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CASE REPORT
Year : 2010  |  Volume : 56  |  Issue : 2  |  Page : 146-149

Tumefactive demyelinating lesion: Experience with two unusual patients


1 Department of Neurology, Chhatrapati Shahuji Maharaj Medical University, Lucknow - 226 003, India
2 Department of Radiotherapy, Chhatrapati Shahuji Maharaj Medical University, Lucknow - 226 003, India
3 Department of Neurosurgery, Chhatrapati Shahuji Maharaj Medical University, Lucknow - 226 003, India

Date of Submission16-Jan-2010
Date of Decision01-Mar-2010
Date of Acceptance14-Jun-2010
Date of Web Publication8-Jul-2010

Correspondence Address:
R K Garg
Department of Neurology, Chhatrapati Shahuji Maharaj Medical University, Lucknow - 226 003
India
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DOI: 10.4103/0022-3859.65292

PMID: 20622396

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 :: Abstract 

Tumefactive demyelinating lesion, a variant of multiple sclerosis, is a solitary large demyelinating lesion, which mimics cerebral neoplasm. Distinguishing tumefactive lesions from other etiologies of intracranial space-occupying lesions is essential to avoid inadvertent surgical or toxic chemotherapeutic interventions. We report two unusual cases of tumefactive demyelinating lesion. The first patient presented with recurrent right focal tonic-clonic seizures with secondary generalization of three-month duration. Her neurological examination was normal. Another patient presented with right homonymous hemianopia. In this patient, the diagnosis was established after biopsy of the lesion, which revealed perivascular lymphocytic infiltrate and aggregates of foam cells in white matter with relatively uninvolved grey matter, suggestive of tumefactive demyelinating lesion. Administration of intravenous methylprednisolone resulted in rapid clinical improvement in both the patients and the brain lesion decreased in size. Both, epilepsy and homonymous hemianopia, are unusual manifestations of tumefactive demyelinating lesions. In our cases, cerebral tumors were initial diagnoses. Presence of an open ring or incomplete ring lesions and other magnetic resonance characteristics helped in differentiating demyelinating lesions from other neoplastic and infective diseases of the brain. Differential diagnosis of tumefactive demyelinating lesions, at times, may prove to be a challenging task for the treating physician.


Keywords: Hemianopia, seizure, tumefactive demyelinating lesion


How to cite this article:
Sinha M K, Garg R K, Bhatt M, Chandra A. Tumefactive demyelinating lesion: Experience with two unusual patients. J Postgrad Med 2010;56:146-9

How to cite this URL:
Sinha M K, Garg R K, Bhatt M, Chandra A. Tumefactive demyelinating lesion: Experience with two unusual patients. J Postgrad Med [serial online] 2010 [cited 2014 Sep 2];56:146-9. Available from: http://www.jpgmonline.com/text.asp?2010/56/2/146/65292


The tumefactive demyelinating lesions are solitary large (greater than 2 cm) lesions with mass-like features and post-gadolinium magnetic resonance imaging (MRI) typically showing an incomplete ring enhancement. [1],[2] The clinical and imaging features of these large demyelinating lesions may mimic primary and secondary brain tumor, brain abscess, tuberculous abscess, or other inflammatory disorders (like sarcoidosis), and may require a brain biopsy for diagnosis. [3] The incidence of tumefactive demyelinating lesions has been estimated at 0.3 cases per 100 000 per year. [4] They occur more frequently in women in the second and third decades of life, with an average age of onset of at least 37 years. [5] Usual clinical manifestations are polysymptomatic and include headache, cognitive changes, mental confusion, aphasia and apraxia. [2] The usual clinical course of tumefactive demyelinating lesions is acute onset, good response to corticosteroids and no clinical or radiological evidence of new lesions in the great majority of patients. [6] The prognosis usually does not depend on the clinical presentation of the lesion. [2] Epilepsy or hemianopia are unusual manifestations of tumefactive demyelinating lesions. We are reporting to two such cases.


