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Published on: April 24, 2020
Lennox-Gastaut syndrome and idiopathic intracranial hypertension
Jason A Correia1, Patrick M Schweder, Peter J Mews
1Department of Paediatric Neurosurgery, Starship Children's Hospital, Park Road, Grafton, Auckland, New Zealand. jason_correia@yahoo.com
Insights
Lennox-Gastaut Syndrome, a severe childhood epilepsy, was found to be associated with symptomatic idiopathic intracranial hypertension in a 15-year-old male. This rare comorbidity required surgical intervention for cerebrospinal fluid pressure management.
Area of Science:
- Neurology
- Pediatric Neurology
- Epileptology
Background:
- Lennox-Gastaut Syndrome (LGS) is a severe, treatment-resistant childhood epilepsy characterized by specific EEG findings, multiple seizure types, and developmental delay.
- Idiopathic Intracranial Hypertension (IIH) presents with elevated cerebrospinal fluid pressure without an identifiable intracranial cause, often leading to headaches and visual disturbances.
Observation:
- A 15-year-old male with a diagnosis of Lennox-Gastaut Syndrome presented with symptoms suggestive of elevated intracranial pressure.
- Diagnostic workup confirmed the coexistence of symptomatic Idiopathic Intracranial Hypertension in the context of established Lennox-Gastaut Syndrome.
Findings:
- This case represents the first documented association between Lennox-Gastaut Syndrome and symptomatic Idiopathic Intracranial Hypertension.
- The patient's condition necessitated the surgical placement of a ventriculoperitoneal shunt to manage the elevated cerebrospinal fluid pressure.
Implications:
- This finding expands the known clinical spectrum of Lennox-Gastaut Syndrome, highlighting a potential, albeit rare, neurological comorbidity.
- Further research may be warranted to explore the underlying mechanisms and prevalence of Idiopathic Intracranial Hypertension in epilepsy syndromes.
- Recognition of this association is crucial for appropriate diagnosis and management of patients with refractory epilepsy and neurological comorbidities.
Abstract:
Lennox-Gastaut Syndrome is a severe childhood epilepsy syndrome characterised by the diagnostic triad of a slow spike and wave pattern on electroencephalogram, multiple seizure types and developmental delay. Idiopathic intracranial hypertension is a syndrome characterised by raised cerebrospinal fluid pressure in the absence of an intracranial mass lesion or ventricular dilatation and often headache. We present the first reported case of Lennox-Gastaut Syndrome associated with symptomatic idiopathic intracranial hypertension in a 15 year old male, requiring cerebrospinal fluid diversion by means of ventriculoperitoneal shunting.
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