[Systems of fibers in the basal ganglia].
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Basal Ganglia↗
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[Positron emission tomography and basal ganglia functions].
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Animals↗
[Functions of the basal ganglia].
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Animals↗
[Anatomy and physiopathology of the basal ganglia].
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Basal Ganglia↗
The isomers of thioctic acid alter C-deoxyglucose incorporation in rat basal ganglia.
Nigral cell death in Parkinson's disease is associated with decreased reduced glutathione (GSH) levels, impaired complex I activity and inhibition of alpha-ketoglutarate dehydrogenase (alpha-KGDH) in substantia nigra. Thioctic acid exerts antioxidant activity through a thiol-disulphide redox couple and is an essential cofactor for alpha-KGDH. However, it is not known whether or not thioctic acid enters basal ganglia or exerts beneficial effects in Parkinson's disease. As a global measure of altered cerebral function, the effect of R- and S-thioctic acid on 14C-2-deoxyglucose (14C-2DG) incorporation was investigated in rats. Rats were treated with either R- or S-thioctic acid (50 mg/kg IP) or 0.9% saline acutely or for 5 days and 14C-2DG incorporation in basal ganglia was assessed. Following acute administration, R- but not S-thioctic acid caused an overall increase in 14C-2DG incorporation that was significant in both substantia nigra zona compacta and zona reticulata. R-thioctic acid also increased the incorporation of 14C-2DG in the medial forebrain bundle, thalamus, and red nucleus. S-thioctic acid decreased 14C-2DG incorporation in the subthalamic nucleus, but increased it in the red nucleus. Following repeated administration, R-thioctic acid no longer increased 14C-2DG incorporation in either zona compacta or zona reticulata of substantia nigra. However, both R- and S-thioctic acid now decreased 14C-2DG incorporation in the subthalamic nucleus. The data suggest that thioctic acid does enter the brain can alter neuronal activity in areas of the basal ganglia intimately associated with the motor deficits exhibited in Parkinson's disease.
Animals↗
Germinoma arising in the basal ganglia in early stage: CT and MR findings.
We report a patient with a germinoma originating in the left basal ganglia. Unenhanced computed tomography (CT) revealed a relatively well-defined high-density lesion, which was slightly enhanced with contrast medium. No tumor stain was noted on angiography. The mass showed hypointensity on T1-weighted magnetic resonance imaging (MRI), and heterogeneous hyperintensity on T2-weighted imaging. The lesion was homogeneously enhanced after the administration of Gd-DTPA. To our knowledge, the MR findings of basal ganglia germinoma have been retrospectively reviewed in just four reported cases, only two of which were examined with Gd-DTPA enhancement. The two cases revealed relatively large masses with cysts and peritumoral edema, and were slightly enhanced after the intravenous injection of Gd-DTPA. Our case was thought to be in the early stage, and its CT and MR findings were slightly different from those of previously reported cases.
Basal Ganglia Diseases↗
[Myelodysplastic syndrome with cyclosporine A-induced encephalopathy presenting with a mass effect on the right basal ganglia after cord blood transplantation].
An 18-year-old female with myelodysplastic syndrome underwent an allogeneic cord blood transplantation in May 2005. The conditioning regimen consisted of total body irradiation, cytarabine and cyclophosphamide. The day of the cord stem cell transfusion was counted as Day 0. For acute GVHD prophylaxis, cyclosporine A (CsA) and methotrexate were used. Engraftment was achieved on Day 30, acute GVHD grade II developed on Day 45 and treatment with methylprednisolone for acute GVHD was started. On Day 68 the patient had generalized seizures accompanied by loss of consciousness, hypertension and left hemiparesis, and was intubated. A cranial CT scan showed a mass effect on the right basal ganglia, and high signal intensities on the T2-weighted and FLAIR images of a MR examination were detected in the bilateral basal ganglia and posterior lobes, the findings of which were compatible with a brain tumor or infectious disease. Since an increased level of apparent diffusion coefficient (ADC) values on the bilateral basal ganglia was noted, we suspected that vasogenic edema had caused the mass effect. She went into remission immediately after CsA treatment was discontinued. Therefore, this case was diagnosed as atypical reversible posterior leukoencephalopathy syndrome (RPLS) associated with CsA. CsA-induced encephalopathy presenting a mass effect in clinical imaging findings is very rare, and an ADC map may be useful for the diagnosis of RPLS.
