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FIGURE 1A: magnification

Left brachial arteriogram, with views of the left hand demon-

strates treme and no

incomplete proximal palmar arch, ex- narrowing of the interosseus branches opacification of the interdigital arteries.

pathectomy and inferior vena cava clipping because of recurrent thrombophlebitis.

Her past history revealed no evidence of lipid ab- normalities, diabetes mellitus or migraine. She had smoked 10 cigarettes daily for many years. There was no history of intake of oral contraceptives, amphetamines or ergot derivatives.

On admission, she was unable to communicate orally, swallow or chew. Her mouth was half opened, and she had minimal voluntary control of her lips, tongue and jaw. There was bilateral facial paresis more severe on the the right side. The soft palate was immobile. No pathological reflexes other than bilateral hyperreflexia were found. Her neurovascular examination was normal. An awake electro- encephalogram was reported as normal. The patient also had normal electrocardiogram, echocardiogram, and chest roentgenogram. Extensive blood and CSF tests were uninformative. Doppler low velocity studies demonstrated decreased low signals through most of the digital arteries and right posterior tibial artery. Radionuclide brain scan and CT scan showed an area of infarction in the left fronto-opercular area. On CT scan there also was a poorly marginated low density area in the right parieto-frontal region. Aor-

tocranial arteriography showed exaggerated tapering of the proximal segments of both middle cerebral artery branches, predominantly at the level of the frontal opercula (fig. 1C-D). The aortic arch and brachiocephalic vessels were unremarkable. Two months later she improved with increased strength in her facial and masticatory muscles, but swallowing difficulties remained essentially unchanged.


The patient had recurrent thrombophlebitis before onset of neurologic dysfunction and digital gangrene. She also had numerous paroxysmal episodes of digital ischemia consistent with Raynaud's syndrome. She did not have evidence of cardiopulmonary, renal, hematologic, gastroenterologic, or collagen vascular disorders. Hyperlipoproteinemia with premature atherosclerosis, as well as intracranial infection and drug abuse, were excluded. There was no evidence of cardiac and extracardiac sources of emboli such as en- docarditis, valvular heart disease, atrial myxoma, or paradoxical embolism arising from leg veins. Histologic examination of vessels from her amputated fingers and toes showed neither vasculitis, such as periarteritis nodosa, nor atherosclerotic plaques.


FIGURE IB: A slightly oblique section of a small muscular artery showing occlusion of lumen. Occasional lymphocytes are present in hyperplastic intima. H&E; Mag X 280.

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