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PubMed1993

[Calcification of the basal ganglia and Fahr disease. Report of two clinical cases and review of the literature].

Computed Tomography (CT) is superior to conventional skull radiographs in detecting calcifications of the basal ganglia. Basal ganglia calcification has been associated with different conditions, abnormality in the calcium-phosphorus metabolism being the most common of these associations. Some patients, without any evident disease, are well documented and called idiopathics of Fahr's syndrome; less numerous patients present familiar trait, with autosomal dominant inheritance. The correlation of neurological impairment with symmetrical calcification of basal ganglia is not so frequent. Here we present two cases of Fahr's disease, in whom CT aspects were very similar, but the neurological manifestations were different. The review of the literature shows, now, that there is no definite pathogenesis of basal ganglia calcifications.

Basal Ganglia↗
PubMedDate not supplied

[Depression and calcinosis of the basal ganglia: apropos of a case].

Idiopathic basal ganglia calcifications is a heterogeneous entity characterized by the association of bilateral and symmetric calcifications of the basal ganglia and the dentae nuclei in the cerebellum, polymorphous neuropsychiatric symptomatology. It is rare, of unknown etiology and often hereditary. We report the case of a 48 years old man, who presented with melancholic depression which has been steadily worsening for more than a year and which had led to a serious suicide attempt. Clinical observation revealed apragmatism, affective dullness and impoverished spontaneous expression; the theme of incapacity predominated but guilt feelings were absent. Neurologic examination was normal except for extra-pyramidal signs. CT-scan revealed the presence of bilateral and extensive calcifications of caudate nuclei, lenticular nuclei and thalamus, which indicated a diagnosis of idiopathic basal ganglia calcification characterized by depression. Moreover, MRI revealed an hypersignal of the white periventricular substance. Phospho-calcic results and the parathormone dosage were normal. Psychometric tests showed that although intellectual capacity seemed to be intact, it was not utilised in practice. Unresponsive to antidepressants and sismotherapy, the patient is still the same one and a half year later, and needs to remain in an institution. Subsequent tests indicate that intellectual deterioration has begun. This case poses the problem of the relationship between depressive syndrome and basal ganglia lesions. We look at studies bearing on this subject and on other clinically similar syndromes (athymhormia, loss of psychic self-activation), in which the interruption or cortico-striato-pallido-thalamo-cortical circuits (particularly the limbic loop), is a physiopathologic mechanism currently invoked.

Basal Ganglia↗
PubMed1996

Evolution of high-intensity basal ganglia lesions on T1-weighted MR in neurofibromatosis type 1.

PURPOSE: To characterize the temporal evolution of the foci of T1 shortening in basal ganglia lesions in patients with neurofibromatosis type 1 (NF-1). METHODS: A retrospective review of MR images of 37 patients with NF-1 revealed 8 patients in whom regions of T1 shortening were noted in the basal ganglia. We reviewed sequential images obtained in these selected patients with special attention to chronological changes in the foci of T1 shortening and their relationship to changes on T2-weighted images. RESULTS: Regions of short T1 in the globus pallidus were observed in 8 patients. In 2 of 3 patients in whom foci of T1 shortening were not identified on the initial imaging study, T1 shortening developed and T2 prolongation diminished after an initial increase. In the third patient, T1 and T2 prolongation appeared simultaneously. Sequential scans in the other 5 patients, in whom areas of increased signal intensity in the globus pallidus were present on both T1-weighted and T2-weighted images on the initial MR examination, showed a diminution in the size of the region of T2 prolongation in 2 patients, an increase in the size of the region of T2 prolongation in 1 patient, a mixed pattern of change in the size of the region of T2 prolongation in 1 patient, and no change in the region of T2 prolongation in 1 patient. During the periods of these T2 changes, the areas of T1 shortening showed no significant interval change. CONCLUSION: The foci of prolonged T2 relaxation in the basal ganglia appear to evolve in a manner similar to the foci of T2 prolongation in the white matter of the posterior fossa. However, the corresponding foci of short T1 in the basal ganglia may evolve with a different time course. In some patients, the foci of short T1 develop at a later time than the T2 prolongation and progress; these foci of short T1 do not appear to regress over periods as long as 90 months. Possible causes of the T1 shortening are remyelination and calcification.

