WED 253 An atypical presentation of sneddon syndrome

A 50-year-old female was admitted following a subacute and increasing headache, numbness in both hands and feet, generalised weakness and confusion.An MRI brain revealed an acute left temporal infarct with multi-focal established infarcts. MR angiography demonstrated marked occlusive disease affecting terminal internal carotid artery and both middle cerebral and posterior cerebral arteries, in a potential Moyamoya pattern.Lumbar puncture, extensive blood tests and echocardiography were unremarkable. A skin biopsy showed intimal thickening of the deep dermal arteries compatible with a diagnosis of Sneddon Syndrome. Livedo reticularis was absent and antiphospholipid antibodies negative. Antiplatelet therapy only was commenced given her seronegativity and Moyamoya.DiscussionSneddon syndrome is an uncommon disorder, characterised as generalised livedo reticularis with stroke (Sneddon, 1965). It is an increasingly recognised cause of ischaemic stroke in young adults, however, its clinical course remains poorly defined in the literature (Boesch et al. 2003). It is increasingly associated with Moyamoya syndrome, posing a challenge in terms of anticoagulation in these patients (Fierini et al. 2015). To our knowledge, this is only the second reported case without livedo reticularis (Marianetti et al. 2011) - highlighting the importance of skin biopsy - and the first with this clinical and radiological combination.

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Focal Cerebral Arteriopathy in Young Adult Patients With Stroke

Stroke ◽

10.1161/strokeaha.119.028343 ◽

2020 ◽

Vol 51 (5) ◽

pp. 1596-1599

Author(s):

Mary Clare McKenna ◽

Noel Fanning ◽

Simon Cronin

Keyword(s):

Ischemic Stroke ◽

Carotid Artery ◽

Internal Carotid Artery ◽

Internal Carotid ◽

Arterial Ischemic Stroke ◽

Tertiary Referral Center ◽

Stroke In Young Adults ◽

Distal Internal Carotid Artery ◽

Cerebral Arteriopathy ◽

Stroke In Young

Background and Purpose— Focal cerebral arteriopathy is monophasic inflammatory stenosis of the distal internal carotid artery or the proximal segment of the middle cerebral artery. It is one of the most common causes of acute arterial ischemic stroke in young children but is a less familiar entity for adult neurologists. Methods— We retrospectively reviewed stroke service radiology records at a tertiary referral center from January 2013 to December 2014. Focal cerebral arteriopathy was defined as nonprogressive unifocal and unilateral stenosis/irregularity of the distal internal carotid artery or its proximal branches. Only patients aged 16 to 55 years with stroke were included. Results— There were 5 cases of focal cerebral arteriopathy: 2 males and 3 females. Three cases were from the cohort of 123 acute presentations of young stroke, and 2 cases were outpatient referrals. The mean age (range) was 43 (32–55) years. The majority presented with recurrent transient ischemic attacks/minor strokes within a single vascular territory over days to weeks. All cases had characteristic radiological features. Interval imaging demonstrated resolution in 1 case and improvement in 3 cases. Functional outcome was excellent with discharge modified Rankin Scale score ranging from 0 to 1. Recurrence occurred in 1 case. Conclusions— Focal cerebral arteriopathy is a rare cause of arterial ischemic stroke in young adults. Follow-up intracranial imaging is essential to differentiate from progressive arteriopathies. Evidence-based treatment warrants further investigation. Prognosis is favorable.

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Cutaneous Polyarteritis Nodosa (cPAN) in ab 5 Years Old Girl

COJ Nursing & Healthcare ◽

10.31031/cojnh.2018.02.000548 ◽

2018 ◽

Vol 2 (5) ◽

Author(s):

Lubna Nazir

Keyword(s):

Case Report ◽

Skin Biopsy ◽

Polyarteritis Nodosa ◽

Clinical Course ◽

Systemic Vasculitis ◽

Separate Entity ◽

Rare Form ◽

Cutaneous Polyarteritis Nodosa ◽

True Incidence ◽

Hands And Feet

Polyarteritis nodosa is a rare form of systemic vasculitis. Cutaneous polyarteritis nodosa (cPAN) is subtype of systemic PAN which predominantly affects the skin, extra-cutaneous findings include fever, malaise, myalgias, arthralgias, and neuropathy. The true incidence of cutaneous PAN is unknown. cPAN is recognized as a separate entity and is essentially a benign disorder which should be distinguished from systemic PAN, as the clinical course and management of the two conditions is different. In this case report, we have described a patient with fever and gangrene of both hands and feet. The patient underwent a very extensive workup in order to determine the cause of her condition, which finally turned out cPAN on skin biopsy

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De novo formation of a carotid web: case report

Journal of Neurosurgery ◽

10.3171/2018.7.jns181579 ◽

2019 ◽

Vol 131 (5) ◽

pp. 1481-1484

Author(s):

