Cases reported "Cerebral Infarction"

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1/192. Localized scleroderma associated with progressing ischemic stroke.

    We present a 73 year-old Japanese woman with localized scleroderma involving the right side of the scalp accompanied by continuous tingling pain, who developed insidiously progressive left hemiparesis. In magnetic resonance imaging of the brain, an infarct first appeared in the watershed region of the right middle cerebral artery territory and subsequently extended to deep white matter accompanied by scattered hemorrhages. Focal stenosis in the M2 portion of the right middle cerebral artery was revealed on magnetic resonance angiography, and the distal vessels were only shown faintly. A biopsy specimen from the sclerotic scalp lesion showed obvious thickening of vessel walls and mild mononuclear cell infiltration. We believe that the progressing ischemic stroke was caused by hemodynamic disturbances from localized sclerotic obstruction of the middle cerebral artery, with an autoimmune pathogenesis.
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2/192. Vertebrobasilar artery territory infarction as an initial manifestation of systemic lupus erythematosus.

    cerebral infarction is a well-documented complication of systemic lupus erythematosus (SLE), that usually occurs several years after the diagnosis of SLE. To our knowledge, however, strokes associated with vertebrobasilar artery involvement were not reported to present as an initial manifestation of SLE. We report two patients, who presented with vertebrobasilar territory infarction as an initial manifestation of SLE. Patient 1 was a 16-year-old girl, who developed dysarthria and ataxia. MRI showed multiple infarcts in the pons, cerebellum and thalamus. Four-vessel cerebral angiography showed multifocal stenoses in the vertebral and basilar arteries with beaded appearance. Patient 2 was a 26-year-old woman, who developed headache associated with dysarthria, dizziness and ataxia. MRI showed multiple infarcts in the cerebellum, medulla, pons, midbrain and thalamus. cerebral angiography revealed occlusion of both vertebral arteries at the first cervical vertebral level with non-visualization of the basilar artery. Both patients were diagnosed as having SLE supported by laboratory results. Although rare, posterior circulation stroke can present as an initial manifestation of SLE, which may be attributed to vasculitis or dissection in the vertebral/basilar artery.
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3/192. Cryptic mucor infection leading to massive cerebral infarction at initiation of antileukemic chemotherapy.

    A 74-year-old man with newly diagnosed acute myelogenous leukemia unexpectedly suffered a massive cerebral infarct on day 2 of induction chemotherapy. Clinically, the hemorrhagic infarct was thought to be due to leukostasis and thrombocytopenia. Necropsy, however, revealed that Zygomycetes-type hyphae had infiltrated cerebral vessels in and near the infarct. The fungal infection was clinically silent otherwise, although fungal elements were also identified in the lung at autopsy. This case illustrates how closely fungal infection may resemble a leukemia-associated cerebrovascular accident.
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4/192. migraine with aura-like headache associated with moyamoya disease.

    A 49-year-old woman was admitted to our hospital because of severe headache. She had a 10-month history of migraine with aura-like headache that occurred every 7 to 10 days and was preceded by photopsia. Brain CT showed cerebral infarction of the left occipital lobe. Bilateral carotid angiograms showed vascular occlusions in the supraclinoid portion of the bilateral internal carotid arteries with telangiectatic vessels acting as collateral channels to the occluded distal carotid arteries, which were consistent with the diagnosis of moyamoya disease. headache resolved gradually and has never developed again after the infarct of the left occipital lobe. Pathophysiological mechanisms of migraine-like headache were discussed. We conclude that borderline perfusion of occipital lobe cortex could be a trigger for the development of migraine with aura-like headache in susceptible patients. In the case of atypical attack of migraine detailed investigation should be done to detect underlying vascular diseases such as moyamoya disease.
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5/192. Aicardi-Goutieres syndrome: a genetic microangiopathy?

    Aicardi-Goutieres syndrome (AGS) (McKusick 225750) is an autosomal recessive disease with onset in the 1st year of life, resulting in progressive microcephaly, calcification of cerebral white matter, thalamus and basal ganglia, generalized cerebral demyelination and a chronic low-grade CSF lymphocytosis, without evidence of infection. We report the autopsy of a patient who died with this disorder at the age of 17 years. Findings were severe microencephaly, diffuse but inhomogeneous cerebral white matter loss with associated astrocytosis, calcific deposits in the white matter, thalami and basal ganglia. neocortex and cerebellar cortex were affected by wedge-shaped microinfarctions. Small vessels showed calcification in the media, adventitia and perivascular spaces. These findings are similar to some previous publications that in retrospect may have been AGS, but this is the first reported cerebral microangiopathy in which the diagnosis AGS was made during lifetime. This report provides evidence that microangiopathy plays a significant role in the pathogenesis of AGS.
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6/192. Failure of a saphenous vein extracranial-intracranial bypass graft to protect against bilateral middle cerebral artery ischemia after carotid artery occlusion: case report.

