LAUSR

A 71-year-old-female, with history of two episodes of transient headache and confusion with normal brain MRI and neuropsychological investigation, presented at the outpatient clinic with a new episode of headache and forgetfulness since 3 weeks. Her family history was negative for stroke and dementia. In the following 2 days, she developed a progressive coma and was admitted at the ICU. At admission, blood pressure was 234/119 mmHg, without signs of papilledema, or hypertensive retinal damage. Brain CT showed large hypodensities in both frontal and left parietal lobe, with some mass effect on midline structures (see Fig. 1). Additional imaging with MRI (see Fig. 2) showed multifocal confluent, T2-hyperintense white matter lesions in both hemispheres, with compression on the left ventricle and subfalcine herniation. There was neither pathological enhancement nor diffusion restriction. T2*weighted images showed multiple corticosubcortical microbleeds in the left frontal and parietal lobe. Based on these findings, differential diagnosis consisted of PRES, CAA-I, progressive multifocal leukoencephalopathy (PML), acute demyelinating encephalomyelitis (ADEM) or low-grade infiltrative glioma. There was no recent infection with CMV, JCV, HSV1, HSV2, and HSV6. Cerebrospinal fluid (CSF) examination showed slight pleocytosis (35 leucocytes per mm3), with normal cultures and PCR, including Toxoplasma gondii and Cryptococcus neoformans. To reduce brain edema and counteract the mass effect, methylprednisolone 1000 mg IV for 5 days and mannitol 20% 70 ml IV 3dd were administered. Hypertension was treated by atenolol and nicardipine IV, later in association with amlodipine PO and clonidine IV with achievement of normotension 10 days after admission. As there was no clinical improvement, despite optimal tension control, frontal lobe brain biopsy was taken. Pathological examination showed cortical oedema with small cerebral microhemorrhages and CAA-positive vessels with perivascular histocytes. Seven days after admission, our patient was mutistic, showed a left hemineglect with slight paresis of her left arm. At day 11, she had incoherent speech, without paresis, with persistent cognitive deficits. She was transferred to a local neurorehabilitation centre for further rehabilitation. Methylprednisolone PO was tapered over several months. Seven months after admission, only minimal cognitive deficits were present. Follow-up brain MRI revealed significant decrease of cerebral edema in both hemispheres. No new microhemorrhages were seen on follow-up imaging.