Ubiquitin in motor neuron disease: study at the light and electron microscope.

Several neurodegenerative diseases, including motor neuron disease (MND), are characterized by formation of abnormal cytoskeleton-derived inclusions which contain ubiquitin (Ubq). We have studied the distribution of Ubq in 26 cases of MND with light and electron microscopic immunocytochemistry. Ubiquitin-positive inclusions were found in neurons of anterior horns in most cases of amyotrophic lateral sclerosis (ALS) but were not present in other forms of MND. Ubiquitin immunoreactivity was observed in 10-15 nm intraneuronal filaments, which were not stained by antibodies to neurofilaments, and on dense bodies of dystrophic neurites throughout the neuropil of anterior horns and pyramidal tracts. Data analysis showed a trend toward lower percentage of Ubq-positive neurons in cases with longer duration of illness or lower number of neurons. A high percentage of Ubq-positive inclusions occurred in cases with an aggressive clinical course, suggesting that ubiquitination takes place at early stages of the disease.     

Synaptic organization of cortico-cortical connections from the primary visual cortex to the posteromedial lateral suprasylvian visual area in the cat

The synaptic organization of the projection from the cat striate visual cortex to the posteromedial lateral suprasylvian cortical area (PMLS) was examined. The anterograde tracer Phaseolus vulgaris leucoagglutinin (PHA-L) was iontophorectically delivered into area 17, and anterogradely labeled fibers were revealed in PMLS by means of an immunocytochemical detection method. Most axons and presumptive terminal swellings were found in layers III and IV. The neuronal elements (n = 190) that were postsynaptic to anterogradely labeled boutons were quantitatively analyzed. All anterogradely labeled cortico-cortical boutons (n = 182) established type 1 synapses. The results show that 83% of the postsynaptic targets were dendritic spines, probably belonging to pyramidal cells. Dendritic shafts constituted 17% of the targets. The dendritic shafts postsynaptic to cortico-cortical boutons were studied for the presence of gamma-aminobutyric acid (GABA) with a postembedding immunogold method. Most dendritic shafts (85%) that were tested were found to be GABA-positive, demonstrating that they originate from local inhibitory neurons. Taking into account that most postsynaptic targets were spines and extending the results of the immunocytochemical testing to the total population of postsynaptic dendrites, it was calculated that at least 14% of targets originated from GABA-positive cells. Thus cortico-cortical axons establish direct monosynpatic connections mainly with pyramidal and to a lesser extent with GABAergic nonpyramidal neurons in area PMLS, providing both feedforward excitation and feedforward inhibition to a visual associational area known to be involved in the processing of motion information. The results are consistent with previously demonstrated deficits in physiological properties of neurons in PMLS following removal of cortico-cortical afferents.     

Intrafollicular blood flow during human ovulation.

Transvaginal ultrasonography with color flow mapping has been used to study changes in intrafollicular blood flow and morphology during follicular rupture and presumed ovulation in one human volunteer. Detailed monitoring started on day 11 of the menstrual cycle and the follicle began to rupture at 14.30 on day 13. This event was preceded by a defined rise and peak in the level of immunoreactive serum luteinizing hormone (LH) by 42 h and 17 h 20 min, respectively. Blood vessels were clearly visible in the inner ring of the follicle (the granulosa layer) at the time of the LH peak and part of the granulosa (probably containing the oocyte) started to detach before the follicle ruptured. The maximum value for the peak blood velocity in the inner vessels was observed 10 s after the start of follicular rupture and there was a concurrent increase in the impedance to blood flow, as reflected by the resistance index and the pulsatility index. The follicle took 14 min 29 s to empty and the corpus haemorrhagicum started to form about 1 min later. These preliminary data suggest that intrafollicular angiogenesis and changes in blood flow can be monitored by a relatively non-invasive technique. Changes in vascularity might be used to predict imminent ovulation and could possibly be identified or modified biochemically to help achieve or avoid a pregnancy.     

Early spontaneous hematoma in cerebral infarct: is primary cerebral hemorrhage overdiagnosed?

