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PubMed1984

Atlas of serotonergic cell bodies in the cat brainstem: an immunocytochemical analysis.

The localization and relative number of serotonergic (5HT) cell bodies in the brainstem of the cat were studied through the use of a specific immunocytochemical technique. A surprisingly large number of 5HT cells were found in regions in addition to the classical raphe nuclei (obscurus, pallidus, magnus, centralis superior, and dorsalis). Foremost among these were: the ventral medulla, just dorsal to the pyramidal tract and inferior olivary complex, and especially the area in and around the lateral reticular nucleus; the dorsal pons, surrounding the central reticular core, and in the central gray area; and a region in the mesencephalon, in and around the interpeduncular nucleus. The advantages and disadvantages of the existing schemas for subdividing and labeling groups of brain 5HT neurons are discussed.

Animals↗
PubMed1990

Projections of two separate cortical areas for rhythmical jaw movements in the rat.

The cortico-bulbar projection from two separate cortical areas which induce different types of rhythmical jaw movements (RJM), and the relationship between these cortical areas were studied with horseradish peroxidase tracing method. One area (A-area) corresponded to the primary jaw motor area and the other (P-area) was located in the agranular insular cortex. Separate descending pathways from two areas passed through the pyramidal tract and projected to the supratrigeminal nucleus, the intertrigeminal region, the dorsal part of the trigeminal sensory complex, and the reticular formation. In the reticular formation, the A-area projected more medially than the P-area did, and the ipsilateral projection from the P-area was more prominent than that from the A-area, although contralateral projections were dominant in the majority of regions. The two areas had only a sparse reciprocal connection. We suppose that the difference in patterns of RJM induced by the two cortical RJM areas may be due to the different projection patterns from these two areas.

Animals↗
PubMed1991

Immunocytochemistry of thyrotropin-releasing hormone in the rabbit medulla oblongata.

The distribution and ultrastructure of thyrotropin-releasing hormone-like immunoreactive (TRH-LI) neurons were examined in rabbit medulla oblongata. TRH-LI cell bodies were located in the ventral region of the medulla oblongata: in the paraolivary and parapyramidal regions, regions in and around the pyramidal tract, the dorsolateral region of the lateral reticular nucleus, and the raphe nuclei. The paraolivary and parapyramidal regions contained most of the TRH-LI cell bodies in the medulla oblongata. TRH-LI neurons processes were densely distributed in the dorsal vagal complex and the area postrema. Electron-microscopic immunocytochemical studies revealed TRH-LI neurons at the obex level in the paraolivary region of rabbits. TRH-like immunoreactivity was localized in larger granular vesicles. TRH-LI somata and dendrites received synaptic inputs from both TRH-LI and unlabeled axon terminals. More than half of the TRH-LI axon terminals made synapses with somata or processes of TRH-LI neurons. These observations, together with previous reports that TRH causes respiratory facilitation, suggest that TRH-LI neurons in the paraolivary region in rabbits may be involved in respiratory functions.

Animals↗
PubMed1993

Selective vulnerability of alpha motor neurons in ALS: relation to autoantibodies toward acetylcholinesterase (AChE) in ALS patients.

The degenerative process in amyotrophic lateral sclerosis (ALS) concerns primarily alpha motor neurons in the spinal cord and brain stem, and neurons forming descending pathways to the cord, especially in the pyramidal tract. Some degeneration of large peripheral sensory nerve fibers often occurs too, but preganglionic autonomic neurons and gamma motor neurons are most often spared in the disease. The vulnerability of alpha motor neurons compared to other types of neurons in ALS is discussed in relation to retrograde axoplasmic transport from peripheral blood of foreign noxious macromolecules, interneuronal transport of such molecules, and neuronal surface structure properties relevant to uptake for retrograde axoplasmic transport. Certain differences in these aspects between alpha motor neurons and other neuronal types exist. Some differences concern the neuronal turnover of acetylcholinesterase (AChE), which could be of special interest in view of the recent demonstration of regular occurrence of autoantibodies towards this enzyme in ALS patients.

Acetylcholinesterase↗
PubMedDate not supplied

Dominantly inherited early-onset non-progressive cerebellar ataxia syndrome.

