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Of brain and bone: The unusual case of Dr. A
Published in Howard J. Rosen, Robert W. Levenson, Neurocase, 2020
J. Narvid, M. L. Gorno-Tempini, A. Slavotinek, S. J. DeArmond, Y. H. Cha, B. L. Miller, K. Rankin
In summary, neuropathological investigations identified cerebral cortical atrophy in an asymmetric pattern, most convincingly in the frontal and temporal lobes. Numerous neurons elaborated tau protein, especially within the hippocampus and temporal lobe. Additionally, scattered tau positive astrocytes were found cerebral cortex and subcortical white matter. These patterns are most consistent with a diagnosis of Pick’s disease.
Immunochemical Approaches to the Diagnosis of Alzheimer Disease
Published in Robert E. Becker, Ezio Giacobini, Alzheimer Disease, 2020
None of the antibodies mentioned so far are disease specific. All of these antibodies appear to recognize antigens present in all neurofibrillary tangle including those in Pick;s disease, progressive supranuclear palsy and Guam-Parkinson’s dementia complex (Wolozin 1987). Hence, the reactivity of the antibodies is technically NFT related reactivity rather than AD related reactivity. It turns out that this relatively broad pathologic specificity does not decrease the potential specificity of the antibody tests for AD. The reason lies in quantitative considerations of the amount of pathology. AD brains have 30 times as much Alz-50 reactivity per a gram wet weight as do other diseases with NFT pathology. The reason for this most likely lies with the extensive neuritic involvement in AD that is absent in other diseases. The potential disease specificity of these tests is yet greater because of spacial considerations. AD affects practically the entire mass of the brain, whereas Pick’s disease affects only the frontal and temporal cortex and PSP affects only the brain stem. If CSF reactivity were related only to the mass of affected tissue then the amount of reactivity present in AD brains should be at least several times greater than that of other diseases with NFT pathology, independent of considerations related to the density of antigen present.
Diseases of the Nervous System
Published in George Feuer, Felix A. de la Iglesia, Molecular Biochemistry of Human Disease, 2020
George Feuer, Felix A. de la Iglesia
In Pick’s disease the clinical patterns are loss of memory and progressive dementia. The brain of affected individuals shows diffuse cortical atrophy which is often very severe inone or both frontal or temporal lobes (Plate 3). There is a moderate to extensive loss of neurons in affected areas and usually intense gliosis. Some of the neurons are greatly distended and large cytoplasmic inclusions replace the nuclei. The accumulation of these lipoprotein particles probably represents a metabolic defect related to the disease. There are changes in the presence of trace elements in the impaired neurons.197
Posterior cortical atrophy: clinical, neuroimaging, and neuropathological features
Published in Expert Review of Neurotherapeutics, 2023
John Best, Marianne Chapleau, Gil D. Rabinovici
The purpose of this review is to provide readers with an overview of Posterior Cortical Atrophy syndrome, including clinical, imaging, pathological, and genetic features, and treatments. Posterior cortical atrophy (PCA) is a term first introduced by Frank Benson and colleagues in 1988 [1]. Benson’s original case series described five patients with early and prominent visual dysfunction, including alexia and visual agnosia. The patients all developed progressive visuospatial and navigational dysfunction (environmental agnosia) with preserved visual acuity. Eventually, most parietal lobe functions were affected, though memory was notably spared until late disease stages. Atrophy of posterior association cortices was noted on computed tomography (CT) or magnetic resonance imaging (MRI). Benson hypothesized that this newly described dementia syndrome represented an atypical presentation of Alzheimer’s disease, Pick’s disease, or possibly a novel clinicopathological entity.
Mixed neuropathology in frontotemporal lobar degeneration
Published in Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 2020
Catherine Pennington, Luca Marini, Elizabeth Coulthard, Seth Love
Tauopathies are frequently seen as co-existent or incidental pathologies in patients with other forms of neurodegeneration (23,24) but the incidence of mixed pathology in those with a primary tauopathy is understudied. An autopsy series of 33 people with neuropathologically diagnosed CBD found coexisting Alzheimer’s spectrum pathology in 3 cases (25). An archival review of 66 cases of Pick’s disease identified proteinopathy consistent with low or intermediate level probability of AD in 3 cases, and occasional alpha-synuclein immunopositivity (26). In our cohort, most of those with FTLD-tau had no other neuropathology present. Why mixed neuropathology is less frequent in FTLD-tau than FTLD-TDP-43 is unclear; this discrepancy was not explained by age at death, or by an excess of any one particular neuropathology with FTLD-TDP.
Fear, Defensive Strategies and Caring for Cognitively Impaired Family Members
Published in Journal of Gerontological Social Work, 2019
Dale Spencer, Laura M. Funk, Rachel V. Herron, Emily Gerbrandt, Lisette Dansereau
Brian was believed by professionals to have Pick’s disease, a frontal lobe dementia affecting personality and behavior, with memory loss normally being less pronounced as a symptom until later in the disease. Brianna’s narrative revealed strong feelings of fear that led to her decision to leave and divorce her husband (i.e., flight response). She experienced physical, emotional, and verbal aggression from Brian, whom she described as previously calm and gentle. Brian’s personality and behavioral changes were so drastic that Brianna wondered, “who is this stranger coming into our lives?” This change in disposition changed the structure of the relationship. As similarly reported by other carer participants, however, Brian’s condition progressed quickly to include scapegoating her (a form of identity threat): “the tables started to turn and he started saying I was the one with dementia. And, ‘you’re mentally ill, I’m not the one.’” This transference and denial was central to Brianna’s narrative and was compounded by a lack of support from other family members, who chose to believe Brian over Brianna. Brianna was in this and other ways embedded in conditions that overdetermined her exposure to fear, intimidation, and violence. She became very scared of her husband, who was verbally abusive, and although he did not physically hit her, he made threatening gestures (e.g., a fist in front of her face). In one example, Brian’s rage toward a stuck door created personal fear: Brianna: We couldn’t get into the house one day; the key wouldn’t go in for some reason. He went berserk, kicking the door and I said, “Brian calm down, we’ll go to the other door.” He just kept kicking; he was just in this rage. So I backed off and went around and opened the door and came. It turned out there was damage done; he almost kicked the door in and [doctor] said in hindsight that I could have called the police then. I could have reported that because I was scared.