Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “BASAL GANGLIA”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 919 records · Page 51Linked to original sources

Focal dystonia secondary to cavernous angioma of the basal ganglia: case report and review of the literature.

The case of a young woman with focal dystonia of the hand due to a cavernous angioma of the basal ganglia is presented. The lesion involved the anterior third of the lentiform nucleus and a large portion of white matter anterior to this nucleus and lateral to the head of the caudate, as shown by magnetic resonance imaging; it was completely removed through a computed tomography-assisted stereotactic craniotomy by microsurgical technique, resulting in the cure of the patient. These facts support the pathophysiological hypothesis of a disruption of the striatopallidothalamic projection to the premotor cortex as the cause of symptomatic dystonia. A review of the reported cases of cavernous angiomas of the deep cerebral gray nuclei shows that this is the first case of cavernous angioma associated with movement disorder.

Adult↗

The syndrome of acute bilateral basal ganglia lesions in diabetic uremic patients.

Acute extrapyramidal movement disorders have rarely been reported in uremic patients. We had previously presented three cases of acute movement disorders with bilateral basal ganglia lesions in uremia, and had proposed that it is not a rare condition. The objective of this study is to establish a more accurate clinical profile of this rarely described clinical syndrome, and to call attention to its common occurrence. We prospectively studied six patients we encountered from March 1996 to June 2001. We also reviewed the clinical records of a large population of uremic patients and identified six more cases. The clinical manifestations, laboratory findings, neuroimages, and clinical outcomes of these 12 patients were analyzed. When possible, each patient was followed up to the present time. Twelve patients had acute onset of movement disorders and bilateral basal ganglia lesions. All of the patients were diabetic. They had acute-onset Parkinsonism or dyskinesias, together with various symptoms such as consciousness disturbance, dysarthria, dysphagia, or ataxia. The main laboratory test results of abnormalities consisted of elevated blood urea nitrogen, creatinine, and metabolic acidosis. They had uniform neuroimaging findings of symmetrical bilateral basal ganglion changes. These changes regressed or disappeared during follow-up. The clinical prognoses were diverse. We believe that this group of patients represents a well-demarcated clinical syndrome, which is not uncommon but has previously been rarely addressed. The underlying mechanism of such lesions may be associated with metabolic, as well as vascular factors.

Basal Ganglia Diseases↗

Basal ganglia haematomas in non-comatose patients: subacute stereotactic aspiration improves long-term outcome in comparison to purely medical treatment.

This study examined whether subacute stereotactic evacuation of basal ganglia haematomas in primarily non-comatose patients is suitable to improve the ultimate outcome of this subgroup of stroke patients. Applying rigorous selection criteria, 56 consecutive non-comatose patients with ganglionic haematomas were treated stereotactically, and 1-year outcomes employing four outcome parameters commonly used to assess outcome were compared with those of 39 similar patients who were treated purely medically. No survival benefit was found in long-term follow-up for either surgical or conservative treatment (total mortality 16.1% vs 28.2%; P=0.121). Among survivors, however, outcome was significantly better in surgical patients. Compared with medical patients, the median Glasgow Outcome Scale score was 1 point higher (P<0.0001) in surgical patients, and the median European Stroke Scale score improvement from baseline to 1-year score was significantly better (P<0.0001). Accordingly, the median Barthel Index score was significantly higher (P=0.002), and the median Modified Rankin Scale score was 1 point lower (P<0.0001). We conclude that primarily non-comatose patients with basal ganglia haematomas can ultimately profit from this form of minimally invasive treatment.

Activities of Daily Living↗

Regional distribution of cholecystokinin binding sites in macaque basal ganglia determined by in vitro receptor autoradiography.

