Isolated Benign Cerebral Vasculitis

Snyder, Bruce D.; McClelland, Robert R.

doi:10.1001/archneur.1978.00500330060012

Cited by 74 publications

(26 citation statements)

References 10 publications

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“…Until recently, the only support for this concept was a limited number of anecdotal case reports of PACNS with similar clinical descriptions with benign outcome (10,11) and several analyses based on retrospective reviews of the PACNS literature documenting differential clinical and laboratory features between patients diagnosed on purely angiographic versus pathologic bases (3,4,9,12). Angiographically defined patients were more likely to be female with acute onset of presentation, along with mild to normal CSF findings and with a better outcome than the pathologically defined patients.…”

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

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“…This condition is very similar to another form of reversible angiopathy, often termed 'isolated benign ce rebral vasculitis' or 'arteritis' [6][7][8][9][10][11][12], in which inflamma tory changes are in fact lacking, so that the term 'acute benign cerebral angiopathy' [3,[13][14][15] is probably more appropriate. The patients are usually women younger than 50 years, who develop an acute neurologic dysfunc tion, which may be confused with that of a subarachnoid hemorrhage, but which is rapidly reversible; angiography shows multisegmental narrowing which resolves within a few weeks, suggesting transient vasoconstriction changes [14][15][16].…”

Section: Discussionmentioning

confidence: 99%

Postpartum Cerebral Angiopathy: Reversible Vasoconstriction Assessed by Transcranial Doppler Ultrasounds

Bogousslavsky¹,

Despland²,

Régli³

et al. 1989

Eur Neurol

139

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Postpartum cerebral angiopathy is a distinct reversible clinicoradiological syndrome which develops shortly after a normal pregnancy. It may belong to the poorly defined group of acute benign cerebral angiopathy, in which multisegmental narrowings of cerebral arteries are also reversible. In a 22-year-old woman with postpartum cerebral angiopathy, sequential transcranial Doppler ultrasounds showed that the flow disturbances began to improve 4 days after onset, and normalized 4 weeks later. A vasoconstriction response to acute severe hypertension is likely to explain this reversible angiopathy.

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“…Before the introduction of the term in 1986, benign TCH was referred to as a variety of terms or clinical circumstances such as benign vascular headache, migrainous vasospasm, crash migraine, benign sexual ('coital') headache type II, benign isolated cerebral vasculitis, and benign angiopathy of the nervous system [42][43][44][45][46][47][48][49]. Although some patients with idiopathic TCH may have a history of migraine, the clinical presentation and temporal profile of TCH is unique.…”

Section: Benign (Idiopathic) Thunderclap Headachementioning

confidence: 99%

Thunderclap headache

Dodick

2002

Current Science Inc

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Thunderclap headache refers to an excruciating headache of instantaneous onset. It occurs as suddenly and unexpectedly as a "clap of thunder." Patients with thunderclap headache may have normal neurologic examination results and normal computed tomographic brain scans, even if they have serious underlying pathology. This has created confusion regarding nosology and the nature and extent of the diagnostic evaluation, which this article discusses.

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Isolated Benign Cerebral Vasculitis

Cited by 74 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

Postpartum Cerebral Angiopathy: Reversible Vasoconstriction Assessed by Transcranial Doppler Ultrasounds

Thunderclap headache

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