Primary Angiitis of the CNS Clinical Presentation

Updated: Nov 03, 2016
  • Author: Mazen Noufal, MD; Chief Editor: Helmi L Lutsep, MD  more...
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Presentation

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]

  • 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]

  • 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]

  • When stroke occurs in the course of PACNS, preceding or concomitant evidence usually points to widespread CNS disease. [2]

  • 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.

  • Spinal cord involvement causing weakness in lower extremities and bladder dysfunction is reported in 5-14% of patients, sometimes before or without any evidence of cerebral dysfunction. [4, 12]

  • 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]

  • 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.

  • 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]

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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]

  1. Acute or subacute encephalopathy that may progress to coma if not treated.

  2. 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.

  3. 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.

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Causes

The causes of PACNS are unknown.

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