Primary angiitis of the central nervous system mimicking dissemination from brainstem neoplasm: a case report

Nabika, Shinya; Kiya, Katsuzo; Satoh, Hideki; Mizoue, Tatsuya; Araki, Hayato; Oshita, Jumpei; Nishisaka, Takashi; Kurisu, Kaoru; Sakamoto, Kazuhiko

doi:10.1016/j.surneu.2007.05.008

Cited by 14 publications

(11 citation statements)

References 11 publications

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“…[3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21] Focal deficit was the most frequent presenting symptom in 12 (54%) patients, followed by headaches in 11 (50%). Imaging revealed a single mass in 13 (62%) patients, supratentorial in 11 4, 6-10, 13, 17, 19, 20 and involving the spinal cord in two.…”

Section: Data From the Literaturementioning

confidence: 99%

Tumor-Like Presentation of Primary Angiitis of the Central Nervous System

Boysson

Boulouis

Dequatre

et al. 2016

Stroke

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Background and Purpose— We aimed to describe the clinical and imaging features of patients with tumor-like presentation of primary angiitis of the central nervous system. Methods— We retrospectively analyzed 10 patients enrolled in the French primary angiitis of the central nervous system cohort, who initially presented tumor-like brain lesions and compared them with other patients within the cohort. Results— The 10 patients with tumor-like presentation in the cohort were younger and had more seizures at diagnosis than the other 75 patients (median of 37 [30–48] years versus 46 [18–79] years; P =0.008; 9 [90%] with seizures versus 22 [29%], P <0.001; respectively). All 10 patients had a biopsy (stereotactic procedure in 7 and open-wedge surgery in 3). Histological findings suggestive of vasculitis were observed in 9 patients in whom conventional cerebral angiography and magnetic resonance angiography were negative. In the remaining patient, vascular imaging demonstrated diffuse bilateral large- and medium-sized vessel involvement (biopsy did not reveal vasculitis). All patients with tumor-like presentation received glucocorticoids, combined with cyclophosphamide in 9 cases. With a median follow-up of 27 (12–130) months, 5 (50%) patients relapsed, but achieved remission again after treatment intensification. Conclusions— Patients with tumor-like presentation of primary angiitis of the central nervous system represent a subgroup characterized with mainly small-sized vessel disease that requires histological confirmation because vascular imaging is often normal. Although relapses are not uncommon, global outcomes are good under treatment with glucocorticoids and cyclophosphamide.

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Section: Data From the Literaturementioning

confidence: 99%

Tumor-Like Presentation of Primary Angiitis of the Central Nervous System

Boysson

Boulouis

Dequatre

et al. 2016

Stroke

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“…5,8,15) Single or multiple mass lesions are also known in PACNS. 8,11,12,19) Approximately 5% of cases with PACNS present as a tumor-like mass lesion. 11) One of the mechanisms of formation of mass lesion is breakdown of the blood-brain barrier of the small vessels by infiltration of inflammatory cells in the perivascular and parenchymal regions, resulting in the mass mimicking enhanced lesion.…”

Section: Discussionmentioning

confidence: 99%

Primary Angiitis of the Central Nervous System Mimicking Tumor-Like Lesion -Case Report-

Tanei

Nakahara

Takebayashi

et al. 2011

Neurol. Med. Chir.(Tokyo)

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A 60-year-old man presented with a rare case of primary angiitis of the central nervous system mimicking a tumor-like lesion and manifesting as slight disorientation, left hemiparesis, and motor aphasia. Computed tomography showed multiple low density lesions in the left frontal lobe, brain stem, and right parietal lobe. Magnetic resonance images revealed a slightly enhanced mass lesion in the right parietal lobe with surrounding brain edema. Serum, cerebrospinal fluid, and other image examinations did not show any abnormal findings, so surgical removal of the right parietal mass was performed. Histological examination revealed that the mass consisted of hemorrhagic infarction without cellular atypia. Proliferations of endothelial cells in small and medium arteries, and infiltration of macrophages in the perivascular space were detected in the infarction tissues. The histological diagnosis was primary angiitis of the central nervous system.

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“…In all subjects rheumatoid factor, antinuclear antibodies, anticardiolipin antibodies, antineutrophil cytoplasm antibodies and lupus anticoagulant were negative. A CSF examination was performed in all patients and revealed a mild increase in the leucocyte count and/or total protein concentration in six cases (n. 1,2,4,5,6,8). Combined MRI abnormalities and angiographic findings suggested PACNS.…”

Section: Case Seriesmentioning

confidence: 97%

“…Since then, single cases or a small number of case studies have been reported. In particular, cases of PACNS were described for their atypical clinical and/or neuroradiological presentation mimicking gliomatosis cerebri, brain tumor or CADASIL [4][5][6]. In collecting a series of cases, authors described their own way of managing PACNS.…”

Section: Introductionmentioning

confidence: 99%