Содержание
- 2. Case 20 YO male Admitted to the Neurology department on April, 11th, 2016 Complaining of gait
- 3. History of present illness 2010 – sudden vertigo, horizontal diplopia, oculomotor abnormalities, gait ataxia. He was
- 4. Brain MRI – T2 (2013)
- 5. Brain MRI – T2
- 6. Brain MRI – T2
- 7. Brain MRI – FLAIR
- 8. Brain MRI – T1 +contrast
- 9. Brain MRI – T1 +contrast
- 10. QUESTIONS Describe the findings of the MRI scans. What is the differential diagnosis? What investigations would
- 11. Differential diagnosis MS ADEM Neurosarcoidosis, Sjögren's syndrome NMO Autoimmune encephalitides CNS vasculitis Primary angiitis of CNS
- 12. Treatment during the next years: May 2014: planned hospitalization to start prophylactic therapy with prednisolone 15
- 13. Examination BP=120/80 mm Hg, Ps=68/min Alert, oriented, normal higher cortical functions. CN: Horizontal nystagmus in left
- 14. Laboratory tests CBA: Leuc.- 4.2 x10*9/l; er 5.18x10*12/l; Hb 159 g/l; lymf. 29%; mon. 9%; neutr.
- 15. Laboratory tests Autoantibodies and markers of vasculitis: anti-nuclear antibodies (ANA), extractable nuclear antigens (ENA), anti-neutrophil cytoplasmic
- 16. Brain MRI – T2 (2016)
- 17. Brain MRI – T2
- 18. Brain MRI - FLAIR
- 19. Brain MRI - FLAIR
- 20. Cervical spine MRI – T2
- 21. Brain MRI – T1 +contrast
- 22. Brain MRI
- 23. Brain MRI - T1 +contrast
- 24. Cervical spine MRI - T1 +contrast
- 25. QUESTIONS What abnormalities do you see at MRI? Now what is the diagnosis, and the disease?
- 26. Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS)
- 27. CLIPPERS is a recently defined inflammatory central nervous system (CNS) disorder, prominently involving the brainstem and
- 28. PATHOGENESIS The pathogenesis of CLIPPERS is poorly understood and ultimately unknown. The perivascular and T cell-predominant
- 29. Core features of CLIPPERS I. Clinical Main: Subacute progressive gait ataxia and diplopia; Other accompanying symptoms:
- 30. Core features of CLIPPERS II. Radiological Numerous punctate or nodular enhancing lesions bilaterally within at least
- 31. Brain MRI – T1 +contrast
- 32. Brain MRI - T1 +contrast
- 33. Core features of CLIPPERS III. Glucocorticosteroid responsiveness Clinical and radiological responsiveness to glucocorticosteroid (GCS)-based immunosuppression. However,
- 34. Core features of CLIPPERS IV. Histopathological White matter perivascular lymphohistiocytic infiltrate with or without parenchymal extension
- 35. Core features of CLIPPERS Differential diagnoses should be excluded e.g. neurosarcoidosis, Sjögren's syndrome, neuro-Behçet's disease, MS,
- 36. «Red flags» No response to treatment with GCS at the beginning or during follow-up. Unusual clinical
- 37. COMPARISON OF 7.0T AND 3.0T MRI The inflammation seen on conventional 1.5T–3.0T MRI only depicts the
- 38. Ultra-high-field MRI reveals perivascular lesions outside the brainstem/cerebellum and tissue damage and also indicates intralesional vascular
- 39. Immunohistochemical stainings for CD45, CD3, CD4, CD8, and CD20 shows prominent CD3 and CD4 T-cell infiltration
- 40. Treatment The initial treatment of choice seems to be a relatively short course of high-dose intravenous
- 41. Back to the case Diagnosis of CLIPPERS is really complicated, especially at the first stages of
- 42. Diagnosis of CLIPPERS in our clinical case was based on: Clinical features: Progressive gait ataxia and
- 43. CONCLUSION Diagnosis of CLIPPERS is challenging, and requires careful exclusion of alternative diagnoses. A specific serum
- 44. References: Buttmann M, Metz I, Brecht I, Brück W, Warmuth-Metz M. Atypical chronic lymphocytic inflammation with
- 45. References: Tallantyre EC, Brookes MJ, Dixon JE, Morgan PS, Evangelou N, Morris PG. Demonstrating the perivascular
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