Acute Disease↗
Endoscopic surgery for spontaneous basal ganglia hemorrhage: comparing endoscopic surgery, stereotactic aspiration, and craniotomy in noncomatose patients.
BACKGROUND: This prospective study aimed to evaluate the safety, neurological outcomes, and cost-effectiveness of 3 surgical procedures for spontaneous basal ganglia hemorrhage. METHODS: Ninety noncomatose patients with basal ganglia hemorrhages were randomized into 3 groups. Group A (n = 30) underwent endoscopic surgery, group B (n = 30) underwent stereotactic aspiration, and group C (n = 30) underwent craniotomy. Waiting time of surgery, length of operation time, and blood loss were compared between all groups. On the second operative day, we evaluated the amount of residual hematoma and the hematoma evacuation rate by computed tomography scan. Surgical mortality and complications were recorded 3 months after the procedure. Neurological outcomes were evaluated by functional independence measure (FIM) score, Barthel index score, and muscle power (MP) of affected limbs 6 months after surgery. We also evaluated the cost-effectiveness of each procedure. RESULTS: There was significant delay in waiting timing of the stereotactic aspiration (172.56 +/- 93.18 minutes; P < .001). Craniotomy had the longest operation time (229.96 +/- 50.57 minutes; P < .001). Blood loss was most significant in the craniotomy (236.13 +/- 137.45 mL; P < .001). The highest hematoma evacuation rate was seen in the endoscopic surgery (87% +/- 8%; P < .01). The mortality rate was 0% in group A, 6.7% in group B, and 13.3% in group C (P = .21). The complication rate was 3.3% in group A, 10% in group B, and 16.6% in group C (P = .62). The most major complications were rebleeding and infection. The FIM score was higher in the endoscopic surgery (79.90 +/- 36.64) than in the craniotomy (33.84 +/- 18.99; P = .001). The Barthel index score was also significantly better in the endoscopic surgery (50.45 +/- 28.59) than in the craniotomy (16.39 +/- 20.93; P = .006). There was more improvement in MP of affected limbs in endoscopic surgery than in craniotomy (P = .004). Endoscopic surgery was more cost-effective than craniotomy using FIM and Barthel index (P < .02 and P < .05, respectively). CONCLUSIONS: Both endoscopic surgery and stereotactic aspiration are minimally invasive and are effective procedures with low complication and mortality rates; however, the waiting timing of stereotactic aspiration is usually longer. Endoscopic surgery may be an appropriate substitute for stereotactic aspiration. It produces good neurological outcomes and aids in rapid hematoma evacuation. Craniotomy may be used for emergency decompression of enlarged hematoma if endoscopic surgery or stereotactic aspiration is not available.
Adult↗
Functional anatomy of the basal ganglia in X-linked recessive dystonia-parkinsonism.
Dystonia is a neurological syndrome characterized by sustained muscle contractions that produce repetitive twisting movements or abnormal postures. X-linked recessive dystonia parkinsonism (XDP; DYT3; Lubag) is an adult-onset disorder that manifests severe and progressive dystonia with a high frequency of generalization. In search for the anatomical basis for dystonia, we performed postmortem analyses of the functional anatomy of the basal ganglia based on the striatal compartments (ie, the striosomes and the matrix compartment) in XDP. Here, we provide anatomopathological evidence that, in the XDP neostriatum, the matrix compartment is relatively spared in a unique fashion, whereas the striosomes are severely depleted. We also document that there is a differential loss of striatal neuron subclasses in XDP. In view of the three-pathway basal ganglia model, we postulate that the disproportionate involvement of neostriatal compartments and their efferent projections may underlie the manifestation of dystonia in patients with XDP. This study is the first to our knowledge to show specific basal ganglia pathology that could explain the genesis of dystonia in human heredodegenerative movement disorders, suggesting that dystonia may result from an imbalance in the activity between the striosomal and matrix-based pathways.
Adult↗
Acute alcohol withdrawal is associated with c-Fos expression in the basal ganglia and associated circuitry: C57BL/6J and DBA/2J inbred mouse strain analyses.
