- The first case of bacillus Calmette-Guérin-induced small-vessel central nervous system vasculitis. [Review]
- CRClin Rheumatol 2018 May 09
- To present an unrecognized vascular complication of bacillus Calmette-Guérin (BCG) therapy administered for superficial bladder carcinoma. We also review the potential mimickers for primary angiitis ...
To present an unrecognized vascular complication of bacillus Calmette-Guérin (BCG) therapy administered for superficial bladder carcinoma. We also review the potential mimickers for primary angiitis of the central nervous system (PACNS) as well as complications of intravesical BCG therapy. An 89-year-old Caucasian man with a history of relapsing high-grade bladder carcinoma treated with intravesical BCG presented with recurring episodes of right upper limb paresthesia with clumsiness and dysarthria. Magnetic resonance imaging of the head revealed multiple predominantly left-sided frontotemporal micronodular peri-vascular lesions. Left frontal lobe biopsy showed non-necrotizing granulomatous vasculitis. Ziehl staining was negative. Initially, he was treated for PACNS but his symptoms relapsed during every attempt to taper the corticosteroids. Six months later, he developed bilateral mycobacterial endophthalmitis, caused by Mycobacterium bovis. Brain biopsy was reviewed and confirmed the presence of perivascular mycobacteria. A retrospective diagnosis of BCG-induced central nervous system vasculitis was made and he was treated with high-dose corticosteroids, moxifloxacin, isoniazid, ethambutol, and rifampicin. BCG is a live attenuated form of Mycobacterium bovis widely used as tuberculosis vaccination and intravesical therapy for superficial forms of bladder cancer. Systemic complications affect roughly 5% of patients and can manifest months or years after the last instillation. Cases of endophthalmitis, meningitis, aortitis, or mycotic aneurysms have been described, but no reports of CNS vasculitis have been found. In disseminated forms of BCG infections, referred to as BCGitis, histopathology usually reveals granulomatous inflammation. Mycobacterial cultures are often negative, making this a diagnostic challenge. This is the first documented case of BCG-induced small-vessel CNS vasculitis. Mycobacterium bovis infection is rare and findings are often nonspecific, making the diagnosis very difficult. Other infectious and non-infectious causes must be ruled out appropriately before considering this entity.
- Pearls & Oy-sters: The critical role of histopathology in diagnosing cancer-associated necrotizing CNS vasculitis. [Journal Article]
- NeurNeurology 2018 Apr 24; 90(17):808-811
- CONCLUSIONS: These cases highlight the importance of comprehensive evaluation of new brain lesions in patients with nonmetastatic solid tumors. Characteristics of new brain lesions in patients with cancer that should raise suspicion of diagnoses other than brain metastasis include (1) primary malignancy without regional or distant metastasis, (2) imaging without discrete mass-like enhancement, and (3) cortically based location of lesions not at the gray-white matter junction.
- Primary lymphomatoid granulomatosis in the central nervous system: A report of three cases. [Journal Article]
- NNeuropathology 2018 Apr 10
- Lymphomatoid granulomatosis (LYG) is a rare lymphoproliferative disorder characterized by infiltration of Epstein-Barr virus (EBV)-positive large atypical B-cells in an angiocentric fashion in a mixe...
Lymphomatoid granulomatosis (LYG) is a rare lymphoproliferative disorder characterized by infiltration of Epstein-Barr virus (EBV)-positive large atypical B-cells in an angiocentric fashion in a mixed inflammatory background. The histologic spectrum of LYG ranges from reactive proliferation to diffuse large B-cell lymphoma according to the number of EBV+ B-cells. It is known that virtually all patients have pulmonary involvement, whereas primary LYG of the other organs has been rarely reported. Herein, we describe three cases of primary LYG of the central nervous system (CNS) without pulmonary lesions, and this is the first collection to be reported in Korea. All of the cases revealed multifocal enhancing necrotic brain lesions masking as metastatic tumors, infection or vasculitis. These patients were successfully managed by corticosteroids and immunomodulating agents without chemotherapy against malignant lymphoma even in grade 3 LYG. We assume that primary CNS LYG might be less aggressive and more controllable than pulmonary LYG. The clinicopathologic characteristics of the cases with a special regard to the differential diagnosis and clinical courses are discussed in combination with an overview of the literature.
