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    Go to the top of the page   ID: 20011128175838 Original case in english  More links about this topic on Pubmed (PubMed Reader)
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    Tuberous sclerosis of the brain / Bourneville-Pringle's disease
    Wolfgang Hirsch, RP Spielmann. Tuberous sclerosis of the brain / Bourneville-Pringle's disease. PedRad [serial online] vol 1, no. 11.
    URL: www.PedRad.info/?search=20011128175838


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

    Wolfgang Hirsch, R.P. Spielmann (Halle)  

     
     Pediatric Radiology CasesEmail Address:

    Viewable for logged on visitors (Log on)  

     
     Pediatric Radiology CasesAge:

    N/A  

     
     Pediatric Radiology CasesGender:

    N/A  

     
     Pediatric Radiology CasesRegion-Organ:

    Head-Brain and brain nerves  

     
     Pediatric Radiology CasesMost likely etiology:

    congenital  

     
     Pediatric Radiology CasesHistory:

    Mental retardation, epilepsy, typical changes in the skin: Adenoma sebaceum: facial angiofibromas in the nasolabial fold - starting at age 1-5. Multiple ungual fibromas, depigmentation of the trunk.  

     
     Pediatric Radiology CasesPathomorphology or Pathophysiology of this disease :

    In this contribution, only the cerebral lesions of a 9 year-old boy are shown.

    Fundamentally, however, Multi-organ illness (phakomatosis) with manifestations possible in:

    Skin: Adenoma sebaceum: facial angiofibromas in the nasolabial fold;

    Eye: retinal hamartomas that can calcify;

    Kidney: Angiomyofibromas, see there;

    Lung and Abdomen: Lymphangioleiomyofibromatosis, see there.

    Heart: benign rhabdomyomas.

     

     
     Pediatric Radiology CasesRadiological findings:


    MRI 1 <- view MRI 1

    MRI 1: Transversal



    MRI 2 <- view MRI 2

    MRI 2: Transversal



    MRI 3 <- view MRI 3

    MRI 3: Transversal



    MRI 4 <- view MRI 4

    MRI 4: Transversal



    MRI 5 <- view MRI 5

    MRI 5: Coronal



    MRI 6 <- view MRI 6

    MRI 6: Transversal



    MRI 7 <- view MRI 7

    MRI 7: Transversal



    MRI 8 <- view MRI 8

    MRI 8: Transversal



    MRI 9 <- view MRI 9

    MRI 9: Transversal



    Intracerebrally, there are 4 types of changes to be found:

    1. Subependymal Hamartomas: They push themselves out under the ependyma into the ventricles, are very small, are principally seen in every ventricle (but most often in the lateral ventricles). Calcifications are seen in rising age. In small children these are hyperintense, but later become isointense to the white matter.

    2. Large-cell tumors: differentiate themselves from above only because of their size and the tendency to grow. They are also more often to be found in the area of the foramen of Monroi. Contrast uptake is possible, but not obligatory.

    3. (Sub)-Cortical Tuber: Round or polygonal, underneath the cortex, and are seen often causing widened gyri. In babies, T1 images are hyperintense and T2 is hypointense. Later they become isointense in T1 and hyperintense in T2. There is a tendency for calcification.

    4. White matter lesions: spanning or linear, these have the same signal intensity characteristics as the (sub)cortical tuber and are composed of dysplastic cell formations. Here too, calcifications are possible.

    Extracerebral manifestations: Kidney, lung, abdomen (see above. In these examples, only cerebral manifestations are shown.)

     

     
     Pediatric Radiology CasesDiagnosis confirmation:

    Total constellation (Consens)  

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

    Relatively typical findings in summation. The difficulty and need to compare this to neurofibromatosis or other cerebral tumors occur only very seldomly.  

     
     Pediatric Radiology CasesCourse / Prognosis / Frequency / Other :

    N/A  

     
     Pediatric Radiology CasesComments of the author about the case:

    N/A  

     
     Pediatric Radiology CasesFirst description / History:

    N/A  

     
     Pediatric Radiology CasesLiterature:

    1. Medline: Medline
    Barkovich AJ
    Pediatric Neuroimaging
    2000  

     
     Pediatric Radiology CasesKeywords:

    Tuberous, sclerosis, brain, Bourneville-Pringle's disease, tuberous sclerosis, mental retardation, epilepsy, skin, Adenoma sebaceum, facial angiofibromas, nasolabial fold, ungual fibromas, depigmentation, kidney, Angiomyofibromas, retinal hamartoma, Lymphangioleiomyofibromatosis, phakomatosis, child, childhood, pediatric radiology  

     
     Pediatric Radiology Cases Cite this article:

    Wolfgang Hirsch, RP Spielmann. Tuberous sclerosis of the brain / Bourneville-Pringle's disease. PedRad [serial online] vol 1, no. 11.
    URL: www.PedRad.info/?search=20011128175838  

     
     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 ]   
     
    Tuberous sclerosis of the brain / Bourneville-Pringle's disease
    Wolfgang Hirsch, RP Spielmann. Tuberous sclerosis of the brain / Bourneville-Pringle's disease. PedRad [serial online] vol 1, no. 11.
    URL: www.PedRad.info/?search=20011128175838


     

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    Tuberous sclerosis of the brain / Bourneville-Pringle's disease
    Other cases by these authors:

    Search Wolfgang Hirsch in Medline Wolfgang Hirsch (17)   
    Search R.P. Spielmann in Medline R.P. Spielmann (2)   

    Tuberous sclerosis of the brain / Bourneville-Pringle's disease  
     
    Tuberous sclerosis of the brain / Bourneville-Pringle's disease
    Wolfgang Hirsch, RP Spielmann. Tuberous sclerosis of the brain / Bourneville-Pringle's disease. PedRad [serial online] vol 1, no. 11.
    URL: www.PedRad.info/?search=20011128175838


     
     
    Tuberous sclerosis of the brain / Bourneville-Pringle's disease
    Wolfgang Hirsch, RP Spielmann. Tuberous sclerosis of the brain / Bourneville-Pringle's disease. PedRad [serial online] vol 1, no. 11.
    URL: www.PedRad.info/?search=20011128175838


     

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    Tuberous sclerosis of the brain / Bourneville-Pringle's disease
    Wolfgang Hirsch, RP Spielmann. Tuberous sclerosis of the brain / Bourneville-Pringle's disease. PedRad [serial online] vol 1, no. 11.
    URL: www.PedRad.info/?search=20011128175838


     




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