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[A case of HTLV-1 associated myelopathy with diffuse white matter lesion of the frontal lobe and continuous lesion of the pyramidal tract on cranial MRI].

In this report, the characteristic findings of cranial MRI of a case with HTLV-1 associated myelopathy (HAM) is described. The patient was a 62-year-old woman with 6 years history of paraplegia. Her main clinical signs were bilateral spastic paraplegia in the lower limb girdle muscles and extremities, paresthesia below the tenth thoracic cord level and urinary disorder. There was bilateral blepharoptosis. Hyperreflexia was observed in the examination of the cranial nerves and upper extremities. She showed no dementia or any other higher cortical dysfunctions. Positive anti-HTLV-1 antibody in the serum and cerebrospinal fluid established a diagnosis of HAM. T2 weighted MRI study revealed the symmetrical diffuse hyperintensity in the subcortical white matter of the frontal lobe and temporal lobe. The hyper-intensity was also observed in the bilateral internal capsule-peduncular base junction and pontine base, which indicated the continuous lesions in the intracranial pyramidal tracts. These MRI findings were different from those of reported cases of scattered lesion in central nervous system. There are some speculations for the nature of the diffuse lesion in the white matter, i.e., fusion of solitary gliosis and/or perivascular cuffing, or diffusely advanced spongy state. The continuous lesion of the pyramidal tract is suspected a systemic demyelination. Although the pathomechanism still remains uncertain, HTLV-1 infection generally affects the spinal pyramidal tract. It seems that this case is selectively affected in the pyramidal tracts in CNS.

Female↗

Pyramidal tract and corticospinal neurons with branching axons to the dorsal column nuclei of the cat.

Extracellular single activity was recorded from pericruciate neurons in anaesthetized, paralysed, artificially ventilated cats. A total of 309 neurons were identified antidromically by stimulation of the dorsal column nuclei (229 from the nuneate nucleus and 80 from the gracile nucleus). The study addressed the question whether pericruciate-dorsal column nuclei neurons (corticonuclear cells) sent collaterals to the ipsilateral red nucleus and/or to the contralateral nucleus reticularis gigantocellularis. Also, the ipsilateral pyramidal tract was stimulated at mid-olivary level, as was the crossed corticospinal tract at C2, Th2 and L2 levels in order to know whether the corticonuclear cells sent their axons to the spinal cord and if so to which level. It was found that more than 95% of the corticonuclear fibres coursed through the pyramidal tract. A significant (28.4%; 88/309) proportion of the the corticonuclear neurons sent collaterals to the red nucleus and/or to the nucleus reticularis gigantocellularis. About 68% (209/309) of the corticonuclear cells did not send their axons to the spinal cord and the remainder were corticospinal neurons. Most of the corticospinal fibres terminated at the cervical level (72/100) and the remaining ended at thoracic (18/100) and lumbar (10/100) segments of the cord. While 63.4% (123/194) of the corticonuclear fibres coursing through the pyramidal tract and ending at supraspinal levels were slow conducting, the great majority of the corticospinal neurons were fast conducting (91/100). The non-corticospinal neurons were significantly slower conducting than the corticospinal cells. The corticogracile neurons were slower conducting than the corticocuneate cells. Of the 88 corticonuclear neurons that sent at least a branch to the sites tested, 50% branched into the red nucleus, 35.2% into the nucleus reticularis gigantocellularis and 14.7% into both nuclei, without significant difference between non-corticospinal and corticospinal cells. Most of the main axons of the corticonuclear cells ended at bulbar and cervical levels (281/309 or 90.9%). The data indicate that pericruciate-dorsal column nuclei neurons form a particular substrate within pyramidal tract cells. They can serve precise functions in motor coordination associated with the selection of their own sensory input. The results are discussed from this point of view.

Animals↗

Effects of 2,5-hexanedione on rat spinal pyramidal tract.

