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Linear areas of echogenicity in the thalami and basal ganglia of neonates: an expanded association. Work in progress.

A retrospective review of 2,320 neonatal cranial sonograms obtained in 1,324 patients identified 25 patients with areas of echogenicity in their thalami and basal ganglia that were of a linear or branching linear distribution. Four of these patients had cytomegalovirus infection. Other major diagnoses encountered included Down syndrome, trisomy 13 syndrome, neonatal asphyxia, non-immune hydrops, and fetal alcohol syndrome. Results of the study suggest a broader etiologic basis for linear areas of echogenicity in the basal ganglia and thalami of children than has previously been reported. These patients warrant complete screening for possible in utero infection and perhaps also chromosomal analysis.

Basal Ganglia↗

Drugs of abuse modulate the phosphorylation of ARPP-21, a cyclic AMP-regulated phosphoprotein enriched in the basal ganglia.

ARPP-21 is a cyclic AMP-regulated phosphoprotein of M(r) 21 kDa that is enriched in the cell bodies and terminals of medium-sized spiny neurons in the basal ganglia. Using a new phosphorylation state-specific antibody selective for the detection of ARPP-21 phosphorylated on Ser(55), we have demonstrated that activation of dopamine D1 receptors increased the level of ARPP-21 phosphorylation in mouse striatal slices. Conversely, activation of D2 receptors caused a large decrease in ARPP-21 phosphorylation. Treatment of mice with either methamphetamine or cocaine resulted in increased ARPP-21 phosphorylation in vivo. Studies using specific inhibitors of protein phosphatases and experiments in mice bearing a targeted deletion of the gene for DARPP-32, a dopamine-activated inhibitor of protein phosphatase-1, indicated that protein phosphatase-2A is primarily responsible for dephosphorylation of ARPP-21 in mouse striatum. These results demonstrate that phosphorylation and dephosphorylation of ARPP-21 are tightly regulated in the striatum. We speculate that ARPP-21 might mediate some of the physiologic effects of dopamine and certain drugs of abuse in the basal ganglia.

Animals↗

Basal ganglia glucose utilization after recent precentral ablation in the monkey.

In the macaque monkey, unilateral ablation of areas 4 and 6 of Brodmann results initially in a significant decrease of glucose metabolic activity in the ipsilateral caudate nucleus, putamen, globus pallidus, substantia nigra, and subthalamic nucleus. The contralateral hemisphere shows nonsignificant but consistently decreased activity in the caudate nucleus, putamen, and globus pallidus. Cerebral blood flow is decreased in the same pattern as the glucose metabolic activity. The change in glucose metabolic activity results from loss of neurons known to project directly from the cerebral cortex to the basal ganglia and also from indirect effects (diaschisis) in basal ganglia structures that do not receive connections from the cerebral cortex.

Animals↗

Adaptive autoregressive identification with spectral power decomposition for studying movement-related activity in scalp EEG signals and basal ganglia local field potentials.

We propose a method that combines adaptive autoregressive (AAR) identification and spectral power decomposition for the study of movement-related spectral changes in scalp EEG signals and basal ganglia local field potentials (LFPs). This approach introduces the concept of movement-related poles, allowing one to study not only the classical event-related desynchronizations (ERD) and synchronizations (ERS), which correspond to modulations of power, but also event-related modulations of frequency. We applied the method to analyze movement-related EEG signals and LFPs contemporarily recorded from the sensorimotor cortex, the globus pallidus internus (GPi) and the subthalamic nucleus (STN) in a patient with Parkinson's disease who underwent stereotactic neurosurgery for the implant of deep brain stimulation (DBS) electrodes. In the AAR identification we compared the whale and the exponential forgetting factors, showing that the whale forgetting provides a better disturbance rejection and it is therefore more suitable to investigate movement-related brain activity. Movement-related power modulations were consistent with previous studies. In addition, movement-related frequency modulations were observed from both scalp EEG signals and basal ganglia LFPs. The method therefore represents an effective approach to the study of movement-related brain activity.

