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The best-known type in this group is called the gangliocytoma, one form of which is the dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease) This is a slowly evolving lesion that forms a mass in the cerebellum; it is composed of granule, Purkinje, and glial cells Reproduced therein, in a disorganized fashion, is the architecture of the cerebellum The importance of distinguishing this disease from other cerebellar tumors is its lack of growth potential and favorable prognosis It should be excised if symptomatic Other forms of gangliogliomas include the desmophilic infantile ganglioglioma, some of the xanthoastrocytomas, and the dysembrioplastic neuroepitheliomas Often they lie near the ventricles, and they may induce hydrocephalus Many of these tumors are rare and affect children mostly; therefore they are not discussed further here Good descriptions are to be found in the monographs of Rus-

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sell and Rubinstein and Levine and Schmidek and in the article by Zentner and coworkers Colloid (Paraphysial) Cyst and Other Tumors of the Third Ventricle The most important of these is the colloid tumor, which is derived, it is generally believed, from ependymal cells of a vestigial third ventricular structure known as the paraphysis The cysts formed in this structure are always situated in the anterior portion of the third ventricle between the interventricular foramina and are attached to the roof of the ventricle (Fig 3115) They vary from 1 to 4 cm in diameter, are oval or round with a smooth external surface, and are lled with a glairy, gelatinous material containing a variety of mucopolysaccharides The wall is composed of a layer of epithelial cells, some ciliated, surrounded by a capsule of brous connective tissue Although congenital, the cysts practically never declare themselves clinically until adult life, when they block the third ventricle and produce an obstructive hydrocephalus Suspicion of this tumor is occasioned by intermittent, severe bifrontal-bioccipital headaches, sometimes modi ed by posture ( ball valve obstruction of the third ventricle) or with crises of headache and obtundation, incontinence, unsteadiness of gait, bilateral paresthesias, dim vision, and weakness of the legs, with sudden falls but no loss of consciousness ( drop attacks, see page 329) Stooping may result in an increase or onset of headache and loss of balance However, this intermittent obstructive syndrome has been infrequent in our experience More often the patient has no headache and presents with the symptoms comparable to those of normal-pressure hydrocephalus Subtle behavioral changes are common and a few patients, as emphasized by Lobosky and colleagues, experience mild confusion and changes in personality that may reach the extreme of psychotic behavior In our experience, chronic headache or gait dif culty is usually present by that time The treatment for many years has been surgical excision, which always carries some risk, but satisfactory results have also been obtained by ventriculoperitoneal shunting of the CSF, leaving the benign growth untouched Decompression of the cyst by aspiration under stereotaxic control has also become an increasingly popular procedure Other tumors found in the third ventricle and giving rise mainly to obstructive symptoms are craniopharyngiomas (see below), papillomas of the choroid plexus, and ependymomas (discussed earlier).

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Arachnoid Cyst ( Localized Pseudotumor ) This lesion, which is probably congenital, presents clinically at all ages but may become evident only in adult life, when it gives rise to symptoms of increased intracranial pressure and sometimes to focal cerebral or cerebellar signs, simulating intracranial neoplasm Seizures may occur but are not characteristic; we have seen only one instance In infants and young children, macrocrania and extensive unilateral transillumination are characteristic features Usually these cysts overlie the sylvian ssure; occasionally they are interhemispheric under the frontal lobes or lie in the pineal region or under the cerebellum They may attain a large size, to the point of enlarging the middle fossa and elevating the lesser wing of the sphenoid, but they do not communicate with the ventricle Rarely, one of these cysts may cover the entire surface of both cerebral hemispheres and create a so-called external hydrocephalus (page 533) The cysts are readily recognized (often accidentally) on the nonenhanced CT scan or MRI, showing a circumscribed tissue defect lled with uid that has the density of CSF (Gandy and Heier) If these cysts are completely asymptomatic, they should be left alone; if symptomatic, additional (MRI) studies are indicated, so as not to overlook a chronic subdural hematoma, which is often associated and may not be visualized on the unenhanced CT scan Suprasellar arachnoid cysts are discussed further on (page 577) The treatment of enlarging and symptomatic cysts is marsupialization or, less preferably, by shunting from the cyst to the subarachnoid space

TABLE 1-9