Sensory axonopathy in hereditary distal spinal muscular atrophy

A girl of 14 year is presented with a distal spinal muscular atrophy (SMA) with autosomal recessive inheritance. The technical findings are in agreement with the diagnosis. Light microscopical examination of sural nerve biopsy, including teased fiber studies and morphometry, showed no abnormalities. Electron microscopical investigation however demonstrated axonal pathology. The question arises if distal SMA is a distal axonopathy mainly of motor nerves, but to some extent also of sensory nerves.     

Smoking and hypertension: risk factors for carotid stenosis

Summary The prevalence of smoking, hypertension and diabetes mellitus was assessed in 221 patients suffering from internal carotid stenosis and compared with the prevalences in two sex- and age-matched control groups composed of subjects having normal Doppler findings and from non-neurological outpatients. Of the subjects with carotid stenosis 27.6% were hypertensive smokers in comparison with 9.5% and 17.2% in the two control groups. The difference of the stenosis cohort from the two control groups was significant (P<0.01 and P=0.016 respectively). There was no statistically significant differences between the occurrence of diabetes and hypertension in non-smokers and patients who smoked. In 394 investigated patients suffering from carotid stenosis or occlusion an obstruction index, based on the Doppler shift frequency, was calculated. This index was lowest in the normotentive non-smokers. It was only insignificantly higher in the hypertensive non-smokers but significantly so in the normotensive smokers. The index was highest in the hypertensive non-smokers. It was concluded that cigarette smoking, especially if associated with hypertension, is a determinant risk factor for carotid stenosis and occlusion.     

Regulation of alcohol consumption by the renin-angiotensin system: a review of recent findings and a possible mechanism of action.

The renin-angiotensin system has traditionally been associated with the regulation of fluid and electrolyte balance. In this review we summarize the data which ascribes a completely new function to this system, i.e., the regulation of alcohol consumption. In addition, we suggest a possible mechanism for this effect based on the concept of a satiety or stop process. The approach taken was to examine the effect on alcohol intake of a wide variety of drug, genetic, dietary, surgical and neurosurgical manipulations, each of which has a range of biological effects characteristic of that manipulation, but all of which share the common property of altering activity in the renin-angiotensin system. The effect of these manipulations on alcohol intake was most parsimoniously explained by reference to their ability to raise or lower activity in the renin-angiotensin system. Any intervention which modulates activity in this system, either directly or indirectly, is likely to have consequences for alcohol consumption.     

Immunoregulators in the nervous system

The nervous system, through the production of neuroregulators (neurotransmitters, neuromodulators and neuropeptides) can regulate specific immune system functions, while the immune system, through the production of immunoregulators (immunomodulators and immunopeptides) can regulate specific nervous system functions. This indicates a reciprocal communication between the nervous and immune systems. The presence of immunoregulators in the brain and cerebrospinal fluid is the result of local synthesis--by intrinsic and blood-derived macrophages, activated T-lymphocytes that cross the blood-brain barrier, endothelial cells of the cerebrovasculature, microglia, astrocytes, and neuronal components--and/or uptake from the peripheral blood through the blood-brain barrier (in specific cases) and circumventricular organs. Acute and chronic pathological processes (infection, inflammation, immunological reactions, malignancy, necrosis) stimulate the synthesis and release of immunoregulators in various cell systems. These immunoregulators have pivotal roles in the coordination of the host defense mechanisms and repair, and induce a series of immunological, endocrinological, metabolical and neurological responses. This review summarizes studies concerning immunoregulators--such as interleukins, tumor necrosis factor, interferons, transforming growth factors, thymic peptides, tuftsin, platelet activating factor, neuro-immunoregulators--in the nervous system. It also describes the monitoring of immunoregulators by the central nervous system (CNS) as part of the regulatory factors that induce neurological manifestations (e.g., fever, somnolence, appetite suppression, neuroendocrine alterations) frequently accompanying acute and chronic pathological processes.     

Extracranial surgery for vertebro-basilar ischaemia (experience of 8 cases).

