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Biomedical subjects

Denise M Damek

Publications and source records attributed to Denise M Damek.

4 recordsLinked to original sources
PubMed2005

Paraneoplastic Retinopathy/Optic Neuropathy.

Paraneoplastic retinopathy and paraneoplastic optic neuropathy comprise a heterogeneous group of ocular syndromes associated with various clinical symptoms and multiple circulating antiretinal antibodies. Current evidence supports an underlying autoimmune mediated process, which is the rationale for the provision of immunosuppressive therapy in addition to antitumor treatment. There are no controlled clinical trials that address the treatment of paraneoplastic retinopathy and/or optic neuropathy. Management decisions must be based on a relatively small number of case reports. There have been no reports of spontaneous visual improvement in these disorders. Therefore, any improvement after treatment is attributable to the therapeutic intervention. With the exception of the paraneoplastic optic neuropathy patient group, most patients show little or no response to immunosuppressive therapy, and only a small percentage of patients have dramatic improvement. However, modest improvements in visual function can improve patient quality of life and functional independence. Prompt diagnosis and initiation of therapy before significant visual loss is seen seems to be a critical factor in treatment success. An increase in serial autoantibody titers may serve as a marker of disease activity and allow initiation of therapeutic interventions before symptomatic visual decline.

Journal Article↗
PubMed2005

Lymphomatosis cerebri as a cause of white matter dementia.

Primary central nervous system lymphoma most often presents as a solitary, isolated lesion in immunocompetent patients. Rarely, the disease presents as a diffuse, infiltrating condition without formation of a cohesive mass, a pattern called lymphomatosis cerebri. We present 3 immunocompetent individuals who developed rapidly progressive dementia. Magnetic resonance imaging features mimicked other disorders of white matter and prompted preoperative diagnoses of Binswanger's disease (subcortical ischemic vascular dementia), unknown leukoencephalopathy, viral infection, or infiltrating glioma. Neuropathologic examination at biopsy (Poon T, Matoso I, Tchertkoff V, Weitzner I Jr, Gade M. CT features of primary cerebral lymphoma in AIDS and non-AIDS patients. J Comput Assist Tomogr . 1989;13:6-9) and autopsy (Schwaighofer BW, Hesselink JR, Press GA, Wolf RL, Healy ME, Berthoty DP. Primary intracranial CNS lymphoma: MR manifestations. Am J Neuroradiol . 1993;10:725-9) demonstrated nonnecrotic, diffusely infiltrating, large-cell B-cell lymphoma of white matter, with relative sparing of gray matter, and without significant leptomeningeal involvement or bulky periventricular disease at autopsy. Microglial and astrocytic reactions, but only subtle myelin pallor, were evident as individual tumor cells permeated the entire brain and spinal cord, albeit with considerable variation in cell density. Individual tumor cells could be identified from the optic nerve to spinal cord, documenting the "whole-brain" nature of the disease. CD20 immunostaining was necessary to fully appreciate the extent of individual lymphoma cell percolation through the white matter. The neurobehavioral deficits manifested by these patients demonstrate that lymphomatosis cerebri is an additional neoplastic cause of white matter dementia and can be added to the growing list of disorders responsible for this syndrome.

Aged↗
PubMed2003

Gliomatosis cerebri: neurobehavioral and neuropathological observations.

BACKGROUND: Gliomatosis cerebri is a rare neoplasm in which individual neoplastic cells diffusely permeate the brain; a cohesive tumor mass may appear late in the disease course, or not at all. The diagnosis can be made either at autopsy or premortem by combining biopsy and neuroimaging findings to demonstrate involvement of more than two lobes of the brain. Extensive hemispheric white matter and corpus callosum infiltration is characteristic, with lesser spread to subcortical and cortical gray matter. Whereas this pattern of localization can be predicted to cause significant disturbances of higher function, the neurobehavioral profile of gliomatosis cerebri patients has not been well described. METHOD: Three patients with gliomatosis cerebri had detailed neurobehavioral assessment, and one had neuropsychological testing early in the disease. Neuropathological investigation focused on the localization of the neoplasm, the correlation between extent of myelin and axon damage with tumor cell density, and the histogenesis of the tumor. RESULTS: The patient with neuropsychological testing had impaired executive function and verbal memory retrieval that reflected bifrontal and left temporal white matter tumor involvement seen on neuroimaging. In the other cases, apathy and fatigue progressed to severe dementia in association with bihemispheric white matter infiltration. Myelin and axon stains and myelin stains showed relative preservation of white matter architecture with severity of damage paralleling increased tumor cell density. Immunostaining for TP53 was found in a high percentage of tumor nuclei in two of three cases, suggesting overlapping features between gliomatosis cerebri and diffuse astrocytomas. CONCLUSIONS: Subtle cognitive and emotional alterations antedate the florid dementia that develops later in the course of gliomatosis cerebri. Clinical, neuroimaging, and neuropathological data suggest that white matter is damaged directly by the tumor and its associated mild edema, although infiltration of subcortical and cortical gray matter also occurs to a variable extent. Strong TP53 immunostaining in gliomatosis cerebri suggests a commonality with diffuse fibrillary astrocytomas that also often show TP53 staining. Gliomatosis cerebri can be considered a cause of white matter dementia resulting from preferential neoplastic disruption of white matter tracts.

Adult↗