Primary Angiitis of the Central Nervous System and Reversible Cerebral Vasoconstriction Syndrome

Introduction: Differential diagnoses of the reversible cerebral vasoconstriction syndrome (RCVS) include all forms of intracranial stenotic disease, such as primary or secondary vasculitis of the central nervous system. Here, we tested the hypothesis that angiographic response to intra-arterial nimodipine application may be helpful in differentiating between RCVS and other entities. Methods: A digital subtraction angiographic (DSA) series of nine consecutive patients with suspected RCVS that were treated by intra-arterial nimodipine due to clinical worsening were retrospectively analyzed. Pre- and post-therapeutic DSA findings of patients with later-confirmed RCVS were compared to those in which another diagnosis was finally made. Results: Intra-arterial nimodipine resulted in a normalization of both the diameter of the main trunks of the cerebral vessels and the caliber of the peripheral vessels in all RCVS patients. This was not the case in the non-RCVS patients, in whom only a slight general vasodilatation was observed. Discussion: Our preliminary results indicate that angiographic response to intra-arterial application might be a helpful differential diagnostic tool in select patients with suspected RCVS.

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Reversible Cerebral Vasoconstriction Syndrome: An Important Cause of Acute Severe Headache

Emergency Medicine International ◽

10.1155/2012/303152 ◽

2012 ◽

Vol 2012 ◽

pp. 1-8 ◽

Cited By ~ 6

Author(s):

Li Huey Tan ◽

Oliver Flower

Keyword(s):

Central Nervous System ◽

Neurological Complications ◽

Severe Headache ◽

Reversible Cerebral Vasoconstriction Syndrome ◽

Diagnostic Features ◽

Therapeutic Implications ◽

Cerebral Vasoconstriction ◽

Primary Angiitis ◽

The Central Nervous System ◽

Acute Headache

Reversible cerebral vasoconstriction syndrome (RCVS) is an increasingly recognized and important cause of acute headache. The majority of these patients develop potentially serious neurological complications. Rigorous investigation is required to exclude other significant differential diagnoses. Differentiating RCVS from subarachnoid haemorrhage (SAH) and primary angiitis of the central nervous system (PACNS) may be difficult but has important therapeutic implications. This paper describes what is currently known about the epidemiology, pathophysiology, clinical, and diagnostic features of the syndrome, an approach to investigation, a summary of treatments, and what is known of prognosis.

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Primary angiitis of central nervous system with cephalic syndrome

Clinical Medicine (Russian Journal) ◽

10.18821/0023-2149-2017-95-6-571-575 ◽

2017 ◽

Vol 95 (6) ◽

pp. 571-575

Author(s):

Natal’ya L. Starikova ◽

E. V. Zalomova

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Corticosteroid Therapy ◽

Systemic Vasculitis ◽

Reversible Cerebral Vasoconstriction Syndrome ◽

Cerebral Vasoconstriction ◽

Primary Angiitis

A man aged 37 years experienced recurrent episodes of intensive headache. MRI revealed multiple symmetric lesions in brain. Systemic vasculitis, reversible cerebral vasoconstriction syndrome and migraine were excluded. Primary angiitis of central nervous system was diagnosed. The patient’s condition rapidly improved on corticosteroid therapy.

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