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MAJOR CATEGORIES OF NEUROLOGIC DISEASE
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chemotherapy used for the treatment of anaplastic oligodendroglioma, as described below The special features of astrocytomas of the pons, hypothalamus, optic nerves, and chiasm, which produce highly characteristic clinical syndromes and do not behave like a cerebral mass, are discussed further on in this chapter
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Figure 31-4 Cystic astrocytoma of the cerebellum MRI demonstrates the large cystic component of the tumor (smaller arrow) and the solid tissue component (larger arrow) (Reproduced by permission from Bisese JH: Cranial MRI New York, McGraw-Hill, 1991)
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In children, the tumor is usually in the cerebellum (Fig 31-4) and declares itself by some combination of gait unsteadiness, unilateral ataxia, and increased intracranial pressure (headaches, vomiting) In contrast to glioblastoma, the average survival period after the rst symptom is 5 to 6 years in cerebral astrocytomas and 8 years or more in cerebellar ones Excision of part of a cerebral astrocytoma, particularly the cystic part, may allow survival in a functional state for many years The cystic astrocytoma of the cerebellum is particularly benign in its overall behavior In such cases, resection of the tumor nodule is of singular importance in preventing a recurrence In recent series, the rate of survival 5 years after successful surgery has been over 90 percent (Pencalet et al) The outcome is less assured when the tumor also involves the brainstem and cannot be safely resected The natural history of the low-grade gliomas is to grow slowly and eventually undergo malignant transformation The duration of progression and the latency of recurrence with modern treatment may extend for many years A survey of the outcome of these lowgrade supratentorial tumors showed that 10-year survival after operation was from 11 to 40 percent provided that 5300 cGy was given postoperatively (Shaw et al) In younger patients, particularly if the neurologic examination is normal or nearly so, radiation can be delayed and the course of the tumor evaluated by frequent imaging procedures A number of studies have come to the conclusion that delaying radiation in younger patients may avoid the consequences of dementia and hypopituitarism (see editorial by Peterson and DeAngelis), but others have suggested that the tumor itself and anticonvulsant drugs cause more dif culty than high-dose radiation Lacking any clear bene t on survival, it seems to us that radiation should be withheld initially Nevertheless, an increase in seizures or worsening neurologic signs then presses one to turn to radiation or further surgery Repeated operations prolong life in some patients; chemotherapy has as yet no established place in the treatment of low-grade pure astrocytomas However, tumors with an oligodendroglial component may respond well to combination
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Gliomatosis Cerebri In this variant of high-grade glioma there is a diffuse in ltration of neoplastic glial cells, involving much of one or both cerebral hemispheres with sparing of neuronal elements but without a discrete tumor mass being identi ed Whether this type of gliomatosis represents neoplastic transformation of multicentric origin or direct spread from one or more small neoplastic foci is not known For these reasons, the tumor is impossible to classify (or to grade) using the conventional brain tumor schemes Many small series of gliomatosis cerebri have been reported since Nevin introduced the term in 1938, but no truly distinctive clinical picture has emerged (Dunn and Kernohan) Impairment of intellect, headache, seizures, and papilledema are the major manifestations and do not set these cases apart on a clinical basis from the malignant astrocytoma, in which the tumor may also be more widespread than the macroscopic picture suggests If there is a syndrome that can be associated early on with gliomatosis, in our experience it has been a nondescript frontal lobe abnormality, sometimes mistaken for depression or a subacute dementia, or pseudobulbar palsy may be the rst manifestation The prognosis is poor, generally measured in months from the time of diagnosis CT and MRI reveal small ventricles and one or more large con uent areas of signal change (Fig 31-5) Imaging studies characteristically show the tumor crossing and thickening the corpus callosum Contrast and gadolinium enhancement tend to be scant,
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Figure 31-5 Gliomatosis cerebri invading both hemispheres FLAIR MRI shows large con uent areas of involvement There is slight enhancement at the edges of the lesions after gadolinium infusion, and the corpus callosum is thickened The patient was mentally slow but had no other neurologic signs
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