Atheromatous lesions of the proximal vertebral artery and the subclavian artery may lead to ischaemic manifestations, occasionally with severe consequences in the vertebro-basilar territory. These transient ischaemic attacks are most often caused by haemodynamic insufficiency rather than thrombo-embolic complications addressed by anticoagulant and antiaggregant treatments. In this study, 8 cases of vertebro-basilar ischaemia (VBI), secondary to subclavian and proximal vertebral artery lesions, are reported. Surgical techniques, subclavian-to-subclavian artery by-pass (5 cases) and vertebral to common carotid artery transposition (3 cases) are described with their respective results. Through a review of the literature, the various operative modalities are discussed in the different pathological conditions of the proximal extracranial vertebro-basilar disease. It appears that the subclavian to subclavian artery by-pass as well as the vertebral-to-common carotid artery transposition are safe surgical procedures with strikingly low morbidity and mortality rates. The widely achieved relief of the ischaemic episodes, undoubtedly makes this surgery an alternative to medical treatment.     

Dimensional measurement of seasonal variation in mood and behavior.

Recent epidemiologic studies have found that the behaviors that characterize seasonal affective disorder (SAD) show seasonal variation in 92%-95% of the general population, suggesting that seasonal variation in behavior and mood is a continuous, dimensional variable extending throughout the general population, defined at the upper extreme by SAD. Research into population seasonality will require a dimensional measure of seasonal variation in mood and behavior that produces a broad, finely graded distribution of seasonality scores sensitive to individual differences throughout the entire range of scores. Accordingly, the Inventory of Seasonal Variation (ISV) was developed as such a measure. This study demonstrated that the ISV has high internal structural validity and is highly sensitive to individual differences in seasonality across its entire range of scores in the normal population. This latter characteristic is not shared by other existing measures of seasonality. Initial external validity of the ISV was supported in that the mean of ISV scores of a SAD sample was found to lie at the 97th percentile of the normal population of scores. Analysis of ISV scores revealed that a winter pattern of seasonality was reported by over 95% of subjects, a pattern that was more pronounced in women than men, while a summer type of seasonality was reported by only 0.6% of the general population.     

Haemangioma of the maxillary antrum

Haemangioma of the maxillary sinus is rare. Clinical diagnosis is of utmost importance for its operative treatment and prevention of hazards. A case is reported for its rarity and some unusual features.     

Electron microscopic immunocytochemical evidence for the existence of bidirectional synaptic connections between growth hormone-releasing hormone- and somatostatin-containing neurons in the hypothalamus of the rat

Synaptic contacts between growth hormone-releasing hormone (GHRH)- and somatostatin-containing neurons were demonstrated in the rat hypothalamus by a double-staining immunocytochemical method at the electron microscopic level. Somatostatin-immunoreactive nerve terminals synapse on GHRH-positive dendrites and cell bodies in the arcuate nucleus. A fine network of GHRH-immunopositive nerve terminals was observed at the light microscopic level in the rostral part of the periventricular nucleus and in the dorsal part of the arcuate nucleus around somatostatin-containing neuronal elements. With the electron microscope synaptic contact between GHRH-containing nerve terminals and somatostatin-containing dendrites are demonstrated. The reciprocal innervation between GHRH- and somatostatin-containing neurons that project to the median eminence and regulate growth hormone secretion must allow them to coordinate their activities.     

Cerebrovascular and metabolic perturbations in delayed heavy charged particle radiation injury

Focal heavy charged particle irradiation of the rabbit brain created defined lesions which were observable by nuclear magnetic resonance (NMR) and positron emission tomography (PET) imaging techniques. The lesions appeared approximately 9-11 months after left partial hemibrain irradiation with 30 Gy (230 MeV/u helium ions), and were restricted to the white matter tracts and deep perithalamic and thalamic regions. 82Rubidium PET and Gadolinium DTPA enhanced NMR imaging were used to detect blood-brain barrier perturbations. 18Fluordeoxyglucose PET studies demonstrated widespread decreases in cerebral glucose uptake in the cortex and thalamus of the irradiated hemisphere. NMR and PET imaging results correlated well with histological findings. Rabbits irradiated with 15 Gy did not demonstrate any abnormalities in the brain with sequential NMR scans through 14 months post-irradiation.