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• Vol.51 No.9
• CLINICA NEUROL, 51: 688−693, 2011
• Vol.51 No.9 contents

Case Report

Diagnosis of pediatric multiple sclerosis initially presenting with tumefactive demyelinating lesion using ¹H-magnetic resonance spectroscopy

Takashi Kageyama, M.D., Ph.D.¹⁾, Yoko Gotoh, M.D.²⁾, Fumie Sano, M.D.²⁾, Takeo Katoh, M.D., Ph.D.⁴⁾, Mituhiko Nambu, M.D., Ph.D.²⁾, Tsutomu Okada, M.D., Ph.D.³⁾ and Toshihiko Suenaga, M.D., Ph.D.¹⁾

¹⁾Department of Neurology, Tenri Hospital
²⁾Department of Pediatrics, Tenri Hospital
³⁾Department of Radiology, Tenri Hospital
⁴⁾Department of Pediatrics, Kyoto University Hospital

We report a case of tumefactive demyelinating lesion (TDL) diagnosed using ¹H-magnetic resonance spectroscopy (¹H-MRS) and conventional magnetic resonance imaging (MRI). A 7-year-old girl was admitted to our hospital with complaints of sleepiness and clumsiness of the right limbs. Neurological examination showed somnolence, right-sided apraxia, and hemiparesis with enhanced tendon reflexes and Babinski sign. Conventional brain MRI revealed extensive hyperintensity in the subcortical white matter of the left frontal lobe in both T₂ weighted and fluid attenuated inversion recovery images. Gadolinium-enhanced T₁ weighted images showed a tumor-like lesion in this area with interrupted rim enhancement, termed open ring sign, and a periventricular lesion along the inferior horn of the right lateral ventricle and a juxtacortical lesion under the right motor cortex. In ¹H-MRS, both single voxel spectroscopy ( SVS ) and chemical shift imaging showed elevation of choline and reduction of Nacetylaspartate in the left frontal lobe lesion. Furthermore, SVS with a short echo time revealed elevated peaks for glutamate/glutamine complex in this lesion. These results suggested the demyelinating nature of this tumorlike lesion, in accordance with the concept of TDL. Based on this diagnosis, we treated the patient with three sets of methylprednisolone pulse therapy, which resulted in the reduction of TDL and neurological improvement. A follow-up study using MRI also demonstrated two more lesions in the corona radiata and internal capsule of the left hemisphere, supporting a diagnosis of multiple sclerosis based on the revised McDonald's criteria (2010). We concluded that ¹H-MRS may be beneficial in the differential diagnosis of TDL.
Full Text of this Article in Japanese PDF (542K)

(CLINICA NEUROL, 51: 688−693, 2011)
key words: tumefactive demyelinating lesion, multiple sclerosis, pediatric, magnetic resonance spectroscopy

(Received: 24-Feb-11)