Supratentorial lymphocytic inflammation with parenchymal perivascular enhancement responsive to steroids (SLIPPERS)
Supratentorial lymphocytic inflammation with parenchymal perivascular enhancement responsive to steroids (SLIPPERS) is a rare inflammatory disorder that shares pathoradiological similarities with chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS).
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Epidemiology
Cases of SLIPPERS are limited to case reports or small case series 1-7, thus, there is no established epidemiological data. It is likely that SLIPPERS is very rare, much rarer than CLIPPERS.
Clinical presentation
The potential clinical presentation from case reports include 1-7:
seizures
focal neurological deficits (e.g. weakness, visual field loss, dysphasia)
headache
cognitive dysfunction
gait dysfunction
Pathology
The aetiopathogenesis is yet to be elucidated. Notably, it has been proposed that SLIPPERS may not be a distinct clinicopathological syndrome, but may instead represent a radiological pattern or appearance that could be seen in a number of existing steroid-responsive inflammatory conditions (e.g. autoimmune GFAP astrocytopathy) 5.
Microscopic appearance
Pathologically, upon brain biopsy, SLIPPERS appears similar to CLIPPERS, with a predominantly T lymphocytic infiltration, often in a perivascular pattern 1-5. There is an absence of granulomas or vasculitic changes 5.
Radiographic features
MRI
MRI brain with gadolinium contrast is the imaging modality of choice, and imaging findings are overall similar to CLIPPERS, except restricted to the supratentorium 1-7. Notably, none of the reported cases in the literature have any pontine, other infratentorial, or spinal cord involvement 1-7.
T1: normal 1,2
T2/FLAIR: patches of hyperintensity involving the white matter 1-3,5-7
DWI/ADC: evidence of facilitated diffusion, in the same location as T2/FLAIR signal changes 2
SWI: may show blooming artifact representing foci of hemorrhage, in the same location as T2/FLAIR signal changes 7
T1 C+ (Gd): patchy, nodular, ‘peppery’ and/or curvilinear regions of contrast enhancement, in the same location as T2/FLAIR signal changes 1-6
MR perfusion: may show hypoperfusion, in the same location as T2/FLAIR signal changes 2
Treatment and prognosis
Management is with glucocorticoids, with good clinical and radiological response 1-7. Relapse has been reported during glucocorticoid tapering, and in this context, steroid-sparing immunosuppression may be employed 3.
History and etymology
SLIPPERS was first coined in a 2015 case series of two patients 3. Since, only a handful of cases have been reported in the literature 1,2,4-7, including one patient's case which has likely been published twice 1,2.
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