Algorithms↗

[A case of chronic toluene intoxication with abnormal MRI findings: abnormal intensity areas in cerebral white matter, basal ganglia, internal capsule, brain stem and middle cerebellar peduncle].

We reported a case of 21 years old man of chronic toluene intoxication with abnormal intensity areas on MRI in cerebral white matter, basal ganglia, internal capsule (especially posterior limb), brain stem and middle cerebellar peduncle. The patient developed various neurological abnormalities such as blurred vision, ataxic speech, gaze evoked horizontal nystagmus, bilateral pyramidal tract sign and limb ataxia after 8 years sniffing of thinner (mainly toluene). MRI examination revealed diffuse high intensity areas in cerebral white matter on T1 weighted image. On T2 weighted image, high intensity areas of deep cerebral white matter, internal capsule (especially posterior limb), cerebral peduncle, ventral pons and middle cerebellar peduncle were noted. Basal ganglia (caudate nucleus, lenticular nucleus and thalamus) were displayed as low intensity area on T2 weighted image. These high intensity areas of internal capsule, brain stem and middle cerebellar peduncle on T2 weighted image would be significant for understanding pyramidal tract sign and cerebellar sign of this case. On the basis of neuropathological descriptions of chronic toluene intoxication, these high intensity areas of T2 weighted image were presumed to be demyelinating lesions of the central nervous system.

Adult↗

Chronic continuous and intermittent L-3,4-dihydroxyphenylalanine treatments differentially affect basal ganglia function in 6-hydroxydopamine lesioned rats--an autoradiographic study using [3H]flunitrazepam.

The effects of chronic 'continuous' and 'intermittent' L-3,4-dihydroxyphenylalanine treatments on GABA receptor function in the basal ganglia of rats with unilateral 6-hydroxydopamine lesions of the medial forebrain bundle was investigated, by autoradiography with [3H]flunitrazepam. The 6-hydroxydopamine lesion itself, increased [3H]flunitrazepam binding in the substantia nigra pars reticulata (+17%, with respect to intact side) and entopeduncular nucleus (+44%), but decreased binding in the globus pallidus of the denervated hemisphere (-20%). 'Intermittent' L-3,4-dihydroxyphenylalanine treatment reduced the [3H]flunitrazepam binding changes observed in the substantia nigra pars reticulata (-13%) and entopeduncular nucleus (-4%), whereas 'continuous' infusion of the same daily dose of L-3,4-dihydroxyphenylalanine had less effect (+14%, substantia nigra pars reticulata; +26%, entopeduncular nucleus). In contrast, the [3H]flunitrazepam binding decrease in the globus pallidus of the 6-hydroxydopamine lesioned animals was unaffected by either regime of chronic L-3,4-dihydroxyphenylalanine treatment. The changes in GABA receptor function implied by these results provide further insight into the pathophysiological effects of L-3,4-dihydroxyphenylalanine treatment on basal ganglia function, following dopamine denervation. In accordance with existing electrophysiological and biochemical evidence on this subject, the main implications of these results include reduced GABA sensitivity of neurons in the entopeduncular nucleus and substantia nigra pars reticulata, following chronic 'intermittent', but not chronic 'continuous' L-3,4-dihydroxyphenylalanine treatment; this may be due to a reversal of the 6-hydroxydopamine induced decrease in the GABA-mediated neurotransmission in the striatoentopeduncular and striatonigral pathways. In contrast, the regulation of GABA receptors in the globus pallidus does not appear to be subject to modulation by chronic L-3,4-dihydroxyphenylalanine administration, suggesting that dopamine replacement in this manner does not modify the 6-hydroxydopamine induced increase in GABA-mediated neurotransmission in the stratopallidal pathway.

Animals↗

Spatiotemporal expression gradients of the carbohydrate antigen (CD15) (Lewis X) during development of the human basal ganglia.