Adolescent↗
PubMed2001

Basal ganglia metabolite abnormalities in minor motor disorders associated with human immunodeficiency virus type 1.

BACKGROUND: Minor motor disorders (MMDs) associated with human immunodeficiency virus type 1 (HIV-1) predict HIV-1 dementia and death. Little is known about the time course and neuropathologic mechanisms of HIV-1 MMDs. OBJECTIVE: To investigate the relationship between HIV-1 MMDs, as assessed by psychomotor speed, and metabolic alterations in the basal ganglia, as detected by proton magnetic resonance spectroscopy. PATIENTS AND METHODS: A total of 32 HIV-1-seropositive patients (10 with no MMD, 8 with incipient MMD, and 14 with sustained MMD, assessed through electrophysiologic testing of psychomotor speed including contraction times; 29 treated with highly active antiretroviral therapy) and 14 HIV-1-seronegative control subjects were examined for cerebral metabolite abnormalities in the basal ganglia by means of magnetic resonance spectroscopy. RESULTS: The 3 patient groups showed significantly different ratios of myoinositol/creatine (P =.02) in the basal ganglia. Whereas patients with no MMD or incipient MMD showed normal ratios, patients with sustained MMD showed higher values for myoinositol/creatine as a sign of glial proliferation. No differences in N-acetyl compounds, indicative of neuronal loss, were found. CONCLUSION: Whereas metabolic alterations in the basal ganglia were not detected in patients with incipient HIV-1 MMD, patients with sustained HIV-1 MMD did have significantly altered metabolic spectra indicative of glial proliferation.

AIDS Dementia Complex↗
PubMed2000

Preliminary findings of antistreptococcal antibody titers and basal ganglia volumes in tic, obsessive-compulsive, and attention deficit/hyperactivity disorders.

BACKGROUND: Previous studies have provided preliminary serological evidence supporting the theory that symptoms of tic disorders or obsessive-compulsive disorder (OCD) may be sequelae of prior streptococcal infection. It is unclear, however, whether previously reported associations with streptococcal infection were obscured by the presence of diagnostic comorbidities. It is also unknown whether streptococcal infection is associated in vivo with anatomical alterations of the brain structures that have been implicated in the pathophysiology of these disorders. METHODS: Antistreptococcal antibody titers were measured in 105 people diagnosed as having CTD, OCD, or attention-deficit/hyperactivity disorder (ADHD) and in 37 community controls without a disorder. Subjects were unselected with regard to their history of streptococcal exposure. Basal ganglia volumes were measured in 113 of these subjects (79 patients and 34 controls). RESULTS: A DSM-IV diagnosis of ADHD was associated significantly with titers of 2 distinct antistreptococcal antibodies, antistreptolysin O and anti-deoxyribonuclease B. These associations remained significant after controlling for the effects of CTD and OCD comorbidity. No significant association was seen between antibody titers and a diagnosis of either CTD or OCD. When basal ganglia volumes were included in these analyses, the relationships between antibody titers and basal ganglia volumes were significantly different in OCD and ADHD subjects compared with other diagnostic groups. Higher antibody titers in these subjects were associated with larger volumes of the putamen and globus pallidus nuclei. CONCLUSIONS: These findings suggest that the prior reports of an association between antistreptococcal antibodies and either CTD or OCD may have been confounded by the presence of ADHD. They also support the hypothesis that in susceptible persons who have ADHD or OCD, chronic or recurrent streptococcal infections are associated with structural alterations in basal ganglia nuclei.

Adult↗
PubMed1997

Spatial distribution of kainate receptor subunit mRNA in the mouse basal ganglia and ventral mesencephalon.

