title: A clinician's dilemma: Sturge-Weber syndrome 'without facial nevus'!!
abstract: Sturge-Weber syndrome (SWS) is a rare, sporadic neurocutaneous syndrome characterized by a classical triad of facial port wine nevus, ipsilateral leptomeningeal angiomatosis (LAM) and glaucoma. The incidence of SWS is 1/50,000 live births, although it is more often underreported. The incidence of SWS without facial nevus is not known, although very few patients without facial nevus have been reported. In these patients, the diagnosis of SWS is made by the findings of computed tomography, magnetic resonance imaging, and histopathology. Here, we report three patients with SWS from our cohort of 28 patients with SWS without facial nevus and discuss their clinical profile and outcome.
authors: Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India.
authors: Sujit A
authors: SA
authors: Jagtap
authors: G
authors: G
authors: Srinivas
authors: Ashalatha
authors: A
authors: Radhakrishnan
authors: K J
authors: KJ
authors: Harsha
keywords: Leptomeningeal angiomatosis; Sturge-Weber syndrome; seizure
references: Semin Cutan Med Surg. 2004 Jun;23(2):87-98
references: 15295918
references: Rev Neurol. 2000 May 1-15;30(9):837-41
references: 10870197
references: Pediatr Neurol. 2000 May;22(5):400-2
references: 10913734
references: AJR Am J Roentgenol. 1992 Apr;158(4):867-71
references: 1546607
references: Neuropediatrics. 1995 Aug;26(4):220-2
references: 8544964
references: Neurology. 1988 Jun;38(6):913-20
references: 3368073
references: Chin Med J (Engl). 1992 Nov;105(11):964-5
references: 1304469
references: Can J Neurol Sci. 2008 Jul;35(3):301-7
references: 18714797
references: Childs Brain. 1983;10(6):387-92
references: 6661936
references: Neurology. 1976 Jul;26(7):629-32
references: 945510
references: Acta Paediatr. 2000 Jun;89(6):740-3
references: 10914976
references: J Child Neurol. 2007 Apr;22(4):479-83
references: 17621534
references: J Neurosurg Sci. 1989 Apr-Jun;33(2):215-8
references: 2795196
references: Pediatr Neurol. 1993 Jul-Aug;9(4):283-8
references: 8216540
references: J Child Neurol. 1998 Dec;13(12):606-18
references: 9881531