Primary Angiitis of the CNS Differential Diagnoses

Updated: Nov 10, 2021
  • Author: Mazen Noufal, MD; Chief Editor: Helmi L Lutsep, MD  more...
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Diagnostic Considerations

Benign angiopathy of the CNS

Among the cases of primary CNS angiitis reported in the literature, many describe patients diagnosed based on cerebral angiography alone without pathological confirmation. These patients had relatively different clinical features and better outcomes than those with pathological diagnoses of PCNSA.

In 1993, Calabrese and colleagues proposed the term benign angiopathy of the CNS (BACNS) to define this subset of cases, the term angiopathy reflects the unconfirmed pathology of the disease and suggests that the angiographic features may be the result of a mechanism other than vascular inflammation, most likely reversible vasoconstriction. An alternative name that has become more commonly used in literature for the disorder is Reversible Cerebral Vasoconstriction Syndrome (RCVS), or Call-Fleming syndrome. [16]

On cerebral angiogram, findings suggesting high probability for angiopathy of the CNS are defined as alternating areas of stenosis and/or ectasia, or vascular beading, in more than one vascular bed. [17]

In a published case series of BACNS (16 patients), 13 were women (83%), with a mean age at diagnosis of 40 years. Acute severe headache was the presenting symptom in 14 patients (88%); 10 patients (63%) had focal neurologic manifestations like stroke, seizures and visual disturbance. Diffuse symptoms such as change of cognition and consciousness occurred in 7 patients (44%).

In the same series, the cerebral spinal fluid (CSF) was examined in 14 patients and was normal in 13. Brain biopsy in 2 patients showed no pathological findings. Associated conditions included the postpartum state, migraine, and use of sympathomimetic drugs like pseudoephedrine and phenylpropanolamine. Although CNS hemorrhages (subarachnoid and/or intracerebral) occur in BACNS, they are felt to be secondary to the vasculopathy, rather than the cause of it. Most patients were treated with a short course of steroids, and calcium channel blockers. All had either complete recovery or improvement with mild-to-moderate residual deficits. No deaths were reported during the follow-up of 128 months. Repeat angiography or MR angiography in 10 patients showed marked improvement in the previously described CNS vascular abnormalities. [17]

Patients with RCVS are usually middle-aged women with an acute or hyperacute presentation (usually featuring severe headache), an abnormal cerebral angiogram, and normal CSF examination. Brain biopsy, when obtained, should be negative for vasculitis. Multiple medications including sympathomimetics, selective serotonine reuptake inhibitors (SSRIs), and chemotherapeutic agents have been linked to RCVS.

Postpartum CNS angiopathy

This condition describes women in the first 2 weeks after delivery with similar clinical and angiographic features of those with benign angiopathy of the CNS. A possible relationship exists with eclampsia and ergot or bromocriptine administration. Treatment is with a short-term course of steroids and calcium channel blockers, with favorable outcomes.

Differential Diagnoses