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Nonconvulsive status epilepticus in a child with congenital bilateral perisylvian syndrome
T Tagawa1, Y Itagaki, M Kobayashi
1Department of Pediatrics, Osaka Kouseinenkin Hospital, Japan.
Insights
Congenital bilateral perisylvian syndrome can present with atypical absence status epilepticus, a rare but serious condition. Prompt recognition and treatment are crucial for managing this neurological emergency.
Area of Science:
- Neurology
- Pediatric Neurology
- Neuroimaging
Background:
- Congenital bilateral perisylvian syndrome (CBPS) is a rare neurological disorder characterized by malformations in the brain's perisylvian regions.
- Patients often exhibit symptoms such as pseudobulbar palsy, intellectual disability, and intractable epilepsy.
Observation:
- A 9-year-old male with CBPS experienced episodes of excessive drooling, fluctuating consciousness, and postural instability.
- Electroencephalogram (EEG) revealed continuous diffuse slow spike and wave activity, suggestive of nonconvulsive status epilepticus.
Findings:
- Neuroimaging (CT and MRI) confirmed bilateral perisylvian malformations and diffuse pachygyria.
- Intravenous diazepam provided only transient improvement, with rapid recurrence of status epilepticus.
- This case suggests atypical absence status epilepticus may be more common in CBPS than previously reported.
Implications:
- The findings highlight the importance of considering atypical absence status epilepticus in children with CBPS, even in the absence of prior reports.
- Early diagnosis and immediate intervention for nonconvulsive status epilepticus are critical for patient outcomes.
- This case expands the known spectrum of neurological manifestations associated with congenital bilateral perisylvian syndrome.
Abstract:
A 9-year-old male with congenital bilateral perisylvian syndrome is described. He had pseudobulbar palsy, mental retardation, and intractable epilepsy. Computed tomography and magnetic resonance images of the brain demonstrated bilateral perisylvian malformations and a diffuse pachygyric appearance. At 8 years of age, he had episodes of excessive drooling, fluctuating impairment of consciousness, unsteady sitting, and frequent head drop that lasted several days. The electroencephalogram demonstrated continuous diffuse slow spike and waves. These findings suggested atypical absence status epilepticus. Intravenous administration of diazepam resulted in transient improvement of clinical and electroencephalographic findings. Status epilepticus recurred within several minutes after diazepam administration. Although no patient has been reported to have a history of status epilepticus among those affected by this syndrome, it seems that atypical absence status can occur more frequently than expected, as seen in Lennox-Gastaut syndrome. After recognition and confirmation of nonconvulsive status epilepticus, immediate treatment must be attempted.