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    Go to the top of the page   ID: 20041110101528 Original case in english  More links about this topic on Pubmed (PubMed Reader)
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    | The Authors | Discussion | Write a Comment to this Case |
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    Juvenile form of Huntington's Chorea
    T Haufe. Juvenile form of Huntington's Chorea. PedRad [serial online] vol 4, no. 11.
    URL: www.PedRad.info/?search=20041110101528


     
     Pediatric Radiology CasesImages to this case: There are MRI-images available for this case. [ MRI ] View all modalities [ All ]   
     Pediatric Radiology CasesAuthor/s:

    T. Haufe (Institut f. Diagn. Radiologie im Städtischen KH Dresden-Neustadt)  

     
     Pediatric Radiology CasesEmail Address:

    Viewable for logged on visitors (Log on)  

     
     Pediatric Radiology CasesAge:

    15 Years  

     
     Pediatric Radiology CasesGender:

    Male  

     
     Pediatric Radiology CasesRegion-Organ:

    Head-Brain and brain nerves  

     
     Pediatric Radiology CasesMost likely etiology:

    congenital  

     
     Pediatric Radiology CasesHistory:

    15 year old boy, normal early development, afterwards cerebral decline with dementia and behavioral disturbance, increased muscle tone and atactic gait. Family hstory positive for neurodegenerative disease with early death of grandmother on father's side.  

     
     Pediatric Radiology CasesPathomorphology or Pathophysiology of this disease :

    Defect on chromosome 4Q, autosomal dominant heredity,
    symptoms result pathophysiologically from a genetically acquired dominance of Dopamine with deficiency of Acetylecholine.  

     
     Pediatric Radiology CasesRadiological findings:


    MRI 1 <- view MRI 1

    MRI 1: T2/Flair transversal: signal intense Putamen and Caput nuclei caudati bilat., symmetrical





    MRI 2 <- view MRI 2

    MRI 2: T1 IR-TSE coronal: Atrophy of the Basal ganglia with widening of the III. Ventricle and of the anterior horns of the lateral ventricles.





    MRI 3 <- view MRI 3

    MRI 3: T1 native transversal: decreased differentiation of the Basal ganglia, diminished waist of the anterior horns of the lateral ventricles due to atrophy of the Caput cuclei caudati bilateral.





    MRI 4 <- view MRI 4

    MRI 4: T1 Spir Gd transversal: no pathologic enhancement detectable



     

     
     Pediatric Radiology CasesDiagnosis confirmation:

    Genetics  

     
     Pediatric Radiology CasesWhich DD would be also possible with the radiological findings:

    Mitochondrial encephalopathy (Morbus Leigh)  

     
     Pediatric Radiology CasesCourse / Prognosis / Frequency / Other :

    About 6% juvenile forms of Huntington's Chorea,
    progressive dementia and behavioral disturbance,
    deadly course after 10-15 years after beginn of illness  

     
     Pediatric Radiology CasesComments of the author about the case:

    N/A  

     
     Pediatric Radiology CasesFirst description / History:

    N/A  

     
     Pediatric Radiology CasesLiterature:

    1. Medline: Medline
    A.James Barkovich
    Pediatric Neuroimaging

    2. Medline: Medline
    Berlit/Seeger
    Neurologie  

     
     Pediatric Radiology CasesKeywords:

    Chorea Huntington, neurodegenerative disease, juvenile Huntington's Chorea, child, childhood, pediatric radiology  

     
     Pediatric Radiology Cases Cite this article:

    T Haufe. Juvenile form of Huntington's Chorea. PedRad [serial online] vol 4, no. 11.
    URL: www.PedRad.info/?search=20041110101528  

     
     Pediatric Radiology Cases Read similar articles: with corresponding keywords
    in the same field: Head-Brain and brain nerves
    or in the region: Head
    or in the tissue/organ: Brain and brain nerves
    or with the etiology: congenital
     
     Pediatric Radiology CasesImages to this case: There are MRI-images available for this case. [ MRI ] View all modalities [ All ]   
     
    Juvenile form of Huntington's Chorea
    T Haufe. Juvenile form of Huntington's Chorea. PedRad [serial online] vol 4, no. 11.
    URL: www.PedRad.info/?search=20041110101528


     

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    Juvenile form of Huntington's Chorea
    Other cases by these authors:

    Search T. Haufe in Medline T. Haufe (4)   

    Juvenile form of Huntington's Chorea  
     
    Juvenile form of Huntington's Chorea
    T Haufe. Juvenile form of Huntington's Chorea. PedRad [serial online] vol 4, no. 11.
    URL: www.PedRad.info/?search=20041110101528


     

    Which diagnosis have other collegues guessed?


    • Mitochondrial encephalopathy (Morbus Leigh)
      Votes: 3 (23 %)


    • Huntington's Chorea
      Votes: 6 (46 %)


    • Periventricular leucencephalomalacia
      Votes: 4 (30 %)


    • Acute disseminated encephalomyelopathy (ADEM)
      Votes: 0 (0 %)



        Total answers: 13

     
    Juvenile form of Huntington's Chorea
    T Haufe. Juvenile form of Huntington's Chorea. PedRad [serial online] vol 4, no. 11.
    URL: www.PedRad.info/?search=20041110101528


     

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    Juvenile form of Huntington's Chorea
    T Haufe. Juvenile form of Huntington's Chorea. PedRad [serial online] vol 4, no. 11.
    URL: www.PedRad.info/?search=20041110101528


     




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