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Callosal agenesis with cyst

A better understanding and new classification

From the Neuroradiology Section (Drs. Barkovich and Simon) and Departments of Neurology, Neurological Surgery, and Pediatrics (Dr. Barkovich), University of California, San Francisco; and the Department of Neurology (Dr. Walsh), Harvard Medical School, Boston, MA.

Address correspondence and reprint requests to Dr. A. James Barkovich, Department of Radiology, Section of Neuroradiology, L 371, University of California, San Francisco, 505 Parnassus Ave., San Francisco, CA 94143; e-mail: jimb{at}radiology.ucsf.edu

OBJECTIVE: To analyze imaging studies of 25 cases of agenesis of the corpus callosum with interhemispheric cyst to assess this malformation itself and associated anomalies.

METHODS: CT (6 patients) and MRI (19 patients) were retrospectively reviewed. The patients were categorized according to morphologic and clinical characteristics.

RESULTS: Based on morphology, the patients were separated into two major types, each with subtypes. Type 1 cysts appear to be an extension or diverticulation of the third or lateral ventricles, whereas Type 2 are loculated and do not communicate with the ventricular system. Type 1a were associated with presumed communicating hydrocephalus but no other cerebral malformations. Type 1b were associated with hydrocephalus secondary to diencephalic malformations prohibiting egress of CSF from the third ventricle into the aqueduct of Sylvius. Type 1c were associated with small head size and apparent cerebral hemispheric dysplasia or hypoplasia. Type 2a (multiloculated cysts) were associated with no abnormalities other than callosal agenesis/hypogenesis. Type 2b were associated with deficiencies of the falx cerebri, subependymal heterotopia, and polymicrogyria (and were almost all in patients diagnosed with Aicardi syndrome). Type 2c were associated with subcortical heterotopia. Type 2d consists of interhemispheric arachnoid cysts. Other than those with Type 2b cysts, gender predominance was overwhelmingly male.

CONCLUSION: Agenesis of the corpus callosum with interhemispheric cyst appears to consist of a heterogeneous group of disorders that have in common callosal agenesis and extraparenchymal cysts, both of which are among the commonest CNS malformations. This article proposes a classification system, based primarily on morphology, by which this complex group of disorders might begin to be better understood.

This article has been cited by other articles:

				S. W. Hetts, E. H. Sherr, S. Chao, S. Gobuty, and A. J. Barkovich Anomalies of the corpus callosum: an MR analysis of the phenotypic spectrum of associated malformations. Am. J. Roentgenol., November 1, 2006; 187(5): 1343 - 1348. [Abstract] [Full Text] [PDF]

				U Moog, M C Jones, L M Bird, and W B Dobyns Oculocerebrocutaneous syndrome: the brain malformation defines a core phenotype J. Med. Genet., December 1, 2005; 42(12): 913 - 921. [Abstract] [Full Text] [PDF]

				N. Tambasco, F. Corea, and V. Bocola Subtotal corpus callosum agenesis with recurrent hyperhidrosis-hypothermia (Shapiro syndrome) Neurology, July 12, 2005; 65(1): 124 - 124. [Full Text] [PDF]

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				A. Stroustrup Smith and D. Levine Appearance of an Interhemispheric Cyst Associated with Agenesis of the Corpus Callosum AJNR Am. J. Neuroradiol., June 1, 2004; 25(6): 1037 - 1040. [Abstract] [Full Text] [PDF]

				A. J. Barkovich Reply AJNR Am. J. Neuroradiol., January 1, 2003; 24(1): 153 - 153. [Full Text] [PDF]