A case of Ullrich disease with distinct pathological change of muscular dystrophy

A case of Ullrich disease was presented. The patient was a 3-year-old girl with torticollis, generalized muscle weakness and acroatonia since birth. High-arched palate, protruded calcaneus, and mild contracture of proximal joints were also recognized. Intellectual development was normal. Serum level of CPK was slightly increased. In histological and histochemical examinations of quadriceps femoris muscle, proliferated connective tissue, marked variation in the muscle fiber diameter, and a lot of degenerated and regenerated fibers were recognized. Minimal injury easily causes subcutaneous hemorrhage, but no abnormality was found in the structure of collagen.     

Behavioral state-specific changes in human hippocampal theta activity

Although there has been extensive examination of the behavioral and physiologic correlates of hippocampal theta activity in animals, the human literature consists of a single case study. We investigated the differential effects of four behavioral states on human hippocampal theta activity in 16 epilepsy surgery patients. Behavioral conditions included resting eyes closed (RC), resting eyes open (RO), eyes open with auditory word activation (AW), and eyes open with visuospatial activation (VS). Hippocampal theta activity decreased during both RO and VS compared to both RC and AW. There were reciprocal changes in delta activity. Comparisons of RO to VS and of RC to AW were nonsignificant. The results demonstrate state-specific changes in human hippocampal theta and are consistent with the animal literature that relates hippocampal theta to sensorimotor integration and forebrain volitional mechanisms.     

Oral health of individuals aged 3-80 years in J?nk?ping, Sweden in 1973 and 1983. I. A review of findings on dental care habits and knowledge of oral health

The aim of the present study was to present data from dental care habits and knowledge of oral health in two cross-sectional studies carried out in 1973 and 1983. A random sample of approximately 1000 individuals evenly distributed in the age groups 3, 5, 10, 15, 20, 30, 40, 50, 60 and 70 years was studied for each of these two years. In the 1983-year examination 80 individuals 80 years of age were also included. All subjects were inhabitants of the community of J?nk?ping, Sweden. A questionnaire about dental care habits and knowledge was used in combination with a clinical and radiographical examination. The participants answered 23-101 questions. The same questions were raised in 1973 and 1983. For most age groups more then 80 per cent visited a dentist yearly in 1983. This yearly attendance was higher than that for 1973. In the 20-year-old group 14 per cent of the individuals received dental care in the PDS in 1973 compared to 63 per cent in 1983. The main reason to visit a dentist in 1973 was by the patient's own initiative while in 1983 the appointments were based on the dentist's initiative. The number of subjects that felt discomfort in connection with a visit to the dentist had decreased between 1973 and 1983. Knowledge about the etiology of caries had increased between 1973 and 1983 was not the case for gingivitis and periodontitis. An increase had occurred in toothbrushing frequency and the use of toothpicks and disclosing tablets but this between 1973 and 1983. In 1983, 98 to 93 per cent of the subjects in the age groups 10-30 years had been exposed to topical fluorides. The corresponding figure for 30-year-olds in 1973 was 1 per cent. It is obvious that during the period 1973 to 1983 there has been an increase in dental attendance, knowledge of dental diseases, oral health and the use of preventive measures.     

Oxybutynin versus propantheline in patients with multiple sclerosis and detrusor hyperreflexia

Hyperreflexia is the most common urological finding in patients with multiple sclerosis. A prospective randomized study was done to compare the effectiveness of 2 commonly used drugs, oxybutynin and propantheline. Of the 34 patients entered into the trial 19 were treated with oxybutynin and 15 with propantheline. The urological symptoms (frequency, nocturia, hesitancy, urgency and urge incontinence) were graded according to severity from 0 to 3. Patients with urinary infection were excluded. Urodynamic examination, consisting of cystometrography and electromyography, was performed in all patients before treatment. Both groups of patients had comparable neurological, urological and urodynamic status before treatment. In 4 patients (21 per cent) treated with oxybutynin and in 4 (27 per cent) treated with propantheline side effects were so severe that the treatment had to be discontinued. Symptomatic response to oxybutynin was good in 10 patients (67 per cent), fair in 2 (13 per cent) and poor in 3 (20 per cent). Propantheline produced good symptomatic results in 4 patients (36 per cent), fair in 1 (9 per cent) and poor in 6 (55 per cent). The mean increase in maximum cystometric capacity on cystometrography was significantly larger in the oxybutynin group than in the propantheline group (144 +/- 115 versus 35 +/- 101). Our results indicate that oxybutynin is more effective than propantheline in the treatment of detrusor hyperreflexia in patients with multiple sclerosis.     

Slowly progressive aphasia without generalized dementia: studies with positron emission tomography

Slowly progressive aphasia without generalized dementia is a degenerative syndrome selectively affecting dominant hemisphere language areas. We report changes in regional glucose metabolism measured by positron emission tomography in two patients with this condition. Striking abnormalities of glucose utilization in the left cerebral cortex were demonstrated in both patients. The findings of other neurodiagnostic studies were relatively unremarkable. The first patient had a 3-year history of progressive anomia and impaired auditory verbal recall. An electroencephalogram was normal, and computed tomography showed mild left perisylvian atrophy. Positron emission tomography revealed profound hypometabolism in the left temporal regions. The second patient also had a 3-year history of progressive anomia. Electroencephalography, computed tomography, and magnetic resonance imaging scans were normal. Positron emission tomography showed a major reduction in left parietal glucose utilization, with a lesser decrement in left temporal metabolism. Neither patient demonstrated significant contralateral or global abnormalities such as those reported in positron emission tomographic studies of Alzheimer's disease with or without focal clinical features. These observations support the concept of adult-onset progressive aphasia without dementia as a clinical syndrome distinct from Alzheimer's disease.