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In children, the proportions differ: astrocytoma, 48 percent; medulloblastoma, 44 percent; ependymoma, 8 percent Seventy percent of gliomas in children are infratentorial; in adults, 70 percent are supratentorial Craniophrayngioma also occurs mainly in younger age groups The incidence of lymphoma was negligible when these series were collected, but it has increased markedly since then (see text)

01 1000 01 1000 02 2000 005 500 005 500 01

Now we go to the 70 Query Designer and access this same query The next illustration shows that we can see the same query within the 70 Query Designer even though we created it in 3x

Table 31-2 Age-speci c frequency of tumor types with age (adapted from the Central Brain Tumor Registry for the United States, 1995 1999; wwwcbtrusorg)

also appears to be increasing even in those with ostensibly normal immune function Classi cation and Grading of Nervous System Tumors Classi cations and grading systems of intracranial tumors abound and are often confusing to the general neurologist Most classi cations have been based on the presumed cell of origin of the neoplasm, while grading systems are meant to be an estimate of the rate of growth and clinical behavior, but the two are often concordant The most recent WHO classi cation represents a current view of tumor specialists but is unfortunately too unwieldy for most clinicians; a discussion of this system can be found in the article by Kleihues and colleagues In order to make this system comprehensible, what follows is an extracted synopsis of (1) modern re nements in the histopathologic study of gliomas, (2) the numerical grading system of Daumas-Duport and coworkers (also known as the St Anne Mayo system), (3) the three-tiered system of Ringertz (which correlates most closely with clinical survival), and (4) the aforementioned new third edition of the World Health Organization s classi cation (which incorporates the Ringertz system) These pathologic systems notwithstanding, it is becoming apparent that there are practical consequences to subclassifying tumors by additional molecular methods (see further on) The astrocytic tumors, the most common forms of glioma, have been subdivided into diffuse well-differentiated astrocytoma (grade II), anaplastic astrocytoma (grade III), and glioblastoma multiforme (grade IV) These represent a spectrum in terms of

Neuroepithelial tumors (glial origin) Pilocytic astrocytoma Glioblastoma Malignant glioma Diffuse astrocytoma Anaplastic astrocytoma Other astrocytoma Oligodendroglioma Anaplastic oligodendroglioma Ependymomas Mixed glioma Embryonal/primitive/ medulloblastoma Meningeal tumors Meningioma Hemangioblastoma Lymphoma Sellar tumors Pituitary adenoma Craniopharyngioma Cranial and spinal nerve tumors Germ cell tumors Local extension from regional tumors Unclassi ed Total

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Now we will open it in the 70 Query Designer and review the information The next illustration shows the query within the 70 Query Designer As you can see, the system realigns the characteristics and key figures in the appropriate fields, since the 3x and 70 versions are quite different

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In addition to the well-known chronic sensory neuropathy associated with chronic renal failure that is discussed later in the chapter, there is a more rapid ( accelerated ) process that has not been widely appreciated as a cause of acute and subacute weakness Most patients in our series were diabetics with stable end-stage renal failure who had been treated by peritoneal dialysis for their long-standing kidney disease (Ropper, 1993) In contrast to the better characterized and less severe chronic uremic neuropathy (page 1149), generalized weakness and distal paresthesias progress in over one or more weeks until a bedbound state is reached In some ways the illness simulates GBS More aggressive dialysis or a change to hemodialysis has little immediate effect, although kidney transplantation is curative Electrophysiologic studies show some demyelinating features but usually not a conduction block There is raised CSF protein concentration (not unexpectedly, for there is usually an element of diabetic neuropathy) A few reported cases have been clinically almost indistinguishable from in ammatory demyelinating polyneuropathy, including, in some, a response to plasma exchange or gamma globulin As with the more

(See page 1031) Some of the neurotoxic effects of Corynebacterium diphtheriae and the mode of action of the exotoxin elaborated by the bacillus are described in Chap 43 Local action of the exotoxin may paralyze pharyngeal and laryngeal muscles (dysphagia, nasal voice) within 1 or 2 weeks after the onset of the infection and shortly thereafter may cause blurring of vision due to paralysis of accommodation, but these and other cranial nerve symptoms may be overlooked At this stage, the cranial neuropathy must be distinguished from that of GBS, botulism, and most of all, from myasthenia gravis A polyneuropathy, coming 5 to 8 weeks later, takes the form

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