The present study was undertaken to investigate central axonal neuropathy caused by 2,5-hexanedione (2,5-HD) administration. The effects of 2,5-HD on rat pyramidal tract were studied electrophysiologically and histopathologically. Male rats were given 0.5% 2,5-HD in their drinking water for 4, 6 or 8 wk. Spinal motor conduction velocity was measured by direct electrical stimulation and was examined histopathologically by electron microscopy. Rats treated with 2,5-HD had reduced spinal motor conduction velocity at 6 wk and their pyramidal tract axons had decreased cross-sectional areas at 4 wk. The mean number of neurofilaments in myelinated axons of rats treated with 2,5-HD for 8 wk was 42.1% of that found in control rats, but the number of microtubules was not significantly different. The ratio of neurofilaments to microtubules in rats treated with 2,5-HD for 4 wk was less than 1.0 in 30.9% of myelinated fibers, while in control rats it was greater than 1.0 in all axons. These changes in spinal pyramidal tract were detected relatively early after 2,5-HD administration. These results in rats indicate the need for further studies of 2,5-HD neurotoxicity in the human central nervous system.

Animals↗

Paw and limb use in skilled and spontaneous reaching after pyramidal tract, red nucleus and combined lesions in the rat: behavioral and anatomical dissociations.

The pyramidal tract and red nucleus send prominent projections to the spinal cord and are thought to co-operate in producing skilled movements. In the present study, skilled reaching for food located on a shelf and spontaneous grasping, handling and eating pieces of pasta were video-recorded and analyzed in control rats, rats with unilateral ibotenic acid lesions of the red nucleus (RN), unilateral pyramidal tract lesions (PT) and combined lesions. The behavioral results suggest that skilled movements are organized as action patterns, easily recognizable and distinctive for each task. In both skilled and spontaneous reaching, PT lesions reduced success more than RN lesions, suggesting a greater role for the PT in guiding limb movements. Both lesions impaired rotatory movements including limb aiming, pronation and supination. RN lesions additionally abolished the arpeggio movement by which the paw is oriented for searching and grasping. Combined lesions were additive as rats lost both rotatory movements and arpeggio. Nevertheless, even after combined lesions, the rats were able to advance the limb, grasp food and withdraw the limb. The sparing following combined lesions suggests that other neural systems as well as compensatory adjustments assist the impaired limb. The results are discussed in relation to the possible distinctive contributions of the rubrospinal and corticospinal tract to the action patterns that comprise skilled movements in rats.

Animals↗

Magnetic resonance signal abnormalities along the pyramidal tracts in amyotrophic lateral sclerosis.

Magnetic resonance imaging (MRI) studies of the brain were reviewed in 16 patients with amyotrophic lateral sclerosis (ALS), representative of a large and homogeneously studied series, 11 of whom showed signal abnormalities along the pyramidal tracts. These were more frequent in patients with more severe upper motor neuron signs but did not correlate with disease severity. Our study suggests that MRI signal abnormalities along the pyramidal tracts are common in ALS and may reflect the severity of pyramidal tract degeneration.

Amyotrophic Lateral Sclerosis↗

Dystopic myelination with hypertrophy of pyramidal tract.

In a case of hypertrophy of the pyramidal tract the cross section of the hypertrophic pyramids was 174% of controls with an increase (148%) in the total population of myelinated fibers. However, there was no commensurate increase in the density of Betz cells. Confined to the hypertrophic tract there was a peculiar anomaly consisting of tubes of thick myelin sheaths that encompassed columns of glial nuclei instead of axons. This type of change, along with the clinical data, may indicate that the lesion originated in the perinatal period when myelin formation is in progress and is susceptible to derangement.

Cell Count↗

Corticostriatal cells in comparison with pyramidal tract neurons: contrasting properties in the behaving monkey.

Antidromically identified neurons projecting to the putamen (CPNs) and pyramidal tract neurons (PTNs) were recorded from motor and premotor cortex of a monkey which performed a load-bearing task with the wrist. CPNs appeared as a uniform population with very slowly conducting axons and low spontaneous activity. In contrast to PTNs, they exhibited weak, mostly insignificant correlation with graded steady-state forces, responded to torque perturbations with remarkably long latency, and seemed to discharge much later with active movement. Collateral branching of PTNs to the putamen was found to be infrequent (1%). We suggest that the putamen receives a cortical message that is strikingly different from that sent down the pyramidal tract.

Animals↗

Diameter of axons and thickness of myelin sheaths of the pyramidal tract fibres in the adult human medullary pyramid.

