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[Acute benign cerebral angiopathy. 6 cases]
Insights
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.
Abstract:
The 6 cases reported here constitute, with 5 previously published cases, a special nosological entity tentatively called "acute benign cerebral angiopathy" by the authors. These 11 cases have in common certain radiological and clinical features. Arteriography shows segmental, multifocal and assymetrical stenoses involving the cerebral arteries between Willis' circle and the terminal arterioles and looking like "strings of sausages". The lesions disappear within one month and present the radiological characteristics of arteritis of medium caliber vessels. The clinical symptoms are suggestive of meningeal haemorrhage or acute cerebromeningeal oedema, with acute repetitive attacks of severe headache and agitation with obnubilation; epileptic seizures and transient neurological deficit may occur. True meningeal haemorrhage confirmed by lumbar puncture is seen in nearly one half of the cases; it seems to be due to alterations in the blood-brain barrier induced by the angiopathy. Intracerebral haematoma may develop, but the disease is usually benign and regresses spontaneously in a few days. None of the usual causes of cerebral arteritis (intra-cranial infection, collagen disease, allergic or toxic angitis) has been found. Pseudo-arteritis (notably spasm of ruptured arterial aneurysms) has been excluded. No aetiological factor common to the 11 cases reported has been elicited, although 6 of the patients had recently given birth and our 6 patients had benign virus infection before or during the clinical manifestations of the disease. In the authors' opinion, the most rewarding line of research would be the role of short acute attacks of arterial hypertension.