- 99mTc-HMPAO brain SPECT in the monitoring of cerebral vasculitis therapy. [Journal Article]
- RERev Esp Med Nucl Imagen Mol 2018 Mar 28
- CONCLUSIONS: In conclusion, brain SPECT seems to be able to monitor the disease in UV, indicating the moment when an improvement of the cerebral perfusion is achieved. In SLE patients this scintigraphic technique did not show a significant improvement in CNS perfusion.
- Erratum: Spinal cord inflammation in children with small vessel primary cns vasculitis. [Published Erratum]
- PCPaediatr Child Health 2017; 22(8):509
- [This corrects the article DOI: 10.1093/pch/pxx086.079.][This corrects the article DOI: 10.1093/pch/pxx086.079.].
[This corrects the article DOI: 10.1093/pch/pxx086.079.][This corrects the article DOI: 10.1093/pch/pxx086.079.].
- Primary central nervous system vasculitis associated with lymphoma. [Journal Article]
- NeurNeurology 2018 Mar 06; 90(10):e847-e855
- CONCLUSIONS: Lymphoma may be diagnosed simultaneously with PCNSV, suggesting an immunologic paraneoplastic mechanism.
- The role of biopsies and autopsies in the diagnosis of cognitive impairment, with emphasis on small vessel diseases: A critical appraisal enriched by personal experience. [Journal Article]
- DNDement Neuropsychol 2017 Oct-Dec; 11(4):356-363
- Acquired and hereditary microangiopathies cause cerebral small vessel diseases (CSVD) that impair cognition. The most frequent is primary angiitis of the CNS (PACNS), whose diagnosis remains challeng...
Acquired and hereditary microangiopathies cause cerebral small vessel diseases (CSVD) that impair cognition. The most frequent is primary angiitis of the CNS (PACNS), whose diagnosis remains challenging, requiring a multidisciplinary approach. Secondary vasculitis, CADASIL, miscellaneous microangiopathies and lymphomas, also cause cognitive impairment. Despite the fact that the need for biopsy has decreased in the era of new neuroimaging methods, biopsies that include small leptomeningeal and parenchymal arterial vessels still remain the gold standard to diagnose PACNS and other CSVD, and to exclude mimics such as infections and malignancies. New approaches for pathological consequences relevant to vascular cognitive impairment such as silent brain lesions, microinfarcts, microbleeds and subtle loss of microstructural integrity, may be detected in autopsies. This article addresses the role of biopsies and autopsies for the diagnosis of cognitive impairment related to small vessel diseases or other inflammatory/ischemic processes, and presents a critical appraisal based on personal experience.
- Central vein sign differentiates Multiple Sclerosis from central nervous system inflammatory vasculopathies. [Journal Article]
- ANAnn Neurol 2018; 83(2):283-294
- CONCLUSIONS: The central vein sign differentiates inflammatory CNS vasculopathies from MS at standard clinical magnetic field strengths. Ann Neurol 2018;83:283-294.
- Progressive Fatal Myelopathy Secondary to Isolated Spinal Cord Vasculitis. [Journal Article]
- FNFront Neurol 2017; 8:705
- A 56-year-old woman with various neurological signs which developed over a 1-year period was admitted for evaluation. MRI showed a markedly abnormal signal in the cervical spine. Despite treatment wi...
A 56-year-old woman with various neurological signs which developed over a 1-year period was admitted for evaluation. MRI showed a markedly abnormal signal in the cervical spine. Despite treatment with IV steroids, she developed a progressive myelopathy, became quadriplegic, and required intubation. Immunomodulatory treatment was ineffective. The patient died 24 days after admission. Histopathological investigation revealed spinal cord necrosis with a lymphocyte predominant meningovascular inflammation involving arteries and veins along with evidence of prior occlusive disease of the anterior spinal artery. The changes were confined to the spinal cord. The present case represents an unusual cause of myelitis for which early and aggressive immunomodulatory treatment may have influenced outcomes.
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- Child Neurology: Primary angiitis of the CNS. [Journal Article]
- NeurNeurology 2017 Dec 05; 89(23):e268-e271