Supratentorial Lymphocytic Inflammation with Parenchymal Perivascular Enhancement Responsive to Steroids (SLIPPERS)-Does it Really Exist?
Fernando FreuaJoão Vitor MahlerPedro Lucas Grangeiro de Sá Barreto LimaIuri Santana NevilleLeonardo Barreira PortellaVictor Hugo Rocha MarussiCarmen Lucia Penteado LancellottiPaulo Ribeiro NobregaGuilherme Diogo da SilvaPublished in: Brain sciences (2023)
Supratentorial Lymphocytic Inflammation with Parenchymal Perivascular Enhancement Responsive to Steroids (SLIPPERS) is a rare variant of the CLIPPERS spectrum with less than ten reports published so far. There is ongoing discussion regarding whether SLIPPERS is a disease entity on its own or just an acronym encompassing many underlying diagnoses, such as sarcoidosis, vasculitis and anti-glial fibrillary acidic protein (GFAP)-associated disease. A 40-year-old woman presented with episodes of language and attention impairment. Magnetic resonance imaging (MRI) revealed T2/FLAIR hyperintense lesions in the subcortical white matter associated with a micronodular, curvilinear perivascular contrast-enhancement. Alternative diagnoses were excluded. There was a remarkable response to steroids. A relapse occurred after six years, and the biopsy showed perivascular T-cell lymphocytic infiltrate, without granulomas, vasculitis, or neoplasia. There was complete resolution of the relapse after steroids. This case represents the longest reported follow-up of a patient diagnosed with SLIPPERS, and brain biopsy after 6 years did not suggest alternative diagnoses. This report contributes to the discussion regarding the possibility that exclusive supratentorial CLIPPERS-like pathology might be an isolated disease entity, but more biopsy-proven cases with a longer follow-up are needed to support this hypothesis. Recently, GFAP astrocytopathy has been characterized and might correspond to a significant number of cases previously diagnosed as CLIPPERS or SLIPPERS.
Keyphrases
- white matter
- magnetic resonance imaging
- contrast enhanced
- oxidative stress
- ultrasound guided
- fine needle aspiration
- computed tomography
- magnetic resonance
- multiple sclerosis
- randomized controlled trial
- autism spectrum disorder
- case report
- high grade
- small molecule
- spinal cord injury
- ionic liquid
- protein protein
- subarachnoid hemorrhage
- adverse drug
- electronic health record
- cerebral ischemia