The developmental expression pattern of the carbohydrate epitope CD15 (Lewis X, Le X) (alpha1-->3-fucosyl-N-acetyl-lactosamine) has been immunocytochemically evaluated in paraffin sections within the human basal ganglia from 10 weeks gestation to three years after birth. At 11 weeks of gestation, CD15 (Le X) positive radial glial cells were located in the anterior and dorsal parts of the lateral ganglionic eminence. Their processes ran from the subventricular zone radially in a highly ordered fashion to the dorsolateral margin of the caudate nucleus and further to the lateral rim of the putamen. At 12 weeks of gestation, strands of CD15 (Le X) material continued to the pial surface, forming a continuous CD15 (Le X) positive borderline separating the accumbens nucleus and olfactory tubercle from the piriform cortex. At 13 weeks of gestation the dorsal putamen was completely CD15 (Le X) immunoreactive along its perimeter and CD15 (Le X) patches, consisting of fine granular material, appeared at the dorsolateral margin of the putamen at this age; while the first CD15 (Le X) patches in the caudate nucleus were observed four weeks later. The matrix compartment of the caudate and dorsal putamen became gradually stained by granular CD15 (Le X) positive material into which CD15 (Le X) immunoreactive somata were embedded. The striking contrast in staining between patch and matrix compartments disappeared shortly after birth. The ventral striatum did not become immunoreactive until the last few weeks before birth. After the formation of CD15 (Le X) positive patches in the striatum (from 12 weeks of gestation), delicate CD15 (Le X) fibres, often accumulated in bundles and related to the striatal patches, became apparent coursing towards the external pallidal lamina and the globus pallidus. Immunoreactivity in the globus pallidus itself was transient, emerging from 16 weeks of gestation, reaching a peak at 21 weeks of gestation and disappearing by birth. Both processes, i.e. the occurrence of CD15 (Le X) striatopallidal fibres and the emerging immunoreactivity in their pallidal target, may be interrelated, so that ingrowing CD15 (Le X) positive axons from the striatum provoke CD15 (Le X) expression in the external and internal pallidum. The variable patterns and intensities of CD15 (Le X) expression are possibly related to periods of maturation of the striatum and the establishment of functional interactions within the basal ganglia. Differential staining of patch and matrix in the developing neostriatum suggests that a distinct phase of cellular adhesion or dishesion mediated by the CD15 (Le X) epitope occurs during establishment of the patch and matrix regions.

Aging↗

Distinct basal ganglia territories are engaged in early and advanced motor sequence learning.

In this study, we used functional MRI (fMRI) at high field (3T) to track the time course of activation in the entire basal ganglia circuitry, as well as other motor-related structures, during the explicit learning of a sequence of finger movements over a month of training. Fourteen right-handed healthy volunteers had to practice 15 min daily a sequence of eight moves using the left hand. MRI sessions were performed on days 1, 14 and 28. In both putamen, activation decreased with practice in rostrodorsal (associative) regions. In contrast, there was a significant signal increase in more caudoventral (sensorimotor) regions of the putamen. Subsequent correlation analyses between signal variations and behavioral variables showed that the error rate (movement accuracy) was positively correlated with signal changes in areas activated during early learning, whereas reaction time (movement speed) was negatively correlated with signal changes in areas activated during advanced learning stages, including the sensorimotor putamen and globus pallidus. These results suggest the possibility that motor representations shift from the associative to the sensorimotor territories of the striato-pallidal complex during the explicit learning of motor sequences, suggesting that motor skills are stored in the sensorimotor territory of the basal ganglia that supports a speedy performance.

Adult↗

Is Broca's area part of a basal ganglia thalamocortical circuit?

The cortex constituting Broca's area does not exist in isolation. Rather, like other cortical regions, Broca's area is connected to other brain structures, which likely play closely related functional roles. This paper focuses on the basal ganglia, a set of subcortical structures that project through topographically organized "channels" via the thalamus to different frontal regions. It is hypothesized that the basal ganglia project to Broca's area. This circuitry is further posited to encompass at least two channels. One channel can be characterized as subserving procedural memory, while the other underlies the retrieval of knowledge from declarative memory. These hypotheses are supported by both anatomical and functional evidence. Implications and issues for further investigation are discussed.

