ISSN:1420-8008    

DOI:10.1159/000107309

出版商:S. Karger AG    

年代:1993

数据来源: Karger

ISSN:1420-8008    

DOI:10.1159/000107310

出版商:S. Karger AG    

年代:1993

数据来源: Karger

摘要:

The neuropathology of frontal lobe degeneration of non-Alzheimer type was reevaluated on the basis of a new material of 13 cases against the background of experiences from earlier published 16 cases. The salient neuropathological feature was an unspecific neuronal degeneration of superficial cortical layers of the frontal and to some extent the temporal lobes without markers for Alzheimer''s, Pick''s or Lewy body diseases and there were no indications so far of a prion etiology. The consistency of histopathological features are taken to indicate a disease entity, also identified by other authors. It is tentatively grouped together with progressive and aphasic dementia, similar cases in the literature and possibly also Pick''s disease and ALS with dementia.

ISSN:1420-8008    

DOI:10.1159/000107311

出版商:S. Karger AG    

年代:1993

数据来源: Karger

摘要:

The group of progressive dementing illnesses that lack distinctive histologic features includes at least four variants: a cortical type, a thalamostriate type, a motor neuronopathy type and a Ieukogliotic type. While the clinical presentation of some cases is that of anterograde amnesia, progressive aphasia and dysexecutive syndrome are the most common initial symptom complexes. A large number of reported cases are familial, although no abnormal gene has been identified. Pathologically, these illnesses are defined by cortical, hippocampal, striatal, medial thalamic, nigral and motor nuclei cell dropout and astrogliosis. In some cases, white matter gliosis is striking. Identification of specific histological or molecular markers of at least some of these conditions will greatly advance our understanding of these specific conditions as well as dementing illnesses in general.

ISSN:1420-8008    

DOI:10.1159/000107354

出版商:S. Karger AG    

年代:1993

数据来源: Karger

摘要:

Seventy-one Japanese cases of presenile dementia with motor neuron disease were reviewed. The clinico-pathological features were: (1) progressive dementia with insidious onset, mostly in the presenile period: (2) neurogenic muscular wasting in the course of illness (ALS- or SPMA-like symptoms); (3) duration from the onset of the illness to death: 2-5 years (average 30.6 months); (4) extrapyramidal symptoms and definite sensory deficits are less commonly present; (5) no characteristic abnormalities in the CSF or EEG; (6) no known consanguinity or familial occurrence; (7) non-specific mild to moderate degenerative changes in the fronto-temporal cerebral cortex, hypoglossal nuclei and spinal cord, and frequently in the substantia nigra. The author was interested in discovering whether the frequency and topology of lesions in the brain of patients with presenile dementia and motor neuron disease differed characteristically from the distribution found in cases of Alzheimer''s disease, Pick''s disease, Creutzfeldt-Jakob disease or progressive subcortical gliosis. Presenile dementia with motor neuron disease might be a new disease entity.

ISSN:1420-8008    

DOI:10.1159/000107312

出版商:S. Karger AG    

年代:1993

数据来源: Karger

摘要:

Frontal lobe degeneration of non-Alzheimer type (FLD) is the second most common primary degenerative dementia in southern Sweden. Clinical findings in 30 FLD cases with postmortem-verified diagnoses are described. FLD starts in the presenium with a mean disease duration of 7.5 years (range 3-17 years). Clinical onset is insidious and slow and the early stage is dominated by personality changes with lack of insight and judgement and signs of disinhibition. A typical feature is progressive loss of expressive speech with stereotyped phrases, late mutism and amimia. Restlessness, changes of oral/dietary behavior and utilization behavior are prevalent as also psychotic features. Temporal and spatial orientation are usually preserved for a long time in contrast to Alzheimer''s disease. Dementia in FLD is similar to that of Pick''s disease and ALS with dementia. Early recognition of FLD seems possible based on standardized clinical evaluation supported by neuropsychological tests, measurement of regional cerebral blood flow and other types of brain imaging. The etiology of FLD is unknown but a positive heredity was reported in 60%.

ISSN:1420-8008    

DOI:10.1159/000107313

出版商:S. Karger AG    

年代:1993

数据来源: Karger

摘要:

A number of causes of progressive mental impairment show a preponderance of frontal lobe symptoms. It is proposed that these disorders can be subdivided into primary and secondary frontal dementias, based on the symptomatology and the site of major pathological disruption. An anatomical explanation, based on variations in cortical and subcortical influences, aids in understanding the clinical differences.

ISSN:1420-8008    

DOI:10.1159/000107314

出版商:S. Karger AG    

年代:1993

数据来源: Karger

摘要:

Three clinical syndromes associated with fronto-temporal cerebral atrophy, studied in one centre are discussed: dementia of frontal type (DFT), DFT and motor neurone disease (MND) and progressive aphasia (PA). The pathological findings in DFT (13 brains), DFT and MND (5 brains) and PA (5 brains) permit a number of clinical pathological groupings. The nosological status of fronto-temporal atrophy is discussed with reference to the literature and it is suggested that a common underlying pathology, including Picks disease as strictly defined by the presence of inclusion bodies, underlies the clinical syndromes, each being determined by the anatomical distribution of the pathology.

ISSN:1420-8008    

DOI:10.1159/000107315

出版商:S. Karger AG    

年代:1993

数据来源: Karger

摘要:

The etiology of frontal lobe dementia of non-Alzheimer type (FLD) is still unknown. There is strong evidence of genetic factors with positive heredity. In this paper, a Swedish family, with several generations affected by FLD is described. In 3 patients typical FLD was confirmed postmortem. The clinical and neuropathological similarities between the patients are impressive.

ISSN:1420-8008    

DOI:10.1159/000107316

出版商:S. Karger AG    

年代:1993

数据来源: Karger

摘要:

We present genetic linkage data in a large family in which non-specific dementia is inherited as an autosomal dominant trait. We have analyzed 45 highly polymorphic microsatellite sequences and excluded a quarter of the genome as the site of the pathogenic mutation in this family.

ISSN:1420-8008    

DOI:10.1159/000107317

出版商:S. Karger AG    

年代:1993

数据来源: Karger