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[Asymptomatic familial basal ganglia calcification with autosomal dominant inheritance: a family report].

We report here a pedigree of basal ganglia calcification with autosomal dominant inheritance. Following a traffic accident, the proband, a seven-year-old boy, was incidentally noted by cranial computed tomography to have calcification of the bilateral basal ganglia. Six affected members spanning three generations, aged from 5 to 57 years, also had calcification in various degree. None of them had clinical symptoms. There were neither abnormal data nor any characteristic physical symptoms associated with parathyroid disorders. There was no consanguinity. Both sexes were affected and the sex ratio was 0.5. Male-to-male transmission was documented. These findings suggested an autosomal dominant trait. The clinico-radiological findings in our pedigree were different from those of the previously reported cases of familial basal ganglia calcification, that infants were affected and that clinical symptoms were absent in elderly patients. These facts suggest our pedigree is a new type of familial basal ganglia calcification with autosomal dominant inheritance.

Basal Ganglia Diseases↗

An avian basal ganglia pathway essential for vocal learning forms a closed topographic loop.

The mammalian basal ganglia-thalamocortical pathway is important for motor control, motor learning, and cognitive functions. It contains parallel, closed loops, at least some of which are organized topographically and in a modular manner. Songbirds have a circuit specialized for vocal learning, the anterior forebrain pathway (AFP), forming a basal ganglia loop with only three stations: the pallial ("cortex-like") lateral magnocellular nucleus of the anterior neostriatum (lMAN), the basal ganglia structure area X, and the medial portion of the dorsolateral thalamic nucleus (DLM). Several properties of this pathway resemble those of its mammalian counterpart, but it is unknown whether all projections in the loop are topographically organized, and if so, whether topography is maintained through the entire loop. After small single- or dual-tracer injections into area X and/or the lMAN of adult zebra finches, we found that the area X to DLM projection is topographically organized, and we confirmed the topography for all other AFP projections. Quantitative analysis suggests maintained topography throughout the loop. To test this directly, we injected different tracers into corresponding areas in lMAN and area X. We found somata retrogradely labeled from lMAN and terminals anterogradely labeled from area X occupying the same region of DLM. Many labeled somata were tightly surrounded by tracer-labeled terminals, indicating the microscopically closed nature of the AFP loop. Thus, like mammals, birds have at least one closed, topographic loop traversing the basal ganglia, thalamus, and pallium. Each such loop could serve as a computational unit for motor or cognitive functions.

Animals↗

[Structural and functional changes in sympathetic ganglia following endotoxin and alpha1-antitrypsin exposure].

Structural and functional organisation of sympathetic ganglia under conditions of endotoxemia was studied in white rats, cats, and dogs. Submicroscopic characteristics of the changes occurring in the rat prevertebral sympathetic ganglia after endotoxin administration or application of endogenous proteinase inhibitor alpha 1-antitrypsin, were assessed as well as ultrastructural bases of the febrile rat ganglionic responses to antipyretic drug administration. Effects of endotoxin on synaptic transmission in inferior mesenteric plexus' ganglia of cats and on electrical activity in inferior mesenteric plexus' ganglia of dogs, were electrophysiologically demonstrated.

Action Potentials↗

Thalamic relay nuclei of the basal ganglia form both reciprocal and nonreciprocal cortical connections, linking multiple frontal cortical areas.

Thalamic relay nuclei transmit basal ganglia output to the frontal cortex, forming the last link in corticobasal ganglia circuitry. The thalamus regulates cortical activity through differential laminar connections, providing not only feedback, but also initiating "feedforward" loops, via nonreciprocal projections, that influence higher cortical areas. This study examines the organization of thalamic connections with cortex from basal ganglia relay nuclei, including ventral anterior (VA), ventral lateral (VL), and mediodorsal (MD) nuclei, in the Macaque monkey. Anterograde and bidirectional tracer injections ([3H]-amino acids, dextran conjugates of Fluorescein, Lucifer Yellow or FluoroRuby, or wheat germ agglutinin) into discrete VA/VL, MD, and frontal cortical sites demonstrate specific thalamocortical connections. VL projections target caudal motor areas (primary, supplementary, and caudal premotor areas), whereas VA projections target more rostral premotor areas (including cingulate and presupplementary motor areas) and MD projects to dorsolateral and orbital prefrontal cortices. Thalamocortical projections innervate cortical layers I and III, and to a lesser extent, layer V. In motor areas layer I projections are more extensive than those to layer III (and V). The complex laminar organization of projections from specific thalamic sites suggests differential regulation of cortical function. Injections of bidirectional tracers into thalamic and frontal cortical sites also show that in comparison to thalamocortical projections, corticothalamic projections to VA-VL and MD are more widespread. These findings demonstrate both reciprocal and nonreciprocal components to the thalamo-cortico-thalamic relay. Together, these experiments indicate a dual role for VA-VL and MD nuclei: (1) to relay basal ganglia output within specific cortical circuits and (2) to mediate information flow between cortical circuits.