Giovanni G. Vercelli ◽

Norbert G. Campeau ◽

Thanila A. Macedo ◽

Elliot T. Dawson ◽

Giuseppe Lanzino

Keyword(s):

Carotid Artery ◽

Internal Carotid Artery ◽

Internal Carotid ◽

De Novo ◽

Filling Defect ◽

Predisposing Factor ◽

Stroke In Young Adults ◽

Lower Shear ◽

Proximal Internal Carotid Artery ◽

Stroke In Young

A carotid web is a shelf-like intraluminal filling defect typically arising from the posterolateral wall of the proximal internal carotid artery. It is recognized as a possible cause of ischemic stroke in young adults. However, its etiopathogenesis is controversial and remains to be fully elucidated. The authors report de novo formation of a carotid web from an intimal dissection documented on serial imaging studies. The findings in this case suggest that a focal intimal dissection could be the underlying cause of a carotid web. Lower shear stress at the posterolateral wall of the proximal internal carotid artery is hypothesized to be a predisposing factor and explains the predilection of a carotid web for this specific location.

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Internal Carotid Artery Dissection and Ischemic Cerebral Infarction in the Setting of Essential Thrombocythemia

Clinical and Applied Thrombosis/Hemostasis ◽

10.1177/1076029610391651 ◽

2010 ◽

Vol 17 (6) ◽

pp. E138-E140 ◽

Cited By ~ 5

Author(s):

T. Freilinger ◽

T. Saam ◽

M. Duering ◽

M. Dichgans ◽

N. Peters

Keyword(s):

Carotid Artery ◽

Internal Carotid Artery ◽

Essential Thrombocythemia ◽

Internal Carotid ◽

Carotid Artery Dissection ◽

Artery Dissection ◽

Cervical Artery Dissection ◽

Stroke In Young Adults ◽

Spontaneous Dissection ◽

Stroke In Young

Cervical artery dissection (CAD) is an important etiology of stroke in young adults. Its etiology is incompletely understood. Here, we report a young woman who presented with acute ischemic stroke in the setting of internal carotid artery (ICA) dissection and essential thrombocythemia (ET). We present a review of previous cases with comorbidity of CAD and ET and discuss the pathophysiological implications of this co-occurrence. In particular, we speculate that ET may increase the susceptibility of cervical vessels to spontaneous dissection, for example, by disturbing the microcirculation within the vessel wall.

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Moyamoya syndrome in an adult with essential thrombocythemia

Neurology International ◽

10.4081/ni.2011.e3 ◽

2011 ◽

Vol 3 (1) ◽

pp. 3 ◽

Cited By ~ 4

Author(s):

Marc Lazzaro ◽

Elizabeth J. Cochran ◽

Demetrius K. Lopes ◽

Shyam Prabhakaran

Keyword(s):

Essential Thrombocythemia ◽

Internal Carotid ◽

Cerebral Arteries ◽

Moyamoya Syndrome ◽

Cerebrovascular Disorder ◽

Hematologic Disorders ◽

Middle Cerebral Arteries ◽

History Of ◽

Progressive Occlusion ◽

Supraclinoid Internal Carotid Artery

Moyamoya syndrome is a rare cerebrovascular disorder characterized by progressive occlusion of the supraclinoid internal carotid artery and proximal portions of the anterior and middle cerebral arteries resulting in an extensive network of collateralized blood vessels and producing a characteristic angiographic appearance. Although the pathophysiology is unclear, hematologic disorders have been associated with development of the moyamoya syndrome. A case report is presented. A 29 year-old female with a history of essential thrombocythemia developed progressive ischemic strokes. Angiography revealed characteristic moyamoya changes and pathologic examination showed intimal hyperplasia with scant collagen fibers and myxoid change. This is the first reported case of moyamoya syndrome in an adult patient with essential thrombocythemia demonstrating histological findings that suggest a shared pathophysiology with moyamoya syndrome in sickle cell anemia.

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ULTRASOUND CHARACTERISTICS OF NON-OBSTRUCTIVE CAROTID ATHEROSCLEROSIS IN CRYPTOGENIC STROKE IN YOUNG ADULTS

10.26226/morressier.58e389b2d462b802923851c3 ◽

2017 ◽

Author(s):

Raphael Buon

Keyword(s):

Young Adults ◽

Carotid Atherosclerosis ◽

Cryptogenic Stroke ◽

Stroke In Young Adults ◽

Stroke In Young

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DIFFICULTIES IN DIAGNOSING MOYA-MOYA DISEASE (CLINICAL CASE)

"Medical & pharmaceutical journal "Pulse" ◽

10.26787/nydha-2686-6838-2020-22-10-48-53 ◽

2020 ◽

pp. 48-53

Author(s):

Novikova I.N. ◽

Popova T.F. ◽

Gribacheva I.A. ◽

Petrova E.V. ◽

Marushchak A.A. ◽

...