 :: Case Reports Top


Patient 1

A 23-year-old right-handed woman presented with recurrent episodes of right focal tonic-clonic seizures with secondary generalization of over three-month duration. Neurological examination was unremarkable. Hemogram (Hemoglobin concentration: 145 g/L, total leukocyte count: 7.2 Χ 10 9 /L with 70% neutrophils) and biochemical investigations (random blood sugar 4.9 mmol/L, serum levels of creatinine: 70 μmol/L, sodium 142 mmol/L, potassium 3.9 mmol/L and calcium 2.2 mmol/L) did not reveal any abnormalities. Anti-nuclear antibody was negative. Human immunodeficiency virus (HIV) serology was negative. Electroencephalogram was normal. MRI brain revealed a rounded well-defined lobulated T1 hypointense mass measuring about 5 cm in left fronto-parietal region, which was hyperintense in T2-weighted and fluid-attenuated inversion recovery (FLAIR) images, with a mild incomplete rim of enhancement in the post-contrast scans [Figure 1]. This was reported to be an astrocytoma elsewhere and the patient was referred for neurosurgical evaluation. Another MRI brain was performed along with proton MR spectroscopy. Repeat neuroimaging demonstrated an increase in the mass effect and edema. There was no encroachment into the adjacent grey matter. Though the mass was large in size, the surrounding edema and mass effect were disproportionately mild. Proton MR Spectroscopy revealed elevated choline peak, presence of glutamate/glutamine peak at 2.4 parts per million and suppressed N-acetyl aspartate (NAA) peak. In view of characteristic imaging appearance, the diagnosis of a tumefactive demyelinating lesion was considered. The patient was treated with intravenous infusion of methylprednisolone (1 g/d for 5 days). Oxcarbazepine in dosage of 600mg per day was given to prevent seizure recurrence. The patient remained seizure-free at 6-month follow-up visit. MRI of the brain performed after 6 months demonstrated regression of the lesion with decrease in the mass effect.

Patient 2

A 48-year-old normotensive man presented with impaired vision. Patient had been noticing difficulty in reading or working during past 1 month. Patient sought an ophthalmologist's opinion after he had a narrow escape while driving car. Detailed history of the patient did not reveal any previous ophthalmologic or neurologic deficit. The visual acuity was 6/6 in both the eyes. The pupils were 4 mm in diameter, equal and reactive to light. There was no relative afferent pupillary defect. Ophthalmoscopy revealed that the optic discs, maculae and peripheral retina were normal in both eyes. Visual field examination showed right homonymous hemianopia. Visual fields were charted with the help of Humphrey visual field analyzer using the central 30-1 and full-field programs. Rest of the neurologic examination was completely normal.

Hemoglobin was 130 g/L, total leukocyte counts 6.8 Χ 10 9 /L with 82% neutrophils, random blood sugar 5.6 mmol/L, creatinine 58 μmol/L, urea 6.1 mmol/L, sodium 144 mmol/L, potassium 4.1 mmol/L and calcium 2.4 mmol/L. Anti-nuclear antibody was negative. HIV serology was negative. Electroencephalogram was normal.

MRI of the brain revealed an irregular well-defined mass measuring 4.8 cm in the left parieto-temporal region involving splenium of corpus callosum with mass effect on adjacent structures. The lesion was hyperintense on T2-weighted and FLAIR images with incomplete rim of enhancement in the post-contrast images [Figure 2]. Initially, a diagnosis of brain tumor was made and the lesion was subjected to biopsy. Histopathology of the biopsied tissue showed moderate perivascular lymphocytic infiltrate and aggregates of foam cells in white matter with relatively uninvolved grey matter, suggestive of tumefactive demyelinating lesion. The patient was treated with methylprednisolone. After six months of follow-up, patient did not report any improvement in visual symptoms. However, a follow-up MRI of the brain demonstrated regression of the lesion with decrease in mass effect and contrast enhancement. The follow-up MRI also showed focal atrophy due to gliosis of the affected brain tissue [Figure 3].


 :: Discussion Top


Tumefactive demyelinating lesions are frequently misdiagnosed. Our patients were evaluated in neurosurgical centers and were initially diagnosed to have brain tumors. In MR study, tumefactive lesions have ill-defined margins, variable amount of cerebral edema, mass effect and there may be variable contrast enhancement of the lesions. In addition, lesions may show central necrosis, cystic degeneration and variable amount of grey matter involvement [7],[8] [Table 1]. Several neoplastic and infective diseases of brain may have similar imaging characteristics.

