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Year : 2008  |  Volume : 56  |  Issue : 2  |  Page : 221-222

MIR in atypical idiopathic inflammatory demyelinating disease treated with methylprednisolone and cyclophosphamide

1 Department of Neurology, Kamillus-Klinik, Asbach, Germany
2 Radiologische Gemeinschaftspraxis, Bad Honnef, Germany

Correspondence Address:
U Kallweit
Department of Neurology, Kamillus-Klinik, Asbach
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.42018

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How to cite this article:
Kallweit U, Pohlau D, Pauleit D, Harzheim M. MIR in atypical idiopathic inflammatory demyelinating disease treated with methylprednisolone and cyclophosphamide. Neurol India 2008;56:221-2

How to cite this URL:
Kallweit U, Pohlau D, Pauleit D, Harzheim M. MIR in atypical idiopathic inflammatory demyelinating disease treated with methylprednisolone and cyclophosphamide. Neurol India [serial online] 2008 [cited 2021 Sep 17];56:221-2. Available from:

The differential diagnosis of multi-focal enhancing lesions in cranial magnetic resonance imaging (MRI) is often difficult and includes a wide spectrum of differential diagnoses including atypical idiopathic inflammatory demyelinating lesions. [1],[2] We report an 18-year-old woman who developed severe multi-focal neurological symptoms within few weeks presenting with somnolence, dysarthria, asymmetric tetraparesis and moderate ataxia. The cerebrospinal fluid (CSF) analysis revealed a mild pleocytosis (17/3 mononuclear cells), a dysfunction of the blood brain barrier and oligoclonal bands without corresponding findings in the serum. The MRI on the day of admission showed large, partly confluent lesions with perifocal edema, T1-hypo-intensity as a correlate of severe tissue damage as well as concentric gadolinium enhancing lesions [Figure 1a]. Immediately, high-dose methylprednisolone (2000 mg/day on seven following days) and high-dose cyclophosphamide (1000 mg = 600 mg/m 2 ) were administered intravenously. A second cyclophosphamide pulse of identical dosage was performed exactly one week later. The MRI on day 7 showed a decrease of mass effect and edema, of lesion volume and especially of gadolinium enhancement [Figure 1b]. Thirteen days after the initiation of the therapy neurological examination revealed mild psychomotor deficit and a latent left-sided hemiparesis. Although the clinical course seemed to be stabile six weeks later, once more 5 x 2 g methylprednisolone and 1 g cyclophosphamide was administered because brain MRI revealed new partly confluent and gadolinium enhancing lesions [Figure 1c]. After 17 months a nuclear facial palsy occurred and brain MRI showed a gadolinium enhancing lesion in the brainstem [Figure 1d]. Due to clinical, laboratory and MRI findings, multiple sclerosis (MS) with dramatic multi-focal onset mimicking a Marburg type of the disease was diagnosed. [3],[4] The further clinical course was relapsing-remitting. Frequent clinical and MRI controls are essential for diagnosis and effects of treatment in malignant forms of idiopathic inflammatory demyelinating diseases.

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1.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. 1    
2.Seewann A, Enzinger C, Filippi M, Barkhof F, Rovira A, Gass A, et al. MRI characteristics of atypical idiopathic inflammatory demyelinating lesions of the brain: A review of reported findings. J Neurol 2008;255:1-10.  Back to cited text no. 2    
3.Johnson MD, Lavin P, Whetsell WP. Fulminant monophasic multiple sclerosis, Marburg's type. J Neurol Neurosurg Psychiatr 1990;53:918-21.  Back to cited text no. 3    
4.Capello E, Mancardie GL. Marburg type and Balò's concentric sclerosis: Rare and acute variants of multiple sclerosis. Neurol Sci 2004;25:361-3.  Back to cited text no. 4    


  [Figure 1a], [Figure 1b], [Figure 1c], [Figure 1d]


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