History
Mode of onset, presenting symptoms, and the course of primary angiitis of the CNS (PACNS) are widely varied, with almost all neurologic signs and symptoms having been reported. [2]
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In a review of 68 patients with a pathological diagnosis from different series, the most common symptoms on presentation were nonfocal, such as headache (58%) and altered mental status (47%). Localizing symptoms frequently seen on presentation were lateralized weakness (32%), aphasia (14%), ataxia (14%) and nonspecific visual complaints (17%). Seizures were among the presenting symptoms in about 15% of patients. [2]
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Although tissue injury from vasculitis is ischemic, PACNS is extremely rare among unselected patients with ischemic stroke. In a retrospective review of more than 4000 cases of stroke in one center over a period of 19 years, only 6 cases (0.15%) of first ischemic stroke were caused by PACNS. [11]
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When stroke occurs in the course of PACNS, preceding or concomitant evidence usually points to widespread CNS disease. [2]
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Hemorrhagic stroke is reported in approximately 11% of patients, with intracerebral bleed most common [4] , usually in the absence of precipitating factors (hypertension, trauma, or coagulopathy). Subarachnoid hemorrhage and spinal subdural hematoma have also been reported.
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In the review of 68 patients with pathological diagnosis, the diagnosis was made more than 6 months after the onset of symptoms in 54% of cases. [2]
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The course is usually progressive, producing a clinical picture of multifocal neurologic dysfunction; however, about 15% of patients have fluctuating and relapsing-remitting symptoms early in the course of the disease.
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Seizures, including intractable status epilepticus, headache, and cognitive decline are common presenting features in children with negative-angiogram small-vessel primary angiitis of the CNS. [13]
Constitutional symptoms commonly seen in systemic vasculitides like fever, fatigue, and weight loss are not usually prominent in PACNS. Only 13% of 31 patients in a recent report had constitutional symptoms. [14]
Physical
Possible findings on neurologic examination are broad, depending on the affected areas in the CNS. Common findings are hemiparesis, impaired cognition or decreased level of consciousness, aphasia, ataxia, and paraparesis. [2]
Despite the diversity of signs and symptoms, Scolding and colleagues recognized 3 broad categories of clinical patterns: [15]
Acute or subacute encephalopathy that may progress to coma if not treated.
A picture of relapsing remitting neurologic deficits similar to multiple sclerosis, but with other symptoms uncommon in multiple sclerosis such as severe persistent headache and seizures.
A picture suggestive of rapidly progressive space-occupying lesion with headache and focal neurologic deficits.
Careful general physical examination is important to detect signs of diseases that may cause a secondary CNS angiitis. Skin rash, purpura, and arthritis are seen in many systemic vasculitides like polyarteritis nodosa and Churg Strauss disease. Wheezing is particularly common in Churg Strauss disease, while sinusitis is common in Wegener granulomatosis. Painful genital and mouth ulcers or uveitis are seen in Behcet disease. Lymphadenopathy and arthritis might be seen in sarcoidosis.
Complications
Complications of primary angiitis of the central nervous system (PACNS) are nonspecific. Patients who are immobile due to neurologic deficits are at higher risk for pneumonia, decubitus ulcers, and deep venous thrombosis (DVT).
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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).
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Low power view of inflamed vessel in the subarachnoid space shows fibrinoid necrosis (pink areas) superiorly.
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Higher power view of small inflamed vessel in the medulla, with fibrinoid necrosis in the wall and chronic transmural inflammation.
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(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.
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(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.
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(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.
