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[Acute benign cerebral angiopathy. 6 cases]
Presse Medicale (Paris, France : 1983)
|October 8, 1983
Summary
Acute benign cerebral angiopathy is a distinct neurological condition characterized by reversible arterial stenosis. This rare disorder presents with severe headaches and neurological deficits, often mimicking subarachnoid hemorrhage.
Area of Science:
- Neurology
- Vascular Neurology
Background:
- A novel nosological entity, tentatively named "acute benign cerebral angiopathy," is proposed based on 11 reported cases.
- This condition shares specific radiological and clinical features distinct from other cerebral arteritis forms.
Observation:
- Radiological findings include segmental, multifocal, and asymmetrical stenoses in cerebral arteries, resembling "strings of sausages."
- Clinical presentation involves severe headaches, agitation, obnubilation, seizures, and transient neurological deficits, often suggesting meningeal hemorrhage or cerebral edema.
Findings:
- Lesions demonstrate radiological characteristics of medium-caliber vessel arteritis, with spontaneous regression within one month.
- Cerebral angiopathy is linked to alterations in the blood-brain barrier, potentially causing meningeal hemorrhage in nearly half of cases.
- While intracerebral hematomas can occur, the disease is typically benign and self-limiting.
Implications:
- The etiology remains elusive, with no common factors identified, though recent childbirth and viral infections were noted in some patients.
- Further research into the role of acute arterial hypertension is recommended.
- Distinguishing this entity from other forms of cerebral vasculitis is crucial for appropriate management.