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bruit that may be audible to the patient ( self-audible bruit ) Other neurologic manifestations are phrenic nerve palsy, numbness of the face, a Horner syndrome, cerebellar ataxia, and temporal lobe epilepsy The jugular foramen is eroded (visible in basilar skull lms and CT) and the CSF protein may be elevated Women are affected more than men, and the peak incidence is during middle adult life The tumor grows slowly over a period of many years, sometimes 10 to 20 or more Treatment has consisted of radical mastoidectomy and removal of as much tumor as possible, followed by radiation The combined intracranial and extracranial two-stage operation has resulted in the cure of many cases (Gardner et al) A detailed account of this tumor will be found in the article by Kramer Carotid Body Tumor (Paraganglioma) This is a generally benign but potentially malignant tumor originating in a small aggregate of cells of neuroectodermal type The normal carotid body is small (4 mm in greatest diameter and 10 mg in weight) and is located at the bifurcation of the common carotid artery The cells are of uniform size, have an abundant cytoplasm, are rich in substance P, and are sensitive to changes in PO2, PCO2, and pH (ie, they are chemoreceptors, not to be confused with baroreceptors) The tumors that arise from these cells are identical in appearance with tumors of other chemoreceptor organs (paragangliomas) Interestingly, they are 12 times more frequent in individuals living at high altitudes Clinically the usual presentation is of a painless mass at the side of the neck below the angle of the jaw; thus it must be differentiated from the branchial cleft cyst, mixed tumor of the salivary gland, and carcinomas and aneurysms in this region As the tumor grows (at an estimated rate of 20 cm in diameter every 5 years), it may implicate the sympathetic, glossopharyngeal, vagus, spinal accessory, and hypoglossal nerves (syndrome of the retroparotid space; see Chap 47) Hearing loss, tinnitus, and vertigo are present in some cases Tumors of the carotid body have been a source of transient ischemic attacks in 5 to 15 percent of the 600 or more reported cases One of the most interesting presentations has been with sleep apnea, particularly with bilateral tumors (see below); respiratory depression as well as lability of blood pressure are common postoperative problems Malignant transformation occurs in 5 percent of cases A similar paraganglioma of the vagus nerve has been reported; it occurs typically in the jugular or nodose ganglion but may arise anywhere along the course of the nerve These tumors may also undergo malignant transformation, metastasize, or invade the base of the skull A carotid body tumor, separate in nature from the glomus jugulare tumor, has been seen in combination with von Recklinghausen neuro bromatosis Familial cases are known, especially with bilateral carotid body tumors (about 5 percent of these tumors are bilateral) The treatment should be surgical excision with or without prior intravascular embolization; radiation therapy is not advised Pituitary Adenomas (See also page 486) Tumors arising in the anterior pituitary are of considerable interest to neurologists because they often cause visual and other symptoms related to involvement of structures bordering upon the sella turcica before an endocrine disorder becomes apparent Pituitary tumors are agelinked; they become increasingly numerous with each decade; by the 80th year, small adenomas are found in more than 20 percent of pituitary glands In some cases, an apparent stimulus to adenoma.

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agents which have been used empirically for many years in the treatment of Parkinson disease and dystonia depends on their capacity to antagonize ACh at sites within the basal ganglia and in projections from the pedunculopontine nuclei Acetylcholine appears also to act on the presynaptic membrane of striatal cells and to in uence their release of neurotransmitters, as discussed below In addition, the basal ganglia contain other biologically active substances substance P, enkephalin, cholecystokinin, somatostatin, and neuropeptide Y which may enhance or diminish the effects of other neurotransmitters, ie, they act as neuromodulators The neurons utilizing these substances and the manner in which they operate are just now being identi ed Of the catecholamines, dopamine has the most pervasive role in the function of the basal ganglia It can be said that it is among the most important in brain disease, since disturbances of dopamine signaling are essential abnormalities of several CNS disorders including Parkinsonism, schizophrenia, attention de cit disorder, and drug abuse Within the basal ganglia, the areas richest in dopamine are the substantia nigra, where it is synthesized in the nerve cell bodies of the pars compacta, and the termination of these bers in the striatum In the most simpli ed models, stimulation of the dopaminergic neurons of the substantia nigra induces a speci c response in the striatum namely, an inhibitory effect on the already low ring rate of neostriatal neurons However, the effects of dopamine have proved more dif cult to resolve, in large part because there are now ve known types of postsynaptic dopamine receptors (D1 to D5), each with its particular anatomic distribution and pharmacologic action This heterogeneity is re ected in the excitatory effect of dopamine on the small spiny neurons of the putamen and an inhibitory effect on others The ve types of dopamine receptors are found in differing concentration throughout various parts of the brain, each displaying differing af nities for dopamine itself and for various drugs and other agents (Table 4-2; see Jenner) The D1 and D2 receptors are highly represented in the striatum, D3 in the nucleus accumbens, D4 in the frontal cortex and certain limbic structures, and D5 in the hippocampus In the striatum, the effects of dopamine act as a class of D1-like (D1 and D5 subtypes) and D2-like (D2, D3, and D4 subtypes) receptors Stimulation of the D1 class stimulates adenyl cyclase, while D2 receptor binding inhibits this enzyme Whether dopamine functions in an excitatory or inhibitory manner at a particular synapse is determined by the local receptor As mentioned earlier, excitatory D1 receptors predominate on the small spiny putamenal neurons that are the origin of the direct striatopallidal output pathway, while D2 receptors mediate the inhibitory in uence of dopamine on the indirect striatopallidal output, as indicated in Fig 4-4 Some of the clinical and pharmacologic effects of dopamine are made clear by considering both the anatomic sites of various receptors and their physiologic effects For example, it appears that drug-induced parkinsonian syndromes and tardive dyskinesias (described further on) are prone to occur when drugs are administered that competitively bind to the D2 receptor, but that the newer antipsychotic drugs, which produce fewer of these effects, have a stronger af nity for the D4 receptor However, the situation is actually far more complex, in part due to the synergistic activities of D1 and D2 receptors, each potentiating the other at some sites of convergence, and the presence on the presynaptic terminals of nigrostriatal neurons of D2 receptors, which inhibit dopamine synthesis and release Even these intricacies do not capture the complexity of neural.

Normalize According to Next Group Level Result/ This option will calculate a percentage based on to Overall Results/ to Unrestricted Overall Results the characteristic value combinations Normalize according to the next group level will generate a % based on the characteristic combinations found directly adjacent to the amounts column To Overall Results will calculate the % based on the total results row of the query To Unrestricted Overall Results will calculate the % based on the total amounts discounting any filters in the report Rank Number/Olympic Rank Number This option will generate a ranking number based on the amounts for each characteristic value and display that ranking number in the amount field Olympic Ranking means that there are no ties This option will hide the values in this column either for the single values or results

Within basal ganglia Striatum LGP STN MGP/SNpr SNpc Outside basal ganglia Nucleus accumbens Frontal cortex Limbic structures Hippocampus Hypothalamus Olfactory tubercle Pituitary Brainstem Drug af nities Dopamine Bromocriptine Pergolide Ropinirole Pramipexole

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