Cerebellar Superficial Siderosis in Cerebral Amyloid Angiopathy

Abstract

Background and Purpose: Although evidence accumulates that the cerebellum is involved in cerebral amyloid angiopathy (CAA), cerebellar superficial siderosis is not considered to be a disease marker. The objective of this study is to investigate cerebellar superficial siderosis frequency and its relation to hemorrhagic magnetic resonance imaging markers in patients with sporadic and Dutch-type hereditary CAA and patients with deep perforating arteriopathy–related intracerebral hemorrhage. Methods: We recruited patients from 3 prospective 3 Tesla magnetic resonance imaging studies and scored siderosis and hemorrhages. Cerebellar siderosis was identified as hypointense linear signal loss (black) on susceptibility-weighted or T2*-weighted magnetic resonance imaging which follows at least one folia of the cerebellar cortex (including the vermis). Results: We included 50 subjects with Dutch-type hereditary CAA, (mean age 50 years), 45 with sporadic CAA (mean age 72 years), and 43 patients with deep perforating arteriopathy–related intracerebral hemorrhage (mean age 54 years). Cerebellar superficial siderosis was present in 5 out of 50 (10% [95% CI, 2–18]) patients with Dutch-type hereditary CAA, 4/45 (9% [95% CI, 1–17]) patients with sporadic CAA, and 0 out of 43 (0% [95% CI, 0–8]) patients with deep perforating arteriopathy–related intracerebral hemorrhage. Patients with cerebellar superficial siderosis had more supratentorial lobar (median number 9 versus 2, relative risk, 2.9 [95% CI, 2.5–3.4]) and superficial cerebellar macrobleeds (median number 2 versus 0, relative risk, 20.3 [95% CI, 8.6–47.6]) compared with patients without the marker. The frequency of cortical superficial siderosis and superficial cerebellar microbleeds was comparable. Conclusions: We conclude that cerebellar superficial siderosis might be a novel marker for CAA.

Decreased Cerebrospinal Fluid Amyloid β 38, 40, 42, and 43 Levels in Sporadic and Hereditary Cerebral Amyloid Angiopathy

“…Symptomatic D‐CAA was defined by the presence of one or more symptomatic ICH(s) in combination with either confirmed carriership of the c.2077G > C mutation or one or more first‐degree relatives with D‐CAA. The D‐CAA CSF samples were collected in the context of a D‐CAA natural history study of LUMC (the AURORA study) 17 …”

“…This protocol included SWI, T2, and FLAIR sequences. Further details are described in Koemans et al 17 …”

“…Patients with D-CAA were included if they had an available CSF sample. Presymptomatic D-CAA was defined as 17 The prevalence of hypertension was assessed in all patient groups and controls, except in the patients with AD (see Tables 1-3).…”

Superficial cerebellar microbleeds and siderosis associated with meningeal carcinomatosis

“…CMBs may arise from a broad spectrum of different etiologies, such as CAA, hypertensive vasculopathy, brain abscess and primary intracranial tumors. Recent studies have shown that superficial cerebellar microbleeds and siderosis may be new markers of CAA ( 3 , 4 ). The distribution of CMBs and the clinical course of patients can be useful to identify the etiology of CMBs.…”

An Uncommon Presentation of Cerebral Amyloid Angiopathy

“…MRI shows also microhaemorrhages, defined as petechial lesions with a diameter less than 5 mm that are generally asymptomatic and located at the cortical-subcortical junction predominantly located in the posterior half of the brain: this distribution pattern is considered to be characteristic for CAA [11]. Cerebellar superficial siderosis has been considered a novel marker for CAA [12]. Other MRI findings of patients affected by CAA are leukoencephalopathy and ischemic lesions.…”