Cholecystokinin binding sites were labeled with [3H]cholecystokinin-8, [125I]cholecystokinin-33, and [125I]cholecystokinin-8 in major structures of macaque basal ganglia by in vitro receptor autoradiography. Analysis of autoradiograms revealed areas of heavy cholecystokinin binding in the neostriatum and substantia nigra that were set off, often quite sharply, from the adjacent globus pallidus and subthalamic nucleus where labeling was, by contrast, very light. Heavy label characterized the ventromedial and posterior parts of the caudate nucleus and adjacent putamen, binding was of moderate intensity in central areas of these regions, while, the dorsolateral margin of the head of the caudate and precommissural putamen, the dorsolateral one-third of the body of the caudate, and all but the most medial and ventral portions of the posterior putamen lateral to the pallidum were sparsely labeled. The pattern of cholecystokinin binding within the neostriatum was mottled; patches of reduced label stood out from the background of more prominent binding. However, those patches were only imperfectly correlated with the striosomal organization of both the caudate nucleus and putamen as revealed by acetylcholinesterase staining. Cholecystokinin binding in the substantia nigra was also intricately patterned. Moderately dense, vertically orientated bands of label were found in the dorsal one-third to half of the pars reticulata, providing a marked contrast to the near background levels in the ventral pars reticulata and overlying pars compacta. The present study shows that heavy cholecystokinin binding is confined to particular areas within the primate basal ganglia; the pattern of label within the substantia nigra and neostriatum can be linked to intrinsic and afferent connections of these structures. The confinement of binding sites to the dorsal pars reticulata suggests an association with dendrites of pars compacta neurons which invade this region; this interpretation is consistent with recent evidence of depletion of nigral cholecystokinin binding sites in macaques following chemical lesion of dopaminergic cells of the par compacta. In the neostriatum the distribution of binding shows overlap with its topographically organized corticostriatal innervation; portions of heavily labeled striatum coincide with regions innervated by association cortex of the frontal and temporal lobes, whereas regions of diminished binding correspond to areas innervated mainly by sensory and motor cortex. These latter findings suggest that cholecystokinin may have a particularly strong influence on cognitive aspects of striatal function.

Animals↗

PET imaging of dopamine receptors in human basal ganglia: relevance to mental illness.

The development of PET and in vivo ligand-binding techniques over the past decade has allowed the analysis of dopamine receptor functions in the basal ganglia of human subjects. Using ligands selective for the different subtypes of dopamine receptors, their gross distribution, total number of binding sites and affinity have been determined in the caudate-putamen of the living human brain. Recent studies in young, drug-naive schizophrenic patients failed to demonstrate a consistent alteration in the densities or affinities of D2 dopamine receptors in the basal ganglia of these subjects, contradicting the view that elevated densities of D2 dopamine receptors are a major pathophysiological mechanism in this disorder. PET measurements of D2 dopamine receptor occupancy in relation to clinical antipsychotic drug treatment demonstrated that all chemically different categories of antipsychotic drugs induced a marked occupancy of D2 dopamine receptors. This effect was dose-dependent and fully reversible. It appeared earlier than the antipsychotic effect and was also present in neuroleptic-resistant patients. Resistance to neuroleptic drugs is in all probability related to heterogeneity of biological factors causing schizophrenia. Some, but not all, antipsychotic drugs also induced a significant D1 dopamine receptor occupancy. This effect was most marked for the unconventional drug clozapine, which showed about the same degree of D1 as D2 dopamine receptor blockade when given in clinical doses.

Basal Ganglia↗

Time-sequential change of amino acid neurotransmitters--GABA, aspartate and glutamate--in the rat basal ganglia following middle cerebral artery occlusion.

To clarify the involvement of neurotransmitters in the remote metabolic effect following focal ischaemia, amino acid neurotransmitter candidates--GABA, aspartate and glutamate--were measured time-sequentially in the basal ganglia following middle cerebral artery occlusion in the rat, which is known to produce a change in glucose metabolism and blood flow in the remote basal ganglia. GABA and aspartate content decreased in the ipsilateral substantia nigra from the second to the 28 th postoperative day. The same tendency was observed in the ipsilateral globus pallidus. Glutamate did not change significantly in either nucleus. In the contralateral side, GABA increased significantly in the substantia nigra throughout the experimental period and in the striatum during the first seven days. These results indicate that the destruction of strionigral tract by focal ischaemia induced the decrease of inhibitory GABA in the ipsilateral substantia nigra. The increase of metabolism in this nucleus may be caused by the decrease of the inhibitory effect.

Animals↗

Beta-adrenergic receptor subtypes in the basal ganglia of patients with Huntington's chorea and Parkinson's disease.

The density of [125I]iodo-cyanopindolol binding to beta-1 and beta-2 adrenergic receptors was studied in post mortem basal ganglia samples of Huntington's chorea and Parkinson's disease patients using autoradiography. Whereas no significant changes were observed in sections from Parkinson's and Huntington's chorea grade 2 patients, a nearly complete loss of beta-1 binding sites was observed in the basal ganglia of Huntington patients at later stages of the disease. The concentration of beta-2 receptors was increased by a factor 2 in the posterior putamen of all choreic cases. These results are consistent with the view that beta-1 receptors are predominantly located on a subpopulation of neurons which degenerate at late stages of Huntington's chorea, while beta-2 receptors are present mainly on glial elements.

Aged↗

Hypothesis III. Tourette syndrome is only one of several causes of a developmental basal ganglia syndrome.

