Gliomatosis Cerebri

[Ane__0__0]

    
      Re: gliomatosis cerebri
    


[ Follow Ups ] [ Post Followup ] [ The Neurology Forum ] [ FAQ ]



Posted by CCFNeuro MD on June 11, 1997 at 14:06:07:

In Reply to: gliomatosis cerebri posted by joanne on June 04, 1997 at 20:06:45:

: Doctors:
     My uncle recently died of an (at the time) unknown nuerologic problem.  The autopsy report
  states that he had diffuse gliomatosis.  I am just finishing my first year of medical school
  so the responsibilty to learn about this disease fell to me.  However, I have had little luck
  finding out information.  Does this occur uni or bilaterally? What glial cells are affected?  
  I was told by my mother that it was the microglial cells, however I have found even less on
  microgliomatosis... is this a type of gliomatosis, or did my mother get inaccurate info?  
  How is this diagnosed, and if it can be diagnosed before autopsy, what type oftreatment should
  have been attempted.  Finally,are there any good review articles on this subject?  
=================================================================================================
Hello, Joanne.  I hope you’re enjoying medical school.  I’m not surprised to hear you have had a difficult time finding information on gliomatosis cerebri;  its an uncommon disorder.   I must confess, I am not an expert in this subject.   The term “gliomatosis cerebri” refers to a neoplastic disorder in which there is proliferation of glial cells.  In essence, it is a very , very diffuse, infiltrative tumor, which can involve both the grey and white matter of the brain. As you know,  glial cells include astrocytes, oligodendrocytes, ependymal cells and microglia. ( Astrocytes play a structural role as well as help regulate the composition of the extracellular fluid, oligodendrocytes form myelin, ependymal cells line the ventricles and central canal of the spinal cord, and the microglial cells are “macrophages of the brain”. )  As far as I know, the abnormal cell proliferation can be composed of any of the different types of glial cells, with the possible exception of the microglial cells, although astrocytes are probably the most common.(I am not sure on this point, though).  Gliomatosis cerebri is typically bilateral.  It is extremely difficult to diagnose and is often .  The appearance on imaging studies  is of a rather diffusely abnormal signal (rather than a focal mass) and other diagnostic possibilities are usually raised such as leukoencephalopathies and demyelinating diseases, dysmyelinating disease, and infections such as SSPE (subacute sclerosing panencephalitis), to name a few.  Biopsy is necessary to make the diagnosis antemortem, but can be nondiagnostic.  Unfortunately, the prognosis is very poor.   Treatment includes whole brain radiation and chemotherapy but I do not know whether treatment actually prolongs survival.  I found a review article which was  published in Surgical Neurology 36(6): 431-40, 1991 December by Ross  et al.  I have not read it, but it sounds like it may be helpful to you.  Good luck with your studies.  
Please note that this information is provided for general medical education purposes only.