Temporal evolution of electroencephalographic abnormalities in Creutzfeldt-Jakob disease.

Frequent serial EEG investigations of three patients with neuropathologically confirmed Creutzfeldt-Jakob disease lasting 13, 24 and 68 weeks revealed typical periodic activity of short duration with stereotyped bilateral sharp waves at the 7th, 8th, and 12th week, respectively, after the onset of symptoms. During the later stages, there were several deviations from this typical pattern. However, periodic activity was preceded between the 3rd and 9th week by intermittent localized or lateralized delta rhythms, which gradually changed into periodic activity. This early temporal evolution of EEG abnormalities may be helpful in the early diagnosis of Creutzfeldt-Jakob disease when accompanied by other investigations to exclude other causes of intermittent delta rhythms.

Serial computed tomography findings in Creutzfeldt-Jakob disease.

Serial CT investigations of 3 patients with histologically confirmed Creutzfeldt-Jakob disease revealed persisting slight brain atrophy to progressive extreme atrophy corresponding to the absolute, not the individual duration of illness. No correlation was observed between CT findings and the patients' condition or electroencephalographic results. In one case with a duration of about 16 months and a terminal brain weight of 750 g a massive bilateral, later unilateral subdural hygroma appeared which probably was caused by retraction of the brain showing an enormous atrophy.

Absence of both internal carotid arteries.

A 63-year-old man developed a slight left hemiparesis. CT scan showed an intracerebral tumour, which was later identified as glioblastoma multiforme. Angiographic examination revealed the absence of both internal carotid arteries. Blood supply of anterior and middle cerebral arteries was provided by communication between a tortuous megadolichobasilar artery and the circle of Willis through enlarged posterior communicating arteries. The case is reported with reference to clinical symptoms as well as angiographic and anatomical findings of 17 comparable cases mentioned in literature.

Disorganization of glycolytic and gluconeogenic pathways in skeletal muscle of aged persons studied by histometric and enzymatic methods.

Histometric data obtained by the point counting method, and the enzyme patterns of glycolysis, gluconeogenesis, fatty degradation and energy transfer have been determined in the same muscle specimens of m. vastus lateralis from 12 untrained patients between the ages of 4 and 78 years who suffered no disturbance of the neuromuscular system. Activities of 18 enzymes have been related to pure muscle weight corrected for fatty and connective tissue content, as well as to single fibre weight. A comparable muscle enzyme pattern was found in persons of around 20 years old and around 70 years old when expressed per gram of single fibre weight. However, in terms of grams of pure muscle weight, a significant activity decrease with age was obtained for 6-phosphofructokinase, triosephosphate dehydrogenase and phosphoenolpyruvate carboxykinase, whereas activity of hexose diphosphatase increased with age as also did 3-hydroxyacyl-CoA dehydrogenase activity. Five other cytoplasmic enzyme activities involved in glycolysis and energy transfer did not change significantly with age, nor did lysosomal acid phosphatase. The mitochondrial enzyme activities of gluconeogenesis (for example, pyruvate carboxylase, malic enzyme) were diminished to a lesser extent as also the auxiliary enzymes glutamic-oxaloacetic transminase and glutamic-pyruvic transaminase; glutamate dehydrogenase activity remained unchanged. The findings indicate a distinct disorganization of cytoplasmic glycolysis and gluconeogenesis pathways in presenile human skeletal muscle, confirming the histometric data already described. They cannot be explained by changes with age in numerical or areal ratio of type I and type II fibres.

[Symptomatology, diagnosis, and treatment of nervous tissue affecting toxoplasmosis in adult (author's transl)]. / Zur Symptomatik, Diagnostik und Therapie der zentralnervösen Erwachsenen-Toxoplasmose.

The neurological manifestations of three adult cases with acquired toxoplasmosis appearing like a focal lesion, multiple sclerosis and with symptoms indicating a cerebellar lesion are reported. The discussion with respect to the literature shows the following results: 1. The diagnosis of mono- and oligosymptomatic toxoplasmosis with neurological symptoms is only approximate after the introduction of the indirect immunofluorescence test too. 2. The toxoplasmosis of the nervous system in the adult is not characterized by a special syndrome like the connatale form. In contrary, it often is mimicking many well known neurological or psychiatric syndromes. 3. There may be an exacerbation of a latent infection in cause of immunosuppressive therapy or radiation of malignancy or after transplantations. 4. Treatment with the combination of pyrimethamine and sulfonamids today is handled less in spite of the possible complications and of doubtful serological results, because an early diagnosis and onset of treatment is important for a good restitution.

The symptomatology, morphology and biochemistry of glycogenosis type II (Pompe) in the adult.

The mild, generalized myopathy (glycogenosis type II) of a 23-year-old male, previously thought to have progressive muscular dystrophy, was studied clinically, electro-myographically, biochemically and with light- and electron microscopes. However, the history and clinical aspects, as well as the registration of high frequency discharges in the electromyogram first made the diagnosis uncertain. This kind of spontaneous activity has been found in nearly all cases reported in the literature. Light microscopic and histochemical examinations show vacular degeneration and glycogen storage in muscle fibres. With the electron microscope we found free dispersed glycogen in the cytoplasm and membrane-bound glycogen, glycogen-filled lysosomes. Biochemical measurements of the muscle enzymes, involved in the glycogen breakdown, were normal except for acid alpha-1,4-glucosidase, which was deficient. The evidence of these findings in this abortive form of glycogenosis type II is discussed and compared with the few cases found in the literature.