In an attempt to gain knowledge of the possible functions of kainate receptors, we have used in situ hybridization to examine the regional and cellular expression patterns of glutamate receptor subunits GluR5-7, KA1 and KA2 in the adult mouse basal ganglia, known to play a pivotal role in the translation of motivation into actions. Kainate receptor subunits were found to be differentially expressed in the circuitry forming the basal ganglia. They differ from each other in expression levels and their spatial localization. GluR6 appeared as the key subunit for the descending gamma-aminobutyric acid (GABA)ergic-glutamatergic pathways, with highest message levels in the caudate putamen, globus pallidus and subthalamic nucleus as well as in the nucleus accumbens and olfactory tubercle. GluR7 exhibited highest expression in the ascending nigrostriatal and mesolimbic dopaminergic neurons. GluR5 had a restricted distribution pattern, with high expression in the ventral pallidum, the islands of Calleja and pars compacta of the substantia nigra. KA2 was usually coexpressed with GluR6, although with a generally lower level of expression. Finally, KA1 mRNA was barely detectable in these neuronal circuits. These data suggest that kainate receptors in general may be involved in the functions associated with the basal ganglia, with a key role in the control of the central dopaminergic transmission. Thus, they might be implicated in the neurodegenerative and psychic disorders associated with an impairment of the basal ganglia.

Animals↗
PubMed1998

Characterisation of the adventitial rectal ganglia in the male rat by their immunohistochemical features and projections.

In recent years, considerable progress has been made in characterising the neural circuitry of the pelvic plexus, particularly in the male rat. However, the small ganglia on the adventitial surface of the rectum remain largely unstudied. We have used immunohistochemistry and retrograde tracing techniques to determine the content and projections of these neurons. The adventitial ganglia contain 600-1,000 neurons. All of these are immunoreactive for choline acetyltransferase, 44% are immunoreactive for calbindin, and 35% are immunoreactive for vasoactive intestinal peptide. Very few (1-5%) adventitial neurons contain tyrosine hydroxylase or neuropeptide Y. In contrast, most adventitial neurons are surrounded by varicose axons that do contain tyrosine hydroxylase or neuropeptide Y. Retrograde tracing studies showed that the primary targets of adventitial neurons within the bowel are the internal anal sphincter and the circular muscle directly adjacent to the sphincter. However, more adventitial neurons project out of the gut wall than to targets within the bowel. These are most likely to be viscerofugal and rectospinal neurons. Combining the immunohistochemical and tracing observations, these studies suggest that the rat adventitial ganglia do not represent an additional source of pelvic (autonomic postganglionic) neurons but, instead, that they are comprised primarily of viscerofugal and rectospinal neurons. This is very different from the adventitial rectal ganglia of the cat, which represent merely an extension of the pelvic plexus.

Animals↗
PubMed1999

Innervation of tracheal epithelium and smooth muscle by neurons in airway ganglia.

The neurochemical profiles of neurons in ferret tracheal ganglia has been characterized, but their projections to smooth muscle and epithelium in ferret trachea has not been examined. The purpose of this study is to determine the location of cell bodies that project VIP-, SP-, and NPY-containing fibers to the ferret tracheal smooth muscle and epithelium. Segments of ferret trachea were cultured for 0, 1, 3, or 7 days, some in the presence of 3 microm capsaicin. VIP, SP, or NPY nerve fiber density was measured using morphometric procedures. A retrograde tracer, rhodamine-labeled microspheres, identified neurons projecting to the epithelium. The density of SP fibers in the epithelium was reduced after culture, but VIP innervation was not different. In tracheal smooth muscle, the density of VIP- and SP-IR fibers was not different during the culture period, but NPY fiber density was reduced at all culture times. Capsaicin treatment did not affect nerve fiber density in the tracheal smooth muscle but produced a significant reduction in the density of epithelial VIP- and SP-IR nerve fibers after 1 day. Rhodamine-labeled microspheres were identified in VIP-containing nerve cell bodies of the ferret tracheal plexus. VIP innervation to the airway epithelium in ferret originates both from cell bodies in airway ganglia and cell bodies in sensory ganglia. The pathway from airway ganglia suggest the existence of a local reflex mechanisms initiated by epithelial irritation.

Animals↗
PubMed1998

Differential modulation of dopamine D1-receptor binding and mRNA expression in the basal ganglia by the D1-receptor antagonist, SCH-23390.

