The dementia of dementia praecox

A group of 18 long-stay patients with a diagnosis of schizophrenia were compared with a group of 10 age-matched subjects who had been institutionalized by reason of physical disease, on performance on tests of intellectual function; and with a group of agematched healthy subjects, both on tests of intellectual function, and radiographically, using the technique of computerized axial tomography (EMI scan) of the brain. By comparison with the normal controls the patients with schizophrenia had increased cerebral ventricular size (assessed as cross-sectional area) and, by comparison with both control groups, showed substantial impairments on intellectual testing. The differences in ventricular area between patients and controls remained significant (P < 0.01) after four patients who had been leucotomized had been excluded. Within the non-leucotomized patient group ventricular area was unrelated to previous neuroleptic medication, ECT or insulin coma therapy, but there was a significant relationship between ventricular area and intellectual impairment (P < 0.01). Intellectual impairment, as assessed by the Withers & Hinton test battery, the Inglis paired associate learning test, and the digits-backward test, was greater (P < 0.05) in patients with negative features (affective flattening, retardation, poverty of speech) than in those without such features. Premorbid occupational histories suggested that nearly all of these patients had at one time functioned at an adequate intellectual level. The findings suggest that within the group of patients with schizophrenia there is a subgroup whose illnesses have hitherto been considered typically schizophrenic, who have severe intellectual impairment associated with evidence of structural brain disease. The size of this subgroup and the significance of the cerebral changes remain to be determined.     

Primary alcoholic dementia and alcohol-related dementia

Clinicopathological issues regarding so‐called ‘alcoholic dementia’ remain under debate. Although clinical observation favors the diagnosis of primary alcohol dementia, caused by direct alcohol neurotoxicity, further confirmation from neuropathological and biochemical perspectives is warranted. Repeat episodes of subclinical Wernicke–Korsakoff’s syndrome may partially account for the chronic state of primary alcoholic dementia, thus supporting the notion that primary alcoholic dementia exists in continuum with chronic and subclinical types of Wernicke–Korsakoff’s syndrome. Diagnostic criteria for alcohol related dementia, as detailed by Oslin et al., represent a purer form of alcoholic dementia and are useful for the scientific discussion of this condition.     

Family history of dementia: dementia with lewy bodies and dementia in Parkinson's disease

Parkinson's disease with dementia (PD-D) and dementia with Lewy bodies (DLB) may result from the same neurodegenerative process with different temporal and spatial courses. The authors report an association between DLB and family history of dementia in a comparison study between patients with a clinicopathological diagnosis of PD-D and DLB. Findings suggest that positive family history for dementia is associated with DLB with a yet unknown mechanism.     

Multi-infarct dementia

Fifty-two patients presenting with dementia were divided into a group in whom clinical features suggested an ischaemic basis (multi-infarct dementia) and a group in whom a primary degenerative process seemed more likely. Focal EEG changes and angiographic evidence of ischeamic areas and atheromatous disease of intracranial vessels were more common in the “ischaemic” than in the primary degenerative group. CBF was significantly reduced in the former but the regional pattern was equally distorted in the two groups. These findings strengthen the belief that the ischaemic score can identify those patients whose dementia is associated with vascular disease.     

Thalamic dementia

Report with complementary clinical examinations and detailed neuropathological findings of a case of subacute progressing "thalamic dementia", interpreted as combined systemic degeneration of the dorsal and medial thalamic nuclei. For the development of the EEG changes, which were followed from beginning of the disease, a slowly advancing reduction of the function of the meso-diencephalic activating system proved responsible. The inferior olives were symmetrically atrophied and the fasciculus tegmenti centralis was on both sides completely degenerated. It is to be considered that the inferior olives are directly subordinated to the medial thalamic nuclei by the way of the fasciculus tegmenti centralis. In the cerebellum nerve cell groups and fiber bundles, which are closely connected with the reticular system, are degenerated. The systemic medial degenerations of the thalamus belong to the abiotrophies in the sense of Gowers original conception.     