A mother and daughter with suspected dominantly inherited, early-onset, non-progressive cerebellar ataxia syndrome have been reported. A review of the literature and the clinical features of the present cases revealed the nosologic features of this rare disorder, possibly dominant inheritance, floppiness and delayed milestones preceding early-onset mild cerebellar ataxia, non-progressive clinical course, retained or even brisk tendon reflexes without pyramidal tract involvement, normal or slightly delayed intelligence, and occasional nystagmus. Neuroimaging reveals selective involvement of the cerebellum, which is prominent in the vermis and the anterior part of the hemispheres.

Adult↗
PubMed1994

Ethylmalonic aciduria: an organic acidemia with CNS involvement and vasculopathy.

Five infants from 3 families, one Egyptian, two Yemeni, are described with a progressive encephalopathy, four of whom have been studied in detail. All patients showed vascular lesions of the skin, characterized by waxing and waning petechiae and ecchymoses. Acrocyanosis was present in three patients. All patients showed retinal lesions characterized by tortuous veins. Protracted diarrhea was not a consistent finding, although they had metabolic crisis in association with diarrhea. They did not show failure to thrive. The neurologic symptoms were indicative of a progressive pyramidal tract disease. Three patients died following sudden emergence of severe basal ganglia, putaminal and head of caudate lesions. In one patient the CT changes in brain were suggestive of infarction. The patients who died manifested pulmonary congestion, or wet lung, and respiratory difficulties during the terminal stage of the disease. In all patients before and during the terminal event, mild-to-moderate hematuria, and in two RBC in CSF, was observed. In one patient there was mild hemoperitoneum at the terminal event. The urine organic acids indicated increased excretion of ethylmalonic, methylsuccinic, glutaric, and adipic acids. The patients invariably showed lactic acidosis, but no ketosis, during and in between the acidotic attacks of the disease. The acylcarnitine profile in blood of two patients showed a pronounced increase in C4 and C5 carnitine esters. In three patients, biopsies from petechiae indicated absence of an immune event, showing only fresh hemorrhage. An immunologic study in one patient was normal for the suppressor:cytotoxic lymphocyte ratio and concentration of interleukin-2 receptor during and in between hemorrhagic attacks. The cytochrome c oxidase activity in fibroblasts was normal. The rate of oxidation of glucose, leucine, isoleucine, valine, propionate and butyrate by fibroblasts was normal. The disease is not responsive to treatment with riboflavin, ascorbic acid, vitamin E, glycine, or carnitine. One patient remained stable on prolonged large doses of methylprednisolone. The biochemical defect leading to ethylmalonic aciduria in this disease remains unknown.

Acidosis↗
PubMed1984

Joseph Babinski: the phenomenon of the toes.

Joseph Babinski first described the plantar cutaneous reflex that bears his name in two papers in 1896 and 1898. He linked the sign, which he termed "the phenomenon of the toes," to disturbances of the pyramidal tract. In the nearly 90 years that have elapsed since the original description, little significant elaboration has been made upon Babinski's initial works. Although the reflex is best known in conjunction with organic disease involving structural injury to the corticospinal tract, it may be present in a number of toxic and metabolic disturbances. These are diverse, and no satisfactory explanation exists to explain the presence of the reflex in such clinical entities. In some instances the reflex reverts to normal following improvement of the underlying condition.

France↗
PubMed1996

Enhancement of inhibitory mechanisms in the motor cortex of patients with cerebellar degeneration: a study with transcranial magnetic brain stimulation.

