Grand Rounds

1Department of Pediatrics, MLN Medical College, Prayagraj, Uttar Pradesh, India, 2National Diagnostics, New Delhi, India

Address for Correspondence: Dr. Anubha Shrivastava, 84, Lukerganj, Prayagraj, Uttar Pradesh 211016, India. Email: anubhashrivastava@rediffmail.com


Keywords: acute necrotizing encephalopathy, childhood

Clinical Problem :
A 9-year-old girl presented with convulsions followed by unconsciousness. She had fever and cough for one week. On examination, Glasgow coma score (GCS) was 8/15. There were no meningeal signs or focal weakness. Fundus examination was normal. Other systemic examination was normal. Investigations showed hemoglobin 11g/dl, total leucocyte count 4200/cumm with 52% polymorphs and 39% lymphocytes, serum bilirubin 0.5mg/dL with normal transaminases and INR of 1.9. Arterial blood gas showed pH of 7.38 and bicarbonate 23mEq/L. Lumbar puncture showed clear cerebrospinal fluid (CSF) with normal pressure, 71.4mg/dL glucose, 16.2 mg/dL protein, 4 cells/cumm (mostly polymorphs and few degenerated cells) and no bacteria. Nasopharyngeal swab was negative for Covid-19 RT-PCR. Blood and CSF was negative for Japanese Encephalitis IgM. Testing for influenza virus was unavailable at our centre. MRI brain showed symmetrical hyper-intense signal intensity on T2W/FLAIR, restricted diffusion on DWI and signal drop (apparent diffusion coefficient) involving bilateral ganglio-thalamic complexes, brain stem and cerebral peduncles (Figure 1). Injection ceftriaxone (100mg/kg/day in 2 divided doses), vancomycin (60mg/kg/day in 4 divided doses) and acyclovir (45mg/kg/day in 3 divided doses) were started intravenously (IV). Oseltamivir was unavailable. Convulsions were controlled with levericetam (40mg/kg/day in 2 divided doses). Intravenous Immunoglobulin (IVIG) (30mg/kg/day for 3 days) followed by oral prednisolone (2mg/kg/day in 2 divided doses) were also given. On 3rd day, patient showed improved sensorium and no further convulsions. On follow-up at three months, distal athetoid movements remained but repeat MRI brain showed resolution.

Figure 1. MRI brain shows thalamic hyperintensity on T2W and restricted diffusion in bilateral thalamus on DWI.

What is the diagnosis?
What is the diagnosis?
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