The first patient presented with seizures as the sole manifestation. Seizures have been reported to be present in 6% of patients with tumefactive demyelinating lesions in one series. [2] In a case series, 2 out of 18 patients with tumefactive demyelinating lesions had seizures along with hemiparesis. [9] Seizures along with hemianopia and hemiparesis was reported in an Indian child as well. [10] However, seizure as an isolated presenting feature of tumefactive demyelinating lesion has not been reported.

The second patient presented with right homonymous hemianopia as the only manifestation. Most cases of homonymous hemianopia are caused by visual cortex stroke. Homonymous visual field defects are unusual in multiple sclerosis and tumefactive demyelinating lesions. [11] In a series of 850 patients with 902 episodes of homonymous hemianopia, 629 (70%) resulted from stroke. Non-stroke causes of homonymous hemianopia included head trauma, brain tumor, neurosurgical procedures and miscellaneous conditions. In 13 (1.4%), episodes were because of multiple sclerosis. [12] Luchinetti et al, reported visual field defects along with other symptoms in 10% of their patients with tumefactive demyelinating lesions. [2] Tumefactive demyelinating lesions in patients presenting with homonymous visual field defects anatomically involve optic radiations in parietal and temporal lobes of cerebral hemispheres. [13],[14]

Distinguishing the tumefactive demyelinating lesions from neoplasm is important, since a misdiagnosis can lead to inadvertent brain irradiation or surgery. Some MR features are more suggestive of tumefactive demyelinating lesions than of brain tumor. These include incomplete rim enhancement, mixed T2-weighted iso- and hyperintensity of enhanced regions, absence of a mass effect and absence of cortical involvement. [15] A variety of enhancement patterns have been observed in patients with tumefactive demyelinating lesions. These patterns include ring (single and multiple), heterogenous, diffuse, punctate and concentric. Ring enhancement is the most frequent. Several studies have suggested that the pattern of open ring enhancement pattern is typically associated with demyelination. In a recent series, authors demonstrated complete ring enhancement (closed ring) pattern in majority of their patients with tumefactive demyelinating lesions. [2] Magnetic resonance spectroscopic metabolite information can be helpful in the differential diagnosis of tumefactive demyelinating lesions. Demonstration of elevation of the glutamate/glutamine peaks is beneficial in the differential diagnosis of tumefactive demyelinating lesions because elevation of these peaks is not seen in neoplastic diseases of the brain. [16] A patient with atypical ring-enhancing lesions and mass effect may require brain biopsy to establish the diagnosis. [1],[2],[17]

The histopathologic characteristics of tumefactive demyelinating lesions include the preservation of axons, the loss of myelin, the presence of macrophages and astrocytic proliferation. Sometimes, even histopathological features may mimic a low-grade astrocytoma because of the hypercellular nature of these lesions and the frequent presence of atypical reactive astrocytes and mitotic figures. When demyelinating lesions have a marked inflammatory component, they may also be confused with a primary central nervous system lymphoma. [2],[18]

The two cases highlight the importance of considering tumefactive demyelinating lesions in the differential diagnosis of intracranial mass lesions. Timely diagnosis can save patient from potentially harmful aggressive treatment.

