The Elusive SLIPPERS Syndrome (Supratentorial Lymphocytic Inflammation with Parenchymal Perivascular Enhancement Responsive to Steroids): A Case Report and Literature Review

Abstract

Background

In 2015, the term “SLIPPERS” was created to refer to a rare type of encephalomyelitis called CLIPPERS syndrome that affects the pons and sometimes other nearby structures, but in this case, it primarily affects the supratentorial region. This variation of the condition is responsive to treatment with steroids.

Case Description

We report the case of a patient who presented with seizures and visual field deficit and had typical radiological and histopathological characteristics of SLIPPERS syndrome.

Conclusion

Although the literature is inundated with CLIPPERS syndrome, its supratentorial variant is extremely rare. To our knowledge, this is fourth case of SLIPPERS syndrome to be reported in literature and serves to enhance clinicopathological understanding of this elusive entity.

Keywords:

• CLIPPERS
• SLIPPERS
• corticosteroids
• neuroinflammation
• perivascular infiltration

Abbreviations

ANA, Anti-nuclear antibody; ANCA, Anti-neutrophil cytoplasmic antibody; Anti-dsDNA, Anti-double stranded DNA; Anti-DNase B, Anti-Deoxyribonuclease B; CBC, Complete blood count; CLIPPERS, Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids; CRP, C-Reactive protein; CSF, Cerebrospinal fluid; CT, Computed tomography; CNS, Central nervous system; ESR, Erythrocyte Sedimentation Rate; FLAIR, Fluid Attenuation Inversion Recovery; GCS, Glasgow Coma Score; HIV, Human Immunodeficiency Virus; HBV, Hepatitis B Virus; HCV, Hepatitis C Virus; HSV, Herpes Simplex Virus; MDT, Multiple Disciplinary Team; MRI, Magnetic resonance imaging; SLIPPERS, Supratentorial Lymphocyte Inflammation with Parenchymal Perivascular Enhancement Responsive to Steroids; T2W, T2 Weighted; T1W, T1 Weighted.

Institutional Approval

Institutional approval was taken from Medical Research Council Qatar.

Consent

The patient gave informed consent for the publication of their case details and any accompanying images.

Acknowledgments

Special thanks to Dr Mohsin Khan for reviewing this article in a short period of time.

Disclosure

The authors report no conflicts of interest in this work.

Additional information

Funding

Open access funding is provided by Qatar National Library.