Keyword(s):

Cerebral Angiography ◽

Neurological Disorders ◽

Clinical Case ◽

Carotid Arteries ◽

Internal Carotid ◽

Cerebral Arteries ◽

Moya Moya Disease ◽

Middle Cerebral Arteries ◽

Internal Carotid Arteries ◽

Made In

Moya-Moya disease is a rare progressive chronic cer-ebrovascular disease characterized by a narrowing of the lumen of the intracranial segments of the internal carotid arteries, as well as the initial segments of the anterior and middle cerebral arteries with the devel-opment of a network of small vascular anastomoses. Violations of blood supply due to occlusion lead to the development of ischemic strokes in the correspond-ing pools, and ruptures of vascular anastomoses - to the development of hemorrhagic strokes, causing a variety of neurological disorders. The article presents a clinical case of Moya-Moya disease in a 31-year-old patient. The disease was manifested by acute disorders of cerebral circulation in ischemic and hemorrhagic types. The diagnosis was made in accordance with the diagnostic criteria of the disease based on the data of endovascular cerebral angiography.

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Epidemiology and characteristics of ischaemic stroke in young adults in Aragon

Neurología (English Edition) ◽

10.1016/j.nrleng.2019.05.009 ◽

2021 ◽

Author(s):

H. Tejada Meza ◽

J. Artal Roy ◽

C. Pérez Lázaro ◽

M. Bestué Cardiel ◽

O. Alberti González ◽

...

Keyword(s):

Young Adults ◽

Ischaemic Stroke ◽

Stroke In Young Adults ◽

Stroke In Young

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Prevalence and outcome of young stroke patients with middle cerebral artery stenosis

BMC Neurology ◽

10.1186/s12883-021-02125-8 ◽

2021 ◽

Vol 21 (1) ◽

Author(s):

Wenjuan Xu ◽

Xiaoyu Zhang ◽

Huan Chen ◽

Zhangning Zhao ◽

Meijia Zhu

Keyword(s):

Risk Factors ◽

Logistic Regression ◽

Risk Factor ◽

Factor Score ◽

Unfavorable Outcome ◽

Stroke Patients ◽

Young Stroke ◽

Stroke In Young Adults ◽

Risk Factor Score ◽

Stroke In Young

Abstract Background Etiologies of acute ischemic stroke in young adults are heterogeneous. Middle cerebral artery (MCA) stenosis is a common finding in Asians which may be an important cause of stroke in young adults. However, studies of stroke in young Asian populations are rare. Our study was to investigate the prevalence and outcome of young stroke patients with MCA stenosis in Chinese populations. Methods Young patients with MCA territory infarction between January 2013 and September 2018 were retrospectively recruited. Subjects were defined as stenosis group (MCA stenosis ≥50%) and no-stenosis group (MCA stenosis<50% or no stenosis) by their MCA stenosis. For patients in stenosis group, they were categorized as uni-MCA stenosis subgroup and multiple stenosis subgroup. Demographic data, risk factors, imaging feature and complications were compared between groups. Prevalence of MCA stenosis and risk factor score (score ≥ 2 or 3) in different age groups were investigated. Modified Rankin Scale (mRS) was used for evaluating functional outcome at discharge (unfavorable outcome: 3–6). Binary logistic regression was performed to determine independent risk factors of unfavorable outcome. Results Two hundred forty-nine young stroke patients were included in our study and 110 (44.2%) patients were defined as stenosis group. 55 (50%) patients were categorized as uni-MCA stenosis subgroup and 55 (50%) were multiple stenosis subgroup. The most common traditional vascular risk factors included hypertension, hyperlipemia, smoking, hyperhomocysteinemia and alcohol consumption. Prevalence of risk factor score ≥ 2 or 3 increased with age, but not incidence of MCA stenosis. By TOAST classification, the most common etiologies were large-artery atherosclerosis (41.0%) and small vessel disease (33.7%). Compared with no-stenosis group, patients in stenosis group were more likely to have large territorial infarct, develop complications and have unfavorable outcome. No significant difference was found between patients in uni-MCA stenosis and multiple stenosis subgroups except history of stroke/TIA, risk factor score ≥ 3 and silent infarct. By logistic regression, hypertension (OR = 3.561; 95%CI, 1.494 to 8.492; p = 0.004), NIHSS scores at admission (OR = 1.438; 95%CI, 1.276 to 1.620; p = 0,000) and infarct size (p = 0.015) independently predicted unfavorable outcome. Conclusions Forty-four point two percent young Chinese adults with MCA territory infarction had MCA stenosis. Prevalence of MCA stenosis did not increase with age. Patients with MCA stenosis had worse clinical outcome, however, only hypertension, NIHSS scores at admission and infarct size were independent predictors.

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