Tourette syndrome (TS) is a hereditary condition characterized by the presence of chronic, multiple motor tics and is commonly associated with obessive-compulsive behavior, attentional and executive dysfunction, and aggressive behavior. A wide range of other genetic and environmental conditions that interfere with normal basal ganglia developmental processes can produce a clinical syndrome that we call the developmental basal ganglia syndrome (DBGS), and which closely resembles TS. Failure to distinguish TS from other causes of DBGS has probably hampered research efforts aimed at clarifying the neurobiological mechanisms, localizing the genetic defect, assessing therapy, and establishing accurate prevalence rates for TS.

Aggression↗

Role of the basal ganglia in a chemically induced dyskinesia in rat.

A complex syndrome consisting of locomotor hyperactivity and a cervical choreoathetoid dyskinesia was produced in rats after 7 to 10 days administration of 3-3 -iminodipropionitrile. Attenuation of motor abnormalities after lesions of the basal ganglia (BG) but not cortical lesions suggests the importance of descending BG influences in this syndrome. Responses of this syndrome to pharmacologic manipulations further argues for BG involvement. This syndrome may be a possible animal model of human hyperkinetic basal ganglia disorders.

Animals↗

Doppler sonographic confirmation of thalamic and basal ganglia vasculopathy in three infants with trisomy 13.

A prospective review of 1746 head sonographic examinations performed from July 1993 to December 1995 revealed three scans that demonstrated a linear, branching echogenic pattern within the thalamus or basal ganglia. All three of these patients had trisomy 13. This finding has been described previously and is presumed to be of vascular causation. Doppler evaluation of the thalamus and basal ganglia in our three cases confirmed the vascular nature of the echogenic, branching pattern.

Basal Ganglia↗

[Spontaneous regression of basal ganglia lesions in a case of neurofibromatosis type 1 (case report)].

Neurofibromatosis type 1 is characterized on magnetic resonance imaging by optic nerve gliomas, parenchymal gliomas, and foci of prolonged T2 relaxation involving the brainstem, cerebellum, midbrain, internal capsule, and basal ganglia. We report a child with neurofibromatosis type 1 in whom serial magnetic resonance imaging demonstrates spontaneous regression of basal ganglia lesions. These lesions, characterized on magnetic resonance imaging by increased signal intensity on T1- and T2-weighted sequences, involved the globus pallidus and internal capsules in a bilateral fashion.

Basal Ganglia Diseases↗

Action sites of rotation and unit firing induced by l-stepholidine and DA agonists in basal ganglia of 6-OHDA-lesioned rats.

AIM: To elucidate the action sites of l-stepholidine (SPD) in the basal ganglia. METHODS: Counting the rotations after intra-nucleus microinjection and recording the neuron firing by microiontophoresis of SPD and DA agonists in the basal ganglia of 6-hydroxydopamine (6-OHDA)-lesioned rats. RESULTS: The DA immunoreactive substance was markedly reduced in the 6-OHDA-lesioned rats. The intra-neostriatum microinjection of apomorphine (Apo, D1/D2), SK&F 38393 (D1), and SPD elicited remarkable rotation, and the characteristics of SK&F 38393-produced rotation were of long latency and long duration. The intra-substantia nigra pars reticulata (SNR) injection of Apo, SK&F 38393, and SPD induced the rotation response, while the selective D2 agonist quinpirole hydrochloride (Ly171555) did not because of scarce D2 receptors in the SNR. The intraglobus pallidus (GP) injection of DA agonists and SPD failed to evoke rotation, but the GP nucleus still had the contribution to rotation elicited by i.p. injection of DA agonists and SPD in the 6-OHDA-lesioned rats with successive kainic acid (KA) lesion. Besides, the successive lesion of entopeduncular nucleus (EP) on rotation was less important than that of GP nucleus. The microiontophoresis of Apo and SPD into the SNR could evoke the neuron firing, but failed to activate the GP neurons, which were activated by sodium glutamate (Glu) and inhibited by gamma-aminobutyric acid (GABA). CONCLUSION: The action sites of SPD-induced rotation and neuron firing via the D1 receptors are in the neostriatum and SNR instead of GP. The direct neurocircuit through SNR is the most important for rotation of 6-OHDA-lesioned rats.

2,3,4,5-Tetrahydro-7,8-dihydroxy-1-phenyl-1H-3-ben↗

Purification and cDNA cloning of ARPP-16, a cAMP-regulated phosphoprotein enriched in basal ganglia, and of a related phosphoprotein, ARPP-19.

