Gliomatosis cerebri

Gliomatosis cerebri

Infobox_Disease
Name = Gliomatosis cerebri


Caption =
DiseasesDB =
ICD10 =
ICD9 =
ICDO = 9381/3
OMIM =
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eMedicineSubj =
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MeshID = D018302

Gliomatosis cerebri (infiltrative diffuse astrocytosis) is a rare primary brain tumor. It is commonly characterized by diffuse infiltration of the brain with neoplastic glial cells that affect various areas of the cerebral lobes [ [http://rad.usuhs.mil/medpix/radpix.html?mode=single&comebackto=mode%3Dgeo_browse&recnum=923 Gliomatosis Cerebri, MedPix : 923 - Medical Image Database and Atlas ] ] .

While gliomatosis cerebri can occur at any age, it is generally found in the third and fourth decades of life.

Presentation

It may affect any part of the brain or even the spinal cord, optic nerve and compact white matter. Clinical manifestations are indefinite, and include headache, seizures, visual disturbances, corticospinal tract deficits, lethargy, and dementia.

Diagnosis

Before the advent of MRI, diagnosis was generally not established until autopsy. Even with MRI, however, diagnosis is difficult.cite journal |author=Bendszus M, Warmuth-Metz M, Klein R, "et al" |title=MR spectroscopy in gliomatosis cerebri |journal=AJNR Am J Neuroradiol |volume=21 |issue=2 |pages=375–80 |year=2000 |pmid=10696026 |doi= |url=http://www.ajnr.org/cgi/pmidlookup?view=long&pmid=10696026] Typically, gliomatosis cerebri appears as a diffuse, poorly circumscribed, infiltrating non-enhancing lesion that is hyperintense on T2-weighted images and expands the cerebral white matter. It is difficult to distinguish from highly infiltrate anaplastic astrocytoma or GBM.cite web |url=http://www.urmc.rochester.edu/smd/Rad/neurocases/Neurocase01.htm |title=Radiology, University of Rochester Medical Center |accessdate=2008-02-06 |format= |work=]

Prognosis

The prognosis for gliomatosis cerebri is generally poor, with a median survival time of only 12 months. [ [http://www.childrenshospital.org/az/Site963/mainpageS963P0.html Gliomatosis Cerebri - My Child Has - Children's Hospital Boston ] ] Surgery is not practical considering the extent of the disease, standard chemotherapy (nitrosourea) has been unsuccessful, and while brain irradiation can stabilize or improve neurologic function in some patients, its impact on survival has yet to be proven.Fact|date=February 2007

Notes


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