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GM2 activator Arylsulfatase A (sulfatidase), sulfatide activator (saposin B) Galactocerebrosidase -Galactosidase A Glucocerebrosidase Sphingomyelinase Cholesterol esteri cation Ceramidase -Galactosidase B

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Wernicke disease and the Korsakoff amnesic state are common neurologic disorders that have been recognized since the 1880s Wernicke disease (originally called polioencephalitis hemorrhagica superioris) is characterized by nystagmus, abducens and conjugate gaze palsies, ataxia of gait, and mental confusion These symptoms develop acutely or subacutely and may occur singly or, more often, in various combinations Wernicke disease is due speci cally to a de ciency of thiamine and is observed mainly though far from exclusively in alcoholics The Korsakoff amnesic state (Korsakoff psychosis) is a unique mental disorder in which retentive memory is impaired out of proportion to all other cognitive functions in an otherwise alert and responsive patient.

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In the past 50 years there has been delineated a group of neurologic disorders that occur in patients with carcinoma or some other type of neoplasia even though the nervous system is not the site of metastases or direct invasion or compression by the tumor. These so-called paraneoplastic disorders are not speci c or con ned to cancer, but the two conditions are linked far more frequently than could be accounted for by chance. They assume special importance because the neurologic syndrome in many cases becomes apparent before the underlying tumor is found. Some of the paraneoplastic disorders that involve nerve and muscle namely, polyneuropathy, polymyositis, and the myasthenic-myopathic syndrome of Lambert-Eaton are described on pages 1194 and 1259, respectively. Here we present several other paraneoplastic processes that involve the spinal cord, cerebellum, brainstem, and cerebral hemispheres. Comprehensive accounts of the paraneoplastic disorders may be found in the writings of Posner, Darnell and Posner, and of Dropcho. Some of these disorders are associated with IgG autoantibodies (Table 31-5), but it should be remarked that although certain antibodies are associated with speci c syndromes, they are not invariably linked to particular cancers. Furthermore, the

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This amnesic disorder, like Wernicke disease, is most often associated with alcoholism and malnutrition, but it may be a symptom of various other diseases that have their basis in lesions of the medial thalami or the inferomedial portions of the temporal lobes, such as infarction in the territory of the temporal lobe branches of the posterior cerebral arteries, hippocampal damage after cardiac arrest, third ventricular tumors, or herpes simplex encephalitis A Korsakoff type of memory disturbance may also follow lesions that involve the basal septal nuclei of the frontal lobe Transient impairments of retentive memory of the Korsakoff type may be the salient manifestations of temporal lobe epilepsy, concussive head injury, and a unique disorder known as transient global amnesia The anatomic basis of the Korsakoff amnesic syndrome is discussed further in Chap 21.

geographic poles (not likely). Earth rotates with respect to the stars, completing a full circle approximately every 23 hours and 56 minutes. In a few minutes, a star that is straight overhead will move noticeably down toward the western horizon. This effect is exaggerated when you look through a telescope. The greater the magnification, the more vividly apparent is the rotation of Earth. The next time you get a chance, set up a telescope and point it at some star that is overhead. Use the shortest focal-length eyepiece that the telescope has so that the magnification is high. Center the star in the field of view. If that star is exactly overhead, then its celestial latitude and longitude correspond to yours. For example, if you re on the shore of Lake Tahoe, your approximate latitude is 39 N and your approximate longitude is 120 W. If you have a telescope pointing straight up and a star is centered in the field of view, then that star s celestial coordinates are close to 39 N, 120 W. However, this won t be the case for long. You will be able to watch the star drift out of the field of view. Theoretically, a star stays exactly at a given celestial longitude coordinate (x, y) for an infinitely short length of time in essence, for no time at all. However, the celestial latitude of each and every star remains constant, moment after moment, hour after hour, day after day. (With the passage of centuries, the celestial latitudes of the stars change gradually because Earth s axis wobbles slowly. However, this effect doesn t change things noticeably to the average observer over the span of a lifetime.)

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