Amino Acids↗

[Adrenoreception characteristics of the neurons of the superior cervical and caudal mesenteric sympathetic ganglia of the cat].

Experiments were conducted on the supeior cervical and the caudal mesenteric sympathetic ganglia of a cat; it was shown that dophamine (DA), similarly to noradrenaline (NA) and adrenaline (A), depressed the cholinergic conduction. The activity of DA in the superior sympathetic ganglion was less than that of the NA and A 2- and 3-fold, respectively, and in the caudal mesenteric ganglia DA was 50 times more active than NA by the capacity to depress the cholinergic conduction. The effects of DA and NA in the superior cervical ganglia were eliminated by dyhydroergotamine, phentholamine and haloperidol, but not by tropaphen and chlorpormazine. In the caudal mesenteric ganglia the inhibitory effect of NA was decreased by phentholamine, dihydroergotamine and chlorpromazine, but not by haloperidol. On the contrary, haloperidol and chlorpromzine decreased the depressive effect of DA on the cholinergic conduction in the caudal mesenteric ganglion, whereas phentholamine, dihydroergotamine and deseryl proved to be ineffective. It is supposed that the manifestation of the dopaminergic mechanism of inhibition of cholinergic conduction in the caudal mesenteric sympathetic ganglion could underlie the dilatation of the mesenterial and renal vessels and its hypotensive action caused by DA.

Action Potentials↗

Reappraisal of the motor role of basal ganglia: a functional magnetic resonance image study.

The importance of the basal ganglia in controlling motor function is well known. However, neuroimaging studies have failed to show either movement-rate dependence or different activation patterns caused by self-initiated (SI) and externally triggered (ET) movements in the basal ganglia-thalamo-motor loop. We herein report the functional magnetic resonance image (fMRI) mapping of sequential left-hand finger movements at five different rates under SI and ET conditions. Significant movement-rate dependence was found in the whole right basal ganglia-thalamo-motor loop only during the SI task. Network analysis also showed strong interactions within this loop during SI movement, whereas interactions were present only from the premotor cortex to the putamen via the sensorimotor cortex during the ET task. Furthermore, psychophysiological interaction analysis confirmed the different modulation between the two tasks in the putamen. fMRI provides evidence that the basal ganglia-thalamo-motor loop plays a key role in controlling the rate of sequential finger movements in SI movement but not in ET movement.

Adult↗

[Intramural ganglia of the human gallbladder: morphological and functional observations].

The purpose of this study was the reinvestigation of the intrinsic innervation of human gall bladder with an immunohistochemical technique named peroxidase anti-peroxidase (PAP). The antigen demonstrated was the S100 protein normally present in the surface of glial cells, Schwann cells and satellite cells in ganglia. The tissues used were taken from 20 human gall bladders, fixed after surgery. This technique is not specific to demonstrate adrenergic or cholinergic innervation but it reveals just myelinated fibers. The current study was undertaken in order to study the organization and the function of plexus of nerves and ganglia present in the wall of the gall bladder. The neck of the gall bladder was the region in which the higher number of nerve cells and nervous fibers was present. The technique used has demonstrated ganglionated plexus and nerves in submucosal layer, fibromuscular and adventitial layer according to the enteric nervous system. All ganglia are postganglionic stations related with preganglionic cholinergic fibers. These results confirm that the intramural ganglia of the gall bladder are analogous to those of the enteric nervous system according to their common origin.

Autonomic Fibers, Preganglionic↗

[Role of the basal ganglia in the occurrence of paradoxical sleep dreams (hypothetical mechanism)].

