摘要:

A case with central nervous symptoms in type V hyperlipoproteinemia was described. The 39-year-old male patient suffered from abdominal pains after fatty meals since childhood. After the age of 31 he developed attacks of cephaleas. Headaches were associated with vertigo, paresis and paresthesia of the limbs and loss of consciousness in some instances. During antilipemic treatment combined with a diet of restricted fat and carbohydrate content the patient became free of complaints. Authors emphasize the impairment of cerebral circulation and tissue hypoxia in the development of cerebral disturbances in hyperlipoproteinemia. The mechanism of the development of hypoxia is discussed.

ISSN:0014-3022    

DOI:10.1159/000114746

出版商:S. Karger AG    

年代:1976

数据来源: Karger

ISSN:0014-3022    

DOI:10.1159/000219341

出版商:S. Karger AG    

年代:1903

数据来源: Karger

摘要:

An aggregation of 14 cases of amyotrophic lateral sclerosis (ALS) was encountered in two families in Minnesota. Although the classical clinical features of ALS predominated, some members of one family showed, in addition, extrapyramidal signs, peripheral sensory impairment in the upper and lower limbs and mild mental fallout. Autosomal dominant inheritance with incomplete penetrance was the most likely mode of transmission. Pathological changes were the same as those seen in sporadic ALS although one patient also showed degeneration of the substantia nigra. These two families were compared to others in the literature and an effort was made to refine the classification of familial ALS.

ISSN:0014-3022    

DOI:10.1159/000114747

出版商:S. Karger AG    

年代:1976

数据来源: Karger

摘要:

Female patient, aged 63 died after 5 years of progressive dementia. Autopsy revealed multiple sclerosis, diffuse lymphoplasmocytic encephalitis, and Alzheimer’s disease. Possible pathogenetic connections between these disease entities are discusse

ISSN:0014-3022    

DOI:10.1159/000114748

出版商:S. Karger AG    

年代:1976

数据来源: Karger

ISSN:0014-3022    

DOI:10.1159/000219342

出版商:S. Karger AG    

年代:1903

数据来源: Karger

摘要:

The case report of a 23-year-old man with polymyositis and some myasthenic features is presented. The biopsy findings suggest a preferential involvement of the neuromuscular junctions by the inflammatory process, a fact which explains the clinical syndrome.

ISSN:0014-3022    

DOI:10.1159/000114749

出版商:S. Karger AG    

年代:1976

数据来源: Karger

摘要:

A particular form of congenital non-progressive myopathy in a 5-year-old boy is described. The morphological features include predominant atrophy and rarity of type II muscle fibres, internally placed nuclei and myofibrillar degeneration in affected fibres.

ISSN:0014-3022    

DOI:10.1159/000114750

出版商:S. Karger AG    

年代:1976

数据来源: Karger

ISSN:0014-3022    

DOI:10.1159/000219343

出版商:S. Karger AG    

年代:1903

数据来源: Karger

摘要:

The influence of long-term corticosteroid and ACTH therapy in two sisters was followed up. The periods of observation were 4 and 2 years. The high levels of corticosteroids obviously reversed the fatal progress of the disease. The relapses in the elder sister occurred during longer periods of discontinued corticosteroid therapy. In the younger the therapy was continuous. There was no relapse except for a short one appearing after the tooth extraction and discontinuation of the therapy for 10 days. Some correlation between neurological signs and symptoms and porphobilinogen and uroporphyrin levels were found. The question arises how it is possible to influence the genetically determined disease by corticosteroids or ACTH.

ISSN:0014-3022    

DOI:10.1159/000114751

出版商:S. Karger AG    

年代:1976

数据来源: Karger

摘要:

A neuropathological description is presented of a solitary giant abscess located in the oblongate medulla of a child aged two. This abscess destroyed almost all the morphological structures in the medulla and caused considerable constriction and translocation of the IVth ventricle. Apart from the exceptional occurrence of an abscess in this brain region, the interesting features of this case are the slow development of the abscess (probably 8 months) and the existence of a well-formed connective tissue capsule, demarcating the lesion from its surroundings. The diagnostic difficulties and clinical and histopathological correlation are also discussed.

ISSN:0014-3022    

DOI:10.1159/000114752

出版商:S. Karger AG    

年代:1976

数据来源: Karger