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Benjamin D. Schanker, Brian P. Walcott, Brian V. Nahed, Kristopher T. Kahle, Yan Michael Li and Jean-Valery C. E. Coumans

familial aggregation and clustering of CM, suggesting a genetic basis. 1 , 6–9 , 13 , 15 , 17 , 22 , 27 , 28 , 34 , 37–40 , 42 , 44 , 45 , 49 , 51 , 52 A recent large retrospective series of 500 cases spanning the past 2 decades found the prevalence of familial CM to be about 3%, 41 and a past study of 364 patients with CM found that 12% of patients had a close relative with CM and/or syringomyelia. 27 We report on a series of 3 family pairs in whom a CM was present ( Table 1 ). TABLE 1: Cases of familial Chiari malformation * Case No

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Familial frontonasal dermoid cysts

Report of four cases

Jeremy L. Plewes and Ivan Jacobson

I ntracranial dermoid cysts, although rare, are well documented. 6, 12, 15, 16 They are usually located in the posterior fossa and the spinal canal, but have also been reported in the subfrontal 11 and median nasal regions. It is difficult to ascertain the true incidence of these tumors in either the frontal or nasal sites as some are not reported and some may be incorrectly diagnosed, particularly the nasal variety. Familial nasal dermoid cysts have also been described. In all, seven families are documented, with 19 patients affected. 1, 2, 8–10, 14 No

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Jonathan Gaillard, Jennifer Klein, Daniel Duran, Armide Storey, R. Michael Scott, Kristopher Kahle and Edward R. Smith

M oyamoya is a progressive arteriopathy characterized by narrowing of the internal carotid arteries and their intracranial branches, resulting in compensatory collateral vessel development and increased risk of stroke. Initially described as an idiopathic process, there is now improved recognition and diagnosis of moyamoya associated with various genetic and systemic disorders, in addition to moyamoya-specific mutations. However, detailed study of the prevalence of familial risk of moyamoya remains limited in the pediatric population. Here we present our

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Maki Mukawa, Tadashi Nariai, Yoshiharu Matsushima and Kikuo Ohno

recognized as a susceptibility gene for MMD 4 , 11 in an East Asian population, the necessity of investigating other compounding factors to explain the disease presentation was also indicated. Therefore, it should be important to compare the clinical features of MMD between sporadic and familial cases using objective parameters such as MRI and an intelligence scale. While most cases of MMD appear to be sporadic, genetic factors of MMD appear to be involved in the pathogenesis of the disease. Familial MMD is estimated to comprise approximately 10%–15% of all reported MMD

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Norbert Roosen, Christian De La Porte, Michel Van Vyve, Claude Solheid and Paul Selosse

I n contrast to astrocytomas and glioblastomas, which have occasionally been reported as occurring in members of one family, the familial appearance of oligodendrogliomas has only been described once. 8 We present two patients, mother and daughter, both of whom were operated on for an oligodendroglioma. Case Reports Case 1 This 37-year-old mother was admitted for evaluation of grand mal epilepsy. The neurological examination was normal. A right posterofrontal focus was noted in the electroencephalogram, and right carotid angiography demonstrated an

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Familial gliomas

Report of four cases

Howard H. Kaufman and Ronald Brisman

posterior fossa, one with malignant histological characteristics and the other relatively benign and cystic. Discussion Although many cases of familial gliomas have been reported, 1, 11, 17 the question remains as to whether the familial occurrence is due to chance alone or to genetic factors. Hauge and Harvald 8 reviewed almost 4000 relatives of over 300 families in which gliomas occurred, together with 2000 relatives in about 250 normal families; they concluded that “… familial occurrence of gliomata and meningiomata seems to be a rare phenomenon

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Amparo Wolf, Huda Alghefari, Daria Krivosheya, Michael D. Staudt, Gregory Bowden, David R. Macdonald, Sharan Goobie, David Ramsay and Matthew O. Hebb

C erebellar liponeurocytomas are typically low-grade tumors that arise in the cerebellar hemispheres or vermis and contain cells of neuronal, astrocytic, and lipomatous differentiation. 17 The biology and long-term outcomes of these tumors are undefined, and to our knowledge, familial occurrences have not been described. We present here the case of a young woman with a cerebellar liponeurocytoma and multiple immediate family members, including her mother, with similar lesions. This report describes the tumors from the patient and her mother and provides a

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Andrés M. Lozano and Richard Leblanc

vascular degeneration added to congenital predisposition may be important in the formation of aneurysms. The role of inheritance in the genesis of cerebral aneurysms is well established for certain disorders such as Type IV Ehlers-Danlos syndrome, Marfan's syndrome, pseudoxanthoma elasticum, and polycystic kidney disease. 22 In addition, a large number of familial cases of cerebral aneurysms not associated with a recognized inherited disorder have been reported. It is therefore of interest to compare the demographic and pathological features of familial cerebral

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Familial arteriovenous malformations

Report of four cases in one family

Michael C. Boyd, Paul Steinbok and Donald W. Paty

was neurologically well. Fig. 1. Case 1. Left: Preoperative computerized tomography scan showing a large right temporal cyst (C) and one of two enhancing mural nodules (arrow) . Right: Photomicrograph of the resected arteriovenous malformation, which consists of abnormal venous and arterial structures. Elastic van Gieson, × 13.5. A later examination disclosed a few pigmented spots on his lower lip of which he had apparently not been aware. However, no other cutaneous lesions or stigmata of a familial vascular disease were found. Case 2

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Timothy C. Ryken, Robert A. Robinson and John C. VanGilder

origin of subependymomas is uncertain, ultrastructural studies indicate that the cell of origin may be the bipotential subependymal cell, which normally differentiates into either ependymal cells or astrocytes. 2, 5 Two previous instances of familial occurrence in patients with subependymoma have been reported in the literature. Clarenbach, et al. , 4 described in 1979 the simultaneous occurrence of fourth ventricular subependymomas in monozygotic twins, both of whom became symptomatic at 22 years of age. In 1987, Honan, et al. , 7 reported the familial