After perfusion fixation with a mixture of paraformaldehyde, glutaraldehyde, and potassium-chromate 4 adult human medullary pyramids were examined with the electron microscope. The myelin sheaths showed only mild signs of post mortal destructions. The periodicity of the lamellae is preserved and varies about 17 nm. For quantitative purposes specimens were taken at random from regions with an extend of half a mm2 on the cross section of the pyramid. On the average 62 lamellae (s = 5, n = 200) built up 1 micron myelin sheath in the 4 brains. The myelin sheath thickness and the inner circumference of the myelin sheath are measured. The axon diameter is calculated from the inner circumference. We obtained an average number of 66,000 fibres, s = 11,000 per mm2. Fibres with a total diameter below 4 microns amount to 87.9%, fibres from 4 to 10 microns to 10.77%, and larger than 10 microns to 1.4%. An axon diameter of about 0.5 micron combined with a thickness of the myelin sheath of 0.3 micron was most often found in all 4 cases. The fibres showed a broad variation of myelin thickness. In a given group of the small axons the thickest myelin sheath exceeded 3 to 4 times the value of the thinnest one. The coefficient of correlation between axon diameter and myelin sheath thickness was calculated as r2 = 0.56. No correspondence exists between frequency maxima and the ratio g, axon diameter to total diameter. Below a total fibre diameter of 5 microns the ratio g scatters broadly about 0.6, fibres larger than 5 microns exceed the value of 0.6. From the morphological data only large sized fibres can evoke a strong effect on compound action potentials if the method of reconstruction of the action potential from fibre size frequency distribution is employed.

Adult↗

[An autopsy case of amyotrophic lateral screlosis (ALS): magnetic resonance imaging and pathological findings of the pyramidal tract].

A 59-year-old woman was diagnosed as amyotrophic lateral screlosis (ALS) on the basis of neurological and electromyographical findings, and died after about 4 years course. Magnetic resonance imaging (MRI) on coronal planes through the internal capsule revealed high signal area almost limited to the pyramidal tract; the high signal area was more restricted in proton weighted imaging than in T2 weighted imaging. Histological lesion of the precentral gyrus was slight, while loss of myelinated fibers in the posterior limb of the internal capsule was remarkable. Extent of the pathological lesion in the posterior limb of the internal capsule well corresponded to the high signal area in proton weighted imaging. Comparison of the pyramidal tract pathology in the corona radiata and the internal capsule in Klüver-Barrera preparations with the MRI findings obtained 2 years and 8 months before the autopsy suggests us that the T2 weighted imaging of ALS brains may detect involvement of the pyramidal tract as early as or even earlier than histological changes become manifest.

Amyotrophic Lateral Sclerosis↗

A transient pyramidal tract projection from the visual cortex in the hamster and its removal by selective collateral elimination.

During the early postnatal development of the neocortex in rats there is an axonal projection from the occipital cortex (which includes the visual cortex) to the spinal cord which is subsequently completely removed through a process of selective collateral elimination. In order to determine whether a similar phenomenon occurs during the development of the hamster cortex, we have injected the retrogradely transported fluorescent dye Fast Blue (FB) into the pyramidal decussation of hamsters at various ages. In adult hamsters such an injection results in a band of labeled neurons confined to layer V and to about the rostral two-thirds of the neocortex; no labeled cells are seen in the occipital cortex. However, a similar FB injection made during the first postnatal week results after a 4-day survival in a continuous band of FB-labeled layer V neurons spread throughout the tangential extent of the neocortex, including the occipital cortex. A similar continuous band of FB labeled layer V neurons is seen throughout the tangential extent of the neocortex including the occipital region in hamsters injected during the first postnatal week but allowed to survive until the fourth week (i.e., after the restriction of the widespread neonatal pattern has occurred). Injections of the anterograde tracer wheat germ agglutinin conjugated to horseradish peroxidase made into the occipital cortex, or for comparison, into more rostral cortical regions in hamsters ranging in age from neonates to adults, reveal that the extension of pyramidal tract axons is staggered along the anterioposterior axis of the cortex such that axons originating from the posterior regions lag behind those arising from more rostral areas. The transient occipital projection appears to reach a maximum around the end of the first postnatal week: a large number of labeled occipital axons is seen in the medullary pyramidal tract, and some of these can be followed through the pyramidal decussation and into the dorsal funiculus of the spinal cord. Injections into the occipital cortex on P16 label only a few fibers in the medullary pyramidal tract, and none is labeled in hamsters injected as adults.(ABSTRACT TRUNCATED AT 400 WORDS)

Animals↗

Direct synaptic linkage of ventrolateral nucleus of thalamus terminal with cat fast pyramidal tract neuron.

