Possible primary central nervous system vasculitis presentıng with confusion and hallucination: a case

Abstract

Primary central nervous system vasculitis (PCNSV) is a rare inflammatory disease of the vessels of the brain and spinal cord without signs of systemic vasculitis. Due to the difficulty of diagnosis, it is difficult to determine the exact incidence. Clinical findings may include a wide variety of neurodeficides and are subacute-chronic. The biopsy or cerebral angiography recommended for definitive diagnosis does not have as high specificity as expected. The presence of a prodromal clinic, the presence of neurodeficitis, the elimination of systemic findings, the presence of atypical lesions on MRI, and abnormal CSF analysis can also lead to a diagnosis of PSSSV. Immunosuppressive agents such as steroids and cyclophosphamide are used in the treatment. In this article, we present a case with abnormal CSF findings and a diagnosis of possible PSSSV using MR spectroscopy.

Keywords

• vasculitis,confusion,central nervous system

Konfüzyon ve halüsinasyon ile prezente olan olası primer santral sinir sistemi vasküliti: bir olgu

Abstract

Primer santral sinir sistemi vasküliti (PSSSV), sistemik vaskülit belirtisi olmadan beyin ve omurilikteki damarlarının nadir bir inflamatuar hastalığıdır. Tanı zorluğu nedeniyle tam insidansını belirlemek zordur. Klinik geniş nörodefisitler içerebildiği gibi subakut-kronik seyirlidir. Kesin tanı için önerilen biyopsi veya serebral anjiografinin beklendiği kadar yüksek özgüllüğü yoktur. Prodromal bir kliniğin bulunması, nörodefisit varlığı, sistemik bulguların ekartasyonu, MRG’de atipik lezyonların varlığı, anormal BOS analizi ile de olası PSSSV tanısına ulaşılabilir. Tedavide steroid ve siklofosfamid gibi immunsüpresif ajanlar kullanılmaktadır. Bu yazıda anormal BOS bulgularının olduğu ve MR spectroskopiden faydalanılarak olası PSSSV tanısı alan olgu sunulmuştur.

Keywords

• vaskülit, konfüzyon,santral sinir sistemi

References

1. 1. Chen SH, Sur S, Sedighim S, Kassi A, Yavagal D, Peterson EC, Starke RM. Utility of diagnostic cerebral angiography in the management of suspected central nervous system vasculitis. J Clin Neurosci 2019; 64: 98-100.
2. 2. Salvarani C, Brown Jr RD, Calamia KT, Christianson TJ, Weigand SD, Miller DV et al. Primary central nervous system vasculitis: analysis of 101 patients. Ann Neurol 2007; 62: 442-51
3. 3. Rice CM, Scolding JS. The diagnosis of primary central nervous system vasculitis, Pract Neurol 2019; 0: 1-7
4. 4. Younger DS, Coyle PK. Central nervous system vasculitis due to infection. Neurol Clin 2019; 37: 441-63.
5. 5. Younger DS. Central nervous system vasculitis due to substance abuse. Neurol Clin 2019; 37: 425-40.
6. 6. Younger DS. Treatment of vasculitis of the nervous system. Neurol Clin 2019; 37: 399-423.
7. 7. Younger DS. Autoimmune encephalitides. Neurol Clin 2019; 37: 359-81.
8. 8. Birnbaum J, Hellmann DB. Primary angiitis of the central nervous system. Arch Neurol 2009; 66: 704-09.

9. 9. Calabrese LH, Mallek JA. Primary angiitis of the central nervous system: report of 8 new cases, review of the literature, and proposal for diagnostic criteria. Medicine (Baltimore). 1988; 67: 20-39.
10. 10. Vollmer TL, Guarnaccia J, Harrington W, Pacia SV, Petroff OAF. Idiopathic granulomatous angiitis of the central nervous system: diagnostic challenges. Arch Neurol 1993; 50: 925–30
11. 11. Salvarani C, Brown RD Jr, Calamia KT et al. Primary central nervous system vasculitis: analysis of 101 patients. Ann Neurol 2007; 62: 442–51.
12. 12. Duna GF, Calabrese LH. Limitations of invasive modalities in the diagnosis of primary angiitis of the central nervous system. J Rheumatol 1995; 22: 662–67.
13. 13. Harris KG, Tran DD, Sickels WJ, Cornell SH, Yuh WT. Diagnosing intracranial vasculitis: the roles of MR and angiography. AJNR Am J Neuroradiol 1994; 15: 317–30.
14. 14. Mehdiyev Z, Öztürk V. Primer Santral Sinir Sisteminin Vasküliti. Turkiye Klinikleri J Neurol-Special Topics 2014;7: 72-76