Biomedical subjects

H Braak

Publications and source records attributed to H Braak.

221 records · Page 13Linked to original sources

PubMedDate not supplied

The progression of Alzheimer's disease from limbic regions to the neocortex: clinical, radiological and pathological relationships.

Alzheimer's disease (AD) is characterised by the gradual accumulation of neurofibrillary pathology in selected regions of the brain. Earlier studies indicate that the accumulation of neurofibrillary tangles is associated both with decline in patient's cognitive performance as well as with medial temporal lobe atrophy on CT scans. There are also indications that progression through the pathological stages of AD is associated with decline in cognitive functions. The results of this study indicate that progression of disease, especially beyond the boundaries of the limbic regions, is associated with marked decline in the cognitive performance of patients suffering from AD. However the clinical manifestations of early pathological stages are not so well defined. We also found that the atrophy of the medial temporal lobe on CT scans is related to the progression of pathology. Atrophy is most apparent when the disease reaches its isocortical stages and is not marked in the limbic stages of the disease. The additive effect of pathologies co-existing with AD is apparent in reduced cognitive scores, while the atrophy of limbic structures, as measured on CT scans, seems to be mainly attributable to AD-related pathology.

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PubMedDate not supplied

Staging of Alzheimer-type pathology: an interrater-intrarater study.

The staging method proposed by Braak and Braak is based on the sequential accumulation of neurofibrillary pathology in the cerebral cortex. Unlike the currently used diagnostic criteria for Alzheimer's disease (AD) it does not take into consideration the age of the patients and whether they were demented or not for the establishment of the pathological stage of the disease. To examine the interobserver reliability of the method we performed an inter- and intrarater study using the Braak staging method in 41 brains. The agreement between the examiners and between the diagnoses of the same examiner at different times was almost perfect, the kappa statistics reaching values above 0.90. These findings indicate that the staging, relying on the differential distribution of neuritic pathology in the brain in AD, is a reliable and reproducible method for the description of AD-related pathology. This makes it suitable for brain-banking and research purposes.

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PubMedDate not supplied

Adult neuronal ceroid-lipofuscinosis.

Among the different clinical forms of neuronal ceroid-lipofuscinosis (NCL), the adult type is the least frequent, most sporadic and most difficult one to diagnose. Clinical symptomatology differs from the classical childhood NCL forms in that ocular symptoms are absent while changes of behavior, dementia and seizures dominate the clinical picture. Excessive accumulation of NCL-specific lipopigments has largely been explored in the nervous system, where pigmento-architectonic investigations disclose layer-specific cortical pathology similar to but less pronounced than that of juvenile and protracted juvenile NCL. Ultrastructural analysis of lipopigments in adult NCL reveals diversity of lipopigment fine structure, but less impressive than in the childhood forms of NCL. Abnormal accretion of lipopigments outside the nervous system has rarely been demonstrated and requires ampler documentation, making in vivo diagnosis of adult NCL often difficult and sometimes equivocal. Adult NCL is now frequently considered identical to "Kufs' disease". However, in the past, the latter term has comprised a heterogeneous spectrum of lipidoses the NCL-nature of which had not been unequivocally established. Thus, one may either speak of "Kufs' syndrome" or abandon this term altogether. Although patients afflicted with adult NCL may suffer from Kufs' disease, not all who have and had Kufs disease may have or have had adult NCL. The current debate on adult NCL centers around scepticism concerning many of the earlier reports, on incorporating diagnostic studies of non-CNS organs in presumptive patients and on distinguishing adult NCL from "atypical" patients or forms of NCL, as well as other disorders marked by non-specific abnormal accumulation of lipofuscin.

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PubMedDate not supplied

Projection neurons of basolateral amygdaloid nuclei develop meganeurites in juvenile and adult human neuronal ceroid lipofuscinosis.

In human neuronal ceroid lipofuscinosis (NCL) meganeurite formation is seen in specific portions of the basolateral amygdaloid complex. Proceeding from the lateral nucleus via the basal nuclei to the accessory basal nucleus, pathological changes, from severe to mild, are found. The parvocellular granular nucleus and the intercalated cell masses remain unchanged. Only the projection cells of the lateral, basal, and accessory basal nucleus develop lipopigment filled meganeurites.

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PubMedDate not supplied

Morphological changes of the isocortex in morbus Parkinson.

An increasing number of pyramidal cells in the third layer of the human neocortex with pigment-filled expansions of their proximal axon is correlated with advancing age. Comparison between cases of morbus Parkinson and an age-matched control group reveals that the number of these pathologically altered pyramidal cells is significantly higher in cases of morbus Parkinson than in controls.

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