Bilateral basal ganglia involvement in a patient with Griscelli syndrome.
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.
Ashrafi
Mahmoud Reza
MR
Children hospital Medical Center, Tehran University of Medical Sciences, Pediatric Neurology, Tehran 1419733151, Iran. mr_ashrafi@yahoo.com
Mohseni
Meysam
M
Yazdani
Shahrooz
S
Alizadeh
Houman
H
Ramyar
Asghar
A
Aghamohammadi
Asghar
A
Izadyar
Mina
M
Mahjoub
Fatemeh
F
Heris
Javad Ahmadian
JA
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*