title: Encephalocraniocutaneous lipomatosis, a rare neurocutaneous disorder: report of additional three cases.
abstract: OBJECTIVE
  Encephalocraniocutaneous lipomatosis (ECCL) is a rare congenital neurocutaneous disorder. It was described by Haberland in 1970 and is also called Haberland syndrome. It is characterized by unilateral skin lesions such as lipomas, connective tissue nevi, and alopecia with ipsilateral ophthalmological and cerebral malformations with or without psychomotor and mental retardation and early-onset seizure.
  
  
  METHODS
  We present three pediatric cases (two boys, one girl) with ECCL. All the patients' sociodemographic, clinical, and neuroradiological data was collected.
  
  
  RESULTS
  We describe two male (5 and 1.3 years old) and one female (15 years old) cases. All patients have unilateral left-sided alopecia with ipsilateral ocular lesion and the cerebral lesion. All patients were born at term; their past history and family histories were unremarkable. Their electroencephalograms showed hemispheric asymmetry. All of the cases had right-sided mild to moderate hemiparesis. In addition, our second case is having optic glioma and this case is the fifth case with glioma associated with ECCL.
  
  
  CONCLUSIONS
  We describe three additional cases with ECCL which is an extremely rare neurocutaneous syndrome. Also, case 2 has optic glioma and according to the literature this is the fifth case of low-grade gliomas with ECCL. We suggest that patients who have ocular lesion and ipsilateral skin lesion must be examined for ECCL, and the patients must be followed up with cerebral MRI once a year for low-grade gliomas.
authors.affiliation: Department of Pediatric Neurology, Eskisehir Osmangazi University Hospital, Meselik, Eskisehir, Turkey. ozankocak79@gmail.com.
authors.forename: O
authors.initials: O
authors.lastname: Kocak
authors.affiliation: Department of Pediatric Neurology, Eskisehir Osmangazi University Hospital, Meselik, Eskisehir, Turkey. coskunyarar@hotmail.com.
authors.forename: C
authors.initials: C
authors.lastname: Yarar
authors.affiliation: Department of Pediatric Neurology, Eskisehir Osmangazi University Hospital, Meselik, Eskisehir, Turkey. kbcarman@gmail.com.
authors.forename: K B
authors.initials: KB
authors.lastname: Carman
mesh_terms: D000293:Adolescent; D002675:Child, Preschool; D005128:Eye Diseases; D005260:Female; D006801:Humans; D007223:Infant; D008068:Lipomatosis; D008297:Male; D020752:Neurocutaneous Syndromes
keywords: Choristoma; Encephalocraniocutaneous lipomatosis; Haberland syndrome; Optic glioma