The excitatory state of the primary motor cortex can be studied by measuring either the postexcitatory inhibition after transcranial magnetic single stimulation (pI-S) or the refractory period with magnetic double stimulation (rP-D). The cerebellum may influence the excitability of the motor cortex by cerebellar inputs and outputs from side loops of transcortical projections. Therefore, we studied pI-S and rP-D in 24 patients with autosomal dominant cerebellar ataxia or idiopathic cerebellar ataxia, who were allocated to one group (Group A) with mild to moderate ataxia (n = 11) and to another group (Group B) with severe ataxia (n = 13). The results were compared with those obtained in 21 normal age-matched control subjects. The central motor conduction time (CMCT) was delayed in approximately half of the patients, demonstrating that the degenerative process, beyond the cerebellum, also affects the pyramidal tract. Mean CMCT was significantly delayed only in patients of Group B. pI-S was prolonged in 10 of our 24 patients; incidence of pathology in pI-S did not differ between the two patient groups. In 5 patients with normal CMCT, pathological pI-S results were found. Mean pI-S was prolonged in the whole patient group and in both subgroups as well. rP-D was prolonged in two patients of Group B only, but mean rP-D was significantly prolonged in the whole patient group. Prolonged postexcitatory inhibition and refractory period may be a consequence of a transient facilitation of cortical inhibitory interneurons, which results in a decreased excitability of primary motor cortex in patients with cerebellar degeneration.

Adult↗
PubMed2004

Precision grasps of children and young and old adults: individual differences in digit contact strategy, purchase pattern, and digit posture.

The evolutionary origins and variations of the precision grip, in which an object is held between the thumb and other digits, are poorly understood. This is surprising because the neural basis of this grasp pattern, including the motor cortex and pyramidal tract have received extensive study. Most previous work has shown that features of an object to be grasped (external factors) determine grasp patterns. The objective of the present study was to investigate individual differences (central factors) in use of the pincer and other precision grips. The grasping patterns of male and female young adults, older adults and children were examined as they reached (with both left and right hand) for five small beads (3-16 mm diameter). Frame-by-frame analysis of grasping indicated a high degree of variability in digit contact strategies, purchase patterns and digit posture both within and between subjects. (1) The contact strategies consisted of five variations, depending on whether the thumb or the index finger dragged or stabilized the bead for grasping. (2) Purchase patterns consisted of seven different types of precision grips, involving the thumb and various combinations of other digits. (3) There were four variations stemming from the posture of the non-grasping digits. Grip patterns of the left and right hands were correlated in individual subjects, as were strategies used for different bead sizes. Females displayed slightly more variability in grasp patterns than did males, and digit width (obtained from photocopies of the subjects' hands) was weakly correlated with the grasp patterns used. Although it was expected that the pincer would be used for all objects, it was preferentially used for only the smallest object except for older adults who used the pincer grasp on most objects. The variability in digit contact strategies, purchase patterns, and posture of the non-grasping digits indicates that central factors (innate or learning-induced architecture of the left parietal cortex) make important contributions to the selection of a grasping pattern. These individual differences are discussed in relation to the neural control of grasping and its potential contribution to understanding the evolution, development, and pathology of the precision grip.

Adolescent↗
PubMed2004

Quantification of Uhthoff's phenomenon in multiple sclerosis: a magnetic stimulation study.

OBJECTIVE: To quantify temperature induced changes (=Uhthoff phenomenon) in central motor conduction and their relation to clinical motor deficits in 20 multiple sclerosis (MS) patients. METHODS: Self-assessment of vulnerability to temperature and clinical examination were performed. We used motor evoked potentials to measure central motor conduction time (CMCT) and applied the triple stimulation technique (TST) to assess conduction failure. The TST allows an accurate quantification of the proportion of conducting central motor neurons, expressed by the TST amplitude ratio (TST-AR). RESULTS: Temperature induced changes of TST-AR were significantly more marked in patients with prolonged CMCT (P=0.037). There was a significant linear correlation between changes of TST-AR and walking velocity (P=0.0002). Relationships were found between pronounced subjective vulnerability to temperature and (i) abnormal CMCT (P=0.02), (ii) temperature induced changes in TST-AR (P=0.04) and (iii) temperature induced changes in walking velocity (P=0.04). CMCT remained virtually unchanged by temperature modification. CONCLUSIONS: Uhthoff phenomena in the motor system are due to varying degrees of conduction block and associated with prolonged CMCT. In contrast to conduction block, CMCT is not importantly affected by temperature. SIGNIFICANCE: This is the first study quantifying the Uhthoff phenomenon in the pyramidal tract of MS patients. The results suggest that patients with central conduction slowing are particularly vulnerable to develop temperature-dependent central motor conduction blocks.

Adult↗
PubMed2006

Intramedullary spinal cord metastases in a patient with small-cell lung cancer.

