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Camille Frances - One of the best experts on this subject based on the ideXlab platform.
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strokes in Sneddon Syndrome without antiphospholipid antibodies
Annals of Neurology, 2015Co-Authors: L Bottin, Camille Frances, Dominique De Zuttere, Pierreyves Boelle, I P Muresan, Sonia AlamowitchAbstract:Objective Sneddon Syndrome (SS) is characterized by the association of a livedo reticularis with stroke. Clinicoradiological features of its neurological manifestations, its prognosis, and the frequency of associated cardiac valvulopathy remain poorly known, particularly in the absence of antiphospholipid antibodies (APL). The objectives were to assess the clinicoradiological pattern of SS without APL (SSAPL−) and its midterm prognosis. Methods Clinical data, transthoracic echocardiograms, and brain imaging of 53 consecutive patients (83% women) with SSAPL−, followed up at our institution between 1991 and 2011, were reviewed. Results Seventy-four strokes were reported; 76% were ischemic strokes (IS), 15% transient ischemic attacks, and 9% hemorrhagic strokes. Heart valve lesions were found in 50% of the cases. Brain imaging showed 177 IS of 3 different types: large territorial (43%), small distal corticosubcortical (14%), and small deep (23%) IS. No significant association was found between the valve involvement and the presence of territorial IS. After a mean follow-up of 7.4 years, 82% of patients had a modified Rankin Scale score ≤ 2. The ischemic event recurrence rate was 20%, with a similar annual rate in the antiplatelet group (3%) compared to the anticoagulation group (2.7%). Interpretation SSAPL− is not only a neurocutaneous disorder, but is frequently associated with heart valve involvement. The latter does not influence the IS type, which suggests that strokes are caused by vasculopathy of the small and medium-size cerebral arteries. Our results show no progression toward a serious disability in the majority of the cases and a moderate recurrence rate under antiplatelet therapy. Ann Neurol 2015;77:817–829
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Sneddon Syndrome without antiphospholipid antibodies in 55 cases clinical radiological and pathophysiological features s43 005
Neurology, 2012Co-Authors: L Bottin, Camille Frances, Dominique De Zuttere, Pierreyves Boelle, I P Muresan, Sonia AlamowitchAbstract:Objective: To study the pathophysiological mechanisms of ischemic strokes (IS) and the prognosis of a cohort of patients with a Sneddon Syndrome without antiphospholipid antibodies (SSAPL-). Background SS is characterized by the association of a livedo reticularis (LR) with strokes. The clinical and radiological features of the neurological manifestations, its prognosis and the frequency of associated cardiac injury remain poorly known, particularly in the absence of APL. Design/Methods: Review of clinical data and brain imaging in 55 consecutive patients (83% women) with SSAPL-, followed up at our institution between 1991 and 2011. Results: LR was first noticed at a mean age of 32 years and the first stroke at 43; 53% had hypertension. Seventy-four strokes were reported, including 76% of IS, 15% of TIA and 9% of hemorrhagic strokes. A mitral or aortic valvulopathy was found in 52% of the 42 patients with an echocardiography. Brain imaging showed 177 IS of 3 different types: large territorial (43%), small distal cortical subcortical (14%) and lacunar (23%) IS; 70% of the IS were located in the superficial territory of the middle cerebral artery. No significant link was found between the valvulopathy and the presence of territorial IS. Other brain abnormalities were frequent: cortical and subcortical atrophy (>90%) and slight leucopathy. After a mean follow-up of 7.4 years, 82% of patients had a modified Rankin score ≤2. Global IS recurrence rate was 12% with a similar annual rate in the antiplatelet group (1.6%) compared to the anticoagulation group (1.5%). Conclusions: SSAPL- is not only a neuro-cutaneous disorder, frequently involving a valvulopathy. This one does not influence the IS type suggesting IS being caused by the small and medium cerebral arteries vasculopathy. Our results show no progression to a serious handicap in the majority of cases and a small recurrence rate under antiplatelet therapy. Disclosure: Dr. Bottin has nothing to disclose. Dr. Frances has nothing to disclose. Dr. De Zuttere has nothing to disclose. Dr. Boelle has nothing to disclose. Dr. Muresan has nothing to disclose. Dr. Alamowitch has nothing to disclose.
