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on the nding of ipsilateral or bilateral anosmia or ipsilateral or bilateral blindness often with optic atrophy and mental changes The tumors may reach enormous size before coming to the attention of the physician If the anosmia is unilateral, it is rarely if ever reported by the patient The unilateral visual disturbance may consist of a slowly developing central scotoma Abulia, confusion, forgetfulness, and inappropriate jocularity (Witzelsucht) are the usual psychic disturbances from compression of the inferior frontal lobes (see page 394) The patient may be indifferent to or joke about his blindness Usually there are radiographic changes along the cribriform plate The MRI is diagnostic Except for the largest tumors, surgical removal is possible Meningioma of the Tuberculum Sella Cushing was the rst to delineate the syndrome caused by this tumor All of his 23 patients were female The presenting symptoms were visual failure a slowly advancing bitemporal hemianopia with a sella of normal size Often the eld defects are asymmetrical, indicating a combined chiasmal optic nerve involvement Usually there are no hypothalamic or pituitary de cits If the tumor is not too large, complete excision is possible If removal is incomplete or the tumor recurs or undergoes malignant changes, radiation therapy of one type or another is indicated The outlook is then guarded; several of our patients succumbed within a few years Glioma of the Brainstem Astrocytomas of the brainstem are relatively slow-growing tumors that in ltrate tracts and nuclei They produce a variable clinical picture, depending on their location in the medulla, pons, or midbrain Most often, this tumor begins in childhood (peak age of onset is 7 years), and 80 percent appear before the 21st year Symptoms have usually been present for 3 to 5 months before coming to medical notice In most patients the initial manifestation is a palsy of one or more cranial nerves, usually the sixth and seventh on one side, followed by long tract signs hemiparesis, unilateral ataxia, ataxia of gait, paraparesis, and hemisensory and gaze disorders In the remaining patients the symptoms occur in the reverse order ie, long tract signs precede the cranial nerve abnormalities Patients in the latter group survive longer than those whose illness begins with cranial nerve palsies The combination of a cranial nerve palsy or palsies on one side and motor and/or sensory tract signs on the other always indicates brainstem disease Headache, vomiting, and papilledema may occur, usually late in the the illness, occasionally early The course is slowly progressive over several years unless some part of the tumor becomes more malignant (anaplastic astrocytoma or glioblastoma multiforme) or, as rarely happens, spreads to the meninges (meningeal gliomatosis), in which instance the illness may terminate fatally within months The main problem in diagnosis is to differentiate this disease from a pontine form of multiple sclerosis, a vascular malformation of the pons (usually a cavernous hemangioma), or a brainstem encephalitis, and to distinguish the focal from the diffuse type of glioma (see below) The most helpful procedure in diagnosis and prognosis is MRI (Fig 31-19) A careful imaging and clinical study of 87 patients by Barkovich and coworkers has emphasized the importance of distinguishing between diffusely in ltrating and focal nodular tumors In the more common diffuse type, there is mass effect with hypointense signal on T1-weighted MRI and heterogeneously increased T2 signal, which re ects edema and tumor in ltration These diffusely in ltrating tumors, usually showing an asymmet-.

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Figure 31-19 Pontine glioma T1-weighted MRIs demonstrate a cystic mass with prominent peripheral gadolinium enhancement The patient was a 3-year-old male with progressive cranial nerve and long tract de cits

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rical enlargement of the pons, have a poorer prognosis than the focal or nodular tumors, which tend to occur in the dorsal brainstem and often protrude in an exophytic manner In a few instances of diffuse brainstem glioma, surgical exploration is necessary to establish the diagnosis (inspection and possibly biopsy) However, the histologic characteristics of a minute biopsy specimen of the tumor are not particularly helpful in determining prognosis or treatment and the general practice is to avoid surgery unless the tumor exhibits unusual clinical behavior or does not conform to the typical MRI appearance of the diffuse type Treatment The treatment is radiation, and if increased intracranial pressure develops as a result of hydrocephalus, ventricular shunting of CSF becomes necessary Adjuvant chemotherapy has not been helpful (Kornblith et al) A series of 16 patients treated by Pollack and colleagues emphasizes the fact that the focal and exophytic brainstem tumors are almost all low-grade astrocytomas; these tumors, in contrast to the more diffuse type, usually respond well to partial resection and permit long-term survival because they recur only slowly and do not undergo malignant transformation Gangliocytomas or mixed astrogangliocytomas are rare imitators of nodular glioma in the brainstem The rarer cystic glioma of the brainstem (Fig 31-19), a pilocytic tumor like its counterpart in the cerebellum, is treated by resection of the mural nodule and, as mentioned earlier, has an excellent prognosis Landol et al emphasized the longer survival in adults with pontine glioma (median 54 months) as compared to children Most of the patients with pontine tumors with which we are familiar proved to have malignant gliomas Glioma of the Optic Nerves and Chiasm This tumor, like the brainstem glioma, occurs most frequently during childhood and adolescence In 85 percent of cases, it appears before the age of 15 years (average 35 years), and it is twice as frequent in girls as in boys (see Cogan) The initial symptoms are dimness of vision with constricted elds, followed by bilateral eld defects of homony-.

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