MRI findings in Hirayama’s disease: flexion-induced cervical myelopathy or intrinsic motor neuron disease?

Hirayama’s disease is a benign juvenile form of focal amyotrophy affecting the upper limbs. Previous studies have suggested that the disorder is a neck flexion induced cervical myelopathy. We report clinical and magnetic resonance imaging findings in nine patients with Hirayama’s disease. Cervical imaging of seven patients revealed spinal cord changes consisting of focal atrophy and foci of signal alterations. On neck flexion a forward movement and mild reduction in the anteroposterior diameter of the lower cervical cord against the vertebral bodies was noted in affected individuals as well as in five normal controls. In contrast to earlier reports, none of our patients showed complete obliteration of the posterior subarachnoid space. Measurement of the anteroposterior spinal cord diameter in each vertebral segment (C4–C7) revealed no significant differences in the degree of spinal cord flattening between the two groups. Furthermore, two of our patients had significant degenerative changes in the cervical spine (disc herniation, retrospondylosis) contralateral to the clinically affected side. These degenerative changes resulted in a marked cord compression on neck flexion but were not associated with ipsilateral clinical abnormalities or spinal cord alterations. Our results argue against a flexion-induced cervical myelopathy and support the view that Hirayama’s disease is an intrinsic motor neuron disease. Received: 15 March 1999 Received in revised form: 25 May 1999 Accepted: 1 June 1999     

Selective up-regulation of P- and R-type Ca2+ channels in rat embryo motoneurons by BDNF

Cultured spinal cord motoneurons from day 15 rat embryos (E15) represent a useful model to study Ca2+ channel diversities and their regulation by neurotrophins. Besides the previously identified L-, N- and P-type channels, E15 rat motoneurons also express high densities of R-type channels. We have previously shown that the P-type channel is nearly absent in 60% of these cells, while the R-type contributes to approximately 35% of the total current. Here, we show that chronic preincubation of cultured rat motoneurons with high concentrations (20-100 ng/mL) of brain-derived neurotrophic factor (BDNF) caused a selective up-regulation of the P- and R-type current density available after blocking N- and L-type channels, with no changes to cell membrane capacitance. N- and L-type channels were either not affected or slightly down-modulated by the neurotrophin. The onset of BDNF up-regulation of P/R-type currents had a half-time of 12 h and reached maximal values of approximately 80%. High concentrations of nerve growth factor (NGF; 50-100 ng/mL) had no effect on P/R currents, while BDNF action was prevented by the kinase inhibitor K252a and by the protein synthesis inhibitor anisomycin. These results suggest that chronic applications of BDNF selectively up-regulates the Ca2+ channel types which are most likely to be involved in the control of neurotransmitter release in mammalian neuromuscular junctions. The signal transduction mechanism is probably mediated by TrkB receptors and involves the synthesis of newly functionally active P- and R-type channels. Our data furnish a rationale for a number of recent observations in other laboratories, in which prolonged applications of neurotrophins were shown to potentiate the presynaptic response in developing synapses.     

CNS pattern of metabolic activity during tonic pain: evidence for modulation by beta-endorphin

CNS correlates of acute prolonged pain, and the effects of partial blockade of the central beta-endorphin system, were investigated by the quantitative 2-deoxyglucose technique in unanaesthetized, freely moving rats. Experiments were performed during the second, tonic phase of the behavioural response to a prolonged chemical noxious stimulus (s.c. injection of dilute formalin into a forepaw), or after minor tissue injury (s.c. saline injection). During formalin-induced pain, local glucose utilization rates in the CNS were bilaterally increased in the grey matter of the cervical spinal cord, in spinal white matter tracts and in several supraspinal structures, including portions of the medullary reticular formation, locus coeruleus, lateral parabrachial region, anterior pretectal nucleus, the medial, lateral and posterior thalamic regions, basal ganglia, and the parietal, cingulate, frontal, insular and orbital cortical areas. Pretreatment with anti-beta-endorphin antibodies, injected i.c.v., led to increased metabolism in the tegmental nuclei, locus coeruleus, hypothalamic and thalamic structures, putamen, nucleus accumbens, diagonal band nuclei and dentate gyrus, and in portions of the parietal, cingulate, insular, frontal and orbital cortex. In formalin-injected rats, pretreated with anti-beta-endorphin, behavioural changes indicative of hyperalgesia (increased licking response) were found, which were paralleled by a significant enhancement of functional activity in the anterior pretectal nucleus and in thalamo-cortical systems. A positive correlation was found between the duration of the licking response and metabolic activity of several forebrain regions. These results provide a map of the CNS pattern of metabolic activity during tonic somatic pain, and demonstrate a modulatory role for beta-endorphin in central networks that process somatosensory inputs.     

