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A Model for Epilepsy of Infectious Etiology using Theiler's Murine Encephalomyelitis Virus
Published on: June 23, 2022
Acute encephalopathy with biphasic seizures and late restricted diffusion temporally associated with human bocavirus
Manjinder Singh Randhawa1, Tarlochan Singh Randhawa2, Suresh Kumar Angurana3
1Division of Pediatric Critical Care, Department of Paediatrics, Advanced Paediatric Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Insights
Acute encephalopathy with biphasic seizures and late restricted diffusion (AESD) is a distinct neurological condition in children. Human bocavirus infection was identified in a case presenting with AESD, highlighting potential infectious triggers.
Area of Science:
- Pediatric Neurology
- Infectious Diseases
- Neuroimaging
Background:
- Acute encephalitis is diagnosed symptomatically, with a specific subtype, acute encephalopathy with biphasic seizures and late restricted diffusion (AESD), identified in Asian children.
- AESD presents with biphasic seizures and encephalopathy following an initial febrile illness, often showing characteristic MRI findings like the 'bright tree appearance'.
Observation:
- A case report details a female infant exhibiting the typical clinico-radiological features of AESD.
- Human bocavirus was detected in the patient's stool sample, suggesting a potential etiological link.
- The infant received immunomodulatory therapy (steroids) and antiepileptic drugs.
Findings:
- The patient presented with the characteristic biphasic seizure pattern and encephalopathy of AESD.
- Human bocavirus was identified as a potential trigger in this AESD case.
- Despite treatment, the infant experienced persistent cognitive impairment but remained seizure-free post-treatment.
Implications:
- This case supports the hypothesis of infectious triggers, specifically human bocavirus, in AESD.
- The findings underscore the variable prognosis of AESD, with potential for long-term neurological sequelae like cognitive impairment.
- Further research is warranted to elucidate the role of human bocavirus in AESD pathogenesis and to optimize treatment strategies.
Abstract:
Acute encephalitis is a syndromic diagnosis. In the last two decades, a unique clinico-radiological entity, named acute encephalopathy with biphasic seizures and late restricted diffusion (AESD), has been reported in children from Asia. It is characterised by an acute febrile illness with seizures and encephalopathy, with some initial improvement followed by a second flurry of seizures and deep encephalopathy, 3-4 days later. MRI may show a pattern of 'bright tree appearance'. An aetiological agent may not always be identified but an infectious trigger is proposed. Immunomodulatory therapy has been tried with variable results. The prognosis is variable, and children are usually left with neurological sequelae including epilepsy and cognitive impairment. We describe a female infant who presented with the typical clinico-radiological syndrome of AESD and human bocavirus was identified in the stool. She received steroids and antiepileptic drugs. She has persistent cognitive impairment at follow-up but remained seizure free.
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