Presenile dementia in Israel

A nationwide epidemiologic study of presenile dementia of the Alzheimer type (PDAT) with onset through age 60 years was carried out in Israel. The Israeli National Neurologic Disease Register and clinical records of all patients discharged from hospitals between 1974 and 1983 with a neurologic or psychiatric diagnosis suggestive of dementia were reviewed. A total of 71 Jewish patients with onset of PDAT between 1974 and 1978 was ascertained. The age at onset in these patients ranged from 43 to 60 years. The median survival was 8.1 years, with slightly longer survival if onset occurred before age 55 years, even after correction for expected mortality according to age and sex. The average annual incidence rate per 100,000 population at risk was 2.4 in the population aged 40 through 60 years. Although the incidence rates were slightly greater for women, the difference between the rates by sex was not statistically significant. The age- and sex-adjusted incidence of PDAT per 100,000 population was significantly higher in those born in Europe or America (2.9) than in those born in Africa or Asia (1.4). No significant difference in survival was found between these two groups. The curve of the incidence rates by age for PDAT in Israel is continuous with that for senile dementia of the Alzheimer type collected by similar methods elsewhere, which suggests that one disease process may account for both conditions.     

Presenile dementia: Sufferers,carers and services

An extensive search was made for people with presenile dementia (PSD) still under 65 years living in the Southampton Health District. Twenty-seven sufferers were identified. People with PSD had a high degree of cognitive, behavioural and self-care disabilities and carers showed considerable stress. Services were received from the old age psychiatry team, and to a lesser extent from social services and the Community Rehabilitation Unit. Community psychiatric nurses were the commonest form of support. The four sufferers in receipt of no services were less impaired. Identified needs included more counselling and advice and specific provision for the more physically active sufferer.     

Presenile dementia in xeroderma pigmentosum]

Neurological manifestations of xeroderma pigmentosum, a rare autosomal recessive neurocutaneous syndrome, are variable. The association with progressive mental retardation, usually with onset in childhood, is well known. We present a case of x.p. with progressive presenile dementia. This combination has, to our knowledge, not yet been reported in the literature. Although no hints on another aetiology have been found, the coincidental combination of x.p. with M. Alzheimer has to be taken into consideration. CT scan and MRI showed a marked cerebral atrophy.     

Presenile dementia presenting as aphasia.

A focal aphasic syndrome was the first and outstanding manifestation of a degenerative, presenile dementia in a 67 year old man. Computerised axial tomography showed a strikingly dilated left Sylvian fissure--particularly in its posterior aspect--in the presence of moderate diffuse cortical atrophy. The radiographic findings correlated well with the clinical data. This is the first report of an aphasic disturbance of language as the initial symptom is presenile dementia.     

Presenile dementia in polycystic lipomembranous osteodysplasia

We present a 36-year-old woman with a 3-year history of cognitive decline followed by development of a small stepped gait and urinary and fecal incontinence. Workup revealed multiple bone cysts documented by X-ray and idiopathic hyperprolactinoma. An MRI confirmed the CT finding of massive bilateral basal ganglia calcification. This is the first case of polycystic lipomembranous osteodysplasia described in Germany. We conclude that patients with presenile dementia, psychosis, or early-onset Parkinsonism associated with basal ganglia calcification should undergo X-rays of hand and feet to rule out polycystic lipomembranous osteodysplasia.     

Presenile non-Alzheimer dementia with motor neuron disease and laminar spongiform degeneration

We describe the clinicopathological findings in three autopsy cases of presenile dementia with motor neuron disease. These patients had a relatively rapid course involving dementia and muscle weakness with a distal pattern of atrophy in the upper extremities. Postmortem examination revealed features of motor neuron disease and spongiform cortical degeneration. The latter change was most marked in the second layer of the frontal or temporal cortex and included minimal to mild neuronal cell loss and mild to moderate gliosis. In this report we relate these patients' laminar spongiform degeneration to three other conditions; frontal lobe dementia, primary progressive aphasia and dementia lacking a distinctive histology. These three conditions and presenile dementia with motor neuron disease may fall within the spectrum of the non-Alzheimer type frontotemporal degenerative dementia.     