A hypothetical mechanism of the basal ganglia involvement in the occurrence of paradoxical sleep dreams and rapid eye movements is proposed. According to this mechanism, paradoxical sleep is provided by facilitation of activation of cholinergic neurons in the pedunculopontine nucleus as a result of suppression of their inhibition from the output basal ganglia nuclei. This disinhibition is promoted by activation of dopaminergic cells by pedunculopontine neurons, subsequent rise in dopamine concentration in the input basal ganglia structure. striatum, and modulation of the efficacy of cortico-striatal inputs. In the absence of signals from retina, a disinhibition of neurons in the pedunculopontine nucleus and superior colliculus allows them to excite neurons in the lateral geniculate body and other thalamic nuclei projecting to the primary and higher visual cortical areas, prefrontal cortex and back into the striatum. Dreams as visual images and "motor hallucinations" are the result of an increase in activity of definitely selected groups of thalamic and neocortical neurons. This selection is caused by modifiable action of dopamine on long-term changes in the efficacy of synaptic transmission during circulation of signals in closed interconnected loops, each of which includes one of the visual cortical areas (motor cortex), one of the thalamic nuclei, limbic and one of the visual areas (motor area) of the basal ganglia. pedunculopontine nucleus, and superior colliculus. Simultaneous modification and modulation of synapses in diverse units of neuronal loops is provided by PGO waves. Disinhibition of superioir colliculus neurons and their excitation by pedunculopontine nucleus lead to an appearance of rapid eye movements during paradoxical sleep.

Basal Ganglia↗

[Precocious puberty caused by hCG-producing germinoma involving the bilateral basal ganglia and cerebral white matter without 1ypical radiologic findings: case report].

An 8-year-old boy presented with a rare case of germinoma involving the bilateral basal ganglia and cerebral white matter manifesting as precocious puberty. Magnetic resonance (MR) imaging at the initial presentation demonstrated mild hyperintense areas in the bilateral basal ganglia and corpus callosum on T1-weighted images, and a small hyperintense spot in the right internal capsule on T2-weighted images. Human chorionic gonadotropin (hCG) level was elevated in the cerebrospinal fluid (CSF), so we strongly suspected that threre was a hCG-producing germinoma originating in the bilateral basal ganglia. Stereotactic biopsy was performed. Histological examination revealed two-cell pattern germinoma. After three cycles of combination chemotherapy consisting of ifosfamide, cisplatin, and etoposide, followed by whole brain irradiation with a total dose of 24 Gy, the CSF hCG level fell below the detection limit, but MR imaging demonstrated no significant change. Intracranial hCG-producing germinoma should be suspected in patients presenting with precocious puberty and elevated CSF hCG level. Moreover, slight intensity change on MR imaging is important to identify germinoma arising from the basal ganglia in the early stage.

Basal Ganglia Diseases↗

Interactions of the thymic polypeptide hormone thymopoietin with neuronal nicotinic alpha-bungarotoxin binding sites and with muscle-type, but not ganglia-type, nicotinic acetylcholine receptor ligand-gated ion channels.

Studies were conducted to assess the ability of the thymic polypeptide hormone thymopoietin (TPO) to interact with proto-typical ganglia-type or muscle-type nicotinic acetylcholine receptor ion channels (nAcChoR). Also investigated were interactions of TPO with neuronal nicotinic alpha-bungarotoxin binding sites (nBgtS), which share many features with nAcChoR and may belong to an extended nAcChoR family but do not appear to function as simple ligand-gated ion channels. TPO and alpha-bungarotoxin (Bgt) share the capacity for high affinity (IC50 values in the nanomolar range) interaction with nBgtS, which are expressed as high affinity radioiodinated Bgt binding sites by cells of the SH-SY5Y or IMR-32 human neuroblastomas. TPO and Bgt also share the capacity for high affinity interaction with muscle-type nAcChoR, which are expressed as high affinity binding sites for radioiodinated Bgt or tritium-labeled acetylcholine by cells of the TE671/RD human clone or the BC3H-1 mouse muscle cell line or on membrane preparations from Torpedo electroplax. TPO and Bgt act acutely as high affinity antagonists of muscle-type nAcChoR functional responses, which are measured using an isotopic rubidium ion efflux assay, on TE671/RD or BC3H-1 cells. In contrast, neither TPO nor Bgt are effective, at doses of up to 1 microM, as antagonists of ganglia-type nAcChoR function on SH-SY5Y or IMR-32 cells, nor are they potent as inhibitors of high affinity tritium-labeled acetylcholine binding to sites on putative ganglia-type nAcChoR expressed by SH-SY5Y or IMR-32 cells. These data indicate that some members of the extended nAcChoR family, including nBgtS and functional muscle-type nAcChoR but not ganglia-type nAcChoR, can interact with either Bgt or TPO. The results suggest that TPO may be an endogenous ligand active in both the nervous and immune systems and that some of its actions may be mediated via nBgtS or via functional blockade of muscle-type nAcChoR.

Acetylcholine↗

[Pathomorphology of adrenergic and cholinergic structures of sympathetic nerve ganglia in experimental burn injuries].

