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PT neurons were recorded from monkeys performing a trained motor task that included tonic maintenance of a limb position, slow and controlled and then quick wrist movements. The results suggest that many of slowly conducting PTNs have preferential relations with specific aspects of slow or tonic motor acts.
OBJECT: The goal of this study was to use diffusion-tensor (DT) magnetic resonance (MR) imaging to track fibers combined with cortical stimulation mapping to delineate descending motor pathways. Subcortical localization of motor pathways in relation to a glioma may provide critical information to guide tumor resection and prevent surgical morbidity. METHODS: Eleven adult patients harboring gliomas underwent MR imaging 1 day prior to image-guided intraoperative cortical motor mapping and tumor resection. Screens depicting 27 cortical motor sites on a surgical navigation system were saved to launch DT imaging of fiber tracks of descending motor pathways. The position and organization of motor tracts were visualized by fiber tracking. Tracks from 16 motor stimulation sites followed descending pathways from the precentral gyrus, through the corona radiata and internal capsule, and into the cerebral peduncle. These tracks were also observed on DT images to diverge along crossing white matter bundles (four patients) and to terminate or deviate in regions of peritumoral vasogenic edema (five patients). CONCLUSIONS: The use of precise intraoperative cortical mapping information and DT images of fiber tracks can reveal the course of motor pathways beneath the cortex. The subcortical fiber tracks generated are consistent with the known anatomical course and somatotopic organization of the motor tract in relation to its cortical origins. Tracking fibers by using DT imaging in combination with functional localization has the potential to reduce surgical morbidity by revealing subcortical connections of the functional cortex.
A lacunar infarction occurred in the posterior limb of the internal capsule in a 33-years-old Colombian woman, showed an antero-posterior (A-P) course of the corticospinal pathway. Anatomical and histological studies had suggested this course, but to the author's knowledge, this could be the first reported case of a living patient with such features, confirming those previous reports about them.
The results suggest : (1) that PT neurons sending axons to motor nuclei innervating distal forelimb muscles branch less than other PT neurons; (2) that a group of PT neurons located close together may influence various species of motoneurons, but that this influence is the strongest on motoneurons of one species; (3) that individual PT neurons terminate only on a small fraction of a given motoneuron group.
PURPOSE: Diffusion tensor imaging can evaluate the cerebral white matter quantitatively using fractional anisotropy (FA) and also can extract a certain tract by tractography, but these two have been used separately and not combined. The purpose of this study was to assess the clinical feasibility of ROI analysis using diffusion tensor tractography (DTT) in patients with amyotrophic lateral sclerosis (ALS). MATERIALS AND METHODS: Sixteen patients with ALS (9 limb-onset type, 7 bulbar-onset type) and nine age-matched volunteers were studied. DTT of the corticobulbar tract (DTT-CBT) and corticospinal tract (DTT-CST) were visualized by free software (dTV/VOLUME-ONE). Regions-of-interest (ROIs) were semi-automatically placed on the tracts defined by DTT methods, and FA values within the ROIs were measured. RESULTS: Mean FA values of ALS patients in the ROIs along the DTT-CST (bulbar-onset: 0.574, limb-onset: 0.594) were significantly lower than those of controls (DTT-CST: 0.629) (p<0.05). The mean FA of DTT-CBT of the bulbar-onset type (0.509) was significantly lower than that of the limb-onset type (0.558) and that of volunteers (0.561). CONCLUSION: DTT could segmentate certain white matter tracts and evaluate them quantitatively. It could depict the subtle changes between subtypes of ALS as well as the changes between the patients and volunteers.
We determined central motor conduction time (CMCT) (motor cortex to root C-8 and motor cortex to root S-1) as well as the amplitude of the compound muscle action potentials in the hypothenar and the abductor hallucis muscles on both sides in 44 patients with definite MS. We compared the values with standards obtained from 86 healthy controls and correlated them with the degree of clinical deficit of the limbs examined. Thirty-nine patients (88.6%) showed a prolonged CMCT. By comparison, only 74.4% of patients had abnormal visual evoked potentials.
Three children, two females and one male, born from unrelated parents show brachycephaly, ogival palate, blindness from 5-6 months and progressive piramidal symptoms. Two subjects had since sixth monthy epileptic seizures, the other one died at 5 th month. In one subject died at 28 month age, central nervous system autoptic examination shows neuronal cortical immaturity, lack of corpus callosum, visual and piramidal patways hypoplasia. The serial EEG recording shows three caracteristic aspects: a) altough a marked changes, the organization of the electric cerebral activity was partially preserved; b) a epileptogenic potentiality was present, that is caracteristic of electro-clinic immage of this subject; c) a suggestive and peculiar tendency to asincrony and asimmetry of epileptic tendency in two hemispheres.
