Sneddon syndrome – imaging findings

Sneddon syndrome (SS) is a rare progressive non-inflammatory thrombotic vasculopathy affecting small/medium-sized blood vessels of unknown origin. It is strongly associated with the presence of antiphospholipid antibodies (AA). The presence of livedo reticularis and cerebrovascular disease are hallmark features. The condition is far more common in young women. We report a case of SS in a 43 year-old male with a two-year history of progressive cognitive impairment consistent with dementia syndrome, and major personality changes, besides livedo reticularis and cerebral angiographic pattern of vasculitis. AA were borderline. The recognition of skin blemishes that precede strokes should raise the hypothesis of SS. AA are elevated in more than half of cases, but their role in the pathogenesis or association of positive antibodies and SS remains unclear. Dementia syndrome in young patients should be extensively investigated to rule out reversible situations. Typical skin findings, MRI and angiography may aid diagnosis.

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“…Cerebral atrophy is described in SS as a progressive complication due to involvement of small arteries. 2 , 4 , 5 , 7 …”

Section: Discussionmentioning

confidence: 99%

Cognitive and psychiatric changes as first clinical presentation in Sneddon syndrome

Fabiani¹,

Filho

Koppe³

et al. 2018

Dement. neuropsychol.

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“…Livedo reticularis is a dermatological disorder marked by mottled purplish discoloration of the skin due to sluggish venous blood flow [2,3]. It is the characteristic symptom of Sneddon's syndrome [4,5]. It can be due to brain stem infarction rarely, as the case we will discuss here.…”

Section: Introductionmentioning

confidence: 99%

Livedo reticularis caused by brain stem infarction: Case report and literature review

Xu¹,

Pei²

2018

Int J Case Rep Images

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Introduction:With brainstem stroke, symptoms can arise depending on the exact location of the lesion with respect to the long tracts, brainstem connection, and cranial nerve nuclei. Livedo reticularis is very characteristic for Sneddon's syndrome. It is rarely caused by dysfunction of brain stem as described is this case. Case Report: A 50-year-old man complained of sudden onset dysarthria, dysphagia. Upon admission, neurological examination revealed unilateral weakness of the upper and lower face in the right side, Horner's sign in the right side, severe dysphagia, and mild dysarthria, as well as insufficient temperature and pinprick sensation in left side, livedo reticularis in the extremities of the left limbs up to wrist and ankle. Brain MRI scan showed a lesion in the right medulla, which was responsible for the syndrome. Infarction in the right RVM (rostral ventral medulla) caused decreased sympathetic outflow causing livedo reticularis in the left limbs. Conclusion: In this case, Livedo reticularis in the left limbs was caused by brain stem infarction in the right, it was not Sneddon's syndrome. This case might add discussion of other possible causes of livedo reticularis and the relevant neuroanatomy, which could explain disturbances of sympathetic nerve activity causing livedo reticularis.

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Sneddon syndrome – imaging findings

Cited by 2 publications

References 4 publications

Cognitive and psychiatric changes as first clinical presentation in Sneddon syndrome

Cognitive and psychiatric changes as first clinical presentation in Sneddon syndrome

Livedo reticularis caused by brain stem infarction: Case report and literature review

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