Cerebral vasculitis, a diagnostic labyrinth

A diagnosis of cerebral vasculitis is frequently considered in patients with new or progressive neurological symptoms for which there is no other explanation. A clinician considering a diagnosis of cerebral vasculitis should be well aware of alternative diagnoses, since these are generally more common. Several consecutive examinations are required for diagnosing cerebral vasculitis, because there is no diagnostic procedure that is highly sensitive as well as highly specific. The added value of the different procedures may depend on the type of blood vessels involved. Standard MRI examinations are sensitive but not specific. Special MRI techniques now make it also possible to make images of the vessel wall itself. Catheter angiography remains important, especially when noninvasive angiographic techniques do not reveal any abnormalities. Brain biopsy can provide proof of cerebral vasculitis and also serves to exclude mimicking conditions.</p

The diagnostic value of skin biopsies in Sneddon syndrome

Background Sneddon syndrome (SS) is defined by widespread livedo reticularis (LR) and stroke. There is no single diagnostic test for SS and diagnosis can be solely based on clinical features. This cross-sectional case-control study aimed to determine the diagnostic value of skin biopsies in SS patients. Materials and methods We studied skin biopsies from patients with a clinical diagnosis of SS or isolated LR. We also studied controls with vitiligo or normal skin. Biopsies were considered standardized if 3 biopsies were taken from the white centre of the livedo and reached until the dermis-subcutis border. Biopsies were scored for features of an occlusive microangiopathy without knowledge of the clinical features. Sensitivity and specificity of the biopsy findings were calculated with the clinical criteria as the reference standard. Results We included 34 SS patients, 14 isolated LR patients and 41 control patients. Biopsies of 17 patients with SS (50%), 4 with isolated LR (31%) and 10 control patients (24%) showed at least one artery in the deep dermis with a thickened vessel wall combined with recanalization or neovascularization (sensitivity 50% and specificity 69% with LR as reference). Standardized biopsies increased the sensitivity to 70%. In a post hoc analysis the combination of an occlusive microangiopathy and the presence of a livedo pattern in the superficial dermis increased the specificity to 92%. Conclusions Standardized skin biopsies can support the clinical diagnosis of SS. An occlusive microangiopathy as the only positive criterion for the diagnosis of SS had insufficient specificity for a definite diagnosis

Cerebrale vasculitis, een diagnostisch doolhof

A diagnosis of cerebral vasculitis is frequently considered in patients with new or progressive neurological symptoms for which there is no other explanation. A clinician considering a diagnosis of cerebral vasculitis should be well aware of alternative diagnoses, since these are generally more common. Several consecutive examinations are required for diagnosing cerebral vasculitis, because there is no diagnostic procedure that is highly sensitive as well as highly specific. The added value of the different procedures may depend on the type of blood vessels involved. Standard MRI examinations are sensitive but not specific. Special MRI techniques now make it also possible to make images of the vessel wall itself. Catheter angiography remains important, especially when noninvasive angiographic techniques do not reveal any abnormalities. Brain biopsy can provide proof of cerebral vasculitis and also serves to exclude mimicking conditions