An electron microscopic study on the synaptic connections between neurons of ventrolateral nucleus of thalamus (VL) and pyramidal tract neurons (PTNs) in cat motor cortex was conducted by means of the anterograde degenerating procedure coupled with horseradish peroxidase (HRP) intracellular staining. Following VL lesions, a large majority of the degenerating terminals were found to terminate on dendritic spines and a few on the dendritic shaft. An asymmetric type synapse formed by a VL degenerating terminal and the dendritic shaft of a branch of apical dendrite of a labeled fast pyramidal tract neuron was demonstrated.

Animals↗

[A case of hereditary motor and sensory neuropathy type I with optic atrophy, neural deafness and pyramidal tract signs].

A case of hereditary motor and sensory neuropathy (HMSN) type I with optic atrophy, neural deafness and pyramidal tract signs was described. The patient was a 53-year-old man who had suffered from difficulty in walking, decreased visual acuity since age 16 years. These symptoms were slowly progressive. At the age of 37, he was pointed out optic atrophy, positive pyramidal tract signs. Distal muscle weakness with atrophy of four limbs was prominent at the age of 50. Since then, he noticed progressive hearing loss with blindness. His elder sister was diagnosed Charcot-Marie-Tooth disease. On neurological examination, he showed to have optic atrophy without retinitis pigmentosa and neural deafness. Also he showed mild degree of muscle weakness and atrophy in four limbs, severe in the distal part of lower limbs. Deep tendon reflexes were absent in all limbs with right Babinski sign. Superficial sensation was decreased slightly in the distal parts of four limbs. Deep sensation was markedly decreased in the leg. There were no cerebellar signs. Audiometric examination revealed bilateral neural hearing loss. There were no findings of spinal cord compression on spinal MRI. On the nerve conduction studies, sensory nerve action potential was not elicited in all nerves tested. Motor nerve conduction velocity of the right median was 41.1 M/sec, also ulnar nerve 44.7 M/sec, but M-wave was not elicited with the electrical stimulation of other tested nerves. On sural nerve biopsy, the density of myelinated fibers was severely decreased. Well-myelinated axon surrounded by onion bulb formation was observed in electron microscopic examination.(ABSTRACT TRUNCATED AT 250 WORDS)

Charcot-Marie-Tooth Disease↗

Junctional specializations between growth cones and glia in the developing rat pyramidal tract: synapse-like contacts and invaginations.

The ultrastructure of contacts between axonal growth cones and glial cells in the developing pyramidal tract was examined by serial sectioning at the third cervical spinal cord segment in 0-, 2-, and 4-day-old rats. Junctional specializations, composed of synapse-like contacts and invaginations, were frequently observed at the contact zone between growth cones and glial elements. The synapse-like contacts consist of clear, round vesicles of 43 +/- 6 nm in the presynaptic growth cone, a pre- and a postsynaptic density, separated by a cleft of 12.1 +/- 0.9 nm. The invaginations consist of small protrusions of the growth cone into the glial element. The invaginated glial membrane is coated. Within the glial element, close to the invagination, frequently organelles were observed that closely resemble endosomes and prelysosomes. Therefore, it is suggested that the invagination represents a stage in endocytosis or possibly phagocytosis of the protruding part of the growth cone by the glial cell. The junctional specializations are formed by growth cones and, less frequently, by axon shafts. The targets of these specialized contacts are, in general, immature glial cells located within the tract area. Occasionally, however, invaginations were also observed into myelinating oligodendrocytes, suggesting that the population of immature target cells includes oligodendrocyte precursors. With regard to the functional significance of these temporary growth cone-glial contacts, several possibilities are discussed, including the suggestion that outgrowing pyramidal tract axons provide immature glial cells with chemical messages, which may influence the timing of glial cell maturation in the tract.

Animals↗

A quantitative analysis of axon outgrowth, axon loss, and myelination in the rat pyramidal tract.

