Encefalitis de Rasmussen de inicio tardío: presentación de un caso y revisión de la literatura

Introduction

Rasmussen's encephalitis (RE) is a rare neurological disorder that causes continuous partial epilepsy, cognitive decline, and hemiparesis in children, with no sex preference. It is characterized by affecting a single cerebral hemisphere, with possible aphasia if it affects the dominant side. Histologically, it shows perivascular inflammation and hemispheric atrophy in frontotemporal and insular areas. Radiologically, cortical atrophy is observed on MRI. Although it typically begins in childhood, it can appear in adolescence or adulthood as adult-onset RE.

Clinical case

The case of a 46-year-old female patient is presented, with the onset of the disease at 18 years of age.

Discussion

This is an uncommon disease that classically presents in childhood. However, approximately 10% of cases develop in adults. The disease progresses through three phases: prodromal with focal seizures, acute with increased seizures and brain volume loss, and residual with hemispheric atrophy and neurological deficits. Diagnosis is supported by clinical criteria and studies such as MRI and EEG. In the presented case, the disease presentation was atypical, constituting a diagnostic challenge. Treatment includes immunotherapy and, in severe cases, hemispherectomy.

Conclusion

RE in adults presents clinical challenges due to its rarity and distinctive manifestations compared to pediatric cases, including a better prognosis and slower progression with fewer cognitive deficits and continuous epilepsy.