Changes in adreno- and cholinergic structures of sympathetic nerve ganglia (superior cervical, stellate, and splanchnic ganglia of the solar plexus) were studied in 15 male white rats aged 5-7 months, b. m. 200-250 g, 3, 7, and 11 days after burn injury (Stages IIIA, IIIB, involving 20-25% of body surface) and in 5 reference animals. The sections were treated in 2% glyoxylic acid solution and by the Karnovsky-Roots technique. Reduced catecholamine concentrations were revealed in sympathetic nerve ganglia neurons in the early periods after burn injury; the mediator reserves are recovered to a certain measure in later periods after thermal injury. The detected shifts in the sympathetic nerve ganglia neurons correlate with the detected shifts in the cardiovascular system.

Adrenergic Fibers↗

The permeability of capillaries among the small granule-containing cells in rat superior cervical ganglia: an ultrastructural lanthanum tracer study.

The permeability of blood capillaries associated with small granule-containing (SGC) cells in rat superior cervical ganglia was investigated at ultrastructural level by employing ionic lanthanum as an electron dense tracer. In rat superior cervical ganglia, the majority of blood capillaries were nonfenestrated. Both fenestrated and nonfenestrated capillaries were observed in the area associated with SGC cells. Lanthanum tracer was observed in the luminal surface, the interendothelial cleft and the subendothelial perivascular spaces of both fenestrated and nonfenestrated capillaries associated with SGC cells. The external lamina of the Schwann cell which surrounded the neurons, nerve fibres and SGC cells were clearly delineated by the lanthanum tracer. Furthermore, the perineuronal space, the periaxonal space, and the pericellular space of the SGC cells were readily accessible to the lanthanum ion. The results demonstrated an absence of blood-nerve barrier, blood-ganglionic and blood-SGC cell barrier to the lanthanum ion in the parenchymal area of the SGC cells in rat superior cervical ganglia. It is proposed that lanthanum may pass through the endothelial cells via 1) the fenestrae of fenestrated capillaries, 2) the intercellular junctions of both fenestrated and nonfenestrated capillaries, i.e., a paracellular pathway; and 3) the process of endocytosis/exocytosis, i.e., a transcellular pathway, to reach the subendothelial space and be distributed in the parenchyma of SGC cells in rat superior cervical ganglia.

Animals↗

The association cortex and the basal ganglia: a neuroanatomical view upon their relationship based on hodological studies.

A general view of the neuroanatomical relationship between the association cortex and the basal ganglia is presented in this article. Firstly, a definition is provided upon which part of the cerebral cortex will be taken into account for that anatomical correlation. Secondly, the corticostriatal projections as a whole, and particularly those from the prefrontal, parieto-temporo-occipital, and limbic cortices are evaluated within the basal ganglia circuits. Thirdly, a functional interpretation is offered upon the diencephalic and mesencephalic influences reached by the basal ganglia nuclei related principally to those areas of the cerebral cortex aforementioned. Finally, some speculations are formulated hoping to throw some light upon the anatomical and physiological significance of the basal ganglia in health and disease.

Animals↗

[Basal ganglia germinoma treated with interstitial brachytherapy; case report].

A case of primary intracranial germinoma in the left basal ganglia treated with interstitial brachytherapy was reported. A 15-year-old boy was referred to our hospital for evaluation of right hemiparesis. A CT scan showed a slightly hyperdense mass with multiple cystic low density in the left basal ganglia. The mass was heterogeneously enhanced after intravenous administration of contrast material. T1 weighted image showed a slightly hyperintense mass with cystic components and the mass was heterogeneously enhanced with Gd-DTPA. T2 weighted MR image showed a mixed intensity mass and peritumoral edema. Stereotactic needle biopsy and implantation of 3 catheters for interstitial brachytherapy were performed simultaneously using BRW CT guided stereotactic apparatus. After the histological diagnosis was confirmed to be two cell pattern germinoma, 9 iridium-192 seeds were inserted into the catheters and maintained for 10 days to give 35Gy of irradiation at the tumor periphery. Subsequent CT scans showed marked tumor regression and the clinical symptoms were improved. Germinoma originating in the basal ganglia is rare and hard to diagnose previous to biopsy. Histological confirmation is essential before initiation of treatment because germinoma is commonly thought to be radiosensitive tumor. The interstitial brachytherapy enables selective irradiation of the tumor and actually causes no complications such as bone marrow suppression or cerebral atrophy. The neuroradiological findings, especially of CT scan and MRI, were presented and the strategy for treatment of germinoma in basal ganglia was discussed.

Adolescent↗

[Connections between spinal ganglia in the pathogenesis of reflex-distant pain syndromes].