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the mystery of Sneddon Syndrome relationship with antiphospholipid Syndrome and systemic lupus erythematosus
Journal of Autoimmunity, 2000Co-Authors: Camille Frances, Jeancharles PietteAbstract:Abstract Since its description in 1965, Sneddon Syndrome (SNS) is usually characterized by the association of an ischemic cerebrovascular disease and a widespread livedo reticularis. The presence of many other manifestations suggests that it is a systemic Syndrome. The prevalence of anti-phospholipid antibodies (aPL) is highly variable, 41% in our experience. Comparison of patients with or without aPL showed that the fishnet of the livedo was clearly larger in aPL-negative patients who nevertheless, did not develop thrombocytopenia. Seizures and clinically audible mitral regurgitation were more frequently observed in aPL-positive patients. These data lead to consider that SNS is not a unique entity. As patients with primary anti-phospholipid Syndrome (APS) and SNS did not differ from those with livedo reticularis, ischemic cerebral events and APS within systemic lupus erythematosus (SLE), there is no reason today to exclude patients with SLE. On one hand, SNS might cover a continuum spectrum joining diverse clinico-biological entities ranging from aPL-negative to SLE-related cases, with primary APS-SNS standing amidst. On the other hand, one might speculate that SNS should be regarded as a nearly similar clinical expression of two distinct disorders, i.e. a peculiar form of APS characterized by preferential arteriolar involvement or on the opposite a primary non-aPL related small artery disease mainly involving brain and skin vessels.
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Sneddon Syndrome with or without antiphospholipid antibodies a comparative study in 46 patients
Medicine, 1999Co-Authors: Camille Frances, T Papo, B Wechsler, J L Laporte, Valerie Biousse, Jeancharles PietteAbstract:: Sneddon Syndrome is characterized by the association of livedo reticularis and cerebral ischemic arterial events (stroke or transient ischemic attack). Reported prevalence of antiphospholipid antibodies is highly variable. We conducted this study to compare the clinical and pathologic features of patients with Sneddon Syndrome according to the presence or absence of antiphospholipid antibodies. Forty-six consecutive patients with Sneddon Syndrome were analyzed. All were examined by the same dermatologist who classified the livedo of the trunk according to the regularity of the fishnet reticular pattern and according to the thickness of the fishnet reticular pattern (> or = 10 mm = large; < 10 mm = fine). Skin biopsies were systematically performed, from both the center and the violaceous netlike pattern in 38 patients. Antiphospholipid antibodies-positive Sneddon Syndrome was defined by the presence of lupus anticoagulant or abnormal titers of anticardiolipin antibodies on repeated determinations. Group I consisted of 27 antiphospholipid antibodies-negative patients and Group II, of 19 antiphospholipid antibodies-positive patients. All patients except I in Group II had irregular livedo reticularis. Large livedo racemosa was more frequently observed in Group I (89%) than in Group II (21%, p < 0.001). On skin biopsy, arteriolar obstruction was detected in only 8 patients (4 in each group). The following parameters were not statistically different between the 2 groups: gender, mean age at detection of livedo, mean age at first clinical cerebral event, hypertension, Raynaud phenomenon, patients with extracerebral and extracutaneous arterial or arteriolar thrombosis or stenosis, patients with venous thrombosis, and women with 2 fetal losses or more. In contrast, seizures (11% in Group I versus 37% in Group II, p < 0.05), mitral regurgitation on echocardiogram (19% versus 53%, p = 0.02), and thrombocytopenia < 150,000/muL (0% versus 42%, p < 0.005) were more frequently observed in Group II. The number of events per year of follow-up was lower with antiplatelet therapy (0.08 versus 0.5) in Group I, but was not different with anticoagulation (0.056 versus 0.06). Antiphospholipid antibodies-negative and -positive patients with Sneddon Syndrome belong to close but different subsets of Sneddon Syndrome.