Dopamine D1-receptor binding in the basal ganglia is differentially regulated by subtype nonspecific dopamine antagonists such as the antipsychotic, Fluphenazine. The purpose of the present study was to determine the relative contributions of D1 and D2 receptor systems in the regulation of basal ganglia D1-receptor binding. Rats were injected twice daily for 21 days with saline, the D1-receptor antagonist, SCH-23390, the D2-receptor antagonist, Raclopride, or both SCH-23390 and Raclopride. Dopamine D1-receptor levels (as indicated by [125I]SCH-23982 binding) and mRNA expression were measured using receptor autoradiographic and in situ hybridization histochemical techniques. [125I]NCQ-298 binding to D2-receptors was also measured as a positive control for the effects of Raclopride. SCH-23390 administration independently increased [125I]SCH-23982 binding in a region-dependent manner with the greatest increases occurring in the entopeduncular nucleus. SCH-23390 also increased D1-receptor mRNA expression in specific striatal subregions suggesting that increases in binding were related to changes in receptor synthesis. In addition, Raclopride independently enhanced D2 binding with comparable increases observed in extrastriatal regions and increases of a lesser magnitude in the striatum. These data show that the modulation of basal ganglia D1-receptor binding observed in animals treated with nonselective antagonists is due primarily to the blockade of D1-receptors. The differential enhancement in basal ganglia D1 binding observed after D1-receptor blockade may be due to anatomical or phenotypic heterogeneity within the population of striatal D1-receptor synthesizing neurons. Similarly, the differential enhancement in striatal and extrastriatal D2-receptor binding may be due to differences in the regulation of striatal and extrastriatal D2-receptor synthesizing neurons.

Animals↗
PubMed2001

3',5'-cyclic adenosine monophosphate regulates expression of synaptotagmin in neonatal sympathetic ganglia in vitro.

The expression of the synaptic vesicle protein, synaptotagmin, in developing rat superior cervical ganglia is influenced by transsynaptic factors associated with membrane depolarization. The present study examines the role of cyclic AMP in the regulation of synaptotagmin in neonatal superior cervical ganglia maintained in explant culture. Ganglia were treated for 48 h in vitro with the Na+-channel ionophore, veratridine, or with pharmacological agents that alter cyclic AMP levels. Levels of cyclic AMP and synaptotagmin were determined by radioimmunoassay. Veratridine treatment significantly increased cyclic AMP in cultured ganglia, with a long time course, and also increased synaptotagmin levels. Drugs that elevate cyclic AMP levels significantly increased synaptotagmin levels, with similar magnitude to that produced by veratridine treatment. These pharmacological agents did not alter neuron survival or total ganglionic protein content. No additive effects were observed after combined treatment with veratridine and pharmacological agents that increased cyclic AMP. Agents that blocked adenylyl cyclase blocked the veratridine-induced increase in synaptotagmin levels. The results suggest that regulation of expression of synaptotagmin in neonatal sympathetic neurons is mediated partially by cyclic AMP.

8-Bromo Cyclic Adenosine Monophosphate↗
PubMed1999

Hemichorea and hemiballism associated with contralateral hemiparesis and ipsilateral basal ganglia lesions.

We report on two patients with unilateral hyperkinetic movement disorders associated with contralateral hemiparesis and ipsilateral basal ganglia lesions. The first patient, a 47-year-old woman, had a low-grade astrocytoma located in the right basal ganglia extending into the subthalamic area and the cerebral peduncle. She presented with left hemiparesis, right hemichorea, and intermittent right-sided tremor at rest. The second patient, a 85-year-old woman, had hypertensive hemorrhage to the right posterior basal ganglia, the posterior limb of the internal capsule, the lateral thalamus, and the subthalamic region with accompanying intraventricular bleeding. She developed right-sided transient hemichorea-hemiballism. A videotape illustration of one of the patients is provided. The literature on the rare occurrence of ipsilateral hemichorea-hemiballism is discussed and possible pathomechanisms are reviewed. We postulate that hemiparesis contralateral to basal ganglia lesions might have a conditioning effect on the appearance of ipsilateral dyskinetic movement disorders.

Aged↗
PubMed1984

Afferent and efferent relationships of the basal ganglia.