Basal Ganglia↗

Differential distribution in rat basal ganglia of Met-enkephalin- and Met-enkephalin Arg6 Phe7-like peptides revealed by immunohistochemistry.

Antisera to Met-enkephalin and its heptapeptide variant Met-enkephalin Arg6Phe7 were used in immunohistochemistry to study distribution of immunoreactive material in rat basal ganglia. In the pallidum, the Met-enkephalin antiserum, but not the Met-enkephalin Arg6Phe7 antiserum, revealed abundant nerve terminals, whereas in the caudate-putamen the heptapeptide antiserum demonstrated numerous immunoreactive cell bodies that were not shown by the Met-enkephalin antiserum. The differential distribution of immunoreactivity is compatible with distinct physiological roles for the two peptides in basal ganglia.

Animals↗

The basal ganglia cholinergic neurochemistry of progressive supranuclear palsy and other neurodegenerative diseases.

BACKGROUND: Progressive supranuclear palsy (PSP) is a progressive neurodegenerative disorder involving motor and cognitive dysfunction. Currently, there is no effective treatment either for symptomatic relief or disease modification. This relates, in part, to a lack of knowledge of the underlying neurochemical abnormalities, including cholinergic receptor status in the basal ganglia. AIM: To measure muscarinic M2 and M4 receptors in the basal ganglia in PSP. METHODS: The muscarinic M2 (presynaptic) and M4 (postsynaptic) receptors in the striatum, pallidum and adjacent insular cortex were autoradiographically measured in pathologically confirmed cases of PSP (n = 18), and compared with cases of Lewy body dementias (LBDs; n = 45), Alzheimer's disease (AD; n = 39) and controls (n = 50). RESULTS: In cases of PSP, there was a reduction in M2 and M4 receptors in the posterior caudate and putamen compared to controls, but no significant changes in the pallidum. Cases with AD showed lower M2 receptors in the posterior striatum. Groups with LBD and AD showed higher M2 binding in the insular cortex compared with controls. CONCLUSIONS: The results suggest loss of posterior striatal cholinergic interneurones in PSP, and reduction in medium spiny projection neurones bearing M4 receptors. These results should be taken in the context of more widespread pathology in PSP, but may have implications for future trials of cholinergic treatments.

Aged↗

Pericentric inversion of chromosome 2 and echogenic vasculature in the basal ganglia: a new finding?

Linear branching echogenicities in the thalamus or basal ganglia have been reported in infants with several genetic and nongenetic disorders. In this article, we report 2 cases of newborns with a neurosonographic diagnosis of thalamic/basal ganglia vasculopathy and karyotype analysis showing pericentric inversion of chromosome 2. To our knowledge, there has been no previous mention of an association between these entities.

Basal Ganglia↗

Calcification of the basal ganglia as visualized by computed tomography.

Physiological calcification of the globus pallidus was visualized by computed tomography in 32 patients. The frequency of visualization increased with increasing age. Patients under the age of 40 with calcification of the globus pallidus should be evaluated for disorders associated with pathological calcification of the basal ganglia. Patients of any age with calcification in the lenticular nucleus and elsewhere in the basal ganglia, dentate nucleus, or multiple areas of the cortex should also be evaluated for these disorders.

Adolescent↗

High levels of dopamine activity in the basal ganglia of cigarette smokers.

OBJECTIVE: The authors' goal was to study presynaptic dopamine activity in smoking and nonsmoking human subjects in vivo. METHOD: [(18)F]Fluorodopa ([(18)F]DOPA) uptake K(i) values in the basal ganglia of nine smoking and 10 nonsmoking healthy men were measured with positron emission tomography. RESULTS: Significantly higher [(18)F]DOPA uptake was observed in both the putamen (average 17.3% higher) and the caudate (average 30.4% higher) of smokers than in those of nonsmokers. CONCLUSIONS: Smoking is related to greater dopamine activity in the human basal ganglia. Nicotine-induced dopamine activity may be a relevant mechanism in dependence on cigarette smoking.

