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Table of Contents    
Year : 2019  |  Volume : 67  |  Issue : 3  |  Page : 856-858

Leptomeningeal metastases with subarachnoid hemorrhage mimicking primary angiitis of CNS

1 Department of Neurology, Aster Medcity, Kothad, Kochi, Kerala, India
2 Department of Neurosurgery, Aster Medcity, Kothad, Kochi, Kerala, India
3 Department of Anaesthesia, Aster Medcity, Kothad, Kochi, Kerala, India

Date of Web Publication23-Jul-2019

Correspondence Address:
Dr. Boby V Maramattom
Department of Neurology, Aster Medcity, Kothad, Kochi - 682 027, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.263252

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How to cite this article:
Maramattom BV, Ram A, Narayanan P, Jithendra T, Panikar D. Leptomeningeal metastases with subarachnoid hemorrhage mimicking primary angiitis of CNS. Neurol India 2019;67:856-8

How to cite this URL:
Maramattom BV, Ram A, Narayanan P, Jithendra T, Panikar D. Leptomeningeal metastases with subarachnoid hemorrhage mimicking primary angiitis of CNS. Neurol India [serial online] 2019 [cited 2020 Oct 30];67:856-8. Available from:


Leptomeningeal metastases (LMMs) are devastating complications of malignancies. A combination of large volume cerebrospinal fluid (CSF) examination, ventricular tapping, CSF tumor markers, flow cytometry, and contrast brain and spine magnetic resonance imaging (MRI) may be needed to prove the diagnosis.[1] A 55-year old woman presented with headache and vomiting of 1 week duration. She was a known case of locally advanced carcinoma breast treated in 2010 with surgery, chemotherapy, and radiation therapy. Seven months earlier, in March 2018, she had developed gait ataxia and was found to have a solitary cerebellar metastasis. The positron emission tomography computed tomography (PET CT) did not show any other fluorodeoxyglucose (FDG) avid lesions. She underwent a midline suboccipital craniotomy and excision of the lesion in the same month, followed by CT simulation, contouring, and radiation therapy to the postoperative bed [27.5 Gy in 5 fractions].

She was then started on tamoxifen. She had a past medical history of hypertension and diabetes mellitus. On examination she was drowsy, had neck stiffness without any focal deficits.

MRI brain with contrast 1 month postoperatively had shown postsurgical changes in the right cerebellum with minimal post-contrast enhancement.

On day 1, a new CT brain showed a small hyperdensity in the right high frontoparietal cortical sulci suggestive of subarachnoid hemorrhage (SAH). The postoperative cystic cavity noted in the right cerebellar hemisphere was noted. There were no features of hydrocephalus. The next day, she became drowsier and was noted to have bilateral abducens palsy. A repeat CT scan showed new areas of SAH in both frontal and left parietotemporal regions. Repeat CT brain over the next 2 days showed no new changes. On day 3, when she developed left focal seizures and fluctuating sensorium, she was intubated and mechanically ventilated.

MRI brain showed scattered cortical SAH over the high frontal and parietal regions with small amounts of intraventricular blood in the occipital horns. In addition, susceptibility weighted images (SWI) showed abnormal tortuous vessels over the right parasagittal area. No leptomeningeal contrast enhancement was seen. Hydrocephalus was absent [Figure 1]. Lumbar CSF examination showed xanthochromia without any abnormal cells. The protein value was 65 mg/dl and CSF sugar was normal. The opening CSF pressure was 22 cm of H2O. On day 6, a neurology consultation was obtained. Examination revealed bilateral papilloedema, abducens palsy, and fluctuating sensorium. The possibilities of disseminated intravascular coagulation, radiation vasculopathy, a mycotic aneurysm, or a leptomeningeal metastases were considered.
Figure 1: MRI SWI show [Panel a] intraventricular SAH, [b] left parietal cortical sulcal SAH, [c] abnormal tortous vessels in the parafalcine area bilaterally, and [d] high parietal sulcal SAH

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A coagulopathic work-up [including prothrombin time (PT), activated partial thromboplastin time (a PTT), D-dimer, platelet count, peripheral smear, and thromboelastography (TEG)] was normal. Other routine blood tests, blood cultures, and echocardiography were also normal. Digital substraction angiogram (DSA) was performed on day 7. Right internal carotid artery (ICA) injection showed beading and irregularities of the terminal cortical branches of the right anterior cerebral artery (ACA), terminal branches of the pericallosal artery, and the posterior temporal and parietooccipital branches of right middle cerebral artery (MCA). Injection of left internal carotid artery (ICA) showed beading of the left pericallosal ACA terminal branch [Figure 2]. In view of the posterior predilection of the vascular changes, the differential diagnosis was modified to radiation vasculopathy or leptomeningeal metastases. On day 10, a navigation-guided external ventricular drain placement and meningocortical biopsy were performed through a burr hole. A ventricular catheter was introduced under navigational guidance. The opening pressure was very high (40 cm). The CSF smear was confirmatory of leptomeningeal metastases [Figure 3]. Her family refused further treatment and she was shifted to palliative care.
Figure 2: Panel A shows “Picture in picture” view of the left ICA injection. Zoomed in view demonstrates the left ACA pericallosal artery beading. Panel B shows right ICA injection. Zoomed in view shows beading of the right MCA parietooccipital and posterior temporal branches

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Figure 3: Cytocentrifuged CSF smears showed hypercellular smears with singly scattered, tightly cohesive clusters of large atypical round to oval cells with a high nuclear-cytoplasmic ratio, vesicular chromatin, prominent nucleolus, and moderate amounts of cytoplasm. Scattered multinucleate cells and mitosis were noted. A few cells showed intracytoplasmic mucin vacuoles. The background showed numerous RBCs and few mononuclear cells and lymphocytes and monocytes

