title: Bilateral basal ganglia involvement in a patient with Griscelli syndrome.
abstract: We report a 6-year-old Iranian boy with silvery-gray hair, eyelashes and the eyebrows who was admitted because of seizures and subsequent stupor. He had previous history of acute hemiparesis at 1 year of age and hepatitis-like syndrome 3 months ago. Microscopic examination of the patient's hair shaft revealed different sized clumps of melanin seen in the center of the shafts. Bone marrow aspiration revealed erythroid hyperplasia and erythrophagocytic cells. Bilateral frontal cortical and subcortical high signal lesions, dirty white matter, high signal areas in the upper pons and in both caudates and lentiform nuclei in T2 WI were the brain MRI findings of the patient. He died in the accelerated phase of Griscelli Syndrome (GS) type 2. To our knowledge we report the first case of GS from Iran.
authors.affiliation: Children hospital Medical Center, Tehran University of Medical Sciences, Pediatric Neurology, Tehran 1419733151, Iran. mr_ashrafi@yahoo.com
authors.forename: Mahmoud Reza
authors.initials: MR
authors.lastname: Ashrafi
authors.forename: Meysam
authors.initials: M
authors.lastname: Mohseni
authors.forename: Shahrooz
authors.initials: S
authors.lastname: Yazdani
authors.forename: Houman
authors.initials: H
authors.lastname: Alizadeh
authors.forename: Asghar
authors.initials: A
authors.lastname: Ramyar
authors.forename: Asghar
authors.initials: A
authors.lastname: Aghamohammadi
authors.forename: Mina
authors.initials: M
authors.lastname: Izadyar
authors.forename: Fatemeh
authors.initials: F
authors.lastname: Mahjoub
authors.forename: Javad Ahmadian
authors.initials: JA
authors.lastname: Heris
mesh_terms: D001479:Basal Ganglia / Q000473:pathology / Q000503:physiopathology*; D001921:Brain / Q000473:pathology; D002648:Child; D017809:Fatal Outcome; D005334:Fever / Q000473:pathology / Q000503:physiopathology*; D006801:Humans; D006968:Hypersensitivity, Delayed / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D009460:Neurologic Examination; D009503:Neutropenia / Q000473:pathology / Q000503:physiopathology*; D016116:Piebaldism / Q000473:pathology / Q000503:physiopathology*; D013577:Syndrome; D013921:Thrombocytopenia / Q000473:pathology / Q000503:physiopathology*