    OBJECTIVE AND IMPORTANCE: We present the case of a patient who experienced bilateral middle cerebral artery infarctions after Hunterian ligation and trapping of a ruptured right cavernous aneurysm, despite a high-flow extracranial-intracranial bypass. This is a rare complication, and it highlights the need for further refinements in our understanding of the hemodynamic insufficiency created by major vessel sacrifice. CLINICAL PRESENTATION: The patient was a 59-year-old woman who experienced multiple episodes of massive epistaxis before undergoing angiography, which revealed left internal carotid artery occlusion and an irregular right cavernous aneurysm. The patient was then transferred to our center for treatment. The patient was neurologically intact at presentation, and her epistaxis was controlled by nasal packing. INTERVENTION: The patient underwent an extracranial-intracranial bypass from the external carotid artery to the M2 segment of the right middle cerebral artery, followed by trapping of the aneurysm. Despite evidence of graft patency, the patient experienced bilateral middle cerebral artery distribution infarctions after surgery. CONCLUSION: Although extracranial-intracranial bypasses protect the majority of patients who undergo carotid artery ligation from ischemic complications, this case demonstrates that hemodynamic insufficiency can occur even with a high-flow saphenous vein graft. Better ways to quantitate the hemodynamic needs of the brain after major vessel sacrifice may facilitate matching of the revascularization strategy to the specific needs of each patient, thus further reducing the likelihood of ischemic complications.
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7/192. Vasculopathic changes of cadasil can be focal in skin biopsies.

    Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (cadasil) is a newly described cause of vascular dementia. Pathologic examination shows multiple small infarcts in the deep cerebral white matter together with a nonatherosclerotic, nonamyloid angiopathy involving the media of small cerebral arteries. Ultrastructurally, characteristic granular material is present in the basal lamina of vascular smooth muscle cells in cerebral and extracerebral blood vessels. The ultrastructural changes have also been demonstrated in skin biopsies of affected patients; consequently, some investigators have recently recommended skin biopsies for the diagnosis of cadasil. This study describes a 54-year-old male with a family history for strokes who had clinical and radiological features suggestive of cadasil. A skin biopsy was performed to confirm the diagnosis. Initially, the characteristic vasculopathic changes of cadasil were not identified within small blood vessel walls. However, multiple deeper sections in other areas showed electron-dense material associated with vascular smooth muscle cells, characteristic of cadasil. Subsequent genetic testing demonstrated a single nucleotide substitution at position 659 on chromosome 19p13.1 causing an amino-acid change (Cys --> Phe), a finding indicative of cadasil. The involvement of blood vessels within the dermis makes skin biopsy a useful adjunct in the diagnosis of cadasil. However, as illustrated by this case, the findings may be focal, requiring a thorough evaluation of the entire biopsy specimen.
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8/192. Intracranial deployment of coronary stents for symptomatic atherosclerotic disease.

    Intracranial percutaneous transluminal balloon angioplasty (PTA) has been used as a technique of last resort in the treatment of intracranial atherosclerotic disease when medical and surgical alternatives have failed or cannot be applied. The major risks associated with PTA include intracranial vessel rupture and abrupt vessel dissection causing occlusion. angioplasty techniques in the extracranial circulation have been improved by the development of safe stent technology in combination with potent antiplatelet agents. We report three successful cases of symptomatic intracranial atherosclerotic disease in middle-aged adults treated by endovascular PTA followed by deployment of coronary stents.
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9/192. Vertebral and carotid artery dissection following chiropractic cervical manipulation.

    A 50-year-old woman presented a sudden left occipital headache and a posterior circulation stroke after cervical manipulation for neck pain. Magnetic resonance imaging documented a left intracranial vertebral artery occlusive dissection associated with an ipsilateral internal carotid artery dissection with vessel stenosis in its prepetrous tract. This is the first reported case showing an associate vertebral and carotid artery dissection following cervical manipulation. Carotid dissection was asymptomatic and, therefore, its incidence may be underestimated. We emphasize that cervical manipulation should be performed only in patients without predisposing factors for artery dissection and after an appropriate diagnosis of neck pain.
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10/192. diffusion-weighted echo planar imaging of acute brain infarction: a preliminary report.

    The clinical usefulness of diffusion-weighted echo planar imaging (DW-EPI) was studied in 55 patients with acute brain ischemia. Ischemic lesions were identified on DW-EPI as hyperintense regions in all patients before changes were detected by conventional magnetic resonance imaging techniques in 12 cases studied earlier than 6 hours after onset. The earliest case was verified on DW-EPI at 50 minutes after onset. The ultra-fast imaging technique took less than 2 minutes to perform even for restless patients. Three patients had cardioembolic middle cerebral artery occlusion, and emergent percutaneous transluminal recanalization was carried out. Chronological changes in the signal of brain ischemia on DW-EPI depended on the site and size of the lesion, lacunar infarct of basal ganglia, and/or massive infarct due to major vessel occlusion, and were affected by associated hemorrhagic events. Coronal DW-EPI could more easily demarcate ischemia in the brainstem and/or cerebellum than axial scans when susceptibility artifacts were present. Coronal scans also demonstrated the site and direction of the pyramidal tract and its anatomical correlation with the lesions. DW-EPI has potential for the diagnostic and therapeutic planning of patients with acute brain ischemia.
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