PART 4 in Microsoft Office

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PART 4
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MAJOR CATEGORIES OF NEUROLOGIC DISEASE
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erythema nodosum, thrombophlebitis, polyarthritis, ulcerative colitis, and a number of neurologic manifestations, some of them encephalitic or meningitic in nature The most reliable diagnostic criteria, according to the International Study Group that assembled data on 914 cases from 12 medical centers in 7 countries, were recurrent aphthous or herpetiform oral ulceration, recurrent genital ulceration, anterior or posterior uveitis, cells in the vitreous or retinal vasculitis, and erythema nodosum or papulopustular lesions The nervous system is affected in about 30 percent of patients with Behcet disease (Chajek and Fainaru); the manifestations are: recurrent meningoencephalitis, cranial nerve (particularly abducens) palsies, cerebellar ataxia, and corticospinal tract signs There may be episodes of diencephalic and brainstem dysfunction resembling minor strokes A few postmortem examinations have related these small foci of necrosis to a vasculitis, including perivascular and meningeal in ltration of lymphocytes There may also be cerebral venous thrombosis The neurologic symptoms usually have an abrupt onset and are accompanied by a brisk spinal uid pleocytosis (lymphocytes or neutrophils may predominate), along with elevated protein but normal glucose values (in one of our patients, 3000 neutrophils per cubic millimeter were found at the onset of an acute meningitis) As a rule, neurologic symptoms clear completely in several weeks, but they have a tendency to recur, and some patients are left with persistent neurologic de cits Rarely, the clinical picture is that of a progressive confusional state or dementia (see the reviews of Alema and of Lehner and Barnes for detailed accounts) The cause of Behcet disease is unknown We have been unable to detect virus particles in the margins of an ulcerative mouth lesion A pathergy skin test the formation of a sterile pustule at the site of a needle prick is listed as an important diagnostic test by the International Study Group, but on the basis of admittedly limited US expeirence, we and our colleagues have found it to be of questionable value Administration of corticosteroids has been the usual treatment, on the assumption of an autoimmune etiology Since the episodes of disease naturally subside and recur, evaluation of treatment is dif cult
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polycythemia and thrombocythemia; and paroxysmal nocturnal hemoglobinuria The study by Martinelli and colleagues, mentioned earlier in the chapter, attributed 35 percent of cases of cerebral vein thrombosis to a mutation in the factor V or the prothrombin gene Averback, who reported seven cases of venous thrombosis in young adults, has emphasized the diversity of the clinical causes Two of his patients had carcinoma of the breast and one had ulcerative colitis A few cases will follow head injury or remain unexplained A stroke in a patient suffering from any one of these systemic conditions should suggest venous thrombosis, though in some instances eg, postpartum strokes arteries are occluded as often as veins The somewhat slower evolution of the clinical stroke syndrome, the presence of multiple cerebral lesions not in typical arterial territories, and its greater epileptogenic and hemorrhagic tendency favor venous over arterial thrombosis Super cial Thrombosis of Cortical Veins Certain syndromes occur with suf cient regularity that they suggest thrombosis of a particular vein or sinus The signature features of isolated thrombosis of super cial cortical veins are the presence of large super cial (cortex and subjacent white matter) hemorrhagic infarctions and a marked tendency to partial seizures Hemiparesis, incomplete hemianopia, and aphasia, any of which may uctuate over days, are also characteristic according to Jacobs and colleagues These variable syndromes re ect the inconstant location of the main surface veins Thrombosis of the vein of Labbe causes infarction of the underlying superior temporal lobe, and occlusion of the vein of Trolard implicates the parietal cortex Quite often, in our experience, the focal de cit worsens immediately after a partial seizure The intracranial pressure is not elevated, as it is when the dural venous sinuses are occluded The diagnosis is now made by careful examination of the MR venogram or by the venous phase of the conventional angiogram Cortical vein thrombosis should be suspected in the situation of multiple hemorrhagic infarctions in one hemisphere without a source of embolism or atherothrombosis Dural Sinus Thrombosis Sagittal and Lateral Sinus Thrombosis In the case of sagittal sinus thrombosis, intracranial hypertension with headache, vomiting, and papilledema may constitute the entire syndrome (this is the main consideration in the differential diagnosis of pseudotumor cerebri, Chap 30) or it may be conjoined with hemorrhagic infarction Paraparesis, hemiparesis, uctuating unilateral or bilateral sensory symptoms, or aphasia result only if the thrombosis propagates to surface veins Focal or odd sensory or motor seizures occur on the same basis but are not as common as with cortical vein thrombosis The common radiologic picture that results from occlusion of the superior sagittal sinus is of bilateral super cial paramedian parietal or frontal hemorrhagic infarction or edemetous venous congestion In the case of CT scan with contrast infusion, a lack of dye opaci cation in the posterior sagittal sinus can be observed with careful adjustment of the viewing window ( empty delta sign ) The spinal uid pressure is increased and the uid may be slightly sanguinous Lateral sinus thrombosis causes hemorrhagic infarction of the temporal lobe convexity, usually with considerable vasogenic edema The enhanced CT scan, arteriography (venous phase), and MR venography greatly facilitate diagnosis by directly visualizing the venous occlusion Once a venous thrombosis be-
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