Abstract
Unilateral somatic and intracranial hypoplasia (USICH) does not represent a true neurocutaneous disorder, but most of its features including unilateral hypoplasia involving the upper and lower extremities, the breast and the trunk, bilateral acral abnormalities affecting especially the middle phalanges, mental retardation and partial epilepsy, overlap with the clinical and imaging findings of other neurocutaneous diseases and/or complex malformation syndromes described in this book, such as oculocerebrocutaneous/ Delleman syndrome (OCCS), encephalocraniocutaneous lipomatosis (ECCL), Proteus syndrome, Parry- Romberg syndrome, scleroderma “en coup de sabre” and Goldenhar syndrome.
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References
De Souza N, Chaudhuri R, Bingham J, Cox T (1994) MRI in cerebellar hypoplasia. Neuroradiology 36: 148–151.
Granados-Alzamora V, Pascual-Pascual SI, Pascual-Castroviejo I (2003) Hipoplasia unilateral de hemisferio cerebeloso: ¿una alteración de origen vascular? Rev Neurol 36: 841–845.
Muller J (1990) Changes in cerebral sulcation in simple absence of the corpus callosum: evidence of rules controlling sulcation patterns. Can J Neurol Sci 17: 343–344.
Pascual-Castroviejo I, Tendero A, Martínez-Bermejo A, Lopez-Terradas JM, Casas C (1975) Persistence of the hypoglosal artery and partial agenesis of the cerebellum. Neuropediatrie 6: 184–189.
Pascual-Castroviejo I (1978) Vascular and nonvascular intracranial malformations associated with external capillary hemangiomas. Neuroradiology 16: 82–84.
Pascual-Castroviejo I (1978a) Vascular changes in cerebellar development defects. Neuroradiology 16: 58–60.
Pascual-Castroviejo I (1985) The association of extracranial and intracranial vascular malformations in children. Can J Neurol Sci 12: 139–148.
Pascual-Castroviejo I, Viaño J, Moreno F, Palencia R, Martínez Fernández V, Pascual-Pascual SI, Martinez-Bermejo A, Garcia-Penas JJ, Roche MC (1996) Hemangiomas of head, neck, and chest with associated vascular and brain anomalies. A complex neurocutaneous syndrome. AJNR Am J Neuroradiol 17: 461–471.
Pascual-Castroviejo I, Pascual-Pascual SI, Viaño J, Martinez V (1997) Unilateral somatic and intracranial hypoplasia. Neuropediatrics 28: 341–344.
Sarnat HB (1992) Cerebral Dysgenesis. Embryology and Clinical Expression. New York: Oxford University Press, pp. 62–71.
Wood B, Dimmick JE (1992) Skeletal system. In: Dimmick JE, Kalousek DK (eds.) Developmental Pathology of the Embryo and Fetus. Philadelphia: JB. Lippincott Company, pp. 401–423.
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Pascual-Castroviejo, I. (2008). Unilateral Somatic and Intracranial Hypoplasia. In: Ruggieri, M., Pascual-Castroviejo, I., Di Rocco, C. (eds) Neurocutaneous Disorders Phakomatoses and Hamartoneoplastic Syndromes. Springer, Vienna. https://doi.org/10.1007/978-3-211-69500-5_60
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DOI: https://doi.org/10.1007/978-3-211-69500-5_60
Publisher Name: Springer, Vienna
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