Lennox-Gastaut Syndrome and idiopathic intracranial hypertension

Correia, Jason; Schweder, Patrick; Mews, Peter; Patel, Rakesh; Law, Andrew

doi:10.1016/j.jocn.2010.01.025

Cited by 5 publications

(3 citation statements)

References 9 publications

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“…2,3 Several etiologies including hypoxic-ischemic encephalopathies, meningoencephalitis, tuberous sclerosis complex, cerebral malformations, monogenic disorders, chromosomal abnormalities, tumors (such as hypothalamic hamartomas) or idiopathic intracranial hypertension can be involved. [1][2][3][4][5] Despite their rare occurrence some inborn errors of metabolism, such as biotinidase defects or disorders of creatine metabolism, have a relevant importance because of the availability of specific effective treatments. 2 Recent data demonstrated that LGS children with history of birth hypoxia or other perinatal events have more severe epilepsy with an earlier onset and a higher incidence of delayed milestones.…”

Section: Epidemiologymentioning

confidence: 99%

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Lennox–Gastaut Syndrome: A State of the Art Review

Mastrangelo

2017

Neuropediatrics

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Lennox-Gastaut syndrome (LGS) is a severe age-dependent epileptic encephalopathy usually with onset between 1 and 8 years of age. Functional neuroimaging studies recently introduced the concept of Lennox-Gastaut as "secondary network epilepsy" resulting from dysfunctions of a complex system involving both cortical and subcortical structures (default-mode network, corticoreticular connections, and thalamus). These dysfunctions are produced by different disorders including hypoxic-ischemic encephalopathies, meningoencephalitis, cortical malformations, neurocutaneous disorders, or tumors. The list of etiologies was expanded to pathogenic copy number variants at whole-genome array comparative genomic hybridization associated with late-onset cases or pathogenic mutations involving genes, such as , or. Various clinical trials demonstrated the usefulness of different drugs (including rufinamide, clobazam, lamotrigine, topiramate, or felbamate), ketogenic diet, resective surgery, corpus callosotomy, and vagus nerve stimulation in the treatment of epileptic manifestations. The outcome of LGS often remains disappointing regarding seizure control or cognitive functioning. The realization of animal models, which are still lacking, and the full comprehension of molecular mechanisms involved in epileptogenesis and cognitive impairment would give a relevant support to further improvements in therapeutic strategies for LGS patients.

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Section: Epidemiologymentioning

confidence: 99%

“…71 In these subjects death often results from an accident during seizures or complications of status epilepticus. 4,5…”

Section: Differential Diagnosismentioning

confidence: 99%

Lennox–Gastaut Syndrome: A State of the Art Review

Mastrangelo

2017

Neuropediatrics

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“…12 Idiopathic intracranial hypertension has otherwise previously not been reported in LGS. We report our patient who was successfully treated with CSF diversion in the form of an LP shunt initially, followed by a VP shunt.…”

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confidence: 96%

Surgical resection and adjuvant radiotherapy for a large pineal hemangiopericytoma

Jian

Han

Yang

et al. 2010

Journal of Clinical Neuroscience

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Neuroimaging Features of Intracranial Hypertension in Pediatric Patients With New-Onset Idiopathic Seizures, a Comparison With Patients with Confirmed Diagnosis of Idiopathic Intracranial Hypertension: A Preliminary Study

et al. 2021

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Seizures have been reported in association with idiopathic intracranial hypertension in pediatric patients. Magnetic resonance imaging (MRI) signs of intracranial hypertension have not been investigated before in pediatric patients with new-onset idiopathic seizures. MRI scans of 182 pediatric patients were retrospectively analyzed for imaging findings of intracranial hypertension, including 46 patients with new-onset idiopathic seizures and elevated cerebrospinal fluid opening pressure, 40 patients with new-onset idiopathic seizures and normal opening pressure, 56 patients with confirmed idiopathic intracranial hypertension, and 40 age- and sex-matched controls. The optic nerve sheath diameter is significantly larger in the group with new-onset idiopathic seizures and elevated opening pressure (mean diameter of 6.02 ± 0.45 mm) compared to patients with new-onset idiopathic seizures and normal opening pressure (mean diameter of 5.24 ± 0.50 mm) ( P value <.001). The cutoff value of 6.0 mm for optic nerve sheath diameter showed 63% sensitivity and 88% specificity to differentiate pediatric patients with seizures and elevated opening pressure from those with seizures and normal opening pressure. Conclusion A cutoff value of 6.0 mm for optic nerve sheath dilation may be used as a screening imaging marker to suspect elevated opening pressure with specificity of 88% in pediatric patients with new-onset idiopathic seizures.

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Lennox-Gastaut Syndrome and idiopathic intracranial hypertension

Cited by 5 publications

References 9 publications

Lennox–Gastaut Syndrome: A State of the Art Review

Lennox–Gastaut Syndrome: A State of the Art Review

Surgical resection and adjuvant radiotherapy for a large pineal hemangiopericytoma

Neuroimaging Features of Intracranial Hypertension in Pediatric Patients With New-Onset Idiopathic Seizures, a Comparison With Patients with Confirmed Diagnosis of Idiopathic Intracranial Hypertension: A Preliminary Study

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