DESCRIPTIONPosterior reversible encephalopathy (PRES) is a clinicalradiological syndrome characterised by brain symmetric lesions of vasogenic oedema, in white matter, basal ganglia or cerebral cortex, preferentially in posterior topography. 1 Typical manifestations are headache and altered mental status. Epileptic seizures are also frequent, although status epilepticus (SE) is a rare occurrence. 2 Chemotherapy may trigger PRES. Nevertheless, no single chemotherapy agent was consistently implicated. 2 Two cases are presented: a young man (patient 1) with a Hodgkin lymphoma, under ABVD chemotherapy (adriamicin, vinblastine, bleomicine, dacarbazin), and an adult woman (patient 2), under docetaxel and gemcitabine treatment due to a femoral sarcoma. Both patients were admitted with a non-convulsive SE. Brain resonance MRI depicted bilateral, predominantly parietal-occipital lesions, hyperintense on T2/ fl uid attenuated inversion recovery and with no water restriction on diffusion-weighted MRI/apparent diffusion coeffi cient maps, consistent with vasogenic oedema ( fi gure 1 ); patient 2 also presented intracranial vasospasm on magnetic resonance angiography. Both patients were successfully treated for the SE with antiepileptic drugs plus blood pressure control and nimodipine. The fi rst patient repeated ABVD afterwards without recurrence of PRES. In the second patient, no further chemotherapy regimens were performed due to neoplasm refractoriness. Furthermore, both patients showed complete resolution of cytotoxic oedema on repeated MRI.The association between PRES and platinum, taxanes, vinca alkaloids, biological agents and combination chemotherapy, is increasingly recognised. The exact mechanism of toxicity remains unknown. Vasospasm and loss of cerebrovascular auto-regulation, with the development of vasogenic oedema is a hypothesis. If PRES is left untreated, cytotoxic oedema and permanent neurological defi cit may occur, 2 hence the need for an early recognition is required.
The clinical phenotype of frontotemporal dementia patients carrying progranulin (GRN) mutations is known to be heterogeneous. We present a patient with corticobasal syndrome and a family with progressive aphasia and behavioral features who were found to have the same p.Gln257Profs*27 mutation. These cases depict the variability of GRN mutation carriers regarding clinical presentation and age of onset. In addition to giving a detailed report of a GRN mutation, we highlight the importance of searching for the presence of GRN mutations in selected sporadic cases and suggest a broadening of GRN genetic screening to better understand the clinical spectrum of these mutations.
A 70-year-old male was admitted with a 2-week progressive course of severe cognitive impairment, scoring three on the Mini Mental State Examination. MRI of the brain showed confluent hyperintense areas in T2/FLAIR in the periventricular and subcortical white matter, extending to right parietal cortex and basal ganglia. Intra-arterial angiography was unremarkable. A targeted stereotactic brain biopsy disclosed a leukocytoclastic vasculitis. The patient improved on steroids. Leukocytoclastic vasculitis adds to the spectrum of histopathologic subtypes of primary angiitis of the central nervous system.
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