We present the case of a 60-year-old male smoker with gait disturbance, lower limb sensory disturbance, and urinary difficulties of subacute onset and progressive course. He had been diagnosed 10 months earlier with limited stage small-cell lung cancer, had received chemotherapy, thoracic radiotherapy, and prophylactic cranial irradiation, and was in remission on follow-up. Examination revealed bilateral pyramidal tract signs, a T9 sensory level, and loss of vibration and position sense in the lower limbs. Gadolinium-enhanced MRI of the cervical and thoracic spinal cord revealed two enhancing intramedullary lesions consistent with metastases. Intramedullary metastases are exceedingly rare, accounting for only 3.4-6% of myelopathies in cancer patients, and they usually coexist with brain or leptomeningeal metastases. The presence of multiple metastases is even rarer, with two lesions found in only 10% of patients with intramedullary disease.

Journal Article↗
PubMed2006

Superficial siderosis due to papillary glioneuronal tumor.

Superficial siderosis of the central nervous system is a rare, progressive, irreversible and debilitating neurological disease characterized by the deposition of haemosiderin in the leptomeninges and the subpial layers of the brain and spinal cord. The main clinical findings are progressive bilateral sensorineural hearing loss, cerebellar ataxia and pyramidal tract signs. The present report describes a 49-year-old woman who presented with intermittent headache of 5 years duration. The pain had become more severe in the previous 6 months. Neurological examination revealed nothing abnormal. Computed tomography showed a cystic mass with apparent internal haemorrhage in the right frontal lobe and T(2)-weighted magnetic resonance imaging showed material of low signal intensity coating the entire surface of the brain. The mass was completely excised via craniotomy. A histopathological study identified the mass as a papillary glioneuronal tumour. The patient recovered well and is still neurologically normal 1 year later. This is the first documented case of superficial siderosis caused by this type of tumour.

Brain Neoplasms↗
PubMed2006

High-affinity GABA uptake and GABA-metabolizing enzymes in pig forebrain white matter: a quantitative study.

GABA receptor activation in central nervous white matter may be protective during white matter hypoxia in the adult, and it may modify axonal conduction, especially in the developing brain. GABA uptake is important for the shaping of the GABA signal, but quantitative data are lacking for GABA uptake and GABA-metabolizing enzymes in central nervous white matter. We report that high-affinity uptake of GABA in adult pig corpus callosum, fimbria, subcortical pyramidal tracts, and occipital white matter is approximately 20% of that in temporal cortex gray matter. Tiagabine (0.1 microM), an antiepileptic drug that specifically inhibits the GAT-1 GABA transporter inhibited GABA uptake 50% in temporal cortex and 60-68% in white structures. This finding indicates that GAT-1 is an important GABA transporter in white matter and suggests that white matter GABA uptake is inhibited during tiagabine therapy. GABA transaminase activity in white structures was approximately 20% of neocortical values. Glutamate decarboxylase (GAD) activity in white structures was only 4% of that in neocortex (7-12 pmol/mg tissue x min(-1) versus approximately 200 pmol/mg tissue x min(-1)). Since white matter activity of citrate synthase of the tricarboxylic acid cycle was approximately 25% of neocortical values ( approximately 0.4 nmol/mg tissue x min(-1) versus approximately 1.5 nmol/mg tissue x min(-1)), the low GAD activity suggests a slower metabolic turnover of GABA in white than in gray matter.

4-Aminobutyrate Transaminase↗
PubMed2006

Two different reorganization patterns after rehabilitative therapy: an exploratory study with fMRI and TMS.