In 51 patients with primary brain lesions, who fulfilled the criteria of brain death, sequential recording of transcranial magnetic evoked potentials (TMEPs) and somatosensory evoked potentials (SEPs) were performed. In all comatose patients with apnoeic cranial nerve areflexia the TMEP could not be elicited, while the response after cervical magnetic stimulation was always preserved. Similarly, no cortical SEPs were preserved in apnoeic cranial nerve areflexia; however, the cervical somatosensory response was preserved in 44%. In deteriorating patients with coma grade III TMEPs were preserved in 3 instances, while cortical SEPs were already absent. Current brain death criteria, however, were not challenged, as TMEPs were absent in all 51 patients, at the latest when apnoea was noted.
A Japanese family with atypical type I familial amyloidotic polyneuropathy (FAP) in Iiyama, Japan was studied. Most of the family members have dysfunctions in the central nervous system, in addition to typical symptoms of type I FAP. The transthyretin (TTR, also called prealbumin) gene of the atypical FAP(FAP-IY) was analyzed with recombinant DNA techniques and a RIA method. FAP-IY was found to have the mutation responsible for the methionine-for-valine substitution at position 30 of TTR, as in the case of typical type I FAP. However, analysis of DNA polymorphisms in the TTR locus showed that FAP-IY has a genetic background differing from that of the typical type I FAP. These observations lead to the consideration that a genetic factor(s) involved in the dysfunction of the central nervous system may locate in a chromosome region in close proximity to the TTR gene.
We evaluated somatosensory evoked potentials (SEPs) to tibial nerve stimulation in 39 patients with sporadic motor neuron disease using multiple scalp derivations (earlobe reference). SEPs were altered in 22 of 29 amyotrophic lateral sclerosis (ALS) patients, whereas they were unaffected in 10 progressive muscular atrophy (PMA) patients. The main changes involved the amplitude and the field distribution of the early P40 and N37 cortical potentials with different modalities varying from a selective loss of the P40 potential (33% of tested sides) to absence of all early cortical SEPs (22% of tested sides). The later components following N50 were generally spared. The commonly used Cz-Fz montage was inadequate for detecting these alterations. Central afferent conduction was slightly affected. The selective loss of cortical SEPs and their close correlation with clinicoelectrophysiologic evidence of central motor system involvement strongly support a cortical origin of the SEP alterations in ALS. We suggest that neuronal loss in the somatosensory cortex may selectively affect the generator sites of the cortical SEPs to lower limb stimulation.
Using magnetic resonance imaging, we studied 23 patients with motor deficit associated with cerebrovascular disease of the internal capsule. According to the severity of the motor deficits, 23 patients were divided into three groups (severe group...9, moderately severe group...8, mild group...6). A coronal T2-weighted image was obtained along a straight line between the front edge of the medulla and the deepest point of the interpeduncular cistern in a midsagittal T1-weighted image. It was revealed that wallerian degeneration extended continuously from part of the internal capsule down to the pons or medulla or the decussation in all patients. The area of wallerian degeneration in the pons and the area of half the pons were calculated from the coronal T2-weighted image. Moreover, the wallerian index was calculated as: (area of wallerian degeneration in pons divided by area of half the pons) X 100. Values of the wallerian index +/- SD were 26.4 +/- 5.1 in the severe group, 19.2 +/- 5.6 in the moderately severe group, and 10.0 +/- 5.4 in the mild group. There were significant differences among the three groups. We concluded that the area of wallerian degeneration was related to the severity of motor deficits.
Three-dimensional MRI data sets were obtained from 12 young adult patients with congenital spastic hemiparesis caused by unilateral periventricular white matter lesions. The impact of these lesions on corticospinal projections to the upper and lower extremities was assessed on reconstructed semi-coronal planes following anatomical landmarks of somatotopic organization in the precentral gyrus and in the internal capsule: a more anterior plane running through the hand-knob of the precentral gyrus and the anterior portion of the posterior limb of the internal capsule representing projections to the upper extremity, and a more posterior plane running through the top of the precentral gyrus and the middle portion of the posterior limb of the internal capsule representing projections to the lower extremity. In addition, the total lesion extent was determined volumetrically, and Wallerian degeneration was assessed qualitatively in the internal capsule and quantitatively by measuring brainstem asymmetry. We found a strong correlation between motor dysfunction of the upper and lower limb and the lateral extent of the periventricular lesion measured on the respective semi-coronal planes. The total lesion volume and the degree of Wallerian degeneration correlated less strongly, both reaching statistical significance only with motor impairment of the hand.
