Cerebral small vessel disease in patients with spontaneous cerebellar hemorrhage

Spontaneous cerebellar-intracerebral hemorrhage (ICH) can be associated with both cerebral amyloid angiopathy (CAA) and hypertensive small vessel disease (HTN-SVD, i.e. arteriolosclerosis). To better understand the underlying microangiopathy of cerebellar-ICH, we aimed to evaluate the spatial distribution of supratentorial cerebral microbleeds (CMBs) and neuropathologic profiles in these patients.

We enrolled consecutive cerebellar-ICH patients. Clinical variables and MRI markers specific for CAA and HTN-SVD were assessed. Patients were classified into categories according to the topography (strictly-lobar, strictly-deep, and mixed) of supratentorial CMBs and comparisons were performed. Available neuropathological material was reviewed to evaluate the presence and severity of arteriolosclerosis and CAA.

Ninety-eight cerebellar-ICH patients were enrolled. Fifty patients (51%) had at least one supratentorial CMB. Twelve patients (12%) had strictly lobar-CMBs, 12 patients (12%) showed strictly deep-CMBs and mixed-CMBs (lobar and deep CMBs) were present in 26 cerebellar-ICH patients (27%). In multivariable analysis, cerebellar-ICH patients with mixed-CMBs were associated with higher prevalence of hypertension (OR 4.9, 95% confidence interval [CI] 1.2-20, p = 0.017) but with lower prevalence of severe centrum-semiovale enlarged perivascular spaces (OR 0.2, CI 0.05-0.8, p = 0.024) when compared to cerebellar-ICH patients with strictly lobar-CMBs. Vascular risk factors and neuroimaging characteristics were similar between strictly deep-CMBs and mixed-CMBs. Six patients had available neuropathological material for analyses and they all showed some degree of arteriolosclerosis.

Cerebellar-ICH patients frequently show supratentorial CMBs. The mixed-CMBs pattern appears to be the most common. Our radiological and pathological results suggest that the majority of cerebellar-ICH patients harbor HTN-SVD as dominant microangiopathy.