Creutzfeldt-Jakob disease segregating in a three generation Danish family.

A three generation family is presented in which rapidly progressive, early-onset Creutzfeldt-Jakob disease without typical EEG changes segregates as an autosomal dominant disease. An aspartic acid to asparagine mutation at codon 178 of the prion gene, PRNP, co-segregates with the disease. As expected, the disease allele also carries the valine codon of the polymorphic valine/methionine codon 129 of the gene. In family members homozygous for this valine codon the disease was more rapidly progressive than in a heterozygous family member, who had a variant clinical phenotype. Definite neuropathological diagnosis required prion staining with specific antibodies.

[A symptom profile of hospitalization-requiring depression among the elderly related to clinical subgroups. A way to improve diagnostics]. / Symptomprofil ved indlaeggelseskraevende depression hos aeldre, relateret til kliniske undergrupper. En genvej til forbedret diagnostik.

UNLABELLED: The objectives were to study clinical symptoms of major depression in later life by using relevant assessment scales of psychopathology, behavioural disorders, and physical functioning. The evaluations on admission and at discharge were based on interdisciplinary observations and interview of patients. The study was carried out during twelve months in a psychogeriatric university department and encompassed 202 patients. All (N = 80) (40%) patients who had a principal diagnosis of major depression are accounted for. Mean age was 79.5 years. Results of the assessments all showed significant improvements (p = 0.0000-0.0024). The overall results of the assessments show that the key symptom of sadness is often missing, muted, or overshadowed by other symptoms, and that behavioural disturbancies and functional disability may be important symptoms in geriatric depression. IN CONCLUSION: adequate treatment of major depression in the elderly significantly improves psychopathology, behavioural disorders and physical functioning. When dealing with mental disorders in this age group, awareness of the five clinical presentations in major geriatric depression, i.e. dementoform, somatoform neurotiform, eretic and classic geriatric depression, may target the diagnostic procedure.

Psychopathologic and functional outcome in the treatment of elderly inpatients with depressive disorders, dementia, delirium and psychoses.

The objectives of this report were to investigate the functional implications and the possible rehabilitation potentials of dementia, delirium, and psychosis in elderly inpatients, compared with that in depression. During 1 year, all patients in a psychogeriatric university clinic were assessed on admission and at discharge with a selection of rating scales and diagnosed according to ICD-10 by consultants with no knowledge of the results of the ratings. All patients who had a principal diagnosis of major depression, dementia, delirium, or a psychosis are discussed. Ratings were made for psychopathology, behavioral disorders, depressive statements, intellectual functioning, activities of daily living, and gait. Depressive and delirious patients improved their status significantly (p < or = .03) in all six assessments, and patients with dementia improved their psychopathology status (p = .002), but the other assessments were unchanged. Results from the small sample (n = 8) of psychotic patients were mainly inconclusive, but there was a tendency for improvement with respect to psychopathology and gait.

[A clinic for the study of dementia--110 consecutive patients]. / Klinik for demensudredning--110 konsekutive patienter.

Case reports of 110 patients referred to a neurological dementia clinic were reviewed to evaluate a standardized diagnostic program. The patients were evaluated by a neurologist, a gerontopsychiatrist, and a neuropsychologist. ICD-10 criteria were used. Fifty-two patients had dementia while 58 had not; of these, 27 suffered from other non-dementia diseases and 31 were without dementia or other psychiatric or neurological disease. Thirteen patients with Alzheimer's disease were treated with tacrine. Four patients underwent cobalamin substitution treatment and seven started antidepressant medication. Ten patients received acetylsalicylic acid (150 mg Q.D.) and two a levo-dopa-type drug. Twenty-six patients were followed by gerontopsychiatric district care. Because only 47% of the patients suspected of dementia actually fulfilled dementia criteria, the evaluation suggests that patients suspected of dementia benefit from a standardized diagnostic program in a specialist setting.

[Improvement of functional abilities after treatment of depression in the elderly]. / Funktionsforbedring ved behandling af depression hos aeldre.

During one year all patients with a diagnosis of depression in accordance with ICD-10, referred to a psychogeriatric department, were assessed at admission and discharge with a number of rating scales: The Brief Geriatric Depression Scale, Katz' ADL-index, the Multidimensional Dementia Assessment Scale, the Mini-Mental State Examination and the Functional Ambulation Classification. The department has an active stepwise treatment strategy: SSRI (selective serotonin reuptake inhibitor), potentiation with mianserin, lithium potentiation, ECT. The 87 depressive patients had a median age of 79 and most had one or more severe somatic conditions. Fifty-nine were severely depressed at admission, 19 at discharge; the number of functionally disabled dropped from 22 to seven and the number of cognitively impaired from 35 to 19. In conclusion, a nihilistic approach to treatment in the very old is unfounded.

[Lewy-body dementia--a new disease entity]. / Lewy-body demens--en ny sygdomsenhed.

Senile dementia of Lewy-body type (SDLB) is a new clinical entity characterized by mild extrapyramidal features and progressive dementia, a relative acute onset of fluctuating loss of memory and confusion, frequently accompanied by visual hallucinations. The syndrome is more frequently identified in women than in men and it occurs after the age of 60 years. A case history is presented and the diagnosis discussed on the basis of literature concerning Lewy-body dementia (LBD).

Calcification of the central nervous system in a new hereditary neurological syndrome.

A case of a new hereditary neurological condition with extensive calcifications of the central nervous system is described. The calcium deposits were especially localized to the leptomeninges, the first layer of the cerebral and cerebellar cortex, and along the ventricular wall. The neuropathological findings were in accordance with the clinic. The case was familial and the pedigree suggested an X-linked recessive inheritance.