Source:Pediatric Neurology
Author(s): Ayaz M. Khawaja, Sandipan Pati, Yu-Tze Ng
The hypothalamic hamartoma (HH) is an abnormal hyperplastic and heterotropic tissue growing in a disorganized fashion, with an estimated incidence of 1 per 50,000-100,000. The HH has been shown to cause intrinsic epileptogenesis leading to gelastic seizures. Surrounding cortical structures may develop secondary epileptogenesis. Persistent seizures due to HHs can be debilitating and result in significant cognitive and behavioral impairment. Early recognition and treatment is important in controlling seizures and preventing further cognitive deterioration. Some patients may experience an improvement in cognition and behavior with early treatment, lending support to the hypothesis that HH is a reversible epileptic encephalopathy. The outcome of epilepsy associated with HH has significantly evolved with the availability of new treatment techniques and an improved understanding of its pathogenesis. Increasing evidence supporting the role of HH in causing gelastic seizures and secondary epileptogenesis has led to surgery being increasingly the definitive treatment. Several minimally invasive procedures have also been devised including neuroendoscopic approaches and different stereotactic radio and laser ablation techniques. However various adverse effects attributable to the specific technique employed in the treatment of HH can occur. We present a comprehensive review of various classification schemes used to divide HHs into different subtypes and the recommended surgical approaches for each. We also reviewed the literature for all the treatment modalities currently available, and compare their efficacy in controlling seizures, and their safety profiles.
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