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The value of positron emission tomography in the diagnosis and monitoring of late infantile and juvenile lipopigment storage disorders (so-called Batten or neuronal ceroid lipofuscinoses).

The value of positron emission tomography in the diagnosis and monitoring of late infantile and juvenile lipopigment storage disorders (so-called Batten or neuronal ceroid lipofuscinoses). Research Abstract Details 

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  • The value of positron emission tomography in the diagnosis and monitoring of late infantile and juvenile lipopigment storage disorders (so-called Batten or neuronal ceroid lipofuscinoses). Abstract Text:

    m philippartM Philippart,e da silvaE da Silva,h t chuganiH T Chugani,m philippartM Philippart,e da silvaE da Silva,h t chuganiH T Chugani,

    Positron Emission Tomography (PET) with 2-deoxy-2 [18F]-fluoro-D-glucose provides a measure of functional brain activity, particularly in the dendritic field. In CLN3 (juvenile neuronal ceroid lipofuscinosis or juvenile Batten disease, with fingerprint inclusions) hypometabolism slowly spreads from calcarine to anterior areas, sparing subcortical structures and brainstem. In CLN2 (late infantile neuronal ceroid lipofuscinosis or Jansky-Bielschowsky disease, with curvilinear inclusions) degeneration is rapid with generalized cortical and subcortical hypometabolism. This is associated with rapidly progressive cerebral atrophy on anatomical neuroimaging. A 4-year-old child with CLN2 scanned with PET 13 months after the clinical onset showed hypometabolism, severe in the thalamus and mild in cortical areas. Three other patients with CLN2 had severe generalized hypometabolism and brain atrophy. Longitudinal PET studies in CLN may provide key insights into degenerative processes.

    The value of positron emission tomography in the diagnosis and monitoring of late infantile and juvenile lipopigment storage disorders (so-called Batten or neuronal ceroid lipofuscinoses). Publishing Authors By Initials

    m philippartM Philippart,e da silvaE da Silva,ht chuganiHT Chugani,m philippartM Philippart,e da silvaE da Silva,ht chuganiHT Chugani,

    For similar tomography, emission-computed research abstracts see: tomography, emission-computed research

    PUBMED ID PMID:

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    The value of positron emission tomography in the diagnosis and monitoring of late infantile and juvenile lipopigment storage disorders (so-called Batten or neuronal ceroid lipofuscinoses). Journal Published:

    PUBLICATION TYPE: Journal Article

    Journal: Neuropediatrics

    VOLUME: 28

    Page Numbers: 74-6

    Journal Abbreviation: Neuropediatrics

    ISSN: 0174-304X

    DAY: 16

    MONTH: Feb

    YEAR: 1997

    The value of positron emission tomography in the diagnosis and monitoring of late infantile and juvenile lipopigment storage disorders (so-called Batten or neuronal ceroid lipofuscinoses). Information

    Number of References:

    LANGUAGE: eng

    NlmUniqueID: 8101187

    The value of positron emission tomography in the diagnosis and monitoring of late infantile and juvenile lipopigment storage disorders (so-called Batten or neuronal ceroid lipofuscinoses). Keywords Mesh Terms:

    KEYWORDS: Tomography, Emission-Computed

    MESH TERMS: radionuclide imaging

    Chemical & Substance for Abstract: The value of positron emission tomography in the diagnosis and monitoring of late infantile and juvenile lipopigment storage disorders (so-called Batten or neuronal ceroid lipofuscinoses). Information

    Substance Name: Fluorodeoxyglucose F18

    Registry Number: 63503-12-8

    Grant and Affiliation Information for The value of positron emission tomography in the diagnosis and monitoring of late infantile and juvenile lipopigment storage disorders (so-called Batten or neuronal ceroid lipofuscinoses).

    AFFILIATION: Department of Pediatrics, UCLA School of Medicine, USA.

    Country: GERMANY

    GERMANY Research PublicationGERMANY Research Publication

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    ACRONYM:

    MEDLINETA: Neuropediatrics

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    The value of positron emission tomography in the diagnosis and monitoring of late infantile and juvenile lipopigment storage disorders so-called Batten or neuronal ceroid lipofuscinoses Related Publications

     

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