Poststroke dementia

Dementia is one of the major causes of dependency after stroke. The prevalence of poststroke dementia (PSD)-defined as any dementia occurring after stroke-is likely to increase in the future. In community-based studies, the prevalence of PSD in stroke survivors is about 30% and the incidence of new onset dementia after stroke increases from 7% after 1 year 48% after 25 years. Having a stroke doubles the risk of dementia. Patient-related variables associated with an increased risk of PSD are increasing age, low education level, dependency before stroke, prestroke cognitive decline without dementia, diabetes mellitus, atrial fibrillation, myocardial infarction, epileptic seizures, sepsis, cardiac arrhythmias, congestive heart failure, silent cerebral infarcts, global and medial-temporal-lobe atrophy, and white-matter changes. Stroke-related variables associated with an increased risk of PSD are stroke severity, cause, location, and recurrence. PSD might be the result of vascular lesions, Alzheimer pathology, white-matter changes, or combinations of these. The cause of PSD differs among studies in relation to the mean age of patients, ethnicity, criteria used, and time after stroke. In developed countries, the proportion of patients with presumed Alzheimer's disease among those with PSD is between 19% and 61%. Patients with PSD have high mortality rates and are likely to be functionally impaired. These patients should be treated according to the current guidelines for stroke prevention.     

Frontotemporal dementia

Frontotemporal dementia (FTD) is a unique neurodegenerative disease that can be differentiated from Alzheimer's disease and other diseases that result in cognitive complaints. The primary anatomic focus of degeneration determines the clinical presentation, which can vary from aphasia to behavioral symptoms. Expanding knowledge about the genetics and biochemistry of FTD may lead to specific treatments.     

Frontotemporal dementia

Frontotemporal dementia (FTD) is an uncommon but important form of degenerative disease. It may make up 50% of dementia cases presenting before age 60. The symptoms are related to the anatomic areas affected. Neary divided the clinical syndromes into "frontotemporal dementia," "progressive nonfluent aphasia," and "semantic dementia." However, the pathology may extend beyond the frontal and temporal lobes and additional symptoms may be found. Although most cases are sporadic, some cases are genetic. The best-known genetic mutation causing FTD is frontotemporal dementia with parkinsonism, linked to the microtubule-associated protein tau on chromosome 17. There are other known genes and chromosome loci related to FTD. The most common pathology found is frontotemporal degeneration with ubiquitin inclusions. In contrast, FTD with Pick bodies is rare. Although there are strategies to help patients and their families, there is no known treatment for the disease.     

Frontotemporal dementia

Frontotemporal dementia (FTD) is a focal clinical syndrome characterised by profound changes in personality and social conduct and associated with circumscribed degeneration of the prefrontal and anterior temporal cortex. Onset is typically in the middle years of life and survival is about 8 years. The presence of microtubule-associated-protein-tau-based pathological features in some patients and the discovery, in some familial cases, of mutations in the tau gene links FTD to other forms of tauopathy, such as progressive supranuclear palsy and corticobasal degeneration. However, more than half of all patients with FTD, including some with a strong family history, show no apparent abnormality in the tau gene or protein, indicating pathological and aetiological heterogeneity. FTD provides a challenge both for clinical management and for theoretical understanding of its neurobiological substrate.     

Vascular dementia

Vascular dementia (VaD) is a term used to describe a particular constellation of cognitive and functional impairment, and is now generally seen as a subset of the larger syndrome of vascular cognitive impairment (VCI). The latter is seen as cognitive impairment in the face of cerebrovascular disease. VCI can be classified clinically by whether patients meet criteria for dementia, and whether the syndrome is distinct or overlaps with primary neurodegenerative diseases, such as Alzheimer's disease. This clinical classification can be further classified by neuroimaging, with subgroups that show cortical infarction, subcortical infarction and white matter changes, each alone or in combination. Understood in this way, VCI is likely the most common form of cognitive impairment in the population. Attempts to treat VaD had varying degrees of success, but it now appears that many forms of VCI might be preventable, especially with good control of vascular risk factors in middle age.     

Hyperlipidemic dementia

The dementia of a patient with hyperlipidemia improved dramatically on treatment with diet and fenofibrate. Rheologic study showed an abnormality of rouleaux disaggregation, and this disappeared as plasma lipid levels fell and mental state improved.