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the natural course of cerebral lesions in Sneddon Syndrome
JAMA Neurology, 1997Co-Authors: Ayman Tourbah, Jeancharles Piette, Marie T Ibazizen, O Lyoncaen, P Godeau, Camille FrancesAbstract:Objectives: To characterize the clinical, biological, and neuroradiological findings in Sneddon Syndrome; to correlate magnetic resonance imaging abnormalities with disability, presence of hypertension and other vascular risk factors, presence of heart valvulopathy on echography, and titer of antiphospholipid antibodies; and to compare these findings in antiphospholipid-positive and antiphospholipid-negative patients. Design: Retrospective review of the records of 32 consecutive patients with livedo reticularis and neurological events, followed up in our institution between January 1991 and August 1995. Patients: Twenty-six patients (20 women and 6 men) who had at least 1 cerebral ischemic arterial event associated with generalized and pathological livedo reticularis. Results: The age at the first cerebral ischemic event ranged from 22 to 58 years. Motor deficit was the most frequent sign (found in 73% of cases). Disability was found in 50%, systemic hypertension in 65%, heart valvulopathy in 61%, and antiphospholipid antibodies in 42% of cases. Patients were classified in 6 groups according to magnetic resonance imaging findings. No correlation was found between the presence of hypertension or other vascular risk factors, valvulopathy, antiphospholipids, and magnetic resonance imaging abnormalities. There was no significant difference between antiphospholipid-positive and antiphospholipid-negative patients except for the presence of antinuclear antibodies. There was a significant correlation between the extent of magnetic resonance imaging abnormalities and disability. Conclusion: The severity of the disease seems to be correlated with magnetic resonance imaging aspects, but not to the presence of antiphospholipid antibodies. Magnetic resonance imaging may help to understand the natural course of the cerebral involvement of the disease.
Veronica C M Kuckkoot - One of the best experts on this subject based on the ideXlab platform.
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Sneddon Syndrome and the diagnostic value of skin biopsies three young patients with intracerebral lesions and livedo racemosa
European Journal of Dermatology, 2008Co-Authors: Catharina M Legierse, Marijke Canningavan R Dijk, C A F M Bruijnzeelkoomen, Veronica C M KuckkootAbstract:Sneddon Syndrome is a rare disorder characterised by generalised livedo racemosa of the skin with extracutaneous neurological symptoms like headache, vertigo, transient ischaemic attacks (TIA), stroke, and seizures. Diagnosis of Sneddon Syndrome is based on these clinical features and positive findings in skin biopsies, namely the histological proof of occlusion of arterioles by intimal proliferation. We describe three cases of young patients with clinical characteristics of Sneddon Syndrome, but in only two cases could this diagnosis be confirmed by skin biopsies. These cases stress the difficulty of diagnosing Sneddon Syndrome and show the additive value of skin biopsies in this process.
Jeancharles Piette - One of the best experts on this subject based on the ideXlab platform.
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the mystery of Sneddon Syndrome relationship with antiphospholipid Syndrome and systemic lupus erythematosus
Journal of Autoimmunity, 2000Co-Authors: Camille Frances, Jeancharles PietteAbstract:Abstract Since its description in 1965, Sneddon Syndrome (SNS) is usually characterized by the association of an ischemic cerebrovascular disease and a widespread livedo reticularis. The presence of many other manifestations suggests that it is a systemic Syndrome. The prevalence of anti-phospholipid antibodies (aPL) is highly variable, 41% in our experience. Comparison of patients with or without aPL showed that the fishnet of the livedo was clearly larger in aPL-negative patients who nevertheless, did not develop thrombocytopenia. Seizures and clinically audible mitral regurgitation were more frequently observed in aPL-positive patients. These data lead to consider that SNS is not a unique entity. As patients with primary anti-phospholipid Syndrome (APS) and SNS did not differ from those with livedo reticularis, ischemic cerebral events and APS within systemic lupus erythematosus (SLE), there is no reason today to exclude patients with SLE. On one hand, SNS might cover a continuum spectrum joining diverse clinico-biological entities ranging from aPL-negative to SLE-related cases, with primary APS-SNS standing amidst. On the other hand, one might speculate that SNS should be regarded as a nearly similar clinical expression of two distinct disorders, i.e. a peculiar form of APS characterized by preferential arteriolar involvement or on the opposite a primary non-aPL related small artery disease mainly involving brain and skin vessels.
