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Fredric B. Meyer, Michael J. Ebersold and David F. Reese

disturbances. However, pain in the C-2 distribution, Brown-Séquard symptoms, and lower cranial nerve findings suggest foramen magnum pathology. Multiple sclerosis was sometimes suggested because of the chronicity, varying degree of weakness, sensory changes, bladder disturbances, gait difficulties, and response to steroids. However, foramen magnum tumors in this series were continually progressive and not fluctuating as suggested by others. 1, 7 In addition, suboccipital pain, the lack of ocular symptoms, 11th cranial nerve deficit, and Brown-Séquard findings are helpful

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Fredric B. Meyer, Thoralf M. Sundt Jr. and Bruce W. Pearson

Within this second group, there is a 32% incidence of bilateral tumors. 23, 45 Therefore, if a positive family history is obtained in the initial evaluation of a patient, early examination of other family members should be performed since the ease of resection is based on the size of the tumor. There appears to be greater incidence of carotid body tumors in people who live at high altitudes 2 or in patients subjected to chronic hypoxia. 9 Clinical Presentation The most frequent presenting symptom in a patient with a carotid body tumor is a palpable neck

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Daniela Lombardi, Bernd W. Scheithauer, David Piepgras, Fredric B. Meyer and Glenn S. Forbes

–1989), pure oligodendrogliomas (1960–1982), cerebellar-type (pilocytic) astrocytomas (1960–1984), and supratentorial pilocytic astrocytomas (1963–1984). In total, 1034, 82, 104, and 51 cases were found, respectively. All AVM cases were reviewed histologically in a search for examples in which oligodendroglial or astrocytic elements were conspicuous. Although lesions in which astrocytes were prominent showed typical features of chronic astrogliosis, we encountered eight AVM cases (0.1%) that showed an apparent increase in the oligodendrocyte content. Paraffin blocks were

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Daniela Lombardi, Bernd W. Scheithauer, Fredric B. Meyer, Glenn S. Forbes, Edward G. Shaw, Donna J. Gibney and Jerry A. Katzmann

, ranging from 1 to 27 years (mean 6 years) after surgery, disclosed three deaths. One patient died 8 years after surgery of aspiration pneumonia due to lower cranial nerve dysfunction, one died 2 years after surgery with chronic encephalopathy related to poorly controlled hydrocephalus, and the third died of central respiratory depression 3 years after surgery. In none of these cases was there radiological evidence of tumor recurrence or progression. Overall, two patients died perioperatively and three during the follow-up period, but none succumbed to direct tumor

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Marc S. Goldman, Robert E. Anderson and Fredric B. Meyer

following MCA occlusion in rats. Cerebral blood flow, cerebrovascular permeability, and brain edema. J Neurosurg 63: 272–277, 1985 32. Smith AL , Hoff JT , Nielsen SL , et al : Barbiturate protection in acute focal cerebral ischemia. Stroke 5 : 1 – 7 , 1974 Smith AL, Hoff JT, Nielsen SL, et al: Barbiturate protection in acute focal cerebral ischemia. Stroke 5: 1–7, 1974 33. Spetzler RF , Selman WR , Weinstein P , et al : Chronic reversible cerebral ischemia: evaluation of a new baboon

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Brian A. Iuliano, Robert E. Anderson and Fredric B. Meyer

, Teasdale GM , McCulloch J , et al : Recirculation model following MCA occlusion in rats. Cerebral blood flow, cerebrovascular permeability, and brain edema. J Neurosurg 63 : 272 – 277 , 1985 Shigeno T, Teasdale GM, McCulloch J, et al: Recirculation model following MCA occlusion in rats. Cerebral blood flow, cerebrovascular permeability, and brain edema. J Neurosurg 63: 272–277, 1985 37. Spetzler RF , Selman WR , Weinstein P , et al : Chronic reversible cerebral ischemia: evaluation of a new baboon model

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Wouter I. Schievink, Fredric B. Meyer, John L. D. Atkinson and Bahram Mokri

more than several millimeters below the level of the foramen magnum. The cerebellar herniation seen in one of our patients down to the C-2 level with marked distortion of the brainstem and craniomedullary junction is distinctly uncommon and may have been due to the unusual chronicity of the spinal CSF leak (9 years). In patients with intracranial hypotension, sixth cranial-nerve paresis as well as the fifth and seventh cranial-nerve dysfunction observed in one of our patients may be due to traction on the nerve secondary to the “sagging” of the brain. 6 Etiology

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Wouter I. Schievink, Michael R. Puumala, Fredric B. Meyer, Corey Raffel, Jerry A. Katzmann and Joseph E. Parisi

G, Reynolds JJ: Extracellular matrix degradation, in Royce PM, Steinmann B (eds): Connective Tissue and its Inheritable Disorders: Molecular, Genetic, and Medical Aspects. New York: Wiley-Liss, 1993, pp 287–316 27. Novis BH , Young GO , Bank S , et al : Chronic pancreatitis and alpha-1-antitrypsin. Lancet 2 : 748 – 749 , 1975 Novis BH, Young GO, Bank S, et al: Chronic pancreatitis and alpha-1-antitrypsin. Lancet 2: 748–749, 1975 28. Pilz P , Hartjes HJ : Fibromuscular dysplasia and

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Jonathan A. Friedman, Fredric B. Meyer, Douglas A. Nichols, Robert J. Coffey, L. Nelson Hopkins, Cormac O. Maher, Irene D. Meissner and Bruce E. Pollock

factors have been implicated in this process. 17 One possible mechanism is that chronic venous hypertension may dilate the venous end of dural capillaries, ultimately causing ectatic dilation of the entire capillary network and precipitating direct arteriovenous connections. 11, 28 This is analogous to the formation of mucosal arteriovenous malformations in the gastrointestinal tract, which are thought to occur by a similar mechanism. 5 Venous hypertension is a consequence of DAVFs that may worsen as the flow through the fistula increases. If venous hypertension

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Iris Schrijver, Wouter I. Schievink, Maurice Godfrey, Fredric B. Meyer and Uta Francke

. Neurology 43: 609–611, 1993 10. Fukutake T , Sakakibara R , Mori M , et al : Chronic intractable headache in a patient with Marfan's syndrome. Headache 37 : 291 – 295 , 1997 Fukutake T, Sakakibara R, Mori M, et al: Chronic intractable headache in a patient with Marfan's syndrome. Headache 37: 291–295, 1997 11. Furthmayr H , Francke U : Ascending aortic aneurysm with or without features of Marfan syndrome and other fibrillinopathies: new insights. Semin Thorac Cardiovasc Surg 9 : 191