[Sebaceous linear nevus syndrome with hemimegalencephaly. Report of a case].
The case of a 7-year-old girl, born from a monochorionic biamniotic pregnancy (with healthy male twin) is presented. The patient showed a congenital brown-yellowish, raised, rough and oval-shaped nevic neoformation of the right temporo-zygomatic region. At 28 days of age she had a right hemiclonic status epilepticus, and from the 8th month of life she presented right sided partial motor seizures. Moreover from the beginning, her development milestones were delayed. When she was 5 years old an electroencephalogram displayed a sharp asymmetrical background activity, a continuous paroxysmal activity on the right hemisphere and independent focal irritative anomalies on the left posterior region. Her computed tomographic scan demonstrated megalencephaly on the right hemisphere. A biopsy specimen of her skin lesion showed the histological characteristics of a nevus sebaceous of Jadassohn. Taken together the clinical, neuroradiological and dermatological data led to the diagnosis of linear nevus sebaceous syndrome.
A
A
Patrizi
D
D
Pizzino
A
A
Tullini
G
G
Gobbi
M
M
Santucci
D002540:Cerebral Cortex / Q000002:abnormalities*; D002648:Child; D004200:Diseases in Twins; D005153:Facial Neoplasms / Q000151:congenital*; D005260:Female; D006801:Humans; D008607:Intellectual Disability / Q000209:etiology; D008297:Male; D009508:Nevus, Pigmented / Q000151:congenital*; D012626:Sebaceous Gland Neoplasms / Q000151:congenital*; D013577:Syndrome; D014429:Twins, Dizygotic
Sindrome del nevo lineare sebaceo con emimegalencefalia. Su di un caso.