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• Vol.45 No.9
• CLINICA NEUROL, 45: 663−668, 2005
• Vol.45 No.9 contents

Case Report

Progressive multifocal leukoencephalopathy with idiopathic CD4 positive T-lymphocytepenia mimicking a low grade glioma on proton MR spectroscopy. A case report

Norito Kokubun, M.D.¹⁾, Tetsuya Ishihara, M.D.²⁾, Momoka Nishibayashi, M.D.¹⁾, Syun-ichiro Ikeda, M.D.³⁾, Kazuo Nagashima, M.D.⁴⁾ and Koichi Hirata, M.D.¹⁾

¹⁾Department of Neurology, Dokkyo University School of Medicine
²⁾Southern Tohoku Research Institute for Neuroscience
³⁾Ikeda Neurosurgical Center Hospital
⁴⁾Laboratory of Molecular and Cellular Pathology, Hokkaido University School of Medicine

A 61-year-old man with no history of HIV infection developed a subacutely progressive dementia and left hemiparesis. Brain MRI showed a high intensity lesion in the right frontal lobe on T2 weighted image. There was no contrast enhancement after gadolinium-DTPA administration. ¹H-MRS revealed a marked decrease in the n-acetyl aspartate/creatine ratios and an increase in the choline/creatine ratio. A lactate peak also was present. A low-grade glioma was suspected and he was admitted to our hospital. On examination, there was a mild dementia and left hemiparesis. A peripheral blood count revealed lymphocytopenia (426/mm³) with a CD4/CD8 ratio of 0.28. No evidence of HIV infection, malignancies or collagen disease was found. A brain biopsy revealed no tumor cells but instead demyelinated brain tissue with large nucleated cells. JC virus antigen was detected in the cells of the demyelinated lesions. A diagnosis of PML associated with idiopathic CD4 positive lymphocytopenia was made. There are only a few reports concerning ¹H-MRS findings in patients with PML and the present case illustrates the difficulty of making a differential diagnosis between PML and glioma.

(CLINICA NEUROL, 45: 663−668, 2005)
key words: PML, idiopathic CD4+lymphocytopenia, MRI, ¹H-MRS, glioma

(Received: 6-Dec-04)