Nondegenerative Dementias and Encephalopathies

Nondegenerative dementias are a diverse but important group of cognitive disorders because they may be reversible with treatment. Thus, evaluation is important when a nondegenerative dementia is suspected. Many causes of nondegenerative dementia result in what is known as subcortical dementia, which is thought to be primarily due to damage to the frontal subcortical connections. Typical clinical features include inattention, bradyphrenia (slowed thought process), executive dysfunction (difficulties planning and sequencing tasks), apathy, psychomotor slowing, and mood disorders. Gait apraxia and urinary difficulties may coexist. Cortical features such as agnosia, seizures, aphasia, and ideomotor apraxia are typically absent.

A Comparative Study of the Behavioral Profile of the Behavioral Variant of Frontotemporal Dementia and Parkinson’s Disease Dementia

<b><i>Background:</i></b> Executive dysfunction is the common thread between pure cortical dementia like the behavioral variant of frontotemporal dementia (bvFTD) and subcortical dementia like Parkinson’s disease dementia (PDD). Although there are clinical and cognitive features to differentiate cortical and subcortical dementia, the behavioral symptoms differentiating these 2 conditions are still not well known. <b><i>Objective:</i></b> To evaluate the behavioral profile of bvFTD and PDD and compare them to find out which behavioral symptoms can differentiate between the two. <b><i>Methods:</i></b> Twenty consecutive patients with bvFTD (&#x3e;1 year after diagnosis) and 20 PDD patients were recruited according to standard diagnostic criteria. Behavioral symptoms were collected from the reliable caregiver by means of a set of questionnaires and then compared between the 2 groups. <b><i>Results:</i></b> bvFTD patients had more severe disease and more behavioral symptoms than PDD. bvFTD patients were different from PDD patients due to their significantly greater: loss of basic emotion (<i>p</i> &#x3c; 0.001, odds ratio [OR] 44.33), loss of awareness of pain (<i>p</i> &#x3c; 0.001, OR 44.33), disinhibition (<i>p</i> &#x3c; 0.001, OR 35.29), utilization phenomenon (<i>p</i> = 0.008, OR 22.78), loss of taste discrimination (<i>p</i> &#x3c; 0.001, OR 17), neglect of hygiene (<i>p</i> = 0.001, OR 13.22), loss of embarrassment (<i>p</i> = 0.003, OR 10.52), wandering (<i>p</i> = 0.004, OR 9.33), pacing (<i>p</i> = 0.014, OR 9), selfishness (<i>p</i> = 0.014, OR 9), increased smoking (<i>p</i> = 0.014, OR 9), increased alcohol consumption (<i>p</i> = 0.031, OR 7.36), social avoidance (<i>p</i> = 0.012, OR 6.93), mutism (<i>p</i> = 0.041, OR 5.67), and failure to recognize objects (<i>p</i> = 0.027, OR 4.33). The bvFTD patients were also significantly less suspicious (<i>p</i> = 0.001, OR 0.0295), less inclined to have a false belief that people were in their home (<i>p</i> = 0.014, OR 0.11) and had fewer visual illusions/hallucinations (<i>p</i> = 0.004, OR 0.107) than PDD patients. <b><i>Conclusion:</i></b> Behavioral symptoms are helpful to distinguish bvFTD from PDD, and thus also cortical dementia with frontal-lobe dysfunction from subcortical dementia.

Cerebrall Autosomal Dominant Artheriopathy with Subcortical Infarcts and Leukoencephalopathy

CADASIL is a hereditary cerebrovascular disease that affects small vessels and usually causes recurrent subcortical infarctions with white matter involvement. It is usually closely related to migraine history, in advanced cases a subcortical dementia usually occurs. It is a disease characterized by a picture of progressive deterioration, and in very advanced stages a pseudobulbar paralysis can occur. In 1955 some cases were described that were called hereditary vascular dementia, later in 1977 it was renamed hereditary multi-infarct dementia, in 1991 they came to be referred to as familial arteriopathic leukoencephalopathy [1]. Finally, with the discovery of the gene involved in the disease, it was renamed CADASIL in 1996 by Joutel. As we can see, the pathological discovery of the disease is of recent discovery, thus adopting the various nomenclatures described above.

Speech and language disturbances in a patient with the clinical features of Steele‑Richardson‑Olszewski syndrome

Steele‑Richardson‑Olszewski syndrome also known as progressive supranuclear palsy (PSP) is a neurodegenerative disease of the central nervous system of extrapyramidal type of neuropathy of unknown etiology. This chapter describes the case of 62 patients diagnosed with PSP, the changes they observe are related to selective linguistic dysfunction resulting from subcortical dementia and executive and motor dysfunction, and the functioning of the subject is significantly determined by emotional disturbance.

HIV-Associated Neurocognitive Disorders

HIV causes a chronic form of encephalitis (HIVE) that is clinically characterized by either dementia or mild neurocognitive impairment. Since the introduction of antiretroviral therapy in 1996, the incidence of HIV dementia has decreased by 50%, but the prevalence of mild neurocognitive disorder has increased up to 39%. HIVE is the result of direct microglial infection, interruption of trophic factors, or caused by inflammatory cytokines. HIV enters the brain primarily by the “Trojan horse mechanism”; it is carried by monocytes and lymphocytes that cross the blood–brain barrier. HIV has a predilection for the basal ganglia, deep white matter, and hippocampus, resulting in a subcortical dementia. HIV dementia is a diagnosis of exclusion and other co-infections, cerebrovascular disease, malnutrition, and drug abuse should be ruled out before making the diagnosis. In patients receiving antiretroviral therapy with immunological response, a novel condition termed CD8+ T cell encephalitis was recently described.