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Leptomeningeal metastases (LMMs) are now the commonest cause of malignant brain tumors in adults.[2] Breast cancer is the commonest cancer associated with LMM. Clinical features indicating LMM include a history of cancer, hydrocephalus or raised intracranial pressure (ICP), cranial neuropathies, radiculopathies, or a cauda equina syndrome. Nevertheless, the CSF yield of malignant cells is <75%. The lumbar CSF yield is higher in patients with spinal or cauda equina presentations.[1] Other useful tests include CSF flow cytometry, tumor markers, CSF metabolomic profile, ventricular CSF tapping, leptomeningeal biopsy, or PET-CT.[3] MRI may demonstrate thick or nodular leptomeningeal enhancement, hydrocephalus, or cranial nerve deposits in LMM. Even with improvements in treatment paradigms, the prognosis of LMM remains dismal with a mean survival time of 3--6 months.[4]

In primary angiitis of the central nervous system (PACNS), the MRI findings include multifocal infarcts, leptomeningeal or parenchymal enhancement, linear or lace-like blooming on susceptibility weighted imaging (SWI) with surrounding fluid attenuated inversion recovery (FLAIR) hyperintensity, and linear or central dot-like enhancement in these areas.[5],[6] Hemorrhagic infarcts, parenchymal hematoma (55%) and SAH (26%) can be seen in PACNS.[7] However, cranial or spinal SAH in LMM is very rare.[8],[9]

The sensitivity of DSA for PACNS is approximately 30%. Multifocal and bilateral beading of vessels, focal occlusions, microaneurysms or prolonged cerebral circulation times may be seen.[5] DSA is rarely performed in LMM nowadays as it has been supplanted by other modalities; however, focal basal arterial narrowing is known in LMM.[10] On DSA, the differential diagnosis of arterial beading includes PACNS, reversible cerebral vasoconstriction syndrome, gliomatosis cerebri, leptomeningeal gliomatosis, infectious arteritis, arachnoiditis, radiation vasculopathy, and severe intracranial atherosclerosis.[11],[12]

Our case provides some teaching points. LMM should be considered when DSA shows multifocal arterial beading as in PACNS. MRI findings in our case also mimicked PACNS by demonstrating SAH and abnormal SWI vessel architecture. Thirdly, ventricular CSF tapping should be considered when there is a suspicion of LMM and other diagnostic modalities are inconclusive.

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 » References Top

Chamberlain MC, Kormanik PA, Glantz MJ. A comparison between ventricular and lumbar cerebrospinal fluid cytology in adult patients with leptomeningeal metastases. Neuro Oncol 2001;3:42-5.  Back to cited text no. 1
Lin NU, Lee EQ, Aoyama H, Barani IJ, Barboriak DP, Baumert BG, et al. Response Assessment in Neuro-Oncology (RANO) group. Response assessment criteria for brain metastases: Proposal from the RANO group. Lancet Oncol 2015;16:e270-8.  Back to cited text no. 2
Yoo BC, Lee JH, Kim KH, Lin W, Kim JH, Park JB, et al. Cerebrospinal fluid metabolomic profiles can discriminate patients with leptomeningeal carcinomatosis from patients at high risk for leptomeningeal metastasis. Oncotarget 2017;8:101203-14.  Back to cited text no. 3
Nayar G, Ejikeme T, Chongsathidkiet P, Elsamadicy AA, Blackwell KL, Clarke JM, et al. Leptomeningeal disease: Current diagnostic and therapeutic strategies. Oncotarget 2017;8:73312-28.  Back to cited text no. 4
Mandal J, Chung SA. Primary angiitis of the central nervous system. Rheum Dis Clin North Am 2017;43:503-18.  Back to cited text no. 5
Harsha KJ, Jagtap SA, Kapilamoorthy TR, Kesavadas C, Thomas B, Radhakrishnan N. CNS small vessel vasculitis: Distinct MRI features and histopathological correlation. Neurol India 2017;5:1291-4.  Back to cited text no. 6
Boulouis G, de Boysson H, Zuber M, Guillevin L, Meary E, Costalat V, et al. Primary angiitis of the central nervous system: Magnetic resonance imaging spectrum of parenchymal, meningeal, and vascular lesions at baseline. Stroke 2017;48:1248-55.  Back to cited text no. 7
Chang CC, Kuwana N, Ito S, Koike Y, Kitamura H. Spinal leptomeningeal metastases of giant cell glioblastoma associated with subarachnoid haemorrhage: Case report. J Clin Neurosci 2001;8:56-9.  Back to cited text no. 8
Inci S, Bozkurt G, Gulsen S, Firat P, Ozgen T. Rare cause of subarachnoid hemorrhage: Spinal meningeal carcinomatosis. Case report. J Neurosurg Spine 2005;2:79-82.  Back to cited text no. 9
Wasserstrom WR, Glass JP, Posner JB. Diagnosis and treatment of leptomeningeal metastases from solid tumors: Experience with 90 patients. Cancer 1982;15:759-72.  Back to cited text no. 10
Maramattom BV, Giannini C, Manno EM, Wijdicks EF, Campeau NG. Gliomatosis cerebri angiographically mimicking central nervous system angiitis: Case report. Neurosurgery 2006;58:E1209.  Back to cited text no. 11
Herman C, Kupsky WJ, Rogers L, Duman R, Moore P. Leptomeningeal dissemination of malignant glioma simulating cerebral vasculitis. Case report with angiographic and pathological studies. Stroke 1995;26:2366-70.  Back to cited text no. 12


  [Figure 1], [Figure 2], [Figure 3]


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