Multiple ischaemic strokes and encephalopathy in a patient with CADASIL and COVID-19: A complex association

Introduction

SARS-CoV-2 infection may present with a wide range of neurological manifestations, including ischaemic and haemorrhagic stroke secondary to endothelial damage and a procoagulant state caused by the virus.

We report the case of a patient with COVID-19 who presented multiple strokes; the patient was a carrier of the mutation responsible for cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), which had been asymptomatic until the time of consultation.

Case report

The patient was a 28-year-old man with no relevant history who consulted due to apathy, diarrhoea, and vomiting of 36 h progression. He had no relevant family history of neurological disease. The physical examination revealed bradyphrenia, severe dysarthria, and dysphagia for both solids and liquids. A PCR test for SARS-CoV-2 yielded positive results. During hospitalisation, HIV infection and syphilis were ruled out. The patient presented no metabolic alterations. A biochemical analysis of CSF yielded normal results.

Laboratory analyses for rheumatic and haematological diseases ruled out blood clotting disorders. A contrast brain MRI scan revealed multiple subcortical lesions compatible with acute ischaemic lesions in both hemispheres, predominantly at the level of the centrum semiovale ( Fig. 1 ). Hyperintense lesions were found in the subcortical white matter of both temporal poles ( Fig. 2 ). CT angiography of the brain and neck detected no abnormalities. Transthoracic echocardiography with microbubbles yielded normal results. Brain angiography and positron emission tomography ruled out vasculitis. The patient received treatment with acetylsalicylic acid dosed at 100 mg/day and required feeding with a nasoduodenal tube due to dysphagia. Imaging findings were suggestive of CADASIL; sequencing of the NOTCH3 gene revealed heterozygosity for a pathogenic variant associated with the disease. The patient’s neurological symptoms progressed favourably. At discharge, the patients scored 1 on the National Institutes of Health Stroke Scale due to mild dysarthria. At 3 months from symptom onset, he scored 1 point on the modified Rankin Scale.