Adult↗

[Calcified cysticerci in basal ganglia and Parkinson's syndrome: report of a case].

It is reported the case of a 57 years-old man from Maranhão State who presented Parkinson's syndrome. Images proper to calcified cysticerci were found in the basal ganglia at the CT-scan examination. Considerations are made as to: the low frequency of cysticercosis in the northern region of the country where the patient lives; the association of symptomatic Parkinson's syndrome and cysticercosis of the central nervous system, which is rare; the demonstration of calcified cysticerci in basal ganglia in this situation, which was not registered up to the present time.

Basal Ganglia Diseases↗

Stereotactic interstitial radiosurgery with a miniature X-ray device in the minimally invasive treatment of selected tumors in the thalamus and the basal Ganglia.

The aim of this study was to evaluate the role of interstitial radiosurgery (IR) using the photon radiosurgery system (PRS) in the treatment of selected tumors within the thalamus and the basal ganglia. The PRS is a miniature X-ray generator that was developed for interstitial irradiation. This series included 14 patients (5 with glioblastomas, 4 with low-grade astrocytomas and 5 with metastases) harboring spheroidal lesions with dimensions ranging from 13 to 42 mm (mean 30 mm). After stereotactic biopsy, a radiation dose ranging from 6 to 15.4 Gy (mean 11.3 Gy) was delivered at the target volume margins. Follow-up varied from 3 to 26 months (mean 10.2 months). In the group of glioblastomas, 3 patients died (3-12 months after the procedure) because of tumor progression, while the remaining had tumor control. Two patients with metastases died from systemic disease (4-9 months after the treatment), and 3 were alive and well at the end of the study. Local control was achieved in all metastases. Patients with low-grade astrocytomas were well and imaging studies showed tumor control PRS IR is a minimally invasive procedure for the treatment of selected glial or secondary brain tumors. Compared to conventional radiosurgery (brachytherapy and external radiosurgery), PRS IR presents dose delivery characteristics useful for the treatment of tumors in the thalamus and basal ganglia, without inconveniences such as handling radioisotopes, the need of expensive facilities and radiation protection measures. Although the clinical value needs further investigations, PRS IR seems to be effective in metastases while it provides less benefit in malignant gliomas. PRS IR could have a major role in the treatment of low-grade astrocytomas.

Adolescent↗

Hyperintense basal ganglia on T1-weighted magnetic resonance images in a patient with common variable immunodeficiency associated with elevated serum manganese.

The authors report on a 32-year-old man with common variable immunodeficiency and high signal intensity in basal ganglia on T1-weighted images. No signal alteration on T2-weighted and postcontrast images was observed. The patient had elevated levels of manganese in the serum. The authors conclude that the unusual hyperintensity in the basal ganglia area on T1-weighted images resulted from manganese deposition due to liver dysfunction.

Adult↗

Specificity of the memory deficits associated with basal ganglia dysfunction.

Recent studies comparing the explicit and implicit memory disorders of Huntington's disease (HD) and Alzheimer's disease (AD) patients have yielded new insights into the specificity of the memory disorders associated with basal ganglia dysfunction. Patients with HD have severe deficiencies in retrieving information from either episodic or semantic explicit memory. This retrieval deficit is not confounded by impairments in storage, rapid forgetting, intrusion errors and a deterioration of the structure of semantic knowledge. While HD patients perform normally on implicit memory tasks involving lexical, semantic and pictorial priming, they are impaired on implicit tests which require the initiation and/or modification of central motor programs. It is concluded that the basal ganglia play a specific but important role in memory although the processes they mediate are different from those dependent on the integrity of the hippocampus and diencephalon.

Basal Ganglia Diseases↗