Morphological analysis of the cervical spinal canal,dural tube and spinal cord in normal individuals using CT myelography

To verify the conventional concept of developmental stenosis of the cervical spinal canal, we performed a morphological analysis of the relations of the cervical spinal canal, dural tube and spinal cord in normal individuals. The sagittal diameter, area and circularity of the three structures, and the dispersion of each parameter, were examined on axial sections of CT myelograms of 36 normal subjects. The spinal canal was narrowest at C4, followed by C5, while the spinal cord was largest at C4/5. The area and circularity of the cervical spinal cord were not significantly correlated with any parameter of the spinal canal nor with the sagittal diameter and area of the dural tube at any level examined, and the spinal cord showed less individual variation than the bony canal. Compression of the spinal cord might be expected whenever the sagittal diameter of the spinal canal is below the lower limit of normal, that is about 12 mm on plain radiographs. Thus, we concluded that the concept of developmental stenosis of the cervical spinal canal was reasonable and acceptable.     

Repair of a ruptured aortic arch aneurysm complicated by postoperative paraplegia: Report of a case

We report herein the rare case of a 79-year-old man who suffered permanent paraplegia after undergoing an otherwise successful total arch replacement for a ruptured aortic arch aneurysm. During cardiopulmonary bypass, perfusion to the distal aorta was maintained from the femoral artery, and postoperative aortography showed intact tributaries from the aorta including the intercostal arteries. Postoperative paraplegia is an extremely rare complication of operations on the aortic arch; however, we speculate that the paraplegia in this patient could be attributed either to a steal phenomenon involving the radicular artery, or to the anatomical particularity of the spinal cord artery described by Cole and Gutelius as the segmental system.     

Caustic ingestion in childhood: current treatment possibilities and their complications

In a review of 15 pediatric patients who had ingested caustic substances, the authors describe the diagnostic and therapeutic procedures to be followed as well as the complications that may occur with their use. The cases reported include 1 esophageal rupture caused by balloon dilatation and 1 recurrent stenosis treated with a silastic tutor.     

Responses of long descending propriospinal neurons to natural and electrical types of stimuli in cat

Long descending propriospinal (LDP) neurons (antidromically identified) having cell bodies of origin in the cervical enlargement and projecting axons at least as far as the L2 segment were studied. Extracellular recording of responses to natural and electrical stimuli was done in high-spinal cats.

(1) A receptive field for natural stimuli was found for 123 LDP neurons. An additional 108 LDP cells were not activated by the natural stimuli used, but some of these fired spike potentials in response to electrical stimulation of peripheral nerves of the forelimb. There was no distinction between neurons activated and those not activated by natural stimuli on the basis of location or conduction velocity.

(2) The most effective natural stimuli were mechanical manipulation of the skin (both low and high threshold), movement of joints of the paw, and pressure to the deep tissues, especially to the extensor side of the arm. These modalities of stimuli were most often excitatory, but could be inhibitory as well.

(3) On the basis of modality, 4 subgroups of LDP cells were identified: those which were responsive only to mechanical-cutaneous, joint-movement, or deep-pressure stimuli, and those which responded to several of these modalities of stimuli, the multimodal group. These subgroups could not be distinguished on the basis of conduction velocity.

(4) The receptive fields varied in size from small (one digit) to large (all of a forelimb). For single LDP cells they included ones with single and/or multimodal input from one or both forelimbs and various combinations of excitation and/or inhibition. However, those in the dorsal horn had only ipsilateral receptive fields, mainly of the mechanical-cutaneous type. Cells with bilateral receptive fields were mainly located medially in the ventral gray in laminae VII and VIII.

