Neeraj N. Baheti, MD,
Atma Ram Bansal, MD,
Chaturbhuj Rathore, MD and
Chandrasekhar Kesavdas, MD
From the Departments of Neurology (N.N.B., A.R.B., C.R.) and Imaging Sciences & Interventional Radiology (C.K.), Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India.
Address correspondence and reprint requests to Dr. C. Rathore, Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum–695 011, Kerala, India cbrathore{at}rediffmail.com
A 17-year-old boy presented with left focal seizures and progressivehemiparesis for 10 years. Serial MRIs showed progressive rightcerebral and contralateral cerebellar atrophy (figure). He isseizure-free after right hemispherotomy. Pathology was compatiblewith Rasmussen encephalitis.
(A–C) Brain MRI (1997): Axial (A, B) and coronal (C) T2-weighted fast spin echo images show atrophy of the right cerebral and left cerebellar hemispheres. (D–F) MRI (2007): Corresponding axial (D, E) and coronal (F) T2-weighted fast spin echo images show the progression of atrophy of the left cerebellar hemisphere, right cerebral peduncle, and right cerebral hemisphere. (G–I) MRI (2008): Images (G–I) 1 year after hemispherotomy show no further progression of cerebellar atrophy.
Crossed cerebellar diaschisis and subsequent crossed cerebellaratrophy, due to supratentorial lesions or chronic focal seizures,represents the best evidence of transneuronal depression inhumans.1 Contrary to the original concept of reversible dysfunctionin diaschisis,2 distant areas may undergo irreversible degenerationdepending upon the nature of the primary process. Progressivecrossed cerebellar atrophy has been mainly reported with conditionsassociated with chronic focal seizures,1 presumably relatedto additional transneuronal excitotoxic damage.
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