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Revista de Neurologia

[Reye's syndrome. Description of a case focused on the patient's epileptic seizures].

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P E Jiménez-Caballero
M C Montes-Gonzalo
J M Velázquez-Pérez

Nøgleord

Abstrakt

BACKGROUND

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.

METHODS

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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