A Benign Form of Isolated Angiitis of the Central Nervous System in Puerperium: An Identical Disorder to Postpartum Cerebral Angiopathy?

Sugiyama, Yoichiro; Muroi, Akiko; Ishikawa, Masahiko; Tsukamoto, Tetsuro; Yamamoto, Toshiyuki

doi:10.2169/internalmedicine.36.931

Cited by 18 publications

(12 citation statements)

References 10 publications

(6 reference statements)

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“…First, our case series closely resembles, clinically and angiographically, similar case studies that have been reported in association with other disorders linked to reversible vasoconstriction. These include vasculopathy in the postpartum period (15)(16)(17)(18), vasculopathy associated with pheochromocytoma (19), and that associated with the use of sympathomimetic drugs (20 -32). In each of these examples, the clinical picture is characterized by similar angiographic findings, benign spinal fluid, and a monophasic course.…”

Section: Discussionmentioning

confidence: 99%

Benign angiopathy of the central nervous system: Cohort of 16 patients with clinical course and long‐term followup

Hajj‐Ali

Furlan

Abou-Chebel

et al. 2002

Arthritis & Rheumatism

219

169

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Objective. Benign angiopathy of the central nervous system (BACNS) is a subset of primary angiitis of the central nervous system (PACNS) with favorable outcome, not requiring prolonged immunosuppression. We retrospectively studied the clinical characteristics and prospectively assessed long-term outcome in a cohort of BACNS patients. Methods. Patients meeting the clinical description for BACNS, evaluated and treated by a single investigator, were included. Data on demographics, signs and symptoms, laboratory studies, neuroimaging, brain biopsy, treatment, and complications were recorded. The long-term assessment included a phone interview utilizing the Barthel index and a specifically designed cognitive index. Results. Sixteen cases met the inclusion criteria. Mean age was 40 years (range 10 -66 years) with female:male ratio of 4.3:1 and a mean followup period of 35 months (0 -128 months). Headache was the most common presenting symptom, seen in 88% of the cases, followed by focal symptoms (63%), and diffuse symptoms (44%). All patients had highly abnormal cerebral angiography, and magnetic resonance imaging abnormalities were present in 77% (10 of 13). Severe cerebral spinal fluid (CSF) abnormalities were present in 1 patient (7%). All followup cerebral angiography showed marked improvement. Recovery was demonstrated in 94% of the patients, 6% relapsed, and there were no deaths. Of patients assessed by the Barthel index, 71% showed no disability and 29% had mild disability. Conclusion. There is a PACNS subset characterized by acute presentation (most commonly headache), normal to mildly abnormal CSF findings, female predominance, and highly abnormal cerebral angiography (reversible after treatment), requiring less intensive treatment than has been traditionally used.

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Section: Discussionmentioning

confidence: 99%

Benign angiopathy of the central nervous system: Cohort of 16 patients with clinical course and long‐term followup

Hajj‐Ali

Furlan

Abou-Chebel

et al. 2002

Arthritis & Rheumatism

219

169

View full text Add to dashboard Cite

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“…In 50-70% of cases, it is associated with the intake of vasoconstrictors, mostly ergot alkaloids, which are commonly used to treat postpartum hemorrhage [5,6]. Ducros et al reported that eclampsia/ preeclampsia, selective serotonin reuptake inhibitors, ergotamine tartrate, methergine, and bromocriptine are causes of RCVS [5].…”

Section: Discussionmentioning

confidence: 99%

“…It demonstrates multifocal segmental narrowing and dilatation (string of beads) in large and medium-sized cerebral arteries [2][3][4][5][6][7][8]. Ducros et al reported that noninvasive angiography (MRA or CT angiography) was 80% sensitive in their series compared with the gold standard of catheter angiography [5].…”

Section: Discussionmentioning

confidence: 99%

“…Postpartum Cerebral Angiopathy (PCA) is a reversible clinicoradiological syndrome, characterized by the acute onset of severe headache, focal neurologic deficits, and reversible cerebral segmental vasoconstriction [2][3][4][5][6]. It is diagnosed by angiography, which demonstrates multifocal segmental narrowing in large and mediumsized cerebral arteries, with a similar appearance to vasculitis [2][3][4][5][6][7][8].…”

Section: Introductionmentioning

confidence: 99%

“…It is diagnosed by angiography, which demonstrates multifocal segmental narrowing in large and mediumsized cerebral arteries, with a similar appearance to vasculitis [2][3][4][5][6][7][8]. The process is generally self-limiting; with the resolution of angiographic abnormalities within 4-12 weeks and typically complete resolution of symptoms [1,4].…”

Section: Introductionmentioning

confidence: 99%

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A Case of Postpartum Cerebral Angiopathy

Kurimoto¹

2014

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Primary central nervous system vasculitis mimicking brain tumour: case report and literature review

Khan

Liu

2009

Rheumatol Int

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Primary central nervous system vasculitis (PCNSV) is a rare inflammatory disease causing significant morbidity and mortality. We present a detailed history and clinical course of a patient with PCNSV along with a literature review. A 50-year-old Chinese female presented with a 6-month history of mild to moderate headache and sudden onset of visual loss. Early computed tomography of the brain showed multifocal infarctions. Over 3 weeks after admission, her clinical presentations changed considerably and her condition deteriorated. She became confused then fell in to semicoma, and developed left-sided paralysis and quadriparesis. At about 7 months after her illness, a magnetic resonance imaging of the brain revealed a tumour-like mass lesion within the right parietal lobe resulting in compressed posterior horn of the right lateral ventricle. In the end, a brain biopsy identified extensive vessel inflammation, which established the diagnosis of PCNSV. Steroid and immunosuppressive therapy dramatically improved the patient's condition. Our report highlights the importance of considering PCNSV as a differential diagnosis in patients who are suspected with encephalitis, multiple sclerosis, cerebral haemorrhage, cerebral thrombus, leukoencephalopathy, or brain tumour, etc. while their clinical presentations are exceptionally so changeable. This case also gives emphasis to the value of a brain biopsy in consolidating the diagnosis, and the efficacy of steroid and immunosuppressive therapy in PCNSV.

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A Benign Form of Isolated Angiitis of the Central Nervous System in Puerperium: An Identical Disorder to Postpartum Cerebral Angiopathy?

Cited by 18 publications

References 10 publications

Benign angiopathy of the central nervous system: Cohort of 16 patients with clinical course and long‐term followup

Benign angiopathy of the central nervous system: Cohort of 16 patients with clinical course and long‐term followup

A Case of Postpartum Cerebral Angiopathy

Primary central nervous system vasculitis mimicking brain tumour: case report and literature review

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