Characterization of Extracranial Giant Cell Arteritis with Intracranial Involvement and its Rapidly Progressive Subtype.

OBJECTIVE: The objective of this study was to characterize patients with extracranial giant cell arteritis with intracranial involvement. METHODS: In a multicenter retrospective study, we included 31 patients with systemic giant cell arteritis (GCA) with intracranial involvement. Clinical characteristics, pattern of arterial involvement, and cytokine profiles were assessed. Patients with GCA without intracranial involvement (n = 17), and with intracranial atherosclerosis (n = 25) served as controls. RESULTS: Erythrocyte sedimentation rate (ESR) was elevated in 18 patients (69.2%) with and in 16 patients (100%) without intracranial involvement (p = 0.02). Headache was complained by 15 patients (50.0%) with and 13 patients (76.5%) without intracranial involvement (p = 0.03). Posterior circulation arteries were affected in 26 patients (83.9%), anterior circulation arteries in 17 patients (54.8%), and both territories in 12 patients (38.7%). Patients with GCA had vertebral artery stenosis proximal and, in contrast, patients with atherosclerosis distal to the origin of posterior inferior cerebellar artery (PICA). Among patients with GCA with intracranial involvement, 11 patients (37.9%) had a rapid progressive disease course characterized by short-term recurrent ischemic events. The median modified Rankin Scale (mRS) at follow-up in these patients was 4 (interquartile range [IQR] = 2.0-6.0) and 4 patients (36.4%) died. Vessel wall expression of IL-6 and IL-17 was significantly increased in patients with rapid progressive course. INTERPRETATION: Typical characteristics of GCA, headache, and an elevated ESR, are frequently absent in patients with intracranial involvement. However, differentiation of intracranial GCA from atherosclerosis can be facilitated by the typical pattern of vertebral artery stenosis. About one-third of patients with intracranial GCA had a rapid progressive course with poor outcome. IL-17 and IL-6 may represent potential future treatment targets. ANN NEUROL 2021;90:118-129.

Therapeutic challenges in a patient with POEMS syndrome and recurrent stroke: presentation of a case and review of the literature.

POEMS syndrome includes the clinical features of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes. A severe complication of patients with this syndrome is ischemic stroke, which has been described only occasionally despite the hematologic abnormalities that may be associated with the disease. In the literature, only eight patients have been reported as suffering from multiple or recurrent strokes until now. We present a 54-year-old woman from Sri Lanka with POEMS syndrome who experienced recurrent ischemic strokes followed by an acute abdomen and global heart failure. On the basis of a literature review, we discuss current therapeutic approaches and their possible side effects.

[Importance of surfactant proteins B and D for the differential diagnosis of acute dyspnea]. / Bedeutung der Surfactant-Proteine B und D in der Differentialdiagnostik der akuten Dyspnoe.

BACKGROUND AND PURPOSE: The basis for an optimal therapy of cardiopulmonary diseases is the assessment of an early diagnosis. This implies an evaluation of possible differential diagnoses of acute dyspnea. In numerous studies, natriuretic peptides were characterized as additional, meaningful parameters for the assessment of left ventricular function. Current studies could demonstrate that surfactant proteins B (SP-B) and D (SP-D) are of importance for the differentiation of patients with acute dyspnea. The aim of this study was to compare the values of NT-proBNP (N-terminal brain natriuretic peptide) and surfactant proteins for the assessment of a final diagnosis in patients with acute dyspnea. PATIENTS AND METHODS: NT-proBNP, SP-B and SP-D were measured in 81 patients with acute dyspnea in the emergency room and were correlated with clinical and echocardiographic parameters with respect to the final diagnosis. For this, patients were classified with respect to clinical and echocardiographic parameters in different subgroups concerning the final diagnosis of acute dyspnea. RESULTS: In patients with a cardiac origin of acute dyspnea, plasma levels of NT-proBNP were significantly higher as compared to patients with a noncardiac diagnosis (p = 0.04). SP-D was highest in patients with a cardiac origin of acute dyspnea, but after performing regression analysis it seems to be of less importance for the differential diagnosis of acute dyspnea as compared to NT-proBNP. SP-B plasma levels were not different between the four subgroups. CONCLUSION: NT-proBNP is of importance for the differential diagnosis of acute dyspnea. Although SP-D shows similar changes of plasma levels between the four subgroups, it seems to be of less importance for the differential diagnosis of acute dysnea. SP-B occurs to be of no relevance for the differentiation between cardiac and noncardiac origin of acute dyspnea.