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Neurology India, Vol. 58, No. 1, January-February, 2010, pp. 147-149 Letter To Editor Primary angiitis of central nervous system: Tumor-like lesion R. Shiva Kumar, Atampreet Singh, Chaturbhuj Rathore, Chandrasekharan Kesavadas1 1 Departments of Neurology and Imaging Sciences and Interventional Radiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India. Correspondence Address: Chaturbhuj Rathore, Departments of Neurology and Imaging Sciences and Interventional Radiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, cbrathore@rediffmail.com Date of Acceptance: 30-Nov-2009
Code Number: ni10039 DOI: 10.4103/0028-3886.60417 Sir, A 44-year-old lady presented with progressive dysarthria, right hemiparesis and mild headache of four months duration without any associated systemic symptoms. After magnetic resonance imaging [MRI] of brain and a stereotaxic biopsy at another center, she was diagnosed to have tumefactive demyelination and was treated with pulse dose of methyl prednisolone followed by oral prednisolone (1 mg/kg/day). Due to continued worsening of the neurological state, she was referred to our center. She had global, predominantly posterior aphasia, right hemiparesis (Grade 4/5) and early papilledema. A previous MRI scan showed a heterogeneous, irregular mass lesion involving cortical and subcortical areas over the left parietal, posterior temporal and insular regions with intralesional hemorrhages and mass effect along with a few areas of restricted diffusion within the lesion [Figure - 1]a-f. Investigations for systemic disorders and vasculitis were negative. On the second day of admission, her sensorium deteriorated and weakness progressed rapidly (MRC Grade 2/5). A repeat MRI showed expansion of the lesion and massive intralesional hemorrhages [Figure - 2]a-c. Magnetic resonance spectroscopy MRS revealed normal choline and reduced N-acetylyaspartate NAA levels [Figure - 2]d with a small area of restricted diffusion within the lesion [Figure - 2]e-f. Digital subtraction angiogram only showed evidence of mass effect without any features of cerebral vasculitis [Figure - 2]h. She underwent urgent decompressive surgery and biopsy of the lesion. Neuropathology showed acute lymphocytic vasculitis predominantly involving small-sized vessels [Figure - 3]d. Staining and culture for mycobacteria and fungi were negative. She made a steady improvement with monthly pulse dose of cyclophosphamide (1 mg/ m 2 ) along with oral steroids. At 18 months of follow-up, she had minimal difficulty in comprehension and right hemiparesis [Grade 4, [Figure - 3]a-c]. Primary angiitis of central nervous system vasculitis (PACNS) is a rare inflammatory disorder, affecting small and medium-sized vessels of the brain and spinal cord. As the prognosis is universally poor in the absence of specific therapy, early diagnosis and treatment is essential. Diagnosis is often delayed, due to the extremely heterogeneous clinical presentations and nonspecific MRI features. [1] PACNS presenting as a CNS mass lesion is extremely rare and usually not considered in the differential diagnosis of mass lesions. Approximately 3-5% cases with PACNS can present as CNS mass lesions. [2] These cases probably represent a fulminant form of focal vasculitis and apart from a higher association with amyloid angiopathy, do not differ from more typical cases. [2] Though usually nonspecific, MRI features like involvement of both the grey and white matter, patchy contrast enhancement, intralesional hemorrhages and normal choline with reduced N-acetyl aspartate (NAA) on magnetic resonance spectroscopy (MRS) may help in differentiating it from other mass lesions like tumefactive demyelination or neoplasms. [3] Biopsy, though mandatory and confirmatory, may be falsely negative due to the patchy focal vasculitis process, as was the case in our patient during initial biopsy. [1] A high degree of suspicion along with careful evaluation of the clinical, MRI and pathological data is required to differentiate it from potential mimickers and for timely diagnosis and treatment. Early recognition is also important, as tumor-like vasculitis usually responds poorly to steroids, and early aggressive immunosuppressive therapy with cyclophosphamide may help in improving the outcome. [1],[2] References
Copyright 2010 - Neurology India The following images related to this document are available:Photo images[ni10039f1.jpg] [ni10039f2-3.jpg] |
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