BACKGROUND: The DBA/2J (D2) and C57BL/6J (B6) mouse strains are the most widely studied genetic models of severe and mild acute alcohol withdrawal, respectively. Previous studies have identified quantitative trait loci and genes involved in risk for acute ethanol withdrawal using mapping populations derived from the D2 and B6 strains, but the brain region(s) and circuit(s) by which these genes and their protein products influence ethanol physiological dependence and associated withdrawal remain to be elucidated. METHODS: B6 and D2 were administered a sedative-hypnotic dose of ethanol (4 g/kg) or saline (control) and returned to their home cages where they were left undisturbed for 7 hr, which has been shown in previous studies to correspond to peak acute ethanol withdrawal severity. The mice were then euthanized and assessed for their numbers of c-Fos immunoreactive neurons across 26 brain regions. The question addressed was whether or not ethanol-withdrawn D2 and B6 mice differed in c-Fos induction (neural activation) within circuitry that could explain the severe ethanol withdrawal of the D2 strain and the mild ethanol withdrawal in B6 strain mice. RESULTS: At peak acute ethanol-withdrawal ethanol-withdrawn D2 and B6 mice differed in neural activation within the basal ganglia, including the subthalamic nucleus and the two major output nuclei of the basal ganglia (the medial globus pallidus and the substantia nigra pars reticulata). Genotype-dependent c-Fos induction was also apparent in associated circuitry including the lateral septum, the ventral tegmental area, the nucleus accumbens core, the dorsolateral caudate putamen, the substantia nigra pars compacta, the cingulate and entorhinal cortices, and the ventral pallidum. D2 and B6 mice showed comparable neural activation in the bed nucleus of the stria terminalis, and the nucleus accumbens shell. CONCLUSIONS: The present studies are the first to use immediate early gene product expression to assess the pattern of neural activation associated with acute ethanol withdrawal. Our results point to the involvement of an extended basal ganglia circuit in genetically determined differences in acute ethanol withdrawal. Based on these data, we suggest that quantitative trait genes (QTGs) involved in acute ethanol withdrawal exert their effects on this phenotype via one or more of the brain regions and circuits identified. As more information becomes available that integrates neural circuit and QTG analyses, the precise mechanisms by which QTGs affect ethanol physiological dependence and associated withdrawal will become apparent.
Animals↗
Type 1 GM1 gangliosidosis with basal ganglia calcification: a case report.
This report concerns a 10-month-old boy, admitted to the Veterans General Hospital-Kaohsiung with generalized tonic convulsion and aspiration pneumonia. He was found to have had developmental regression, progressive hypotonia and hepatosplenomegaly since four months of age. Physical examination revealed a large head circumference (97th percentile), frontal bossing, depressed nasal bridge, hepatosplenomegaly, broad hands and short fingers. Neurologic examination showed poor control of eye movement, profound hypotonia, muscle weakness, brisk deep tendon reflexes and Babinski's sign. Hypoplasia of the vertebral bodies with anterior beaking, wedge-shaped metacarpals, spatulated ribs and a J-shaped sella turcica were displayed on bone radiographs. Cranial computerized tomography scans showed diffuse brain atrophy, dilated ventricles and calcification of the bilateral basal ganglia. Vacuolated lymphocytes were noted in a peripheral blood smear. Type 1 GM1 gangliosidosis was diagnosed based on a deficiency of beta-galactosidase activity. To our knowledge, basal ganglia calcification in type 1 GM1 gangliosidosis has never been reported in the literature. We suggest that type 1 GM1 gangliosidosis be considered in the differential diagnosis of patients with an early onset of neurologic decline, organomegaly and basal ganglia calcification.
Basal Ganglia Diseases↗
Evidence for the importance of basal ganglia output nuclei in semantic event sequencing: an fMRI study.