A survey of the known circuitry of the basal ganglia leads to the following conclusions. (1) No complete account can yet be given of the neural pathways by which the basal ganglia affect the bulbospinal motor apparatus. Channels of exit from the basal ganglia originate from the internal pallidal segment, the pars reticulata of the substantia nigra, and the subthalamic nucleus, and each of these is directed in part rostrally to the cerebral cortex by way of the thalamus, in part caudally to the midbrain. The postsynaptic extension of the mesencephalic channels to bulbar and spinal motor neurons is largely unknown. Since the ascending channels are collectively of greatest volume, the notion remains plausible that the basal ganglia act in considerable part by modulating motor mechanisms of the cortex. (2) Recent findings in the rat suggest that the striatum is subdivided into a ventromedial, limbic system-afferented region and a dorsolateral, 'non-limbic' region largely corresponding to the main distribution of corticostriatal fibres from the motor cortex. These two subdivisions appear to give rise to different striatofugal lines, the outflow from the limbic-afferented sector partly re-entering the circuitry of the limbic system. (3) The limbic-afferented striatal sector suggests itself as an interface between the motivational and the more strictly motor aspects of movement. This suggestion is strengthened by evidence that the 'limbic striatum' seems enabled by its striatonigral efferents to modulate not only the source of its own dopamine innervation but also that of a large additional striatal region.

Animals↗
PubMed1984

Functional organization of the basal ganglia: contributions of single-cell recording studies.

Studies of single-cell discharge in the basal ganglia of behaving primates have revealed: characteristic patterns of spontaneous discharge in the striatum, external (GPe) and internal (GPi) globus pallidus, pars reticulata and pars compacta of the substantia nigra, and the subthalamic nucleus (STN); phasic changes in neural discharge in relation to movements of specific body parts (e.g. leg, arm, neck, face); short-latency (sensory) neural responses to passive joint rotation; a somatotopic organization of movement-related neurons in GPe, GPi, and STN; a clustering of functionally similar neurons in the putamen and globus pallidus; greater representation of the proximal than of the distal portion of the limb; changes in neural activity in reaction-time tasks, suggesting a greater role of the basal ganglia in the execution than in the initiation of movement in this paradigm; a clear relation of neuronal activity to direction, amplitude (?velocity) of movement, and force; a preferential relation of neural activity to the direction of movement, rather than to the pattern of muscular activity. Some of these findings suggest that the basal ganglia may play a role in the control of movement parameters rather than (or independent of) the pattern of muscular activity. Loss of basal ganglia output related to amplitude may account for the bradykinesia in Parkinson's disease. The presence of somatotopic organization in the putamen and globus pallidus, together with known topographic striopallidal connections, suggests that segregated, parallel cortico-subcortical loops subserve 'motor' and 'complex' functions.

Animals↗
PubMed2004

Basal ganglia activity remains elevated after movement in focal hand dystonia.

Although previous studies of focal hand dystonia have detected cortical sensorimotor abnormalities, little is known about the role of the basal ganglia in this disorder. We report here that when focal hand dystonic patients performed finger-tapping tasks, functional magnetic resonance imaging showed persisting elevations of basal ganglia activity after the tasks ended. We posit that inhibitory control of the basal ganglia may be faulty in focal hand dystonia, and that the increases we observe in "resting" activity may mask basal ganglia abnormalities in standard imaging contrast analyses.

Adult↗
PubMed2003

Variations in the number and position of human lumbar sympathetic ganglia and rami communicantes.

The purpose of this study is to provide precise anatomical and statistical information about the number and location of lumbar sympathetic ganglia and the number and length of the related rami communicantes, and to consider the neurological pathway for nociception from the low back. Three-hundred ninety-three ganglia and 719 rami communicantes from 50 human cadavers were identified by gross dissection. The number of ganglia in a single lumbar chain ranged from 2 to 6, the mean being 3.9. The mean lengths of rami connected to the 1st and 5th lumbar spinal nerves, respectively, were significantly longer and shorter than those connected to the 2nd, 3rd, and 4th nerves. The lumbar sympathetic ganglia and rami communicantes were not distributed segmentally. The present results may assist in understanding the nociceptive pathway from the low back.

Aged↗
PubMed2005

Evidence for "direct" and "indirect" pathways through the song system basal ganglia.

