6533b85bfe1ef96bd12babee

RESEARCH PRODUCT

Electron microscopic studies on skin and lymphocytes in early juvenile neuronal ceroid-lipofuscinosis.

Seiji KimuraSeiji KimuraHans H. Goebel

subject

Pathologymedicine.medical_specialtyLymphocyteEarly juvenileInfantile neuronal ceroid lipofuscinosisMatrix (biology)BiologyLipofuscinDevelopmental NeuroscienceNeuronal Ceroid-LipofuscinosesLipid dropletmedicineJuvenileHumansLymphocytesChildSkinGeneral Medicinemedicine.diseaseMicroscopy Electronmedicine.anatomical_structureChild PreschoolPediatrics Perinatology and Child HealthUltrastructureNeuronal ceroid lipofuscinosisNeurology (clinical)

description

Skin and lymphocytes of three patients with early juvenile neuronal ceroid-lipofuscinosis (NCL) were ultras trueturally investigated. Fingerprint profiles (FPP), isolated and I or mixed with curvilinear profiles (CLP), in various dermal cells and large, usually single lipopigments delineated by a trilaminar membrane and filled with a granular matrix, FPP and occasionally lipid droplets in lymphocytes were observed in all three patients. Characteristic lipopigments in lymphocytes are an important feature to differentiate between early juvenile NCL and late infantile and juvenile NCL.

yearjournalcountryeditionlanguage
1987-01-01Braindevelopment
10.1016/s0387-7604(87)80088-8https://pubmed.ncbi.nlm.nih.gov/3445918