ARPP-16 (cAMP-regulated phosphoprotein of Mr = 16,000) is a substrate for cAMP-dependent protein kinase and is enriched in the basal ganglia. ARPP-16 has been purified to homogeneity from the soluble fraction of bovine caudate nuclei. An additional substrate for cAMP-dependent protein kinase of Mr = 19,000 (ARPP-19) was found to cross-react with rabbit anti-serum prepared against purified ARPP-16. Immunological analysis indicated that ARPP-16 was enriched in the basal ganglia while ARPP-19 was present in similar levels in all brain regions studied and was also present in non-neuronal tissues. ARPP-19 was also purified to homogeneity from bovine caudate nucleus cytosol. Using oligonucleotide probes based on the partial amino acid sequence of purified ARPP-16, cDNA clones for ARPP-16 and ARPP-19 were isolated from a bovine caudate nucleus cDNA library and sequenced. The predicted amino acid sequences of the two proteins were identical except that ARPP-19 had an additional 16 amino acids at the NH2-terminal. The two cDNA clones share an identical 3'-untranslated region of 756 nucleotides. The cDNA clone for ARPP-16 contained 806 additional nucleotides located 3' to this common sequence. The 5'-untranslated regions of the two clones were entirely different. The results suggest the possibility that ARPP-16 and ARPP-19 are produced by alternative transcription and splicing.

Amino Acid Sequence↗

The genetic causes of basal ganglia calcification, dementia, and bone cysts: DAP12 and TREM2.

BACKGROUND: Polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy (PLOSL), or Nasu-Hakola disease, is a presenile dementia associated with loss of myelin, basal ganglia calcification, and bone cysts. It is caused by recessively inherited mutations in two genes encoding subunits of a cell membrane-associated receptor complex: TREM2 and DAP12. The clinical course of PLOSL has not been characterized in a series of patients with TREM2 mutations. METHODS: The authors compare neurologic and neuroradiologic follow-up data of six patients carrying TREM2 mutations with PLOSL due to defective DAP12 genes. The authors review the known mutations in these two genes. RESULTS: Mutations in DAP12 and TREM2 result in a uniform disease phenotype. In Finnish and Japanese patients with PLOSL, DAP12 mutations predominate, whereas TREM2 is mutated more frequently elsewhere. CONCLUSIONS: Polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy should be considered in adult patients under age 50 years with dementia and basal ganglia calcification. Radiographs of ankles and wrists, and DNA test in uncertain cases, confirm the diagnosis.

Adaptor Proteins, Signal Transducing↗

Infantile spasms with basal ganglia MRI hypersignal may reveal mitochondrial disorder due to T8993G MT DNA mutation.

PURPOSE: To report three cases of infantile spasms (IS) with an abnormal magnetic resonance imaging signal in the basal ganglia (Leigh-like syndrome), due to T8993G mt DNA mutation. PATIENTS AND RESULTS: The first sign was, at the end of the first year of life, IS in one case and the combination of IS with behavior changes in the two other cases. Video EEG polygraphy demonstrated both spasms and hypsarrhythmia, but no other kind of seizures. Vigabatrin or steroids controlled the spasms with a follow-up of several years. All 3 patients had hyperlactatorrhachia (3.47 to 7 mmol/l). Axial hypotonia and dystonia appeared by the end of the first year of life. As in cases with the NARP mutation and onset later in life, neuropathy and retinopathy could also be demonstrated. DISCUSSION: Although it is well established that symptomatic IS with hypsarrhythmia mainly result from cortical lesions, this epileptic encephalopathy may also be generated by lesions in the basal ganglia without evidence of cortical damage. This finding suggests that West syndrome is likely to be caused by age-related dysfunction at any level of a cortico-putaminal loop of hyperexcitability.

Adenosine Triphosphatases↗

Gait function in newly diagnosed children with autism: Cerebellar and basal ganglia related motor disorder.

We investigated gait in newly diagnosed children with autism. From our previous study with 6- to 14-year-olds, we hypothesized that motor symptoms indicative of basal ganglia and cerebellar dysfunction would appear across the developmental trajectory of autism. Two groups were recruited: children with autism (eight males, three females; mean age 5 y 10 mo [SD 9 mo]; range 4 y 4 mo-6 y 9 mo) and a comparison group of typically developing children (eight males, three females; mean age 5 y 9 mo [SD 1 y 1 mo]; range 4 y 3 mo-7 y 2 mo). The GAITRite Walkway was used to gather data from average gait and intra-walk measurements. Experienced physiotherapists analyzed gait qualitatively. Groups were matched according to age, height, weight, and IQ; although not statistically significant, IQ was lower in the group with autism. Spatiotemporal gait data for children with autism were compatible with findings from patients with cerebellar ataxia: specifically, greater difficulty walking along a straight line, and the coexistence of variable stride length and duration. Children with autism were also less coordinated and rated as more variable and inconsistent (i.e. reduced smoothness) relative to the comparison group. Postural abnormalities in the head and trunk suggest additional involvement of the fronto-striatal basal ganglia region. Abnormal gait features are stable across key developmental periods and are, therefore, promising for use in clinical screening for autism.

Adolescent↗