BACKGROUND: One of the cardinal features in multiple system atrophy (MSA) is the white matter pathology: loss of myelin, astrocytosis, and glial cytoplasmic inclusions. The pathological changes of tissue microstructure can modify the diffusion behavior of water molecules, which can be assessed by diffusion tensor imaging (DTI). OBJECTIVES: To explore the hypothesis of white matter degeneration in MSA. METHODS: We studied 11 patients with clinically probable MSA and 10 age-matched controls. DTI was performed in both groups to measure fractional anisotropy (FA) in various regions of interest: the inferior cerebellar peduncle (ICP), middle cerebellar peduncle (MCP), superior cerebellar peduncle (SCP), basis pontis, internal capsule, and corpus callosum. RESULTS: FA values in SCP and corpus callosum showed no significant difference between the MSA group and controls. By contrast, FA values decreased in the MSA group in the MCP, basis pontis and internal capsule. In addition, FA values in the MCP were negatively correlated with ataxia severity in the MSA group. CONCLUSION: The areas showing decreased tissue anisotropy in DTI corresponded well with pathologically vulnerable areas in MSA. In addition, the local tissue anisotropy of MCP decreased in accordance with functional disability. These observations implied that DTI is a feasible method for in vivo evaluation of the white matter pathology in MSA.
An anterograde tracer study has been made of the developing corticospinal tract (CST) in the rat using wheat germ agglutinin-conjugated horseradish peroxidase (WGA-HRP). Analysis of normal Rager stained material revealed that corticospinal axons reach upper cervical spinal cord levels at the day of birth (PO). Postnatal rats ranging in age from one (P1) to fourteen (P14) days received multiple WGA-HRP injections into the cortex of their left hemisphere and were allowed to survive for 24 h. The first labeled CST fibers caudally extend into the third thoracic spinal cord segment at P1; into the eighth thoracic segment at P3; into the first or second lumbar segment at P7 and into the second to third sacral segment at Pg. Thus the outgrowth of the leading 'pioneer' fibers of the CST is completed at P9 but later developing axons are continuously added even beyond P9. Quantitative analysis of the amount of label along the length of the outgrowing CST revealed a characteristic pattern of labeling varying with age. The most striking features of that pattern are: the formation of two standing peaks at the level of the cervical and lumbar enlargements respectively and the transient presence of a smaller running peak which moves caudally with the front of the outgrowing bundle. The standing peaks are ascribed to the branching of the axon terminals at both intumescences, whereas the running peak probably arises by the accumulation of tracer within the growth cones at the tips of the outgrowing CST axons. Factors such as the number of axons, the varying axon diameters, the branching collaterals, the presence of varicosities, the transport rate of the tracer, the uptake of the tracer at the injection site, which possibly may affect the amount of label present in both the entire bundle and in the individual axons are discussed. Current research is focused upon an analysis of the relation between the site of injection within the cortex and the pattern of labeling of the CST. A delay of two days was found between the arrival of the CST axons at a particular spinal cord level and their outgrowth into the adjacent spinal gray. However, combined HRP and electronmicroscopic experiments are necessary to determine the factors behind the maturation of the CST as well as the maturation of the spinal gray.
1. Intracellular records were obtained from motoneurones innervating muscles of the baboon's forearm and hand. Monosynaptic excitatory postsynaptic potentials (EPSPs) were elicited by stimulation of motor cortex (CM EPSPs) and peripheral nerves (Ia EPSPs).2. CM EPSPs were larger on average in motoneurones innervating intrinsic hand muscles and extensor digitorum communis (EDC) than in neurones of other forearm muscles.3. Among motoneurones of the median nerve, the CM EPSP tended to be larger for cells with more rapidly conducting axons than for those with more slowly conducting axons. Among motoneurones of EDC the opposite tendency was found.4. The afferent fibres responsible for the Ia EPSP nearly always had a lower stimulus threshold than that of motor axons in the same nerve. Some observations were made concerning the distribution of heteronymous Ia EPSPs.5. Among motoneurones of a given nerve, those with large Ia EPSPs tended to receive larger CM EPSPs than did cells in which the Ia EPSP was small.6. The results are discussed in relation to problems concerning the pyramidal control of hand and finger movement.
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