1. D KALPANA
Addl. Professor of Pediatric Neurology,
Medical College, Thiruvananthapuram

2.  Definition of terms
 Differential diagnosis
 Points from history/epidemiology
 Investigations
 Supportive management
 Specific management
 Autoimmune encephalitis

3. ENCEPHALOPATHY
• Diffuse disturbance of brain function without inflammation
ENCEPHALITIS
• Dysfunction of brain associated with inflammation
FEBRILE ENCEPHALOPATHY
• a/c onset of fever (<1wk)+alteration of consciousness >12
hrs

4. Febrile
inflammation
ENCEPHALOPATHY
Temp >380C Cellular CSF
Seizures
Alteration of cerebral Imaging /EEG
function suggestive of
Focal neurological signs inflammation

5.  Clinically, a case of Acute Encephalitis Syndrome (AES)
is defined as a person of any age, at any time of year with
the acute onset of fever and at least one of:
 a) change in mental status (including symptoms such as
confusion, disorientation, coma, or inability to talk);
 b) New onset of seizures (excluding simple febrile seizures.
 ( A simple febrile seizure is defined as a seizure that occurs in a child aged 6
months to less than 6 years old, whose only finding is fever and a single
generalized convulsion lasting less than 15 minutes, and who recovers
consciousness within 60 minutes of the seizure)
Bull World Health Organ 2008, 86(3):178-186.

6.  INFECTIONS
 DEMYELINATION - ADEM
 AUTOIMMUNE ENCEPHALITIS
 DRUGS/TOXINS
 COLLAGEN VASCULAR DISORDERS
 SEIZURES –NON CONVULSIVE STATUS
 METABOLIC
 ICSOL

7.  Viral encephalitis
 Herpes simplex type 1. type2
 Varicella zoster
 HHV6
 Epstein Barr virus
 Arboviruses – JE,West Nile,Dengue, Chikun
gunya,
 Rhabdoviruses-rabies
 Orthomyxo –H1N1
 Paramyxo –measles
 HIV

8.  Bacterial
 Meningitis
 Brain abscess
 Sepsis associated encephalopathy
 Leptospirosis
 Typhoid
 M. tuberculosis
 Rickettsial (scrub typhus)
 Parasitic
 Cerebral malaria
 Toxoplasma

11.  Features of infection
 Evidence of CNS involvement
 Features of raised intracranial tension
 Signs and symptoms of meningeal irritation

12. • HSV is sporadic
epidemiology • JE epidemics
h/o animal bites • Rabies
• JE
Mosquito bites • Dengue
• Chikun Gunya
Working/playing • Leptospirosis
in dirty water
Contact withTB • TBM

13. •Maculopaular
•Petechiae/purpura
Fever with rash •Vesicles
•Eschar
•Herpes labialis
Respiratory • H1N1
symptoms
• enteroviruses
Diarrhoea,vomiting • Polio
• Mumps
Parotitis • EB virus
• HIV
• Dengue
Myalgia,arthralgia
• leptospirosis

15. Abnormal Ataxia
ADEM
behaviour/psychosis VZV
HSV Entero virus
Limbic encephalitis
NCSE
Lower cranial nerve
Opisthotonic posture palsies
TBM
Choreoathetosis VASCULITIS
JE,autoimmune Brainstem encephalitis
JE,west nile
Meningeal signs
MENINGITIS Visual loss
ADEM Optic neuritis
meningoencephalitis Hypertensive
encephalopathy
Asymmetric signs and
symptoms
Encephalitis Papilloedema
ICSOL
TBM Hydrocephalus –TBM
ADEM Hypertensive
encephalopathy

16. • EB virus
lymphadenopathy • leptospira
Hypotension • dengue
• Chikungunya
shock • leptospira
• EBVIRUS
• dengue
organomegaly • Lepto
• HIV
• COLLAGEN

17. management

19. SHOCK SEPSIS
INTRCRANIAL SEIZURE
INFECTION • STATUS EPILEPTICUS
• ENCEPHALITIS • NON CONVULSIVE STATUS
• MENINGITIS
• TBM
RAISED ICT METABOLIC
• PAIPPEDEMA • HYPOGLYCEMIA
• GCS<8 • HYPERAMMONEMIA
• ASYMMETIC PUPILS • ACIDOSIS
• DKA
• POSTURING
• DRUGS
• ABSENT DOLL’S EYE

20. CSF STUDY
EEG
IMAGING

21. CONTRAINDICATIONS
IMAGING BEFORE LP IN RAISED ICT
EMPIRICAL ANTIBIOTICS +ACYCLOVIR IF DELAY OF
SEVERAL HRS IS EXPECTED

22.  NOTE THE OPENING PRESSURE
 CELLS
 GRAM STAIN,CULTURE
 PROTEIN
 SUGAR
 VIROLOGICAL STUDIES –PCR,IgM
 TBPCR
 LACTATE

23. BACTERIAL • PMN
MENINGITIS • High protein, low sugar, gram stain
• Few lymphocytes
ASEPTIC • Normal protein
MENINGITIS • Normal sugar
• lymphocytic
VIRAL
• Normal sugar, normal to slightly
ENCEPHALITIS raised protein
• Opalescent, cob web
TUBERCULOUS • Lymphocytic
MENINGITIS • High protein. Low sugar

