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AJNR - American Journal of Neuroradiology

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American Journal of Neuroradiology 24:153, January 2003
© 2003 American Society of Neuroradiology

Letter

Reply

A. James Barkovich^a

^a Neuroradiology Section
Department of Radiology
University of California, San Francisco
San Francisco, CA

We thank Dr. Soto-Ares for his kind comments regarding our article (1) and extend our compliments on his very interesting article in the same issue of the AJNR (2). We agree with his comments that a classification based on embryology, genetics, or signaling molecules is optimal for the description of malformations; indeed, this subject was discussed in our article. However, as Dr. Soto-Ares acknowledges, such a classification is very difficult and we are a long way from having enough knowledge of cerebellar genetics and molecular biology to accomplish such a classification. For the present, we think that a radiologic classification is a useful first step in studying this complicated group of diseases, just as it has proved useful in studying cerebral cortical malformations (3, 4) and anomalies of the cerebral commissures (5, 6).

Regarding Dr. Soto-Ares’ question about the pathogenesis of focal cerebellar cortical dysplasias in the absence of cerebral anomalies, we can suggest several possibilities, including focal ischemia or infection, somatic mosaicism, or different dosages of the same mutation in the same gene in different parts of the CNS. We agree that these are important questions that are not possible to answer by radiologic investigation.

We are delighted to have interested and enlightened colleagues such as Dr. Soto-Ares working in this field of study. The contributions of Dr. Soto-Ares and his colleagues to the study of cerebellar malformations have been both interesting and enlightening (2, 7). As advances in this field result from the cooperation of all those interested in the subject, we are happy that he has found our article worthy of comment and we hope for further interactions with his group and with others who have interest in cerebellar development and maldevelopment.

References

1. Patel S, Barkovich AJ. Analysis and classification of cerebellar malformations. AJNR Am J Neuroradiol2002; 23 :1074 –1087[Abstract/Free Full Text]

2. Soto-Ares G, Devisme L, Jorriot S, Deries B, Pruvo JP, Ruchoux MM. Neuropathologic and MR imaging correlation in a neonatal case of cerebellar cortical dysplasia. AJNR Am J Neuroradiol2002; 23 :1101 –1104[Abstract/Free Full Text]

3. Barkovich AJ, Kuzniecky RI, Dobyns WB, Jackson GD, Becker LE, Evrard P. A classification scheme for malformations of cortical development. Neuropediatrics1996; 27 :59 –63[Medline]

4. Barkovich AJ, Kuzniecky RI, Jackson GD, Guerrini R, Dobyns WB. Classification system for malformations of cortical development: update 2001. Neurology2001; 57 :2168 –2178[Abstract/Free Full Text]

5. Raybaud CA, Girard N. Anatomic MRI study of commissural agenesis and dysplasia of the telencephalon (agenesis of the corpus callosum and related anomalies): clinical correlations and morphogenetic interpretation [in French]. Neurochirurgie1998; 44[suppl 1] :38 –60

6. Barkovich AJ, Simon EM, Walsh CA. Callosal agenesis with cyst: a better understanding and new classification. Neurology2001; 56 :220 –227[Abstract/Free Full Text]

7. Soto-Ares G, Delmaire C, Deries B, Vallee L, Pruvo JP. Cerebellar cortical dysplasia: MR findings in a complex entity. AJNR Am J Neuroradiol2000; 21 :1511 –1519[Abstract/Free Full Text]

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