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Published on: September 20, 2024
A Young Girl with Acute Encephalopathy Who Presented with Status Epilepticus Following a Flu-Like Illness
Noora Mohamed Saqr Aljalahma1, Raafat Hammad Seroor Jadah1
1Department of Pediatrics, Royal Medical Services - Bahrain Defense Force (BDF) Hospital, Riffa, Bahrain.
Insights
Acute necrotizing encephalopathy of childhood (ANEC) is a rare pediatric condition. Early diagnosis and aggressive management are crucial for improving outcomes in children with ANEC.
Area of Science:
- Pediatric Neurology
- Neuroscience
- Rare Diseases
Background:
- Acute necrotizing encephalopathy of childhood (ANEC) is a rare, severe neurological disorder in children.
- It is characterized by rapid deterioration, often following a flu-like illness.
- Thalamic and brainstem lesions are characteristic findings.
Abstract:
BACKGROUND Acute necrotizing encephalopathy of childhood (ANEC) is a rare form of acute encephalopathy in children that is characterized by rapid and progressive deterioration in the patient's clinical condition. Our aim in reporting the case is to highlight the importance of early diagnoses and aggressive early management of ANEC to achieve a better outcome. We report the case of a 5-year-old girl who presented with acute progressive encephalopathy with bilateral symmetrical thalamic involvement and brainstem lesion proceeded by a flu-like illness. CASE REPORT We report a 5-year-old girl who was previously healthy. She presented with rapid progressive encephalopathy and status epilepticus following a flu-like illness. Her magnetic resonance imaging (MRI) showed significance symmetrical bilateral thalamic enhancement with brainstem lesions, which is consistent with the diagnosis of ANEC of childhood, and her initial electroencephalogram (EEG) showed severe encephalopathy. Despite extensive management of ANEC, the patient showed a poor prognosis. CONCLUSIONS ANEC is a rare, specific, devastating condition in the pediatric population. Early diagnosis and aggressive management should be initiated immediately to avoid the high mortality associated with the condition. Furthermore, to highlight the importance of the clear history of the patient who presented with rapid and progressive deterioration in the level of consciousness and status epilepticus proceeded by viral illness, a rapid neuroradiological images such as brain MRI should be obtained to confirm the diagnosis of this rare condition. We also seek to increase awareness of this disorder among healthcare workers and general practitioners.
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