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destructive lesions with remarkable delity, but in some cases, even when the tumor is seen with various studies, it may be dif cult to obtain a satisfactory biopsy Tumors of the Foramen Magnum Tumors in the region of the foramen magnum are of particular importance because of the need to differentiate them from diseases such as multiple sclerosis, Chiari malformation, syringomyelia, and bony abnormalities of the craniocervical junction Failure to recognize these tumors is a serious matter, since the majority are benign and extramedullary, ie, potentially resectable and curable If unrecognized, they terminate fatally by causing medullary and high spinal cord compression Although these tumors are not numerous (about 1 percent of all intracranial and intraspinal tumors), sizable series have been collected by several investigators (see F B Meyer et al for a complete bibliography) In all series, meningiomas, schwannomas, neuro bromas, and dermoid cysts are the most common types; others, all rare, are teratomas, dermoids, granulomas, cavernous hemangiomas, hemangioblastomas, hemangiopericytomas, lipomas, and epidural carcinomas Pain in the suboccipital or posterior cervical region, mostly on the side of the tumor, is usually the rst and by far the most prominent complaint In some instances the pain may extend into the shoulder and even the arm The latter distribution is more frequent with tumors arising in the spinal canal and extending intracranially than the reverse For uncertain reasons, the pain may radiate down the back, even to the lower spine Both spine and root pain can be recognized, the latter due to involvement of either the C2 or C3 root or both Weakness of one shoulder and arm progressing to the ipsilateral leg and then to the opposite leg and arm ( around the clock paralysis, page 52 is a characteristic but not invariable sequence of events, caused by the encroachment of tumor upon the decussating corticospinal tracts Occasionally both upper limbs are involved alone; surprisingly, there may be atrophic weakness of the hand or forearm or even intercostal muscles with diminished tendon re exes well below the level of the tumor, an observation made originally by Oppenheim which we have been able to con rm Involvement of sensory tracts also occurs; more often it is posterior column sensibility that is impaired on one or both sides, with patterns of progression similar to those of the motor paralysis Sensation of intense cold in the neck and shoulders has been another unexpected complaint, and also bands of hyperesthesia around the neck and back of the head Segmental bibrachial sensory loss has been demonstrated in a few of the cases and a Lhermitte sign (really a symptom) of electric-like sensations down the spine and limbs upon exing the neck has been reported frequently The cranial nerve signs most frequently conjoined and indicative of intracranial extension are dysphagia, dysphonia, dysarthria, and drooping shoulder (due to vagal, hypoglossal, and spinal accessory involvement); included less often are nystagmus and episodic diplopia, sensory loss over the face and unilateral or bilateral facial weakness, and a Horner syndrome The clinical course of such lesions often extends for 2 years or longer, with deceptive and unexplained uctuations With dermoid cysts of the upper cervical region, as in the case reported by Adams and Wegner, complete and prolonged remissions from quadriparesis may occur The important diagnostic procedure is MRI (Fig 31-20) and, if this is unavailable, CT myelography Tumors of the foramen magnum should be differentiated from spinal or brainstem-cerebellar multiple sclerosis, Chiari malformation with syrinx, and chronic adhesive arachnoiditis Persistent.

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occipital neuralgia with a foramen magnum syndrome is particularly suggestive of a tumor at that site Treatment is surgical excision (see Hakuba et al) followed by focused radiation if the resection has been incomplete and the tumor is known to be radiosensitive

Once you fill in the values in the variable screen, click Save As to save the variant using the Save Variant dialog box, shown next

REMOTE EFFECTS OF NEOPLASIA ON THE NERVOUS SYSTEM (PARANEOPLASTIC DISORDERS) (Table 31-5)

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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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Opsoclonus-myoclonus-ataxia Ocular movement disorder, gait ataxia Retinal degeneration Scotomas, blindness, disc swelling Antirecoverin (Anti-CAR) Subacute sensory neuropathy and neuronopathy Lambert-Eaton myasthenic syndrome Stiff-man syndrome Chorea Optic neuropathy

Anti-Yo (anti-Purkinje cell) Ovary, fallopian tube, lung, Hodgkin disease (anti-Tr) Anti-Hu (ANNA 1) Small-cell lung, neuroblastoma, Anti-Ma prostate, breast, Hodgkin, testicular (Ma) Anti-Ri (ANNA 2) Breast, fallopian tube, small-cell lung

The Save As User Variant check box is checked by default If you leave that turned on, you will create a local variant If you deactivate that parameter, your screen will change a bit and allow you to enter a technical name for the variant, as shown next This variant will then be a global variant

Distal or proximal sensory loss Proximal fatiguing weakness, autonomic symptoms (dry mouth) Muscle spasms and rigidity Bilateral choreoathetosis Blindness

Small-cell lung, thymoma, renal cell, melanoma Anti-Hu (ANNA-1) Small-cell lung, Hodgkin, other lymphomas Anti-voltage-gated (VGCG) Small-cell lung, Hodgkin, other calcium channel lymphomas Breast Lung, Hodgkin, others Lung

Antiamphiphysin Anti-Hu, CRMP-5 Anti-CRMP-5

In most cases, a particular autoantibody is associated with a speci c tumor type rather than with the clinical syndrome (eg, small-cell lung cancer and polyneuropathy with ANNA 1, breast cancer with anti-Purkinje cell antibody, testicular tumors with anti-Ma) Clinical syndromes similar to each of these may occur with non small cell lung cancer and lymphoma, most often in the absence of detectable antibodies

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