Using the complex of the neurohistological research methods the author studied spinal ganglia, spinal cord (37 dogs) following the removal of groups of the spinal ganglia in the cervical and thoracic sections. Widely spread distant receptor-reflex systems of the connections between spinal ganglia accompanied by dendrites and axones were revealed. The fibres of these systems pass in the Bechterew intermediate regions of the posterior columns of the spinal cord. The straight interneuronal connections between the cervical and lumbar spinal ganglia were found. On the basis of the obtained data the author conducted analysis of the mechanism of the occurrence of reflex-distant pain syndromes in patients with ganglio-radiculoneuralgia in degenerative diseases of the vertebral column. The reflex-distant syndrome occurs due to irritation of spinal ganglion neurons accomplishing distant ganglion connections.

Animals↗

[The change of ACh metabolism in the dorsal horn of spinal cord and spinal ganglia during electroacupuncture analgesia (EAA)].

In this paper a preliminary study was made about the relationship between ACh and the primary input of acupuncture sensation based on the changes of content of ACh, its synthetic enzyme (choline acetylase, or ChAC) and its degradation enzyme (acetylcholinesterase, or AChE) in the dorsal horn of the spinal cord and spinal ganglia. The results were found that: 1) EA at "huantiao" exerted a marked analgesia effect, the acupuncture analgesia was inhibited when the lateral dorsal root was cut off. 2) The content of ACh of the EA group were slightly lower than those of the control group. 3) AChE activity in the spinal ganglia and the dorsal horn increased markedly under electroacupuncture stimulation. 4) The activity of ChAC in the dorsal horn of rats under acupuncture stimulation was significantly higher than those of the control group. 5) ACh content in the spinal ganglia increased obviously when the degradation of peripheral ACh was inhibited by prostigmine. 6) With the lumber dorsal roots excised, AChE activity of the operative side were much lower than those of the intact side during EA stimulation. It suggest that the metabolism of ACh in the dorsal horn of the spinal cord and spinal ganglia change during the course of EAA, and only when signals produced at the acupoints are delivered to the spinal cord via ACh-containing primary somatosensory nerves, can they exert analgesic and therapeutic effect of acupuncture.

Acetylcholine↗

[Influence of electroacupuncture on ACh content and AChE activity in the spinal ganglia of rat].

The spinal ganglion a cell body storage of somatic primary-sensory neurons, is considered as an important region for neurochemical research of primary afferent nerves. The present experiment aims at the observation on the Ach content and its changes in the ganglia of 128 rats by means of biochemical methods under the effect of administration of Neostigmini and application of electroacupuncture. It is found that there are evidence of existence of Ach in the ganglia and when the AchE activity in the periphery nerve system is inhibited, the Ach content of ganglia rose. In addition, during acupuncture (applied at acupoint "Huantiao") analgesia, no apparent changes of Ach content can be visualized in spinal ganglia, however, the level of AchE activity goes up evidently. The foregoing results indicate that in the somatic primary-sensory neuron Ach is one of important neurotransmitters which may influence and take part in the transmission of sensory information through primary afferent nerves.

Acetylcholine↗

[A case of bilateral necrosis of the basal ganglia after hypotensive shocks].

We reported a case with bilateral necrosis of the basal ganglia after hypotensive shocks. The patient was a 69-year-old woman, who fell into a hypotensive shock (B.P. below 40 mmHg) of unknown origin during examination of her bladder cancer and was admitted into CCU. After admission, hypotensive shocks were repeated four times (B.P. below 50 mmHg each time). Neurological examination revealed a left spastic hemiplegia. Brain CT on 10th day showed bilaterally low density areas around the basal ganglia and a diagnosis of brain infarction was made. She gradually presented quadriplegia and symptomatic changes from pyramidal to extrapyramidal signs. Brain CT on 24th day showed bilateral hemorrhagic infarction of the basal ganglia with enhanced effect. On 79th day, she again fell into shock and died. Neuropathological examination of the brain was as follows. 1) laminar necrosis of the deep layers of the cerebral cortex, 2) bilateral necrosis of the hippocampal Sommer sector, 3) bilateral necrosis of the caudate nucleus, putamen and pallidum with neuronal loss and infiltration of fat granule cells, 4) sparing of the internal capsules, 5) bilateral necrosis of the reticular zone of the substantia nigra, 6) foci of fresh necrosis and loss of Purkinje cells in the cerebellum. These lesions are consistent with those of selective vulnerability in hypoxia as described by Scholz et al. An extensive distribution of cerebral as well as basal ganglia necrosis in this case was caused by repeated shocks.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