(5) A comparison of the location of the subtypes of LDP cells revealed that neurons activated by mechanical-cutaneous stimuli were in laminae I and IV–VIII; whereas deep-pressure and multimodal activated neurons were almost exclusively in laminae VII and VIII.

(6) LDP cells receiving input from deep-pressure receptors in the extensor muscles of the arm, joint-movement, or deep-pressure receptors of the paw probably relay position or weight-bearing information about the forelimbs to the lumbosacral spinal cord. This arrangement suggests that LDP neurons function in interlimb coordination and would be active during locomotion. They probably participate also in other reflexes elicited by cutaneous and deep stimuli.

Plasticity of the corticospinal tract following midthoracic spinal injury in the postnatal rat

Rats received a midthoracic spinal cord "overhemisection" including right hemicord and left dorsal funiculus at birth (neonatal operates, N = 15) or 21 days of age (weanling operates, N = 14). In a second experiment neonatal (N = 6), 6-day (N = 3), and 12-day (N = 7) rats sustained a right sensorimotor cortex (SmI) ablation to destroy the left corticospinal tract (CST) at the same time as the spinal injury (double lesion operates). Later (3-12 months) injections of 3H-proline and autoradiography were used to label the left or right CST. The results of the first experiment showed that most right CST axons failed to grow around the spinal lesion in neonatal operates (N = 9). There was an increase in the density of label, mainly to CST projection areas, in a 1-mm zone rostral to the lesion. However, left CST axons bypassed the lesion by growing through the intact tissue in neonatal operates (N = 6). These displaced axons were consistently located within the dorsal portion of the lateral funiculus (dLF) and remained within that location caudal to the lesion, an area normally containing only a few CST axons. In spite of this abnormal position, these axons terminated bilaterally throughout the remainder of the cord in normal CST sites. In weanling operates, CST axons severed by the lesion did not regenerate around the lesion site. An increased density of label over the few spared axons within the left dLF and in CST projection zones immediately caudal to the lesion site suggested axonal sprouting by these axons. The results of the second experiment showed that the lack of growth of right CST axons around this injury in neonatal operates was, at least partially, due to an interaction with left CST axons. In neonatal double lesion operates, right CST axons grew around the spinal injury for a varying distance within the left dLF and distributed bilaterally to normal CST sites. The number of right CST axons bypassing the lesion was related to the configuration of the lesion site. A smaller number of right CST axons bypassed the lesion in 6-day double lesion operates and most terminated within 2-3 mm of the lesion site. Right CST axons failed to grow around this injury in 12-day double lesion operates.(ABSTRACT TRUNCATED AT 400 WORDS)     

The functional restoration approach for chronic spinal disability

The functional restoration approach to treating chronic spinal disability consists of a medically directed, interdisciplinary team approach to physical reconditioning and a cognitive-behavioral crisis intervention procedure for dealing with related psychosocial problems. One- and two-year follow-up studies have demonstrated the clinical efficacy of this approach. The present article describes this approach and summarizes the research documenting its success in treating patients with chronic spinal disability. This article also highlights the pitfalls in misunderstanding and misrepresenting the components of the functional restoration approach when evaluating treatment efficacy.     

Intraoperative ultrasonography of extramedullary spinal tumours

Summary Intraoperative spinal sonography (IOSS) was performed in 28 patients with extramedullary spinal tumours. Images were obtained of 31 tumours, because 2 patients had multiple tumours. There were 17 neurilemmomas, 9 meningiomas and 2 dermoid, cysts. All tumours appeared well-defined and highly echogenic. There was no difficulty differentiating extramedullary spinal tumours from intramedullary nasses excepting case, a big neurilemmoma at the conus medullaris. All images were assessed with reference to the surface of the lesions, the existence of cysts, and movement. Neurilemmomas had smooth surfaces, some cysts, and exhibited a floating movement. Meningiomas had irregular surfaces, were more highly echogenic than neurilemmomas, and adhered tightly to the dura mater. However, some neurilemmomas and meningiomas did not have these, and it was not always possible to differentiate them. Although, IOSS has limitations of resolution, it gave useful information about the size of the tumours, the degree of displacement of the spinal cord, and even about the type of tumour in some cases. It was extremely helpful to the surgeon.