Introduction Acute ischaemic stroke (AIS) has been reported as a relatively infrequent complication of Covid-19. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is the commonest inherited stroke disorder. Our aim was to determine the clinical and radio- logical features of Covid-19 associated AIS in patients with CADASIL.

Methods We performed a systematic review of publications of patients with Covid-19 and CADASIL and included one unpublished case from two such presentations to our center.

Results We identified a total of 6 cases; average age 46 years, all female. Mean time from Covid-19 to AIS was 9.8 days, with no severe infection. Imaging demonstrated multiple infarcts in 66%, mean number of DWI lesions 9.6, 83% sustained infarcts located in the internal borderzones. An initial diagnosis of neuro- inflammation was revised in 50% following genetics, with 83% unaware of the CADASIL diagnosis.

Conclusions This systematic review describes the pattern of COVID-19 associated AIS in CADASIL. Such multiple infarcts, primarily affecting the internal borderzones, have been described in patients with Covid-19 without CADASIL. We discuss possible pathophysiological mechanisms, which may be extrapolated to patients with cerebral small vessel disease, including endothelial injury causing microvascular thrombosis and hypoperfusion due to cerebral blood flow dysautoregulation.