24.  Take at least 5 ml of CSF
 Be sure that it is not mixed with blood
 Sensitivity and specificity are relatively good
 Can be negative very early in HSV and after
10 days of treatment
 Never stop Acyclovir before repeating once
more after 72hrs – if clinical history, EEG and
imaging are suggestive
 Serum/CSF Ig M antibodies useful in JE
 Paired samples – 4 fold rise in titre

25.  MRI is preferable to CT scan-
 CTis advised in unstable patients, delirious
children who cannot be kept still for 30 min

27. JE

28. RABIES

31.  Diffuseslowing suggests encephalopathic
process
 PLEDS in HSE
 Triphasic waves in metabolic encephalopathy
 Non convulsive status epilepticus

33.  Should be suspected in confusion, stupor,
unarousable coma
 Subtle features like eye blinking, nystagmus,
perioral twitching, automatisms may be seen
 May follow convulsive seizures
 EEG is the only diagnostic clue
 Response to diazepam can be demonstrated
in simultaneous EEG recording
 Generalised/complex partial

35.  Maintain
 Normothermia
 Normoglycemia
 Normal electrolyte balance
 Normotension
 Management of raised ICT
 minimal stimulation
 Head end elevation
 Avoid hypotonic fluids
 3% saline
 Mannitol 20% solution
 hyperventilation

36.  Management of
seizures/status
epilepticus
 Identify SIADH and
manage
 Rapid correction of
hyponatremia may
lead to central
pontine
myelinolysis

37.  Abnormal /psychotic behaviour – haloperidol+
phenergan
 Choreoathetosis – dopa blockers
 Dystonia - tetrabenezine, anticholinergics,
muscle relaxants

38.  HSE –ACYCLOVIR I/V 10 mg/kg/dose 8 hrly
x 14 -21 days. (500 mg/m2)Neonates 20
mg/kg/dose
 Oral acyclovir has very low bioavailability
 Oral valacyclovir can be used
 Very costly
 Empirical acyclovir
 Repeat LP after 72 hrs if initial PCR is
negative – and stop Acyclovir after that.
 Other drugs effective - foscarnet

39.  Varicella zoster – acyclovir
 HHV 6 - foscarnet +gancyclovir
 CMV – gancyclovir
 H1N1- oseltamivir
 Rickettsia – doxycycline
 Mycoplasma – azithromycin
 Leptospira – penicillin
 Bacterial meningitis – ceftriaxone+ vancomycin
 ADEM – steroids, IVIG
 Autoimmune encephalitis - immunosuppressants

40.  Even in best centres a definite diagnosis of
encephalitis is reached only in 42% of cases
(Granerod et al)
 ADEM in 21%
 1% autoimmune encephalitis
 37% no definite diagnoses
 Undiagnosed viral infections
 Autoimmune causes
 Unidentified metabolic causes

41.  Poorly understood CNS condition
 Manifests lethargy –delirium
 Pathogenesis
 bacterial invasion of brain
 endotoxins
 derangement of neurotransmitter and
 amino acid and microvascular changes
 Prognosis---serious
 May be seen in patient with
 1. mechnical ventilation
 2.critical ill patient in micu (sedatives, neuromuscular
blocking agents, dyselectrolytemia,hepatic failure may
contribute)

42.  MANIFESTATION MAY BE HIV VIRUS
ITSELF OR ITS NEUROLOGICAL
COMPLICATION D/T OPPORTUNISTIC
INFECTION LIKE
 1. CNS tuberculosis
 2. cytomegalo virus encephalitis
 3. toxoplasmosis
 4. cryptococcal meningitis
 5.syphilis
 6.tumours (primary CNS lymphoma )or drug
related complications

43.  The potentially fatal complication of
falciparum malaria ( most important cause of
unarousable coma in febrile patients in
endemic area )
 SUSCEPTIBILITY
 - childrens
 - pregnant women
 - non – immune adults
 20 % all severe falciparum malaria requires
ICU admission

44.  Selective cytoadherence and
sequestration of parasitized RBC’S in
cerebral venules and
 toxin release at schizont rupture are
possible pathological mechanism
 Systemic complications like hypoglycemia
may contribute to development of coma
 Diagnosis – PS for MP
 Treatment – artesunate is better than
quinine

45.  often presents with fever
 behavioural abnormalities
 psychosis
 movement disorders
 seizures/status
 May be paraneoplastic –
teratoma ovary in young
females
 Often no tumour is
identified
 Antibodies to NMDA
,VGKC receptors
 Treatment – IVIG,
plasmapheresis

46. ?
?
?

47.  A variety of infective and non infective conditions
in children can present as acute febrile
encephalopathy
 Stabilisation of patient and supportive
management helps a lot in reducing morbidity and
mortality
 Identification of specific etiology helps in
institution of specific therapy
 Awareness of Autoimmune encephalitis is
important – another treatable cause like ADEM
 In a significant proportion of cases aetiology is yet
to be identified