Idiopathic hypereosinophilic syndrome (IHS) is characterized by persistent hypereosinophilia (>1500/ mm3 for at least 6 months duration) with evidence of end-organ damage. It should be considered only after the exclusion of all other causes of hypereosinophilia. It could be revealed by encephalopathy. In this case, radiological features of cerebral magnetic resonance imaging (MRI) could contribute to the early diagnosis. We report a 33-year-old man presented to the Neurological Department with an acute altered status mental and paraparesis, which had appeared four weeks before. Eosinophils in repeated blood tests were more than 1500/mm3. FLAIR MRI images showed a typical distribution of multiple lesions in the deep and sub cortical white and gray matter. Diffusion weighted images show multiple high signals in the border zone which was high or low in the apparent diffusion coefficient map image. All other secondary causes of hypereosinophilia were ruled out. The evolution was marked by a dramatic response to steroids with improvement of eosinophilia, brain function and cerebral MRI findings. This case suggests that HIS should be considered when other causes of vascular lesions in cerebral MRI are ruled out. We highlight the necessity of introducing corticosteroids precociously in order to improve prognosis.
 

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