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      Crossed cerebrocerebellar diaschisis in Dyke–Davidoff–Masson syndrome

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      Journal of Pediatric Neurosciences
      Medknow Publications & Media Pvt Ltd

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          Abstract

          Dear Sir, I read an interesting article on magnetic resonance imaging depiction of crossed cerebrocerebellar diaschisis in Dyke–Davidoff–Masson (DDMS) syndrome by Gupta et al.[1] There are still a lot of case reports in various journals from time to time and only rarely some case series.[2] Even then, there are many new or unresolved issues in this diverse syndrome. One such issue is being resolved up to some extent by the present twin case report. Here, I would like to share my views and experience: In one of our study, we had a very remarkable finding in the form of cerebral hemispheric plus cerebellar atrophy in nine patients out of a total of 28 patients of DDMS, of which three patients had diffuse bilateral and one had crossed cerebellar atrophy (CCA) which was associated with left cerebral hemiatrophy[3] In an earlier study, it was concluded that in patients with epilepsy and destructive insults early in life, the extent of the supratentorial lesion as well as the antecedent of status epilepticus plays a major role in the pathogenesis of CCA. Recurrent seizures do not seem to be relevant to the development of CCA[4] The authors in the present case report have concluded that CCA in the setting of DDMS is ipsilateral when insult is before the age of 1 month and is contralateral when the insult is after this age due to differences in anatomy during development, which was consistent in their patients, as both of them had history of insult after 1 month of age A question arises out of our case series that three patients who had diffuse bilateral cerebellar atrophy, where to place them about time of injury according to the authors' explanation of 1 month demarcating age for insult Other remarkable findings of our study included, hippocampal sclerosis (16%), dystonia, hemiparkinsonism, mirror movement, and DandyWalker syndrome, with a conclusion that DDMS can present protean clinicoradiological manifestation, cerebellar atrophy, hippocampal sclerosis, and phenytoin intolerance may be one of them. The drawback with our study was lack of functional neuroimaging. Hence, the aim of further case reports and case series should be on such important aspects of various pathophysiological correlates of varied manifestation so as to manage and take preventive steps to avoid the dreaded disabilities in such patients. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

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          Magnetic resonance imaging depiction of acquired Dyke–Davidoff–Masson syndrome with crossed cerebro-cerebellar diaschisis: Report of two cases

          Acquired Dyke–Davidoff–Masson syndrome, also known as hemispheric atrophy, is characterized by loss of volume of one cerebral hemisphere from an insult in early life. Crossed cerebellar diaschisis refers to dysfunction/atrophy of cerebellar hemisphere which is secondary to contralateral supratentorial insult. We describe magnetic resonance imaging findings in two cases of acquired Dyke–Davidoff–Masson syndrome with crossed cerebro-cerebellar diaschisis.
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            Crossed cerebellar atrophy in patients with precocious destructive brain insults.

            To analyze the frequency and pathogenetic factors of crossed cerebellar atrophy (CCA) in adult patients with epilepsy secondary to destructive brain insults of early development. We studied 51 adult patients with epilepsy and precocious destructive lesions. Patients were divided into 3 groups according to the topographic distribution of their lesions on magnetic resonance imaging: group A, hemispheric (n = 9); group B, main arterial territory (n = 25); and group C, arterial border zone (n = 17). We evaluated the presence of CCA visually and with cerebellar volumetric measurement, correlating it with the clinical data. Other features shown on magnetic resonance imaging, such as the thalamus, brainstem, and middle cerebellar peduncle, were also carefully analyzed. Seven patients (13%) had CCA that was associated with the extent of the supratentorial lesion (6 from group A, 1 from group B, and none from group C; P<.001). Status epilepticus was present in 6 patients from group A and in none from the other groups. There was an association between the antecedent of status epilepticus and CCA (P<.001). All patients had atrophy of the cerebral peduncle ipsilateral to the supratentorial lesion and 4 had contralateral atrophy of the middle cerebellar peduncle. The duration of epilepsy was not associated with the presence of CCA (P =.20). Our data suggest that in patients with epilepsy and destructive insults early in life, the extent of the supratentorial lesion as well as the antecedent of status epilepticus play a major role in the pathogenesis of CCA. Recurrent seizures do not seem to be relevant to the development of CCA.
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              Acquired Dyke-Davidoff-Masson syndrome: Revisited

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                Author and article information

                Journal
                J Pediatr Neurosci
                J Pediatr Neurosci
                JPN
                Journal of Pediatric Neurosciences
                Medknow Publications & Media Pvt Ltd (India )
                1817-1745
                1998-3948
                Apr-Jun 2016
                : 11
                : 2
                : 162
                Affiliations
                [1]Department of Neurology, Dr. S.N. Medical College, Jodhpur, Rajasthan, India
                Author notes
                Address for correspondence: Dr. Khichar Purnaram Shubhakaran, House No. E-22/13, Umaid Hospital Campus, Geeta Bhawan Road, Jodhpur - 342 001, Rajasthan, India. E-mail: drkhicharsk@ 123456gmail.com
                Article
                JPN-11-162
                10.4103/1817-1745.187653
                4991170
                fc4c1baa-4cce-45c7-aae1-9e42939a47bd
                Copyright: © Journal of Pediatric Neurosciences

                This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

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