Song learning in oscine birds relies on a circuit known as the "anterior forebrain pathway," which includes a specialized region of the avian basal ganglia. This region, area X, is embedded within a telencephalic structure considered homologous to the striatum, the input structure of the mammalian basal ganglia. Area X has many features in common with the mammalian striatum, yet has distinctive traits, including largely aspiny projection neurons that directly innervate the thalamus and a cell type that physiologically resembles neurons recorded in the mammalian globus pallidus. We have proposed that area X is a mixture of striatum and globus pallidus and has the same functional organization as circuits in the mammalian basal ganglia. Using electrophysiological and anatomical approaches, we found that area X contains a functional analog of the "direct" striatopallidothalamic pathway of mammals: axons of the striatal spiny neurons make close contacts on the somata and dendrites of pallidal cells. A subset of pallidal neurons project directly to the thalamus. Surprisingly, we found evidence that many pallidal cells may not project to the thalamus, but rather participate in a functional analog of the mammalian "indirect" pathway, which may oppose the effects of the direct pathway. Our results deepen our understanding of how information flows through area X and provide more support for the notion that song learning in oscines employs physiological mechanisms similar to basal ganglia-dependent forms of motor learning in mammals.

Animals↗
PubMed2006

Expression of protein kinase C-substrate mRNAs in the basal ganglia of adult and infant macaque monkeys.

We performed in situ hybridization histochemistry on the monkey basal ganglia to investigate the mRNA localization of three protein kinase C substrates (GAP-43, MARCKS, and neurogranin), of which expression plays a role in structural changes in neurites and synapses. Weak hybridization signals for GAP-43 mRNA and intense signals for both MARCKS and neurogranin mRNAs were observed in the adult neostriatum. All three of the mRNAs were expressed in both substance P-positive direct pathway neurons and enkephalin-positive indirect pathway neurons. In the nucleus accumbens, the hybridization signals for the three mRNAs were weaker than those in the neostriatum. Double-label in situ hybridization histochemistry in the neostriatum revealed that GAP-43 and neurogranin mRNAs were expressed in a subset of MARCKS-positive neurons. While intense hybridization signals for MARCKS mRNA were observed in all of the other basal ganglia regions such as the globus pallidus, substantia innominata, subthalamic nucleus, and substantia nigra, intense signals for GAP-43 mRNA were restricted to the substantia innominata and substantia nigra pars compacta. No signal for neurogranin mRNA was observed in the basal ganglia regions outside the neostriatum and the nucleus accumbens. These results indicate that the protein kinase C substrates are abundant in some specific connections in cortico-basal ganglia circuits. Developmental analysis showed that the expression level in the putamen and nucleus accumbens, but not in the caudate nucleus, was higher in the infant than in the adult, suggesting that synaptic maturation in the caudate nucleus occurs earlier than that in the putamen and nucleus accumbens.

Age Factors↗
PubMed1984

A sympathetic projection from sacral paravertebral ganglia to the pelvic nerve and to postganglionic nerves on the surface of the urinary bladder and large intestine of the cat.

Anatomical and electrophysiological experiments have demonstrated a prominent projection from the sacral sympathetic chain via the pelvic nerve to postganglionic nerves on the surface of the urinary bladder and the large intestine of the cat. Retrograde labeling studies revealed that the pelvic nerve, which is generally believed to carry primarily parasympathetic axons, has a considerable population of sympathetic fibers originating mainly from the S1-S3 paravertebral ganglia. The number of sympathetic neurons projecting to the pelvic nerve (2,100) was about 75% of that projecting to the pudendal nerve (2,900), a somatic nerve which would be expected to carry a large sympathetic fiber constituent. Sympathetic neurons projecting to the pudendal nerve were located primarily in the L6-S2 ganglia. Electrophysiological studies confirmed the presence of a sympathetic pathway from the paravertebral ganglia to the pelvic viscera. Electrical stimulation (thresholds 1.5-3 V) of the lumbar sympathetic chain evoked firing in the pelvic nerve and in postganglionic nerves on the surface of the colon and bladder at latencies of 60-150 msec. The responses were unaffected by cutting the chain one segment rostral to the site of stimulation, but were abolished by the administration of a ganglionic-blocking agent (tetraethylammonium). The responses on the colon and bladder postganglionic nerves were also abolished by transection of the pelvic nerve. The conduction velocity in the sympathetic postganglionic axons was approximately 1 m/second. In summary, these studies indicate that the pelvic nerve, like somatic nerves, receives a prominent projection from the sympathetic chain ganglia. The function of this sympathetic paravertebral pathway and its relationship with prevertebral innervation of the pelvic organs remains to be established.

Adrenergic Fibers↗