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

Aspergillosis
Atrial myxoma with embolization to the brain
Behcet Disease
CADASIL (Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy)
Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome)
Eclampsia
Fibromuscular Dysplasia
Giant Cell Arteritis (Temporal Arteritis)
Granulomatosis with Polyangiitis (GPA, formerly Wegener Granulomatosis)
HIV-1
Henoch-Schonlein Purpura
Herpes Simplex Encephalitis
Histoplasmosis
Infectious endocarditis with septic embolization to the brain
Intravascular lymphoma
Malignant Hypertension
Meningovascular Syphilis
Metastatic CNS Neoplasms
Migraine Headache
Moyamoya Disease
Multiple Sclerosis
Neurosarcoidosis
Paraneoplastic PACNS: Hodgkin's disease
Polyarteritis Nodosa
Primary CNS Neoplasms
Rheumatoid Arthritis (RA)
Sjogren Syndrome
Systemic Lupus Erythematosus (SLE)
Tuberculosis (TB)

Workup

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Media Gallery

Lateral right internal carotid angiogram shows beading of anterior cerebral arteries (arrowheads) and beading (straight arrows), segmental dilatation (curved arrow), and narrowing of middle cerebral arteries (open arrow).
Low power view of inflamed vessel in the subarachnoid space shows fibrinoid necrosis (pink areas) superiorly.
Higher power view of small inflamed vessel in the medulla, with fibrinoid necrosis in the wall and chronic transmural inflammation.
(a) T1-weighted left parasagittal MRI showing hypointense lesions in the cortical and subcortical white matter of the left superior temporal gyrus and frontal and frontoparietal operculum with sulcal obliteration. (b) The postgadolinium MRI at this level showing nodular parenchymal and linear pial enhancement.
(a) T2-weighted axial MRI showing hyperintense lesions in the lentiform nuclei, head of the caudate nuclei and frontoparietal subcortical white matter. (b) On T1-weighted (postgadolinium) axial image, the lentiform nuclei and head of caudate nuclei are heterogeneously hypointense, while subcortical white matter lesions are isointense. Linear areas of pial enhancement are seen bilaterally.
(a) T2-weighted axial MRI showing large, well-defined hyperintense lesions in the left frontal and parietal lobes. (b) Postgadolinium T1-weighted axial MRI showing nodular parenchymal and cortical-pial enhancement of the lesion. A follow up (after 3 mo) T2-weighted axial (c) and postgadolinium (d) T1-weighted axial MRIs showing significant reduction of the lesion size and nodular enhancing areas.