Semantic event sequencing is the ability to plan ahead and order meaningful events chronologically. To investigate the neural systems supporting this ability, an fMRI picture sequencing task was developed. Participants sequenced a series of four pictures presented in random order based on the temporal relationship among them. A control object discrimination task was designed to be comparable to the sequencing task regarding semantic, visuospatial, and motor processing requirements but without sequencing demands. fMRI revealed significant activation in the dorsolateral prefrontal cortex and globus pallidus internal part in the picture sequencing task compared with the control task. The findings suggest that circuits involving the frontal lobe and basal ganglia output nuclei are important for picture sequencing and more generally for the sequential ordering of events. This is consistent with the idea that the basal ganglia output nuclei are critical not only for motor but also for high-level cognitive function, including behaviors involving meaningful information. We suggest that the interaction between the frontal lobes and basal ganglia output nuclei in semantic event sequencing can be generalized to include the sequential ordering of behaviors in which the selective updating of neural representations is the key computation.
Basal Ganglia↗
Frequency of linear hyperechogenicity over the basal ganglia in young infants with congenital rubella syndrome.
Five young infants with congenital rubella syndrome (CRS) underwent cranial ultrasonographic examinations and/or computed tomographic examinations. Only two of these infants were small for their gestational age, and none of them were microcephalic at birth. Deafness and ocular lesions were found in four patients, and congenital heart disease was found in three. All had abnormal ultrasonographic findings: linear-shaped hyperechogenicity over the basal ganglia was noted for five patients, periventricular punctate hyperechogenicity was noted for three, and subependymal cysts were observed in two. Follow-up ultrasonograms for two of the patients showed progressively enlarging hyperechogenic lesions. Calcification was found in both patients examined by means of computed tomography. All patients became microcephalic, with profound global developmental delay. Intracranial calcifications are common findings in patients with CRS. Ultrasonography should be performed for high-risk neonates, regardless of their symptoms. The finding of linear hyperechogenicity over the basal ganglia should prompt a search for all congenital infections, including CRS.
Basal Ganglia↗
Effect of electrical stimulation of the cerebral cortex on the expression of the Fos protein in the basal ganglia.
The protein Fos is a transcription factor which is quickly induced in response to a variety of extracellular signals. Since this protein is expressed in a variety of neuronal systems in response to activation of synaptic afferents, it has been suggested that it might contribute to activity-dependent plasticity in neural networks. The present study investigated the effect of cortical electrical stimulation on the expression of Fos in the basal ganglia in the rat, a group of structures that participate in sensorimotor learning. Results show that the repetitive application of electrical shocks in restricted areas of the cerebral cortex induces an expression of Fos mostly confined to the striatum and the subthalamic nucleus. The induction which can be elicited from different cortical areas (sensorimotor, auditory and limbic areas) does not require particular temporal patterns of stimulation but rather depends on the total number of shocks delivered during a given period of time. Moreover, it appears to be rather independent of the number of spikes discharged by the activated cells. In the striatum, the distribution of immunoreactive neurons is precisely delineated and conforms to the known topographical organization of stimulated corticostriatal projections. As demonstrated using a variety of double labelling techniques (combination of the immunocytochemical detection of Fos with the autoradiography of mu opioid receptors, calbindin immunocytochemistry, in situ hybridization of preproenkephalin and preprotachykinin A messenger RNAs), striatal neurons which express Fos are mostly localized in the matrix compartment and concern equally enkephaline and substance P containing efferent neurons. In the subthalamic nucleus, Fos expression evoked by cortical stimulation is also confined to discrete regions of the nucleus, the localizations corresponding to the primary projection site of the stimulated cortical cells. These results indicate that in addition to its phasic synaptic influence on the basal ganglia, the cerebral cortex could exert a long-term effect on the functional state of this system via a genomic control. Since the basal ganglia are involved in sensorimotor learning and motor habit formation, it is tempting to speculate that the activity-dependent Fos induction at corticostriatal and subthalamic synapses may contribute to consolidate the functionality of the neuronal networks activated during the completion of given motor tasks.
Action Potentials↗
Regression of HIV encephalopathy and basal ganglia signal intensity abnormality at MR imaging in patients with AIDS after the initiation of protease inhibitor therapy.