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Sneddon Syndrome with or without antiphospholipid antibodies a comparative study in 46 patients
Medicine, 1999Co-Authors: Camille Frances, T Papo, B Wechsler, J L Laporte, Valerie Biousse, Jeancharles PietteAbstract:: Sneddon Syndrome is characterized by the association of livedo reticularis and cerebral ischemic arterial events (stroke or transient ischemic attack). Reported prevalence of antiphospholipid antibodies is highly variable. We conducted this study to compare the clinical and pathologic features of patients with Sneddon Syndrome according to the presence or absence of antiphospholipid antibodies. Forty-six consecutive patients with Sneddon Syndrome were analyzed. All were examined by the same dermatologist who classified the livedo of the trunk according to the regularity of the fishnet reticular pattern and according to the thickness of the fishnet reticular pattern (> or = 10 mm = large; < 10 mm = fine). Skin biopsies were systematically performed, from both the center and the violaceous netlike pattern in 38 patients. Antiphospholipid antibodies-positive Sneddon Syndrome was defined by the presence of lupus anticoagulant or abnormal titers of anticardiolipin antibodies on repeated determinations. Group I consisted of 27 antiphospholipid antibodies-negative patients and Group II, of 19 antiphospholipid antibodies-positive patients. All patients except I in Group II had irregular livedo reticularis. Large livedo racemosa was more frequently observed in Group I (89%) than in Group II (21%, p < 0.001). On skin biopsy, arteriolar obstruction was detected in only 8 patients (4 in each group). The following parameters were not statistically different between the 2 groups: gender, mean age at detection of livedo, mean age at first clinical cerebral event, hypertension, Raynaud phenomenon, patients with extracerebral and extracutaneous arterial or arteriolar thrombosis or stenosis, patients with venous thrombosis, and women with 2 fetal losses or more. In contrast, seizures (11% in Group I versus 37% in Group II, p < 0.05), mitral regurgitation on echocardiogram (19% versus 53%, p = 0.02), and thrombocytopenia < 150,000/muL (0% versus 42%, p < 0.005) were more frequently observed in Group II. The number of events per year of follow-up was lower with antiplatelet therapy (0.08 versus 0.5) in Group I, but was not different with anticoagulation (0.056 versus 0.06). Antiphospholipid antibodies-negative and -positive patients with Sneddon Syndrome belong to close but different subsets of Sneddon Syndrome.
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the natural course of cerebral lesions in Sneddon Syndrome
JAMA Neurology, 1997Co-Authors: Ayman Tourbah, Jeancharles Piette, Marie T Ibazizen, O Lyoncaen, P Godeau, Camille FrancesAbstract:Objectives: To characterize the clinical, biological, and neuroradiological findings in Sneddon Syndrome; to correlate magnetic resonance imaging abnormalities with disability, presence of hypertension and other vascular risk factors, presence of heart valvulopathy on echography, and titer of antiphospholipid antibodies; and to compare these findings in antiphospholipid-positive and antiphospholipid-negative patients. Design: Retrospective review of the records of 32 consecutive patients with livedo reticularis and neurological events, followed up in our institution between January 1991 and August 1995. Patients: Twenty-six patients (20 women and 6 men) who had at least 1 cerebral ischemic arterial event associated with generalized and pathological livedo reticularis. Results: The age at the first cerebral ischemic event ranged from 22 to 58 years. Motor deficit was the most frequent sign (found in 73% of cases). Disability was found in 50%, systemic hypertension in 65%, heart valvulopathy in 61%, and antiphospholipid antibodies in 42% of cases. Patients were classified in 6 groups according to magnetic resonance imaging findings. No correlation was found between the presence of hypertension or other vascular risk factors, valvulopathy, antiphospholipids, and magnetic resonance imaging abnormalities. There was no significant difference between antiphospholipid-positive and antiphospholipid-negative patients except for the presence of antinuclear antibodies. There was a significant correlation between the extent of magnetic resonance imaging abnormalities and disability. Conclusion: The severity of the disease seems to be correlated with magnetic resonance imaging aspects, but not to the presence of antiphospholipid antibodies. Magnetic resonance imaging may help to understand the natural course of the cerebral involvement of the disease.
