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|Year : 2012 | Volume
| Issue : 2 | Page : 162-163
A puzzling case of treatable dementia
SA Jabeen, AK Meena, KR Mridula, R Borgohain
Department of Neurology, Nizam's Institute of Medical Sciences, Hyderabad, Andhra Pradesh, India
|Date of Web Publication||14-Jun-2012|
S A Jabeen
Department of Neurology, Nizam's Institute of Medical Sciences, Hyderabad, Andhra Pradesh
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Jabeen S A, Meena A K, Mridula K R, Borgohain R. A puzzling case of treatable dementia. J Postgrad Med 2012;58:162-3
Identifying treatable causes of dementia is important as it can be cured completely with treatment of the underlying etiology. We report a rare case of rapidly progressive dementia, caused by necrotizing neurosarcoidosis, which was treated successfully.
A 50-year-old goldsmith presented with bifrontal headache and progressive memory loss of two months' duration, with single generalized seizure. There were no cranial nerves, motor or sensory symptoms. His past and personal history was unremarkable. General and other systemic examinations were normal. Cognitive assessment revealed impaired attention, concentration, memory and calculation. The mini mental state examination (MMSE) score was 16/30. There were no other deficits. Meningeal signs were absent.
Routine investigations were normal. Magnetic resonance imaging (MRI) brain showed multiple nodular enhancing meningeal granulomas at the skull base [Figure 1]. Cerebrospinal fluid (CSF) analysis revealed elevated protein (385 mg/dl); normal sugar (48 mg/dl) and 14 lymphocytes. There were no malignant cells.
|Figure 1: (a) MRI brain flair axial images showing hyperintensities involving gray matter abutting meninges in basifrontal and temporal lobes. (b) MRI brain gadolinium.enhanced T1.weighted images showing multiple small meningeal granulomas at skull base|
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This patient presented with a clinical picture of rapidly progressive dementia and headache. Imaging and CSF analysis were suggestive of chronic granulomatous meningitis (CGM), usually caused by meningeal infection with bacteria, fungi and parasites, or by inflammatory diseases like sarcoidosis and Wegener's granulomatosis. 
Further workup was done to establish the etiology. Computed tomography (CT) scan of thorax showed mild hilar adenopathy. Serology was negative for vasculitis including cytoplasmic antineutrophilic cytoplasmic antibodies (cANCA). CSF culture was sterile. Serum and CSF venereal disease research laboratory (VDRL) tests were negative. Angiotensin-converting enzyme (ACE) level in CSF was elevated [7.4 U/l (normal <1.5U/l)] and serum was normal [30 U/l (normal < 40U/L)]. Corrected serum calcium was 9.8 mg/dl. Mantoux test was negative
As the above investigations were inconclusive, meningeal biopsy was performed which showed well-formed necrotizing noncaseating granulomas similar to necrotizing sarcoid granulomas first described by Leibow.  Stains for acid-fast bacilli and fungi were negative. There was no evidence of vasculitis. The adjacent brain parenchyma was normal [Figure 2].
|Figure 2: a) Meningeal biopsy showing well-formed epithelioid cell granulomas with central necrosis (H & E ×200). b) Higher magnification of the same granulomas showing necrosis and peripheral mantle of|
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Smears from biopsied specimen showed no organisms. Cultures for mycobacteria, fungi and other fastidious organisms were negative.
Since histopathology revealed necrotizing granulomas, we carefully excluded infection and Wegener's granulomatosis by the above tests. Based on the compatible clinical and imageological picture and after careful exclusion of other etiologies mentioned above, a diagnosis of definite neurosarcoidosis was made using criteria by Zajicek et al.  Presence of necrosis is unusual in neurosarcoidosis but few cases are reported in the literature.  Hence a diagnosis of neurosarcoidosis with necrotizing sarcoid granulomatosis (NS-NSG) was made.
The patient was started on oral prednisolone 60 mg per day for six weeks, and then tapered to 20 mg per day. After one month there was significant improvement. At three months his cognitive functions improved markedly in all domains with MMSE score of 25/30. A repeat MRI of brain showed complete resolution of granulomas [Figure 3].
|Figure 3: Repeat MRI brain flair axial (a) and gadolinium- enhanced T1-weighted (b) images showing marked resolution in granulomas with steroid therapy|
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To our knowledge this is the first case of NS-NSG presenting with dementia with well-demonstrated radiological resolution like in other cases of neurosarcoidosis.  This case illustrates the importance of careful evaluation for treatable causes of dementia in clinical practice.
| :: References|| |
|1.||Finsterer J, Kladosek A, Nagelmeier IE, Becherer A, Matula C, Stradal KH, et al. Chronic granulomatous meningitis with multiple cranial nerve lesions, hydrocephalus, stroke, sinus thrombosis and epilepsy. South Med J 2000;93:1108-11. |
|2.||Liebow AA. The J. Burns Amberson lecture-Pulmonary angiitis and granulomatosis. Am Rev Respir Dis 1973;108:1-18. |
|3.||Zajicek JP, Scolding NJ, Foster O, Rovaris M, Evanson J, Moseley IF, et al. Central nervous system sarcoidosis - diagnosis and management. QJM 1999;92:103-17. |
|4.||Strictland-Marmol LB, Fessler RG, Rojiani AM. Necrotizing sarcoid granulomatosis mimicking an intracranial neoplasm: Clinicopathologic features and review of the literature. Mod Pathol 2000;13:909-3. |
|5.||Ginat DT, Dhillon G, Almast J. Magnetic Resonance Imaging of Neurosarcoidosis. J Clin Imaging Sci 2011;1:15. |
[Figure 1], [Figure 2], [Figure 3]
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