Presenile dementia diagnosed as posterior cortical atrophy

Posterior cortical atrophy (PCA) was originally proposed in 1988 based on five cases of dementia presenting characteristic clinical symptoms. The concept of PCA is still not generally accepted. Herein, we present a case of a presenile female with PCA. A 57‐year‐old woman was brought to the hospital by her older sister. The patient's chief complaints were that she could not drive a car safely and had caused numerous traffic accidents. Construction apraxia and unilateral spatial agnosia were detected by neuropsychological tests. The patient could not write a coherent the letter even though she was well educated. In addition, she demonstrated slight memory disturbance and she needed her sister's support in daily life. Magnetic resonance imaging and single photon emission computed tomography examinations confirmed bilateral posterior atrophy and significant hypoperfusion in the occipital regions. The neuropathological background of PCA remains unclear. Therefore, the concept of PCA should be validated by the accumulation of information from more cases.     

Presenile dementia syndromes: an update on taxonomy and diagnosis

The four major degenerative dementias that often begin in presenescence: are reviewed. These are Alzheimer's disease, frontotemporal dementia, dementia with Lewy bodies, and Creutzfeldt-Jakob disease. Their epidemiological, genetic, and clinical features are reviewed, and controversies in taxonomy arising from recent discoveries described. Particular attention is given to the pathological role of protein aggregation, which appears to be a factor in each disease.     

Presenile dementia: the difficulties of early diagnosis.

A follow-up of 35 patients first diagnosed as having presenile dementia at York Clinic is described. In only 15 cases did progressive deterioration confirm the diagnosis. A retrospective case note study of all the patients is described comparing the clinical features and results of special investigations of those patients in whom the diagnosis was confirmed in this way and those in whom it was not. The results of this study are used to illuminate some of the special difficulties in the early diagnosis of presenile dementia.     

Presenile dementia with motor neuron disease in Japan. A new entity?

A 61-year-old woman suffered the gradual onset of difficulty with memory, concentration, and cognition at age 58. Progressively more severe dementia was accompanied by muscle wasting and fasciculation prominent in hand and bulbar muscles. An electromyogram and a muscle biopsy specimen demonstrated denervation patterns, and a computerized tomographic scan showed considerable cerebral atrophy. This report reviews cases of presenile dementia with motor neuron disease reported in Japan and discusses the possibility of a new clinicopathologic entity.     

Presenile dementia with motor neuron disease in Japan: clinico-pathological review of 26 cases.

The clinico-pathological findings of 26 cases of presenile dementia with motor neuron disease in Japan are reviewed. The characteristic features include: (1) Progressive dementia with slowly progressive onset in the presenile period. (2) Neurogenic muscular wasting during the course of illness. (3) A duration of illness to death of from one to three years. (4) Absence of extrapyramidal symptoms and definite sensory deficits. (5) No characteristic abnormalities in the CSF or EEG. (6) No known parental consanguinity of familial occurrence. (7) Non-specific mild degenerative changes throughout the CNS without evidence of cerebrovascular disease or primary degenerative dementia, but with the presence of pathological findings of motor neuron disease. The possibility that this is a new disease entity is suggested.     

Presenile primary cognitive decline or Alzheimer's dementia: 7-year clinical and neuropsychological follow-up

Early diagnosis of presenile Alzheimer's disease (AD), which would serve for prognosis and for guiding choices of treatment, is still an important, difficult task for the clinical neurologist. We studied 24 patients, 12 of whom had minor cognitive impairment or questionable dementia (PICD) and 12 who met NINCDS-ADRDA criteria for presenile AD (PAD). Using clinical, neuropsychological, neurophysiological and neuroradiological methods, we followed the patients up to two disease end-points: death or untestable condition. This paper concentrates on the main clinical and neuropsychological findings relative to these two end-points. All PAD patients evolved into clinically evident Alzheimer-type dementia, became untestable within 60 months and died within 72 months. Only 3 of the PICD patients became demented; 2 of them died during the follow-up and 1 died eight months later. The other 9 PICD patients showed only moderate cognitive decline, compatible with normal aging processes. Neurophysiological and neuroradiological findings might be an important tool for arriving at a correct early diagnosis, when they are assessed with clinical neuropsychological data.