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[Reye's syndrome. Description of a case focused on the patient's epileptic seizures]
P E Jiménez-Caballero1, M C Montes-Gonzalo, J M Velázquez-Pérez
1Servicio de Neurología, Hospital Virgen de la Salud, 45005 Toledo, España. pjimenez1010j@yahoo.es
Insights
Reye
Area of Science:
- Pediatrics
- Neurology
- Hepatology
Background:
- Reye's syndrome is a severe childhood illness causing encephalopathy and liver damage.
- Often follows viral infections like influenza or varicella.
- Acetylsalicylic acid (ASA) ingestion during viral illness is epidemiologically linked.
Observation:
- A case report details a 20-month-old female with Reye's syndrome post-viral infection and ASA use.
- The patient experienced non-convulsive status epilepticus and later developed Lennox-Gastaut syndrome.
- A hepatic biopsy was deemed appropriate for diagnosis.
Findings:
- The patient's condition progressed, leading to death from pneumonia at 18 years.
- Highlights the potential for Reye's syndrome to mimic inborn errors of metabolism.
- Emphasizes the need to rule out metabolic disorders in suspected Reye's syndrome cases.
Implications:
- Early and aggressive management of cerebral hypertension is crucial for reducing mortality and sequelae.
- Despite decreased incidence, Reye's syndrome requires continued clinical awareness.
- Distinguishing Reye's syndrome from metabolic disorders is vital for appropriate patient care.
Introduction:
Reye's syndrome is an acute disease characterised by encephalopathy and fatty degeneration of the liver that occurs almost exclusively in children. It can cause the death of the patient in up to a third of all cases, generally due to severe cerebral oedema. The aetiopathogenesis of this condition is uncertain and is usually preceded by a viral infection, generally from the influenza or varicella virus. Some studies have shown a strong epidemiological association between the ingestion of acetylsalicylic acid (ASA) during the viral infection and development of Reye's syndrome.
Case Report:
We describe the case of a 20-month-old female who developed Reye's syndrome within the context of a viral infection and the ingestion of ASA. A hepatic biopsy study is appropriate in this syndrome. The patient presented a non-convulsive status during the acute phase and at one year developed Lennox-Gastaut syndrome. She died from pneumonia at the age of 18 years.
Conclusions:
In all patients with clinical features that suggest Reye's syndrome, inborn errors of metabolism that can mimic it must be precluded. Although the incidence of this syndrome has gone down considerably in recent years, it is important to keep it in mind as an early and aggressive diagnosis and treatment of cerebral hypertension will reduce the mortality rate and the sequelae.
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