PURPOSE: To determine whether protease inhibitors cause regression of periventricular white matter signal intensity abnormalities in patients with human immunodeficiency virus (HIV) encephalopathy and whether the changes on magnetic resonance (MR) images correlate with cognitive improvement. MATERIALS AND METHODS: MR images were retrospectively and prospectively analyzed in 16 adult patients with HIV encephalopathy. White matter and basal ganglia signal intensity abnormalities on initial long repetition time (TR) images were compared with those on subsequent long TR images in patients who received and in patients who did not receive protease inhibitors. Clinical correlation was obtained. RESULTS: Of the nine patients receiving protease inhibitors, four showed nearly complete regression, four showed interval stability, and one showed slight progression. Thus, eight patients (89%) demonstrated either stability or improvement in white matter disease, which correlated with cognitive improvement. Of the seven patients not receiving protease inhibitors, six (86%) showed marked progression with a decline in cognitive function and one had no interval change. The difference between the two groups was statistically significant. Of the two patients receiving protease inhibitors who initially had basal ganglia signal intensity abnormalities, one demonstrated resolution and one nearly complete resolution subsequently. CONCLUSION: Although the patient population is small, protease inhibitors may cause regression of periventricular white matter and basal ganglia signal intensity abnormalities in HIV encephalopathy and may have a role in treatment.
AIDS Dementia Complex↗
The nucleus accumbens as part of a basal ganglia action selection circuit.
BACKGROUND: The nucleus accumbens is the ventral extent of the striatum, the main input nucleus of the basal ganglia. Recent hypotheses propose that the accumbens and its dopamine projection from the midbrain contribute to appetitive behaviors required to obtain reward. However, the specific nature of this contribution is unclear. In contrast, significant advances have been made in understanding the role of the dorsal striatum in action selection and decision making. OBJECTIVE: In order to develop a hypothesis of the role of nucleus accumbens dopamine in action selection, the physiology and behavioral pharmacology of the nucleus accumbens are compared to those of the dorsal striatum. HYPOTHESES: Three hypotheses concerning the role of dopamine in these structures are proposed: (1) that dopamine release in the dorsal striatum serves to facilitate the ability to respond appropriately to temporally predictable stimuli (that is, stimuli that are so predictable that animals engage in anticipatory behavior just prior to the stimulus); (2) that dopamine in the nucleus accumbens facilitates the ability to respond to temporally unpredictable stimuli (which require interruption of ongoing behavior); and (3) that accumbens neurons participate in action selection in response to such stimuli by virtue of their direct (monosynaptic inhibitory) and indirect (polysynaptic excitatory) projections to basal ganglia output nuclei.
Animals↗
Anatomical aspects of information processing in primate basal ganglia.
Recent studies with double-anterograde tract-tracing methods have shed new light on the organization of the basal ganglia circuitry in primates. This paper briefly reviews some of these findings and provides a personal interpretation of their possible functional significance. Emphasis is placed on the fact that the striatum and the subthalamic nucleus have multiple representations in the two major output structures of the basal ganglia, namely the internal segment of the globus pallidus and the substantia nigra pars reticulata. It is hypothesized that this multiple representation serves as a means of amplifying and diversifying the striatal and subthalamic influences upon thalamocortical neurons that is mediated through the globus pallidus and the substantia nigra. Furthermore, evidence for the highly ordered organization of the striatopallidal and subthalamopallidal projections, which converge onto single pallidal neurons according to different but highly specific patterns, is taken as an indication that the subthalamic nucleus uniformly excites a vast collection of pallidal neurons, whereas the striatum exerts a more specific inhibitory control upon selected subsets of subthalamically driven pallidal neurons.
Animals↗
Dopamine metabolism increases in post-mortem schizophrenic basal ganglia.
The dopamine-rich regions of post-mortem brains from 6 schizophrenics and 7 controls were analyzed. There were no significant changes in dopamine concentrations in basal ganglia and nucleus accumbens of schizophrenics compared with controls. Schizophrenic basal ganglia (putamen and caudate) showed significantly higher levels of homovanillic acid, and tyrosine hydroxylase activity. Among the schizophrenic patients, markedly high activity of tyrosine hydroxylase was measured in a patient diagnosed as catatonic type. He had not taken antipsychotic drugs for 3 months prior to death. In his relatives, three other schizophrenics were found to the second degree. A remarkable low level of dopamine and a high level of homovanillic acid measured indicate this case would have had an increased turnover rate of dopamine in the dopaminergic nerve terminals. Among the schizophrenic patients, there might be one group whose enzyme activity of dopamine synthesis in the brain is exceptionally high.
Adult↗