Journal of neurosurgery
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Journal of neurosurgery · Feb 2014
ReviewMolecular genetics of paragangliomas of the skull base and head and neck region: implications for medical and surgical management.
Paragangliomas are rare, slow-growing tumors that frequently arise in the head and neck, with the carotid bodies and temporal bone of the skull base being the most common sites. These neoplasms are histologically similar to pheochromocytomas that form in the adrenal medulla and are divided into sympathetic and parasympathetic subtypes based on functionality. Skull base and head and neck region paragangliomas (SHN-PGs) are almost always derived from parasympathetic tissue and rarely secrete catecholamines. ⋯ Collectively these findings demonstrate that paragangliomas of the skull base and head and neck region have a distinct genetic signature from sympathetic-based paragangliomas occurring below the neck, such as pheochromocytomas. Paragangliomas serve as a unique model of primarily surgically treated neoplasms whose future will be altered by the elucidation of their genomic complexities. In this review, the authors present an analysis of the molecular genetics of SHN-PG and provide future directions in patient care and the development of novel therapies.
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Journal of neurosurgery · Feb 2014
Haptoglobin genotype and functional outcome after aneurysmal subarachnoid hemorrhage.
Haptoglobin allele heterogeneity has been implicated in differential reactive oxidant inhibition and inflammation. Haptoglobin α2-α2 has a lower affinity for binding hemoglobin, and when bound to hemoglobin, is cleared less easily by the body. The authors hypothesized that haptoglobin α2-α2 genotype should be less protective for downstream injury after aneurysmal subarachnoid hemorrhage (aSAH) and should portend a worse outcome. ⋯ Patients with aSAH who carry the haptoglobin α2-α2 genotype had a worse outcome. Interestingly, the presence of a single α-2 allele was associated with worse outcome, suggesting that the haptoglobin α-2 protein may play a role in the pathology of brain injury following aSAH, although the mechanism for this finding requires further research. The haptoglobin genotype may provide additional information on individual risk of secondary injury and recovery to guide care focused on improving outcomes.
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Journal of neurosurgery · Feb 2014
Case ReportsAuditory brainstem implants in neurofibromatosis Type 2: is open speech perception feasible?
Patients with bilateral auditory nerve destruction may perceive some auditory input with auditory brainstem implants (ABIs). Despite technological developments and trials in new stimulation sites, hearing is very variable and of limited quality. The goal of this study was to identify advantageous and critical factors that influence the quality of auditory function, especially speech perception. ⋯ This study documents that open-set speech recognition in pure auditory mode is feasible in patients with ABIs. Large tumor volumes do not prevent good outcome. Positive preconditions are short ipsilateral and short bilateral deafness periods and high number of auditory electrodes. Early ability in pure auditory word recognition tests indicates long-term capability of open speech perception.
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Journal of neurosurgery · Feb 2014
Case ReportsAdvanced neuroimaging findings of pseudotumoral hemicerebellitis in an elderly male requiring surgical decompression.
Acute cerebellitis is an inflammatory process that usually affects bilateral cerebellar hemispheres in the pediatric population. Pseudotumoral hemicerebellitis is an extremely rare presentation in which unilateral cerebellar involvement mimics a tumor that can exert significant mass effect on the surrounding structures, which may require surgical intervention. ⋯ Advanced neuroimaging, including MR perfusion and MR spectroscopy, may be helpful in excluding other diagnoses. The authors present the case of pseudotumoral hemicerebellitis in the oldest documented patient, a 73-year-old man who required surgical decompression, and they provide a brief discussion of advanced neuroimaging findings.
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Journal of neurosurgery · Feb 2014
Case ReportsHistopathological assessment of fatal ipsilateral intraparenchymal hemorrhages after the treatment of supraclinoid aneurysms with the Pipeline Embolization Device.
Delayed ipsilateral intraparenchymal hemorrhage has been observed following aneurysm treatment with the Pipeline Embolization Device (PED). The relationship of this phenomenon to the device and/or procedure remains unclear. The authors present the results of histopathological analyses of the brain sections from 3 patients in whom fatal ipsilateral intracerebral hemorrhages developed several days after uneventful PED treatment of supraclinoid aneurysms. ⋯ These findings are suggestive of a potential association between intraprocedural foreign body emboli and post-PED treatment-delayed ipsilateral intraparenchymal hemorrhage.