A quantitative analysis of the development of the pyramidal tract (PT) was carried out at the level of the caudal medulla oblongata and at the sixth cervical spinal segment (C6), in rats ranging in age from embryonic day 20 (E20) to the adult of 90 days postnatally (P90). The axon number in the right medullary PT rises from 27,000 axons at E20 to 391,000 axons at P4. Growth cones are abundant during this period, but can still be observed occasionally at P7. After P4, the axon number is reduced by 62%, to 150,000 in the adult. A rapid axon loss until P14 is followed by a gradual axon loss, continuing beyond the third postnatal week. A similar biphasic axon loss was observed in the cervical PT. At P2 and at P7, concentrations of electron-dense material were observed in 0.5-0.7% of the axon profiles in the medullary PT. Since at P21 this feature was only observed in 0.2% of the axons, it might represent an early sign of axon loss. Myelination starts in the medullary PT at P7. Especially during the third postnatal week, the number of myelinated axons increases rapidly. In the adult rat PT, both at medullary and cervical levels, about one third of the axons are still unmyelinated. The results indicate that the development of the rat PT is characterized by a gradual outgrowth of its fibers and by a protracted, biphasic axon loss. Furthermore, comparing the PT at the medulla, at C3, and at C6, a rostrocaudal decrease in axon number was observed during development as well as at the adult stage. Therefore, no evidence was found for increased axon branching in the tract in the cervical intumescence.

Aging↗

Spontaneous regeneration of the pyramidal tract after transection in young rats.

Spontaneous regeneration of the pyramidal tract after transection of the medullary pyramid was examined in young rats by the anterograde tracing method with wheat germ agglutinin-conjugated horseradish peroxidase. Care was taken to cut the tract as sharply as possible to minimize traumatic injuries. A very sharp cut produced edema-free lesions without subsequent formation of either cysts or scars, whereas a relatively blunt cut produced edema and later scars and/or cysts in the lesion. Regenerated projections in the latter cases were sparse, short, dispersed and largely aberrant as described in previous reports. By contrast, regenerated projections in the former cases were very much similar to normal in various respects: the amount, extension, path, formation of a compact bundle and termination. There was, however, a decisive difference from normal, that is, the additional aberrant projections.

Age Factors↗

[Role of the pyramidal tract in the mechanism of dyspnea and hyperventilation].

Hypoxia evoked by mechanical asphyxia first of all disturbs the generation of potentials in pyramidal tract neurons. Hypoxic work, therefore, causes inadequate contraction of somatic muscles, a difficulty in work performance and respiratory discomfort. Concurrently, the disturbance of the pyramidal tract disinhibits the respiratory center of the brain stem with a result of excessive hyperventilation during hypoxic work.

Afferent Pathways↗

A re-evaluation of the question of ascending fibers in the pyramidal tract.

In 1952 we published a study in the cat with the Glees method, demonstrating the occurrence of degenerating fibers in the pyramidal tract rostral to transections of the tract in the spinal cord. These fibers were interpreted as spinocortical fibers, which have also been described in man. However, other authors have disputed the existence of such fibers. In an attempt to provide more information about this subject, multiple injections of horseradish peroxidase (free and lectin-labeled) were made in the sensorimotor cortex of 4 cats. No retrogradely labeled cells were found in the spinal cord in these cases. Our present and previously reported findings are discussed in the light of other studies of pathological changes in fiber tracts within the central nervous system. Although the present experiments were negative, the degenerating axons previously observed by us in silver sections from the pyramid, pons and internal capsule after lesions of the pyramidal tract in the spinal cord, can not be satisfactorily explained as evidence of retrograde, indirect Wallerian, degeneration of corticospinal fibers.

Animals↗

A quantitative analysis of the development of the pyramidal tract in the cervical spinal cord in the rat.

A quantitative electron microscopic analysis was undertaken of the development of the pyramidal tract, at the level of the third cervical spinal segment, in rats ranging in age from the day of birth to three months old. The axon number was calculated as the product of axon density, determined in a systematic random sample of electron micrographs, and tract area. During the first postnatal week the tract contains thin unmyelinated axons and growth cones. Growth cones are abundant in neonatal rats, but can still be observed occasionally at the end of the first postnatal week, indicating a continuous addition of pyramidal tract axons during the first postnatal week. Myelination starts around P10. By the end of the first postnatal month approximately 50% of the axons have already been myelinated. Myelination proceeds during further maturation, but in the three month old rat 28% of the axons are still unmyelinated. The total number of axons increases rapidly after birth up to 153,000 at the fourth postnatal day. Subsequently, the number of axons is reduced by nearly 50% to 79,000 in the adult rat. The axon loss is most prominent during the second postnatal week, when 32,000 axons are eliminated, but continues for several weeks at a slower rate.

Animals↗