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Epidemiology and subtypes of dementia
Published in Marjolein de Vugt, Janet Carter, Understanding Young Onset Dementia, 2021
Dementia is a syndrome and although degenerative cases are most common, dementia is caused by many different diseases. It is essential to recognise that dementia may be reversible if the correct diagnosis is made and treated. It is also useful to differentiate patients with prominent cognitive deficits arising from pathology in the cerebral cortex (cortical dementia) and those with prominent basal ganglia, thalamic or brainstem pathology (subcortical dementia). Cortical dementia is characterised by clear errors in specific cognitive domains with relatively preserved cognitive speed (Albert et al., 1974). In contrast, subcortical dementia is characterised by profound cognitive slowing, frontal executive dysfunction, and memory retrieval impairment (Albert et al., 1974). These clinical phenotypes do overlap, but the distinction between the two broad categories is useful.
Dementia in Movement Disorders
Published in W. R. Wayne Martin, Functional Imaging in Movement Disorders, 2019
The initial descriptions of progressive supranuclear palsy (PSP), Huntington’s disease, and Wilson’s disease all included accounts of behavioral or intellectual abnormalities. Dementia was one of the cardinal features that permitted PSP to be recognized as a distinct disease process by Steele, Richardson, and Olszewski.1 It was soon noticed that the dementia exhibited by patients with PSP was different from that seen in other diseases. The term “subcortical dementia” was coined to emphasize its special characteristics,2 and it has received much attention as a prototype for dementias associated with movement disorders.
B
Published in Anton Sebastian, A Dictionary of the History of Medicine, 2018
Binswanger Disease A progressive subcortical encephalopathy leading to a classic picture of dementia, which occurs mostly in the fifth and sixth decades of life. Described by a professor of psychiatry at Jena, Otto Ludwig Binswanger (1852–1929) in 1894. See subcortical dementia.
Surgery plus adjuvant radiotherapy for primary central nervous system lymphoma
Published in British Journal of Neurosurgery, 2020
Connor J. Kinslow, Ali I. Rae, Alfred I. Neugut, Christopher M. Adams, Simon K. Cheng, Sameer A. Sheth, Guy M. McKhann, Michael B. Sisti, Jeffrey N. Bruce, Fabio M. Iwamoto, Adam M. Sonabend, Tony J. C. Wang
The role of RT in PCNSL is controversial and still unresolved. Although adjuvant whole-brain radiation therapy (WBRT) after high-dose methotrexate combination chemotherapy (HD-MTX CCT) increases progression-free survival,16 20–30% of patients experience a late-stage neurotoxicity.14,4652,53 This presents as rapidly progressive subcortical dementia and is characterized by psychomotor slowing, executive and memory dysfunction, behavioral changes, gait ataxia, and incontinence.14 Such findings have led to significant disagreements about the role of RT for PCNSL, even among experts.4753,54 While some have stated that RT is no longer relevant for PCNSL,54 others argue that the risk of recurrent disease is twice that of neurotoxicity, and that preventing relapse should, therefore, be the primary focus.53 As an alternative, chemotherapy with reduced-dose WBRT is associated with high rates of complete response and disease control with minimal neurotoxicity.12,4955,56 This treatment strategy is now actively being explored.53
A review and update on the diagnosis and treatment of neuropsychiatric Wilson disease
Published in Expert Review of Neurotherapeutics, 2019
Sean Cleymaet, Katsuko Nagayoshi, Edward Gettings, Justin Faden
In a study examining the cognitive function of 67 patients with WD, patients already showing neurologic symptoms performed significantly worse (p < .05) in all attention focused tasks than patients with WD that were neurologically asymptomatic as measured by the Test of Everyday Attention (TEA) [31]. Intact attention depends heavily on cortical-subcortical processing loops and is aberrant in WD patients secondary to subcortical basal ganglia involvement. In advanced disease, patients will have subcortical dementia symptoms that include slowed thinking, memory loss, and executive dysfunction. Cortical signs such as aphasia, apraxia, and agnosia are usually absent.
Neurofibromatosis type 1: A neuro-psycho-cutaneous syndrome?
Published in Orbit, 2018
Christopher K.H. Burris, Michael A. Stier, Shahriar Salamat, Shari Thomas, Simone Lauderdale, Meisha L. Raven, Maria E. Rodriguez, Devasis N. Reddy, Heather D. Potter, Daniel M. Albert
Though ophthalmologists frequently emphasize NF1’s visually apparent stigmata, the psychosocial and emotional morbidities are often overlooked. Several studies have confirmed the associations between NF1 and anxiety, depression, dysthymia, low self-esteem, poor body image, social withdrawal/isolation, behavioral problems, sleep disturbance, and decreased cognitive functioning. Besides the direct effects of NF1, each of the interventions in this case (brain biopsy, tumor resection, ventriculostomy, chemotherapy) could have caused or contributed to her depressive symptoms.3–7 The relationship between chronic medical illness and depression has been well described, with anxiety disorders being common in cancer patients, patients with kidney disease, liver failure, congestive heart failure, and stroke.8–13 Some of the psychological features in NF1 may be the outcome of the emotional burden of chronically carrying a potentially devastating, progressive disease with outward signs.14,15 Loss of independence related to impaired neurological functioning may also play a role, such as the near total loss of vision and intellectual disability in our patient.16 Corpora amylacea are associated with aging and neurodegenerative processes, but are nonspecific.17 Zoller et al. proposed that the NF1 related cognitive deficits may result from white-matter lesions in the subcortical brain areas due to the proliferation of glial cells, hamartomas, or aberrant myelination and believed the symptoms compared favorably to those seen in subcortical dementia.18 Much is still unknown about the interplay between the neurological, psychological, and social aspects of NF1, and literature on NF1 and suicide is scant. Research in this area needs further study. Though the mechanisms behind the psychological disturbances and cognitive impairment seen in some NF1 patients are still unclear, the need for early intervention with professional psychological evaluation for affected individuals and their families is unquestionable.