Catharina M Legierse - One of the best experts on this subject based on the ideXlab platform.
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Sneddon Syndrome and the diagnostic value of skin biopsies – Three young patients with intracerebral lesions and livedo racemosa
European Journal of Dermatology, 2008Co-Authors: Catharina M Legierse, Marijke R. Canninga-van Dijk, C.a.f.m. Bruijnzeel-koomen, Veronica C. M. Kuck-kootAbstract:Sneddon Syndrome is a rare disorder characterised by generalised livedo racemosa of the skin with extracutaneous neurological symptoms like headache, vertigo, transient ischaemic attacks (TIA), stroke, and seizures. Diagnosis of Sneddon Syndrome is based on these clinical features and positive findings in skin biopsies, namely the histological proof of occlusion of arterioles by intimal proliferation. We describe three cases of young patients with clinical characteristics of Sneddon Syndrome, but in only two cases could this diagnosis be confirmed by skin biopsies. These cases stress the difficulty of diagnosing Sneddon Syndrome and show the additive value of skin biopsies in this process.
-
Sneddon Syndrome and the diagnostic value of skin biopsies three young patients with intracerebral lesions and livedo racemosa
European Journal of Dermatology, 2008Co-Authors: Catharina M Legierse, Marijke Canningavan R Dijk, C A F M Bruijnzeelkoomen, Veronica C M KuckkootAbstract:Sneddon Syndrome is a rare disorder characterised by generalised livedo racemosa of the skin with extracutaneous neurological symptoms like headache, vertigo, transient ischaemic attacks (TIA), stroke, and seizures. Diagnosis of Sneddon Syndrome is based on these clinical features and positive findings in skin biopsies, namely the histological proof of occlusion of arterioles by intimal proliferation. We describe three cases of young patients with clinical characteristics of Sneddon Syndrome, but in only two cases could this diagnosis be confirmed by skin biopsies. These cases stress the difficulty of diagnosing Sneddon Syndrome and show the additive value of skin biopsies in this process.
Ayman Tourbah - One of the best experts on this subject based on the ideXlab platform.
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the natural course of cerebral lesions in Sneddon Syndrome
JAMA Neurology, 1997Co-Authors: Ayman Tourbah, Jeancharles Piette, Marie T Ibazizen, O Lyoncaen, P Godeau, Camille FrancesAbstract:Objectives: To characterize the clinical, biological, and neuroradiological findings in Sneddon Syndrome; to correlate magnetic resonance imaging abnormalities with disability, presence of hypertension and other vascular risk factors, presence of heart valvulopathy on echography, and titer of antiphospholipid antibodies; and to compare these findings in antiphospholipid-positive and antiphospholipid-negative patients. Design: Retrospective review of the records of 32 consecutive patients with livedo reticularis and neurological events, followed up in our institution between January 1991 and August 1995. Patients: Twenty-six patients (20 women and 6 men) who had at least 1 cerebral ischemic arterial event associated with generalized and pathological livedo reticularis. Results: The age at the first cerebral ischemic event ranged from 22 to 58 years. Motor deficit was the most frequent sign (found in 73% of cases). Disability was found in 50%, systemic hypertension in 65%, heart valvulopathy in 61%, and antiphospholipid antibodies in 42% of cases. Patients were classified in 6 groups according to magnetic resonance imaging findings. No correlation was found between the presence of hypertension or other vascular risk factors, valvulopathy, antiphospholipids, and magnetic resonance imaging abnormalities. There was no significant difference between antiphospholipid-positive and antiphospholipid-negative patients except for the presence of antinuclear antibodies. There was a significant correlation between the extent of magnetic resonance imaging abnormalities and disability. Conclusion: The severity of the disease seems to be correlated with magnetic resonance imaging aspects, but not to the presence of antiphospholipid antibodies. Magnetic resonance imaging may help to understand the natural course of the cerebral involvement of the disease.