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Sudden death of an infant with 'an early epileptic encephalopathy'
1Department of Legal Medicine, Osaka City University Medical School, Asahi-machi 1-4-3, Abeno, Osaka 545-8585, Japan. legalmed@med.osaka-cu.ac.jp
Insights
Sudden infant death in a case of Ohtahara syndrome was linked to influenza A viral infection and bacterial bronchopneumonia. This highlights the importance of investigating viral infections in sudden infant deaths, even with clear brain abnormalities.
Area of Science:
- Neurology
- Pathology
- Pediatrics
Background:
- Presents an autopsy case of sudden death in an infant diagnosed with early infantile epileptic encephalopathy with suppression-bursts (Ohtahara syndrome).
- Ohtahara syndrome is a rare and severe form of epilepsy typically presenting in the first few months of life.
Observation:
- The infant experienced recurrent seizures from 3 months of age and collapsed with high fever, leading to death.
- Autopsy and postmortem MRI revealed significant brain atrophy, cortical dysplasia, and signs of olivo-ponto-cerebellar atrophy.
Findings:
- The primary cause of death was determined to be bacterial bronchopneumonia secondary to influenza A virus infection.
- Advanced, asymmetric brain atrophy with cortical dysplasia was noted, particularly in the left temporal and right occipital lobes.
Implications:
- This case underscores the critical need to investigate viral infections, such as influenza A, in sudden infant death cases, even when significant neuropathological findings are present.
- Epidemiological surveys for viral infections are crucial in understanding sudden deaths among physically handicapped infants.
Abstract:
This article reports an autopsy case of sudden death of an infant with an infrequent encephalopathy involving epileptic episodes. The infant was a 1-year and 10-month-old boy, who had a history of the first convulsive seizures in the third month after birth. The clinical diagnosis was described as 'an early infantile epileptic encephalopathy with suppression-bursts' (Ohtahara syndrome). On a winter day, he was collapsed following a high fever and was already dead on the arrival at a hospital. The body was small for the age and poorly nourished. The autopsy and postmortem magnetic resonance imaging scan (MRI) of formalin-fixed brain revealed advanced unsymmetric brain atrophy with cortical dysplasia, which were prominent in the left temporal and right occipital lobes, and sclerotic atrophy of the parahippocampal gyri, additionally showing a feature of the olivo-ponto-cerebellar atrophy. However, the cause of death was pathologically and microbiologically determined as bacterial bronchopneumonia following pulmonary infection of the influenza A virus. In sudden death cases of physically handicapped infants, the investigation of viral infection in consideration of an epidemiological survey is important even when the death can be pathomorphologically explained.