When It Fits a Case of SLIPPERS with Imaging, Histopathology, and Treatment Response
Konrad Kubicki1, Thomas Shoemaker2
1Neurological Sciences, Rush University Medical Center, 2Rush University Medical Center
Objective:

To contribute to the scarce literature on Supratentorial Lymphocytic Inflammation with Parenchymal Perivascular Enhancement Responsive to Steroids (SLIPPERS)—thus, supporting this rare constellation of findings as a distinct clinical entity.  

Background:

In 2015 the term SLIPPERS was coined. Its inaugural use described two patients with clinical, radiographic, and histopathological similarity to Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS). This supratentorial variant has proved to be exceptionally rare, with only several case reports since.

Design/Methods:
NA
Results:

Our 61 year-old female patient presented with severe right-sided headache of 2 weeks and several days of encephalopathy. On examination, she was found to have left hemiparesis. She underwent multiple MRI brain studies which demonstrated nodular areas of enhancement involving the bilateral gangliocapsular region and left periventricular/left caudate. Associated T2 hyperintensities involved the right basal ganglia and left frontal subcortical white matter. Initial cerebrospinal fluid studies revealed a white blood cell count of 450/uL with lymphocytic predominance, protein 125 mg/dL, glucose 67 mg/dL, and negative autoimmune encephalopathy panel.

Subsequently, brain biopsy targeting a left frontal lesion demonstrated mild lymphohistiocytic infiltrate. The inflammatory cells were comprised of CD3+ T-cells with perivascular accumulation and parenchymal extension. There was no evidence of neoplastic, infectious, demyelinating, or vasculitic processes on histopathology.

 Intravenous corticosteroids were trialed outpatient (methylprednisolone 1 gram for 3 days with month-long prednisone taper). MRI brain with and without contrast was repeated after treatment, with interval resolution of the enhancing lesions and associated decrease in prior T2 abnormalities. Clinically, she remained stable with residual mild left hemiparesis. Notably, her presentation resembled the initial clinical presentation in the index case report—headache with hemiparesis.

Conclusions:
This case fits clinical, radiographic, and histopathological features of the recently described SLIPPERS. Additional case studies with long-term follow up may better elucidate the nature of this inflammatory disorder including associated clinical outcomes.
10.1212/WNL.0000000000211567
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