anhe

1. • ANHE is a rare condition secondary to respiratory viruses like influenza and has been recently seen with COVID 19 Virus as well
• It has a fulminant course with high mortality rate which is approximately 30%

2. • A 24year aged gentlemen presented with history of high grade fever since 4days, vomiting’s since 3days, altered sensorium and irritability
since 1day.
• There is no history of headache/blurred vision/seizures.
• Illness was not preceded by diarrhoea. There is no recent history of travel. He was not on any medications
• There were no signs of focal neurological deficits, cranial nerve palsies, or meningeal signs. Remaining systemic examination was non-
contributory.
• He was intubated in view of poor GCS .
• The possibility of acute febrile encephalopathy with differentials of viral encephalitis, cerebral malaria, pyogenic meningitis, severe dengue
and enteric encephalopathy were considered.
LAB PARAMETER RESULT
COVID RT PCR NEGATIVE
TLC 15,200
NEUTROPHILS 60%
LYMPHOCYTES 12%
HEMOGLOBIN 12.9
PLATELETS 25000
CRP 109.50mg/Dl
TOTAL BILIRUBIN 2.96mg/Dl
DIRECT BILIRUBIN 1.79mg/Dl
SGOT 157U/L
SGPT 87U/L
ALP 122U/L
GGT 257U/L
MALARIA ANTIGEN [V & F] NEGATIVE
LEPTOSPIRA IgM NEGATIVE
DENGUE IgM 11.28 [POSITIVE]
CSF ANALYSIS
GROSS EXAMINATION TURBID
TOTAL LEUCOCYTE COUNT 88
NEUTROPHILS 10%
LYMPHOCYTES 90%
PROTEIN 103.2mg/dL
ADA NEGATIVE
TB GENE EXPERT NEGATIVE
VARICELLA ZOSTER VIRUS PCR NEGATIVE

3. • CT Brain was done which revealed multiple hypodensities with haemorrhagic foci.
• MRI brain with contrast was done which revealed irregular contour lesions surrounded by oedema in bilateral frontoparietal temporal occipital lobes and
right cerebellar hemisphere.
• USG Abdomen showed gall bladder wall oedema, mild splenomegaly , mild free fluid collection in abdomen.
• Treatment was commenced empirically with antibiotics, antivirals, anti-oedema measures, antiepileptics, single donor platelet transfusion and mechanical
ventilatory support
• CSF biofire MULIPLEX PCR was done which is negative for Bacteria (Streptococcus pneumoniae, Listeria monocytogenes, Neisseria meningitis) and
Viruses (CMV, Entero virus, HSV 1and 2, HHV 6). Fungi- Cryptococcus was negative. CSF culture was negative. Dengue IgG in CSF was 13.84 units
(>11+ve) and dengue Ig M in CSF was 96.87 units (> 11+ve)
• Diagnosis of dengue necrotising hemorrhagic encephalitis was confirmed by blood and CSF dengue serology and radiological findings . Dexamethasone
was started at a dose 6mg once daily.
• In spite of all medical measures, he gradually worsened and succumbed to death.

4. • Acute necrotising encephalitis is a rare syndrome which is mostly caused by viral infections.
• Usually, ANE develops secondary to viral infections, including influenza A and influenza B, novel influenza A (H1N1), parainfluenza, varicella, human
herpesvirus 6 and herpesvirus 7 (HHV-6 and HHV-7), enterovirus, novel reovirus strain (MRV2Tou05), rotavirus, herpes simplex virus, rubella, coxsackie
A9, and measles, among which the influenza virus and HHV-6 are most common.
• Hypercytokinemia is the key pathogenesis in this disease
• Intravenous glucocorticoids, immunoglobulin, and plasmapheresis should be effective based on the pathogenesis of ANE 1,2.
• Multiple studies demonstrated diffuse cerebral oedema, bilateral symmetrical FLAIR and T2 hyperintensities in thalami, pons, and medulla with
heterogenous or peripheral enhancement on contrast administration 3 .
• In our patient, MRI Brain with contrast showed irregular contour lesions surrounded by oedema in bilateral fronto parietal temporal occipital Lobes and
right cerebellar hemisphere.
• These are not typical findings for dengue encephalitis. As CSF titres for IgM and IgG were positive, we concluded this case to be a case of acute
necrotising haemorrhagic encephalitis with atypical MRI findings

5. 1.L. Bergamino, V. Capra, R. Biancheri et al., “Immunomodulatory therapy in recurrent acute necrotizing encephalopathy ANE1: is it useful?”
Brain and Development, vol. 34, no. 5, pp. 384–391, 2012.
2..R. Manara, M. Franzoi, P. Cogo, and P. A. Battistella, “Acute necrotizing encephalopathy: combined therapy and favorable outcome in a
new case,” Child’s Nervous System, vol. 22, no. 10, pp. 1231–1236, 2006
3. FerreiraML, Cavalcanti CG, Coelho CA, Mesquita SD. Neurological manifestations of dengue: Study of 41 cases. Arq Neuropsiquiatr
2005;63:488-93