CASE DESCRIPTION
A 17-year-old male was admitted to the hospital for persistent seizures.
Symptoms began five years ago. They lasted an average of one minute and
would stop and restart, eventually occurring up to multiple times.
Consciousness was preserved with the events. Physical examination
revealed intellectual and cognitive impairment. Computed tomography
image showed atrophy of the left cerebral hemisphere (Figure 1).
Clinical and imaging features were consistent with the diagnosis of RE.
This patient had been attempted treatment with conventional therapies
including steroids, plasmapheresis, and intravenous immunoglobulin. The
condition had improved, but not completely.
RE is a rare chronic neurological disorder, characterized by unilateral
inflammation of the cerebral cortex, drug-resistant epilepsy,
progressive neurological and cognitive impairment. The etiology is
unknown, but evidence supports an autoimmune basis.1Diagnostic imaging methods, especially magnetic resonance imaging, have
become a mainstay for diagnostic assessment and follow-up in RE.
Usually, in the acute stage, there is a hyperintense signal on the
T2-weighted image that is often present in cortical/subcortical regions
or both. The distribution is heterogeneous and temporal fluctuation.
Thereafter, most patients present with unilateral enlargement of the
ventricular system. RE requires a differential diagnosis from
hemimegalencephaly. Treatment with immunotherapy can improve at an early
stage, at a later stage, hemispherectomy should be
considered.2