We identified 15 patients (63 +/- 8 years) in whom CT showed no bleeding within 6 hours of stroke onset but showed ganglionic or lobar hemorrhage less than 18 hours later, without visible underlying infarct (early spontaneous intra-infarct hematoma [ESIH]). No patient had antithrombotic therapy or a coagulation disorder, but eight had hypertension. The second CT was prompted by rapid worsening (in 10) or because the first CT was not available (in five). Prior transient ischemic attacks, silent infarcts on CT, and a potential cardiac source of embolism were more common in patients with ESIH than in 200 patients with primary cerebral hemorrhage (PCH) admitted during the same period. Distal occlusions were present in four of five patients who underwent intracranial studies within the first 2 days. Most of these patients probably had embolism with early and extensive bleeding in the ischemic area. Our findings suggest that ESIH may be under-recognized, while PCH may be overdiagnosed.     

VISUOMOTOR PERFORMANCE OF NORMAL AND CLUMSY CHILDREN. II: ARM-TRACKING WITH AND WITHOUT VISUAL FEEDBACK

Tracking performance was investigated in normal and clumsy children in two age-groups, six to seven and 10 to 11 years. Target signals moving unpredictably along a straight line had to be tracked, both with and without visual feedback. Performance was described in three ways: (1) performance in the low-frequency range (LF); (2) the delay between target signal and tracking movement (DL); and (3) a measure of tracking quality or over-all similarity in the shape of target signal and tracking movement (Q). Clumsy children in both age-groups had a lower tracking quality (Q) and longer delay (DL) than the normal children. Disturbances in the regulation of attention seemed to affect tracking performance, particularly of the six- to seven-year-old clumsy children. There was no significant difference between normal and clumsy children in the effect of visual feedback on tracking performance. This suggests that clumsiness is not linked to disturbance of integration of visual feedback information and motor processes.     

Myelin-axon relationships established by rat vagal Schwann cells deep to the brainstem surface

The central-peripheral transitional zones of rat dorsolateral vagal rootlets are highly complex. Peripheral nervous tissue extends centrally for up to several hundred micrometers deep to the brainstem surface along these rootlets. In some instances this peripheral nervous tissue lacks continuity with the peripheral nervous system (PNS) and so forms an island within the central nervous system (CNS). In conformity with the resulting complexity of the CNS-PNS interface, segments of vagal axons lying deep to the brainstem surface are myelinated by one or more intercalated Schwann cells, contained in peripheral tissue insertions or islands, at either end of which they traverse an astroglial barrier. Intercalated Schwann cells are thus isolated from contact or contiguity with the Schwann cells of the PNS generally. They are short, having a mean internodal length of around 60% of that of the most proximal Schwann cells of the PNS proper, which lie immediately distal to the CNS-PNS interface and which are termed transitional Schwann cells. The thickness of the myelin sheaths produced by intercalated Schwann cells is intermediate between that of transitional Schwann cells and that of oligodendrocytes myelinating vagal axons of the same calibre distribution. This is not due to limited blood supply or to insufficient numbers of intercalated Schwann cells, the density of which is greater than that of transitional Schwann cells. These factors are unlikely to restrict expression of their myelinogenic potential. Nevertheless, the regression data show that the setting of the myelin-axon relationship differs significantly between the two categories of Schwann cell. Thus, the myelinogenic response of Schwann cells to stimuli emanating from the same axons may differ between levels along one and the same nerve bundle. Mean myelin periodicity was found to differ between sheaths produced by intercalated and by transitional Schwann cells.     

Reasoning in deluded schizophrenic and paranoid patients. Biases in performance on a probabilistic inference task

An experiment is described in which deluded subjects with a diagnosis of schizophrenia or of delusional disorder (paranoia) were compared with a nondeluded psychiatric control group and a normal control group on a probabilistic inference task. Factors relevant to belief formation and maintenance were investigated. Deluded subjects requested less information before reaching a decision and were more ready to change their estimates of the likelihood of an event when confronted with potentially disconfirmatory information. No differences were found between the two diagnostic groups of deluded subjects. The results are discussed in light of prevailing theories of the importance of abnormal experience rather than reasoning biases in the formation and maintenance of delusional beliefs. It is suggested that a reasoning abnormality is involved, which may coexist with perceptual abnormalities.     

False positive-false negative CT scan in late epileptic seizure: a meningioma-glioblastoma association

Summary A false-negative finding on initial CT is reported in a case of supratentorial glioma. This observation was peculiar because the first CT revealed a meningioma which might initially have been related to the clinical symptoms. The term false positive-false negative CT is proposed. The reasons for such CT failures are discussed. The accuracy of clues as to the localization of the glioma provided by EEG is emphasized.