We used two complementary methods to investigate cortical reorganization in chronic stroke patients during treatment with a defined motor rehabilitation program. BOLD ("blood oxygenation level dependent") sensitive functional magnetic resonance imaging (fMRI) and intracortical inhibition (ICI) and facilitation (ICF) measured with transcranial magnetic stimulation (TMS) via paired pulse stimulation were used to investigate cortical reorganization before and after "constraint-induced movement therapy" (CI). The motor hand function improved in all subjects after CI. BOLD signal intensity changes within affected primary sensorimotor cortex (SMC) before and after CI showed a close correlation with ICI (r = 0.93) and ICF (r = 0.76) difference before and after therapy. Difference in number of voxels and ICI difference before and after CI also showed a close correlation (r = 0.92) in the affected SMC over the time period of training. A single subject analysis revealed that patients with intact hand area of M1 ("the hand knob") and its descending motor fibers (these patients revealed normal motor evoked potentials [MEP] from the affected hand) showed decreasing ipsilesional SMC activation which was paralleled by an increase in intracortical excitability. This pattern putatively reflects increasing synaptic efficiency. When M1 or its descending pyramidal tract was lesioned (MEP from the affected hand was pathologic) ipsilesional SMC activation increased, accompanied by decreased intracortical excitability. We suggest that an increase in synaptic efficiency is not possible here, which leads to reorganization with extension, shift and recruitment of additional cortical areas of the sensorimotor network. The inverse dynamic process between both complementary methods (activation in fMRI and intracortical excitability determined by TMS) over the time period of CI illustrates the value of combining methods for understanding brain reorganization.

Aged↗
PubMed2006

Fibers from the dorsal premotor cortex elicit motor-evoked potential in a cortical dysplasia.

OBJECTIVE: To identify the fibers originating from the dorsal premotor cortex (dorsal PMC) that contribute to motor-evoked potentials (MEP), we have applied integrated functional neuronavigation and awake surgery during removal of an epileptic cortical dysplasia located in the right premotor cortex. METHODS: Chronic subdural electrodes were employed for functional mapping during a routine invasive evaluation for intractable epilepsy. After the fibers originating from the dorsal PMC were plotted into the tractography-integrated functional neuronavigation, subcortical MEPs and clinical symptoms were examined during resection of the epileptogenic dorsal PMC. RESULTS: During removal of the epileptogenic area, MEPs were elicited by electrical stimulation of the fibers originating from the dorsal PMC, which were separated from the pyramidal tracts from the precentral gyrus. Resection of the dorsal PMC and its fibers caused a transient dysmetric movement of the left toe without motor weakness. CONCLUSION: Functional corticospinal tract fibers originating from the dorsal PMC can be defined and removed safely under local anesthesia with the aid of integration of functional neuronavigation and subcortical electrical stimulation.

Adolescent↗
PubMed2004

Unilateral periodic limb movement in sleep after supratentorial cerebral infarction.

We report a patient with involuntary right leg movements during sleep, which developed after acute ischemic stroke in the corona radiata. Nocturnal polysomnography confirmed the diagnosis of periodic limb movement in sleep (PLMS). Although the pathogenesis of PLMS is still controversial, it has been proposed that the loss of cortical or subcortical inhibition resulting from the pyramidal tract lesion might cause PLMS. The present case provides evidence to support this hypothesis.

Adult↗
PubMed2006

Mirror movements following cortical resection of polymicrogyria in a child with intractable epilepsy.

Mirror movements may be congenital or acquired. There are few reports of acquired mirror movements in pediatric patients. Further, mirror movements in children with epilepsy have rarely been reported. A 9-year old male, with intractable partial epilepsy resulting from polymicrogyria of the right hemisphere, underwent cortical resection of the right frontotemporoparietal region for a malformation of cortical development. He developed left hemiplegia and mirror movements in the left hand in the postoperative period. Four months after surgery, he remained seizure-free with mild residual left-sided hemiplegia and persistent mirror movements. Mechanisms postulated for mirror movements include aberrant pyramidal tract development and transcallosal inhibitory pathways. The latter mechanism might have contributed to the mirror movements observed in this child. This study is the first report of mirror movements following focal cortical resection for intractable epilepsy due to polymicrogyria.

Cerebral Cortex↗
PubMed1993

Superficial siderosis of the central nervous system--diagnosis by magnetic resonance imaging.

Superficial siderosis of the central nervous system is a rare disorder characterized by the deposition of haemosiderin in the leptomeninges, cranial nerves and spinal cord. The clinical features include sensorineural deafness, ataxia and pyramidal tract signs. We describe two cases demonstrating the characteristic findings on magnetic resonance imaging of widespread peripheral low signal on T2-weighted images.

Adult↗