Sneddon’s syndrome presenting with severe disabling bilateral headache

Cavestro, Cinzia; Richetta, Luca; Pedemonte, Enrico; Asteggiano, Giovanni

doi:10.1007/s10194-009-0109-3

Cited by 11 publications

(5 citation statements)

References 3 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…LR is noticed before cerebrovascular events in more than a half of patients. In some patients, the livedo is first detected at the time of stroke occurrence [ 16 , 17 ]. In rare cases, LR appears after neurological symptoms [ 18 ].…”

Section: Introductionmentioning

confidence: 99%

Sneddon’s syndrome: a comprehensive review of the literature

Xu²,

Liang³

2014

Orphanet J Rare Dis

116

View full text Add to dashboard Cite

Sneddon’s syndrome (SS) is a rare non-inflammatory thrombotic vasculopathy characterized by the combination of cerebrovascular disease with livedo racemosa(LR). The Orpha number for SS is ORPHA820. It has been estimated that the incidence of SS is 4 per 1 million per annum in general population and generally occurs in women between the ages of 20 and 42 years. LR may precede the onset of stroke by years and the trunk and/or buttocks are involved in nearly all patients. The cerebrovascular manifestations are mostly secondary to ischemia (transient ischemic attacks and cerebral infarct). Other neurological symptoms range from headache, cerebral hemorrhage, seizures, cognitive and psychiatric disturbances. The involved internal organs include heart, kidney, and eyes. Histological findings of skin are characteristic and the involved vessels are small to medium-sized arteries at the border of dermis to subcutis with a distinct histopathological time course. The main diagnostic criteria are general LR with typical histopathological findings on skin biopsy and focal neurological deficits. The pathogenesis is related to hypercoagulable state and intrinsic small-vessel vasculopathy. The optimal management remains an unsolved problem and long-term anticoagulation have been recommended for cerebral ischemic events based on the presumed pathogenesis. There are controversial results in treatment of SS with immunomodulatory agents. The aim of this review is to comprehensively discuss this disease.

show abstract

Section: Introductionmentioning

confidence: 99%

Sneddon’s syndrome: a comprehensive review of the literature

Xu²,

Liang³

2014

Orphanet J Rare Dis

116

View full text Add to dashboard Cite

show abstract

“…If livedo is detected, then the diagnosis is established. There are cases in which livedo is diagnosed after a stroke in the germane literature [11,12]. This highly uniform appearance gives us the idea that MRI can be considered a biomarker of this disease.…”

Section: Discussionmentioning

confidence: 98%

Characteristic imaging features of neurovascular involvement in primary Sneddon’s syndrome: an analysis of 12 cases

et al. 2020

View full text Add to dashboard Cite

Objective Sneddon's syndrome is a cerebrocutaneous non-inflammatory progressive distal arteriopathy, characterized by livedo racemosa, stroke, and neuropsychiatric symptoms. Our aim was to highlight the characteristic neuroimaging features of Sneddon's syndrome that might be helpful to clinicians in timely diagnosis of this entity. Methods Twelve patients (median age 49 years, 11 female) with primary Sneddon's syndrome, diagnosed in last 10 years, were analyzed from the perspective of magnetic resonance imaging (MRI) features. In addition, a novel pseudoangiomatosis score was defined for grading angiographic abnormalities (range: 0 to 6). Results Median interval from the onset of neurological symptoms to diagnosis was 6 years. Presentation was with acute stroke in 5, seizures in 3, dementia/speech problems in 2, seizures plus cognitive dysfunction in 1, and chronic progressive hemiparesis in 1. All patients had a typical lesion pattern on MRI. This included multiple (median 3) cortical-subcortical supratentorial and cerebellar non-territorial infarcts, accompanied by multifocal cerebral atrophy. Of note, large territorial infarcts due to cerebral parent artery occlusion, an embolic pattern with multi-territorial involvement on diffusion-weighted imaging, small vessel disease features like severe white matter involvement or lacunar infarcts, and cerebral hemorrhage in the absence of anticoagulation were not observed. MRI lesion severity was not correlated with angiographic arteriopathy severity, clinical stage, or presentation symptoms. Conclusion Sneddon's syndrome is characterized by highly typical clinico-radiological features. Brain MRI has diagnostic value. By knowing the characteristics of the syndrome, misdiagnosis and potentially harmful treatment can be prevented in this entity that might pose a diagnostic challenge.

show abstract

“…Headache is the most reported symptom in almost 50% of patients [ 3 , 6 ]. Two cases were reported describing the headaches experienced by these patients [ 1 , 2 ]. In our observation, headache attacks seem to be holocephalic and with no reported symptoms of (nausea, vomiting, photophobia, and phonophobia).…”

Section: Discussionmentioning

confidence: 99%

“…Sneddon syndrome is a rare, non-inflammatory, occlusive vasculopathy affecting small- and medium-sized arteries of the brain and skin [ 1 , 2 ]. Sneddon syndrome can present with stroke, transient ischemic attack, livedo reticularis, and progressive dementia [ 1 , 2 ]. Very few cases reported a headache as one of the symptoms with no clear association.…”

Section: Introductionmentioning

confidence: 99%

Hidden in the Rash: A Sneddon Syndrome Case Report

Almutawa,

Almalak,

Alotaibi

2023

Cureus

View full text Add to dashboard Cite

This clinical case report aims to highlight the unusual presentation of Sneddon syndrome with a possible association with paroxysmal hemicrania. A medical record review was performed at a tertiary hospital in Riyadh, Saudi Arabia. Data collected include clinical evaluations and laboratory and imaging results. Informed consent was obtained. Hereby, we present a 27-year-old female who presented with multiple stroke attacks, along with severe headaches involving right retro-orbital pain with an eight-year history of spotted skin lesions. Initial unenhanced computed tomography (UCT) brain in the emergency showed left insular cortex hypodensity, revealing acute ischemic insult. Subsequent magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) revealed acute ischemic infarct in the territory of the left middle cerebral artery (MCA) involving the insula and frontoparietal lobe. Further investigations were done, including cerebrospinal fluid (CSF) analysis and autoimmune and infectious workup, which were unrevealing. Skin biopsy of the lesions showed subcutaneous fat necrosis with nonspecific scattered fibrinogen positivity and was labeled as livedo reticularis vs. livedo racemosa. A Sneddon syndrome diagnosis can be very challenging, needing a high index of suspicion to direct the diagnostic investigations. Moreover, the presence of a severe headache is an unusual phenomenon that needs further study.

show abstract

Sneddon’s syndrome presenting with severe disabling bilateral headache

Cited by 11 publications

References 3 publications

Sneddon’s syndrome: a comprehensive review of the literature

Sneddon’s syndrome: a comprehensive review of the literature

Characteristic imaging features of neurovascular involvement in primary Sneddon’s syndrome: an analysis of 12 cases

Hidden in the Rash: A Sneddon Syndrome Case Report

Contact Info

Product

Resources

About