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Refractory epilepsy accompanying acute encephalitis with multifocal cortical lesions: possible autoimmune etiology
Tohru Okanishi1, Yuka Mori, Tetsuya Kibe
1Division of Child Neurology, Institute of Neurological Sciences, Faculty of Medicine, Tottori University, 36-1 Yonago 683-8504, Japan. oknsoknsokns@yahoo.co.jp
Abstract:
We report on a 14-year-old male suffering from acute encephalitis, whose clinical course met the criteria for acute encephalopathy with refractory, repetitive partial seizures (AERRPS). He presented with extremely refractory partial and secondary generalized seizures, and required high-dose barbiturate infusion therapy for 57 days under mechanical ventilation. Seven weeks after onset, the seizures were ameliorated by treatment with sodium bromide, carbamazepine, clobazam, and high-dose phenobarbital. Magnetic resonance imaging on day 14 of admission showed multifocal cortical lesions scattered in the bilateral hemispheres; these disappeared on day 34. Diffuse and mild atrophy of the cerebral cortex, and moderate atrophy of the hippocampus, appeared by day 61. Serum anti-glutamate receptor epsilon2 autoantibodies were detected on day 2. The patient was discharged after 113 days of admission with intractable epilepsy, memory disability, and regression of intelligence. We discuss the etiological significance of the multifocal lesions, which are unusual findings on neuroimaging of AERRPS.
Insights
This study details a teen with acute encephalitis and severe seizures (AERRPS), highlighting unusual brain lesions and long-term cognitive deficits. Treatment involved barbiturates, anti-epileptics, and resulted in intractable epilepsy.
Area of Science:
- Neurology
- Pediatric Neurology
- Neuroimmunology
Background:
- Acute encephalitis can present with severe neurological complications.
- Acute encephalopathy with refractory, repetitive partial seizures (AERRPS) is a rare and severe condition.
- Understanding the neuroimaging and immunological aspects of AERRPS is crucial for patient management.
Observation:
- A 14-year-old male presented with acute encephalitis and extremely refractory seizures meeting AERRPS criteria.
- The patient required prolonged mechanical ventilation and high-dose barbiturate therapy.
- Neuroimaging revealed transient multifocal cortical lesions and later developed cerebral and hippocampal atrophy.
Findings:
- Serum anti-glutamate receptor epsilon2 autoantibodies were detected early in the clinical course.
- Seizures eventually improved with a combination of anti-epileptic drugs and sodium bromide.
- The patient was discharged with intractable epilepsy, memory deficits, and intellectual regression.
Implications:
- The presence of multifocal cortical lesions is an unusual neuroimaging finding in AERRPS.
- Autoantibodies against glutamate receptors may play a role in the pathogenesis of AERRPS.
- This case underscores the severe long-term neurological sequelae associated with AERRPS.
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Encephalitis l: Introduction
Encephalitis ll: Pathophysiology
Epilepsy ll: Types
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Various factors can trigger epilepsy, including genetic factors, brain damage, metabolic causes, and unknown etiology. Diagnosis of epilepsy involves electroencephalography (EEG), which...
Seizures: Classification
Seizures are typically classified into two main categories: focal and generalized seizures.
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Seizures l: Introduction
