Pearls & Oy-sters: Paraneoplastic cerebral vasculitis

Abstract

Pearls cConvexity subarachnoid hemorrhage (SAH) represents 6% to 7% of all cases of subarachnoid hemorrhage, with cerebral amyloid angiopathy and reversible cerebral vasospasm accounting for 55% to 70% of all cases. Oy-sters cConvexity SAH due to cerebral vasculitis of paraneoplastic origin is a rare entity, the exact incidence of which remains undetermined. Case reportA 47-year-old woman was apparently of normal health until 10 days before presentation to our emergency services when she developed sudden-onset, severe holocranial headache and vomiting followed 6 days later by altered sensorium. There was no history of fever or any other systemic complaints. She was admitted and evaluated at another center and was later referred to our hospital. On examination, she had lymphadenopathy in the left cervical, supraclavicular, and left axillary regions. There was a 4 × 3 cm, hard, nontender, ulcerating mass in her left breast. She was in altered sensorium (Glasgow Coma Scale score E3M4V2) with neck rigidity and positive Kernig sign. She had palsy of the left seventh nerve, right third nerve, and bilateral sixth nerves. Deep tendon reflexes were brisk, and plantar responses were bilaterally extensor. Based on history and examination findings, the possibility of carcinomatous meningitis with hemorrhagic leptomeningeal metastases was considered and she was evaluated. Routine hemogram, biochemical investigations, and coagulogram were normal. She had a noncontrast CT scan of the brain done elsewhere, which revealed the presence of bilateral high parietal sulcal SAH (figure, A). CT angiography did not reveal any evidence of aneurysm or arteriovenous malformation. MRI scan of the brain showed SAH in bilateral parieto-occipital regions (figure, B and C), and magnetic resonance venography was normal. In view of the clinical picture and the above investigational profile, an alternative possibility of paraneoplastic reversible cerebral vasospasm with convexity SAH was also considered, and she underwent diagnostic digital subtraction angiography. This revealed extensive and multifocal narrowing of small-sized vessels suggestive of either a vasospasm or cerebral vasculitis ( figure, D). Meanwhile, a CSF examination revealed the presence of malignant cells on cytocentrifugation, confirming the diagnosis of neoplastic meningitis. She was started on IV methylprednisolone for symptomatic relief. Further radiotherapy and chemotherapy including intrathecal methotrexate were planned. However, she progressively deteriorated to Glasgow Coma Scale score 7/15 by day 2 of admission for which she was intubated and given ventilator support. A Tru-Cut biopsy of the breast lesion was performed, which was suggestive of a grade III ductal infiltrating carcinoma. Repeat brain MRI showed resolving SAH and extensive bilateral diffusion restriction suggestive of infarcts in the brain with spinal metastases. She succumbed on day 4 of admission. She underwent an autopsy, which revealed a dense patch of SAH along the inferior surface of the left cerebell...

2021 Guideline for the Prevention of Stroke in Patients With Stroke and Transient Ischemic Attack: A Guideline From the American Heart Association/American Stroke Association

“…Conditions in this category are rare and include lymphomatoid granulomatosis and angiotropic or intravascular lymphoma (also known as angioendotheliomatosis) 736 among other even less common disorders. 737,738 Treating the underlying malignancy also reduces the risk of recurrent stroke. The relatively rare occurrence of neoplastic vasculitis is a challenge for the systematic study of the disease.…”