- Diagnosis and Treatment of Primary Central Nervous System Angiitis. [Review]
- CTCurr Treat Options Neurol 2018 Aug 04; 20(9):38
- Primary central nervous system angiitis (PCNSA) is a rare disease. Varying clinical pictures coupled with lack of sensitive and specific diagnostic tests lead to challenges in management of these pat...
Primary central nervous system angiitis (PCNSA) is a rare disease. Varying clinical pictures coupled with lack of sensitive and specific diagnostic tests lead to challenges in management of these patients. This unfortunately may lead to both under- or over-diagnosis and unnecessary treatment. It is important to recognize the different conditions that may mimic the clinical and radiologic presentation. We present a contemporary literature review that should update physicians who encounter this patient population.
- Amyloid-Beta-Related Angiitis with Distinctive Neuro-Ophthalmologic Features. [Journal Article]
- NNeuroophthalmology 2018; 42(4):237-241
- Amyloid beta-related angiitis (ABRA) is a subtype of cerebral amyloid angiopathy-related inflammation, with distinctive pathology and prognosis compared with cerebral amyloid angiopathy (CAA). On a s...
Amyloid beta-related angiitis (ABRA) is a subtype of cerebral amyloid angiopathy-related inflammation, with distinctive pathology and prognosis compared with cerebral amyloid angiopathy (CAA). On a spectrum of increasing severity, ABRA is considered to be in-between the less aggressive inflammatory-CAA and the more severe primary central nervous system (CNS) angiitis. Whereas retinal pathological changes were described in subjects with primary or secondary CNS angiitis, and non-inflammatory CAA, bilateral posterior pole superficial and peripapillary retinal hemorrhages have not been reported as initial signs in patients with pathology-confirmed ABRA, accompanying neurological spells and characteristic neuroimaging findings.
- [Misleading cerebral arterial wall gadolinium-enhancement in malignant lymphoma]. [Case Reports]
- RSRinsho Shinkeigaku 2018 Jul 27; 58(7):456-459
- A previously healthy, 77-year-old woman presented with gradual cognitive decline and acute gait imbalance. On admission, despite no obvious paralysis, she tilted to the right. Her Mini-Mental State E...
A previously healthy, 77-year-old woman presented with gradual cognitive decline and acute gait imbalance. On admission, despite no obvious paralysis, she tilted to the right. Her Mini-Mental State Examination score was slightly low (23/30). Gadolinium-enhanced, high-resolution T1-weighted MRI showed abnormal arterial wall enhancement at the bilateral middle cerebral and right internal carotid arteries. The combination of arterial and parenchymal enhancement limited to the central nervous system (CNS), normal laboratory data including soluble interleukin-2 receptor, and random skin and bone-marrow biopsies was suggested of primary angiitis of the CNS (PACNS). However, a biopsy specimen from the right insula showed CD20-positive lymphoma cells indicative of diffuse large B-cell lymphoma. After receiving chemotherapy, above-mentioned abnormal findings were significantly improved. Considering the increasing clinical application of high resolution MRI, there is a risk that patients may receive a presumptive diagnosis of PACNS and immunosuppressive treatment without biopsy confirmation. It should be noted that a combination of abnormal arterial wall and linear parenchymal enhancement similar to that found for PACNS on high-resolution MRI may occur in patients with primary central nervous system lymphoma.
- Immune cell profiling in the cerebrospinal fluid of patients with primary angiitis of the central nervous system reflects the heterogeneity of the disease. [Journal Article]
- JNJ Neuroimmunol 2018 Aug 15; 321:109-116
- Primary angiitis of the central nervous system (PACNS) is a rare and heterogeneous inflammatory disease of the CNS vasculature with poorly understood pathophysiology. Comprehensive immune-cell phenot...
Primary angiitis of the central nervous system (PACNS) is a rare and heterogeneous inflammatory disease of the CNS vasculature with poorly understood pathophysiology. Comprehensive immune-cell phenotyping revealed increased frequencies of leukocytes in the cerebrospinal fluid (CSF) of PACNS patients compared to patients with multiple sclerosis, ischemic stroke, and somatoform disorders (n = 18 per group). Changes in the intrathecal immune-cell profile were heterogeneous in PACNS. While proportions of T-cell subsets remained unaltered, some PACNS patients showed a shift toward NK- or B cells. Intrathecal immunoglobulin synthesis was observed in a subgroup of PACNS patients with an increased frequency of antibody producing plasma cells.
- Cerebrospinal Fluid Concentrations of Neuronal Proteins Are Reduced in Primary Angiitis of the Central Nervous System. [Journal Article]
- FNFront Neurol 2018; 9:407
- Primary angiitis of the central nervous system (PACNS) is a rare autoimmune vasculitis limited to the CNS often causing substantial disability. Understanding of this disease is impaired by the lack o...
Primary angiitis of the central nervous system (PACNS) is a rare autoimmune vasculitis limited to the CNS often causing substantial disability. Understanding of this disease is impaired by the lack of available biomaterial. Here, we collected cerebrospinal fluid (CSF) from patients with PACNS and matched controls and performed unbiased proteomics profiling using ion mobility mass spectrometry to identify novel disease mechanisms and candidate biomarkers. We identified 14 candidate proteins, including amyloid-beta A4 protein (APP), with reduced abundance in the CSF of PACNS patients and validated APP by Enzyme-linked Immunosorbent Assay (ELISA) in an extended cohort of patients with PACNS. Subsequent functional annotation surprisingly suggested neuronal pathology rather than immune activation in PACNS. Our study is the first to employ mass spectrometry to local immune reactions in PACNS and it identifies candidates such as APP with pathogenic relevance in PACNS to improve patient care in the future.
- CNS Vasculitis: an Approach to Differential Diagnosis and Management. [Review]
- CRCurr Rheumatol Rep 2018 May 30; 20(7):37
- The goal of this review is to provide an up-to-date approach to diagnosis and management of patients with central nervous system (CNS) vasculitis.
The goal of this review is to provide an up-to-date approach to diagnosis and management of patients with central nervous system (CNS) vasculitis.
- 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.
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- 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.