6533b85bfe1ef96bd12bbf02

RESEARCH PRODUCT

Electron microscopic observation of tonsillar tissue as a diagnostic aid in early juvenile neuronal ceroid-lipofuscinosis.

Hans H. GoebelSeiji Kimura

subject

MalePathologymedicine.medical_specialtyLymphocytePalatine TonsilInfantile neuronal ceroid lipofuscinosisBiologyLipofuscinDiagnosis Differentialstomatognathic systemDevelopmental NeuroscienceNeuronal Ceroid-LipofuscinosesBiopsymedicineHumansLymphocytesChildmedicine.diagnostic_testGeneral MedicineMononuclear phagocyte systemmedicine.diseaseMicroscopy Electronmedicine.anatomical_structureTonsilPediatrics Perinatology and Child HealthSkin biopsyUltrastructureNeuronal ceroid lipofuscinosisNeurology (clinical)

description

An electron microscopic observation in a tonsil of a patient with early juvenile neuronal ceroid-lipofuscinosis (NCL) demonstrated characteristic lipopigments in lymphocytes, i.e., fingerprint profiles (FPP) and granular matrixes. While numerous FPP, curvilinear profiles (CLP) and granular matrixes were found in reticulo-endothelial and plasma cells, tonsillar lymphocytes contained only FPP and granular matrixes as seen in circulating lymphocytes. These findings suggest that a tonsil biopsy, an easy and simple technique, may provide more reliable information than a skin biopsy not only for the diagnosis of but also for differentiating the clinical forms of childhood NCL.

yearjournalcountryeditionlanguage
1987-01-01Braindevelopment
10.1016/s0387-7604(87)80091-8https://pubmed.ncbi.nlm.nih.gov/3445919