Primary central nervous system vasculitis: Comparison of patients with and without cerebral amyloid angiopathy

Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA.
Rheumatology (Oxford, England) (Impact Factor: 4.48). 09/2008; 47(11):1671-7. DOI: 10.1093/rheumatology/ken328
Source: PubMed


To describe the clinical features and outcomes of patients with primary central nervous system vasculitis (PCNSV) and cerebral amyloid angiopathy (CAA) from a large cohort of consecutive patients with PCNSV treated at a single institution.
We identified 101 consecutive patients with PCNSV admitted between January 1983 and December 2003. PCNSV diagnoses were based on findings from a central nervous system (CNS) biopsy (n = 31) and conventional angiography (n = 70). CNS tissue specimens from 49 cases were examined histologically, and 49 were stained for amyloid deposits. Those with vascular amyloid deposits (CAA) were compared with those without histological evidence of amyloid deposition.
Eight cases (26%) with CNS biopsy specimens positive for PCNSV also showed findings of CAA. Compared with patients with PCNSV only, these patients were older at diagnosis, predominantly male, had a more acute onset, a higher frequency of cognitive dysfunction and showed prominent gadolinium-enhanced leptomeningeal lesions with MRI. Histologically, all had a granulomatous vascular inflammatory pattern. Six patients responded promptly to therapy. Outcomes at last follow-up were similar in the two groups.
PCNSV with CAA appears to form a clinical subset of PCNSV. The vasculitis influences the clinical findings to a greater degree than the presence of amyloid deposits in the vessels.

Download full-text


Available from: Dylan V Miller, Dec 19, 2013
  • Source
    • "1 69 F cognitive decline bilateral multifocal (-) (+) perivasculitis steroid alive relapse (+) Salvarani et al. 2008 [29] 8 63* M6 F2 encephalopathy 6 focal neurology 2 headache 3 only aphasia with alexia 1 bilateral 8 multifocal NA (+) 5 (-) 3 vasculitis steroid 3 steroid cyclophosphamide 5 improved 6 died 1 worsened 1 Amick et al. 2008 [30] 1 79 F transient right sided weakness left occipital/parietal NA (-) vasculitis (-) died Alcalay et al. 2009 [31] 1 92 F mental status change bilateral multifocal (+) (+) (-) steroid alive Daniëls et al. 2009 [32] 1 80 F mental status change right sided hemiparesis dysphasia seizure bilateral left hemisphere right parietal/occipital (+) (-) (-) steroid alive relapse (+) Greenberg et al. 2010 [9] "
    [Show abstract] [Hide abstract]
    ABSTRACT: A 56-year-old man noticed discomfort in his left lower limb, followed by convulsion and numbness in the same area. Magnetic resonance imaging (MRI) showed white matter lesions in the right parietal lobe accompanied by leptomeningeal or leptomeningeal and cortical post-contrast enhancement along the parietal sulci. The patient also exhibited higher brain dysfunction corresponding with the lesions on MRI. Histological pathology disclosed β-amyloid in the blood vessels and perivascular inflammation, which highlights the diagnosis of cerebral amyloid angiopathy (CAA)-related inflammation. Pulse steroid therapy was so effective that clinical and radiological findings immediately improved. CAA-related inflammation is a rare disease, defined by the deposition of amyloid proteins within the leptomeningeal and cortical arteries associated with vasculitis or perivasculitis. Here we report a patient with CAA-related inflammation who showed higher brain dysfunction that improved with steroid therapy. In cases with atypical radiological lesions like our case, cerebral biopsy with histological confirmation remains necessary for an accurate diagnosis.
    Full-text · Article · Sep 2011 · Journal of Neuroinflammation
  • Source
    • "Others recommended combination therapy consisting of prednisone and cyclophosphamide for at least 1 year [25,26]. In addition, Salvarani et al. [27] indicated that patients with IACNS which also have vascular deposits of β-peptide generally respond well to immunosuppressive treatment. However, the present case demonstrated good effects of immunosuppressant to patient without indication of amyloid protein deposition. "
    [Show abstract] [Hide abstract]
    ABSTRACT: Isolated angiitis of the central nervous system (IACNS) is a rare but severe vascular disease, which could present like an isolated inflammatory lesion on magnetic resonance imaging (MRI). To date, only a few such cases with tumor-like IACNS have been reported. A 35-year-old woman presented with headache and left-sided weakness. MRI scans initially mislead us to a diagnosis of glioblastoma (GBM). Surgery was performed. The mass was sub-totally resected. Pathological examination confirmed a cerebral vasculitis. Radiological features, such as disproportionate mass effect, striped hemorrhage and abnormal enhancement of adjacent vessels, could be helpful to distinguish a tumor-like IACNS from a GBM. Single therapy with high doses of steroid did not improve the patient's condition. Combined therapy with prednisolone and cyclophosphamide showed great benefit to the patient. No relapse occurred during the period of 18 months follow-up. Although a tumor-like IACNS has no established imaging features, a diagnosis of tumor-like IACNS should be suspected when MRI shows inappropriate presentations of a tumor. Greater awareness of this potential manifestation of IACNS may facilitate more prompt diagnosis and treatment.
    Full-text · Article · Aug 2011 · World Journal of Surgical Oncology
  • Source
    • "Because of various reports regarding success of corticosteroid treatment, we performed a literature review on the use of immunosuppressive agents in CAA-encephalopathy with special attention to its pathological subtypes. A total of 45 patients in 18 articles could be identified (Table 1) [4-6,8-22]. In four patients TGI and PVI co-occurred [8,15,18]. "
    [Show abstract] [Hide abstract]
    ABSTRACT: Cerebral amyloid angiopathy (CAA) is a common but often asymptomatic disease, characterized by deposition of amyloid in cerebral blood vessels. We describe the successful treatment of CAA encephalopathy with dexamethasone in a patient with CAA-related inflammation causing subacute progressive encephalopathy and seizures, which is an increasingly recognized subtype of CAA. The two pathological subtypes of CAA-related inflammation are described and a review of the literature is performed concerning immunosuppressive treatment of CAA-related inflammation with special attention to its pathological subtypes. Immunosuppressive therapy appears to be an appropriate treatment for CAA encephalopathy.
    Full-text · Article · Mar 2010 · Journal of Neuroinflammation
Show more