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AJR 2001; 177:721-722
© American Roentgen Ray Society


An Unusual Central Nervous System Manifestation of Behçet's Disease

Sergi Ganau, Joan Berenguer, Teresa Pujol and Josep M. Mercader

Hospital Clínic Barcelona Barcelona 08036, Spain

A 34-year-old man who had experienced a previous episode of cerebral involvement of Behçet's disease presented with a 9-day history of a throbbing headache and progressive right hemiparesis. CT scans and conventional MR images showed an isointense lesion with a hypointense 2-cm central area with ringlike contrast enhancement on T1-weighted images (Fig. 4A) in the left frontal region. This central area appeared slightly hyperintense on T2-weighted images. Perilesional edema was also observed. Echoplanar MR imaging and MR spectroscopy were performed, with the findings revealing the possibility of a brain abscess. Diffusion-weighted images showed a bright cavity and a markedly decreased apparent diffusion coefficient with perilesional edema (Fig. 4B). Perfusion MR imaging revealed that regional cerebral blood volume was substantially decreased throughout the lesion. MR spectroscopy showed end-products of bacterial breakdown (acetate, cytosolic amino acids, lactate, and succinate) (Fig. 4C). Blood and cerebrospinal fluid cultures were negative, and the patient was empirically treated with antibiotics and corticosteroids, substantially improving the symptoms. Follow-up CT and conventional MR studies revealed a slight increase in the left frontal lesion. Neurosurgery was then performed, revealing an abscess in which gram-positive cocci were identified.



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Fig. 4A. 34-year-old man with Behçet's disease. Contrast-enhanced axial T1-weighted image (TR/TE range, 3300/24-83, number of excitations, 2) shows ring contrast-enhancing lesion in the frontal region with perilesional hypointense edema. Hypointense residual lesion, which corresponds to previous vasculitic episode, is shown in right hemisphere.

 


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Fig. 4B. 34-year-old man with Behçet's disease. Diffusion-weighted image (TR/TE, 8000/105; b value, 1000 sec/mm2) reveals increased signal in left frontal region surrounded by hypointensity that corresponds to edema.

 


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Fig. 4C. 34-year-old man with Behçet's disease. Proton spectrum obtained by point-resolved spectroscopy (Probe-SV; General Electric Medical Systems, Milwaukee, WI; TR/TE, 1500/288, number of excitations, 8) from lesion shows resonances representing (from right to left) cytosolic amino acids (AA), lactate (Lac), alanine (Ala), acetate (Ac), and succinate (Succ).

 

Central nervous system involvement in Behçet's disease is reported in 4-49% of all patients [1]. Lesions are mostly due to vasculitic processes. MR imaging is more sensitive in revealing them than CT is. Nonhemorragic lesions are the most typical, and they are usually hypointense on T1-weighted images and hyperintense on T2-weighted images. Some of the lesions are enhanced after contrast media administration. Homogeneous, heterogeneous, linear, circular, and ring patterns have been described. In our patient, the findings on conventional MR imaging were rather similar to those reported in the literature [1, 2]. To our knowledge, echoplanar MR imaging and spectroscopic findings in Behçet's disease have not been previously described.

Desprechins et al. [3] described the diagnostic clue of diffusion-weighted MR images in patients with cerebral "ring-enhancing" masses. The authors reported three cases of brain abscesses with hyperintensity in the necrotic component [3]. Ernst et al. [4] reported reduced regional cerebral blood volume in toxoplasmatic lesions and attributed that finding to a probable lack of vasculature in the abscess. On MR spectroscopy, lactate (1.3 parts per million) is often observed because of the necrotic tissue, but it is not pathognomonic for abscesses. Nevertheless, the presence of cytosolic amino acids (0.9 parts per million), acetate (1.9 parts per million), and succinate (2.4 parts per million) is highly suggestive of abscesses [5]. These findings in a patient with Behçet's disease suggested a brain abscess.

In our patient, diffusion-weighted imaging, perfusion imaging, and spectroscopy contributed substantially to our making the correct diagnosis.

References

  1. Koçer N, Islak C, Siva A, et al. CNS involvement in neuro-Behçet syndrome: an MR study. AJNR 1999;20:1015 -1024[Abstract/Free Full Text]
  2. Tali ET, Atilla S, Keskin T, Simonson T, Isik S, Yuh WTC. MRI in neuro-Behçet's disease. Neuroradiology 1997;39:2 -6[Medline]
  3. Desprechins B, Stadnik T, Koerts G, Shabana W, Breucq C, Osteaux M. Use of diffusion-weighted MR imaging in differential diagnosis between intracerebral necrotic tumors and cerebral abscesses. AJNR 1999;20:1252 -1257[Abstract/Free Full Text]
  4. Ernst T, Chang L, Witt MD, et al. Cerebral toxoplasmosis and lymphoma in AIDS: perfusion MR imaging experience in 13 patients. Radiology 1998;208:663 -669.[Abstract/Free Full Text]
  5. Burtscher IM, Holtas S. In vivo proton MR spectroscopy of untreated and treated brain abscesses. AJNR 1999;20:1049 -1053[Abstract/Free Full Text]

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