751. Forehead plaque: a cutaneous marker of CNS involvement in tuberous sclerosis.
- Author
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Rama Rao GR, Krishna Rao PV, Gopal KV, Kumar YH, and Ramachandra BV
- Subjects
- Adolescent, Adult, Child, Child, Preschool, Female, Fibrosis, Humans, Infant, Male, Middle Aged, Retrospective Studies, Brain Diseases pathology, Forehead pathology, Skin pathology, Tuberous Sclerosis pathology
- Abstract
Background: Tuberous sclerosis complex (TSC) is a neurocutaneous genodermatosis characterized by hamartoma formation in multiple organs. There are no definite cutaneous markers suggestive of central nervous system (CNS) involvement in TSC., Aims: To study association of forehead plaque seen in tuberous sclerosis patients and CNS involvement in TSC., Methods: This is a retrospective study of 15 cases of tuberous sclerosis in varying age groups - from 1.5 to 50 years. All the cases were thoroughly evaluated with detailed history; clinical examination; and relevant investigations like X-rays of chest, skull, hands and feet; ultrasound abdomen and computed tomography of brain., Results: Out of the 15 cases, CNS involvement was seen in 8 cases. Seizures were present in 8 cases (53.33%) and mental retardation was seen in 6 cases (40%). Computerized tomography of brain revealed subependymal nodules (SENs) in eight cases (53.33%). In addition to SENs, subependymal giant cell astrocytomas and cortical tubers were seen in 2 cases each. Out of these 8 cases having CNS involvement, in 7 cases forehead plaque was observed. In 1 case, no forehead plaque was observed (X 2 = 1.07, P < 0.05)., Conclusion: This study shows that there is a statistically significant relationship between the presence of a forehead plaque and CNS involvement in TSC. Therefore, forehead plaque may be considered as a novel cutaneous marker to know the CNS involvement in TSC at an early stage.
- Published
- 2008
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