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  • Author or Editor: Anand V. Germanwala x
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Stephen J. Johans, Kevin N. Swong, Daniel J. Burkett, Michael P. Wemhoff, Sean M. Lew, Chirag R. Patel and Anand V. Germanwala

Superficial siderosis (SS) of the CNS is a rare and often unrecognized condition. Caused by hemosiderin deposition from chronic, repetitive hemorrhage in the subarachnoid space, it results in parenchymal damage in the subpial layers of the brain and spinal cord. T2-weighted MRI shows the characteristic hypointensity of hemosiderin deposition, classically occurring around the cerebellum, brainstem, and spinal cord. Patients present with progressive gait ataxia and sensorineural hearing impairment. Although there have been several studies, case reports, and review articles over the years, the clear pathophysiology of subarachnoid space hemorrhage remains to be elucidated. The proposed causes include prior intradural surgery, prior trauma, tumors, vascular abnormalities, nerve root avulsion, and dural abnormalities.

Surgical repair of a dural defect associated with SS has been shown to be efficacious at preventing symptomatic progression. There have been several reports of dural defects within the spinal canal treated with surgery. Here, the authors present the first known case of a dural defect of the ventral skull base, namely a clival meningocele, presumed to be causing SS. In this case report, a 10-year-old girl with a history of head trauma at the age of 3 years was found to have a clival meningocele 3 years after her original trauma. On follow-up imaging, the patient was found to have radiographic growth of the meningocele along with evidence of SS of the CNS. The patient was treated conservatively until she began to have progressive hearing loss. It was presumed that the growing meningocele was the source of her SS. An endoscopic endonasal transclival approach with a multilayer dural reconstruction was performed to fix the dural defect and repair the meningocele in hopes of mitigating the progression of her symptoms. At her 12-month postoperative follow-up, she was doing well, with audiometry showing a slightly decreased hearing threshold in the left ear but improved speech discrimination bilaterally. Postoperative MRI showed a stable level of hemosiderin deposition and meningocele repair. Long-term follow-up will be necessary to evaluate for continued clinical stabilization or possible improvement.

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Daniel M. Heiferman, Daphne Li, Joseph C. Serrone, Matthew R. Reynolds, Anand V. Germanwala, Clarence B. Watridge and Adam S. Arthur

Dr. Francis Murphey of the Semmes-Murphey Clinic in Memphis recognized that a focal sacculation on the dome of an aneurysm may be angiographic evidence of a culpable aneurysm in the setting of subarachnoid hemorrhage with multiple intracranial aneurysms present. This has been referred to as a Murphey’s “teat,” “tit,” or “excrescence.” With variability in terminology, misspellings in the literature, and the fact that Dr. Murphey did not formally publish this important work, the authors sought to clarify the meaning and investigate the origins of this enigmatic cerebrovascular eponym.

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Anand V. Germanwala, Jeffrey C. Mai, Nestor D. Tomycz, Ajay Niranjan, John C. Flickinger, Douglas Kondziolka and L. Dade Lunsford

Object

The aim of this paper was to determine prognostic factors for adult medulloblastoma treated with boost Gamma Knife surgery (GKS) following resection and craniospinal irradiation.

Methods

The authors performed a retrospective analysis of 12 adult patients with histologically proven medulloblastoma or supratentorial primitive neuroectodermal tumor who between February 1991 and December 2004 underwent ≥ 1 sessions of GKS for posttreatment residual or recurrent tumors (6 tumors in each group). Before GKS, all patients had undergone a maximal feasible resection followed by craniospinal irradiation. Nine patients also received systemic chemotherapy. Stereotactic radiosurgery was applied to residual and recurrent posterior fossa tumor as well as to foci of intracranial medulloblastoma metastases. The median time interval from initial diagnosis and resection to the first GKS treatment was 24 months (range 2–37 months). The mean GKS-treated tumor volume was 9.4 cm3 (range 0.5–39 cm3).

Results

Following adjunctive radiosurgery, 5 patients had no evidence of tumor on magnetic resonance (MR) imaging, 3 patients had stable tumor burden on MR imaging, and 4 patients had evidence of tumor progression locally with or without intracranial metastases. All patients with tumor progression died. Eight patients survive with a mean cumulative follow-up of 72.4 months (range 21– 152 months). No acute radiation toxicity or delayed radiation necrosis was observed among any of the 12 patients. The majority of patients who achieved tumor eradication (80%) and tumor stabilization (67%) after GKS had residual tumor as the reason for their referral for GKS. The best outcomes were attained in patients with residual disease who were younger, had smaller tumor volumes, had no evidence of metastatic disease, and had received higher cumulative GKS doses.

Conclusions

Single or multiple GKS sessions were a well-tolerated, feasible, and effective adjunctive treatment for posterior fossa residual or recurrent medulloblastoma as well as intracranial metastatic medulloblastoma in adult patients.