list of possible investigations
CLINICAL ASSESSMENT History
Physical examination
Investigations
HISTORY 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
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
EXAMINATION Opportunistic examination and
observation
Vital signs: HR, BP, RR, Spo2, temperature
Mental state, communication, behaviour, orientation, memory etc.
Examination Neurological examination:
› Focal neurological deficit Motor & sensory Cranial nerves & limbs
› Eyes: nystagmus, ophthalmoplegia, pupils, fundoscopy
› Abnormal movement
Examination of other systems
INVESTIGATIONS Initial investigations
› Blood glucose› Blood gases› Urea & electrolytes› LFT› Ammonia› FBC & blood picture› Urine FEME
Prompt identification of treatable cause
INVESTIGATIONS Further tests should be tailored to the
differential diagnoses
Lumbar puncture: CNS infections
Neuro-imaging (Ultrasound, CT, MRI)
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)
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
CNS infection: Neuro-imaging
CT with contrast: Bacterial meningitis: Subdural effusion, meningeal enhancement, abscess formation
CT with contrast: Brain abscess with ring enhancement
Neuro-imaging: TB meningitis
Plain CT: Hydrocephalus CT with contrast: Basal enhancement
Neuro-imaging: Herpes Encephalitis
MRI (T2): Bilateral asymmetric temporal, insular & basifrontal hyper-intensity
Neuro-imaging: Acute Disseminated Encephalomyelitis (ADEM)
MRI, T2 (Lt), FLAIR (Rt): Multiple hyper-intense foci involving the white matter & deep grey matter
Neuro-imaging: Acute Necrotising Encephalopathy of Childhood (ANEC)
MRI (T2, FLAIR, DWI): Bilaterally symmetric signal change in the thalami
Neuro-imaging: Infantile Bilateral Striatal Necrosis (IBSN)
Plain CT: Bilaterally symmetric hypodensity of the caudate nuclei & putamen with mass effect
Autoimmune Encephalitis & Immune Related Encephalopathy
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
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
Intracranial Haemorrhage Traumatic
› Accidental › Non-accidental: Child abuse (Shaken baby
syndrome)
Spontaneous› Vascular malformation› Bleeding disorder
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.
Trauma / Intracranial Haemorrhage
Blood count (platelet), coagulation profile
Neuro-imaging
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
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
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
Tumour/ CNS Malignancy Suggestive features
› Signs & symptoms of raised ICP› Focal neurological deficit› Seizures› Extra-cranial primary malignancy
Neuro-imaging: 1st line investigation
Diffuse Intrinsic Brainstem Glioma
Gliablastoma multiformeMedulloblastoma
Acute Encephalopathy in
ChildrenCase Illustration
Case1 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
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
Urine ME: RBC 5+
ASOT 800
Diagnosis: Hypertensive encephalopathy secondary to post-streptococcus acute gromerulo-nephritis (AGN)
Brain MRI
Posterior Reversible Encephalopathy Syndrome
Case 211 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
Able to talk & walk independently
Activities of daily living: need supervision with some assistance
On anti-epileptic drug, occasional breakthrough seizures
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
Case 3
Video EEG: Non-convulsive status epilepticus
Conclusion 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
Thank you