 
 :: References Top

1.Dagher AP, Smirniotopoulos J. Tumefactive demyelinating lesions. Neuroradiology 1996;38:560-5.   Back to cited text no. 1  [PUBMED]    
2.Lucchinetti CF, Gavrilova RH, Metz I, Parisi JE, Scheithauer BW, Weigand S, et al. Clinical and radiographic spectrum of pathologically confirmed tumefactive multiple sclerosis. Brain 2008;131:1759-75.  Back to cited text no. 2  [PUBMED]  [FULLTEXT]  
3.Jain D, Rajesh LS, Vasishta RK, Radotra BD, Banerjee AK. Demyelinating disease simulating brain tumours: A histopathologic assessment of seven cases. Indian J Med Sci 2006;60:47-52.  Back to cited text no. 3  [PUBMED]  Medknow Journal  
4.Ragel BT, Fassett DR, Baringer JR, Browd SR, Dailey AT. Decompressive hemicraniectomy for tumefactive demyelination with transtentorial herniation: Observation. Surg Neurol 2006;65:582-3.   Back to cited text no. 4  [PUBMED]  [FULLTEXT]  
5.Comi G. Multiple sclerosis: Pseudotumoral forms. Neurol Sci 2004;25:374-9.  Back to cited text no. 5      
6.Kepes JJ. Large focal tumor-like demyelinating lesions of the brain: Intermediate entity between multiple sclerosis and acute disseminated encephalomyelitis? A study of 31 patients. Ann Neurol 1993;33:18-27.   Back to cited text no. 6      
7.Fallah A, Banglawala S, Ebrahim S, Paulseth JE, Jha NK. Case Series: Tumefactive demyelinating lesions: A diagnostic challenge. Can J Surg 2010;53:69-70.   Back to cited text no. 7  [PUBMED]  [FULLTEXT]  
8.Saindane AM, Cha S, Law M, Xue X, Knopp EA, Zagzag D. Proton MR spectroscopy of tumefactive demyelinating lesions. AJNR Am J Neuroradiol 2002;23:1378-86.  Back to cited text no. 8  [PUBMED]  [FULLTEXT]  
9.Malhotra HS, Jain KK, Agarwal A, Singh MK, Yadav SK, Husain M, et al. Characterization of tumefactive demyelinating lesions using MR imaging and in-vivo proton MR spectroscopy. Mult Scler 2009;15:193-203.  Back to cited text no. 9  [PUBMED]  [FULLTEXT]  
10.Puri V, Chaudhry N, Gulati P, Tatke M, Singh D. Recurrent tumefactive demyelination in a child. J Clin Neurosci 2005;12:495-500.  Back to cited text no. 10  [PUBMED]  [FULLTEXT]  
11.Rot U, Ledinek AH, Jazbec SS. Clinical, magnetic resonance imaging, cerebrospinal fluid and electrophysiological characteristics of the earliest multiple sclerosis. Clin Neurol Neurosurg 2008;110:233-8.   Back to cited text no. 11  [PUBMED]  [FULLTEXT]  
12.Zhang X, Kedar S, Lynn MJ, Newman NJ, Biousse V. Homonymous hemianopia in stroke. J Neuroophthalmol 2006;26:180-3.  Back to cited text no. 12  [PUBMED]  [FULLTEXT]  
13.Evangelopoulos ME, Evangelopoulos DS, Potagas C, Sfagos C. Homonymous hemianopsia as the leading symptom of a tumor like demyelinating lesion: A case report. Cases J 2009;2:9366.  Back to cited text no. 13  [PUBMED]  [FULLTEXT]  
14.Murai H, Kiyosawa M, Suzuki Y, Mizoguchi S, Ishii K, Ishikawa K, et al. A case of multiple sclerosis with homonymous hemianopia examined by positron emission tomography. Jpn J Ophthalmol 2004;48:591-3.   Back to cited text no. 14  [PUBMED]  [FULLTEXT]  
15.Kim DS, Na DG, Kim KH, Kim JH, Kim E, Yun BL, et al. Distinguishing tumefactive demyelinating lesions from glioma or central nervous system lymphoma: Added value of unenhanced CT compared with conventional contrast-enhanced MR imaging. Radiology 2009;251:467-75.  Back to cited text no. 15  [PUBMED]  [FULLTEXT]  
16.Cianfoni A, Niku S, Imbesi SG. Metabolite findings in tumefactive demyelinating lesions utilizing short echo time proton magnetic resonance spectroscopy. AJNR Am J Neuroradiol 2007;28:272-7.  Back to cited text no. 16  [PUBMED]  [FULLTEXT]  
17.Schwartz KM, Erickson BJ, Lucchinetti C. Pattern of T2 hypointensity associated with ring-enhancing brain lesions can help to differentiate pathology. Neuroradiology 2006;48:143-9.   Back to cited text no. 17  [PUBMED]  [FULLTEXT]  
18.Tan HM, Chan LL, Chuah KL, Goh NS, Tang KK. Monophasic, solitary tumefactive demyelinating lesion: Neuroimaging features and neuropathological diagnosis. Br J Radiol 2004;77:153-6.  Back to cited text no. 18  [PUBMED]  [FULLTEXT]  


    Figures

  [Figure 1], [Figure 2], [Figure 3]
 
 
    Tables

  [Table 1]

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