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At least 19 recordsLinked to original sources
PubMed1979

Basal ganglia calcification in Down's syndrome.

Basal ganglia calcification has been observed in autopsy material of 7% of children suffering from Down's Syndrome. The incidence of this finding in the general neurological population is approx. 0.3%. The present report is the first case of Down's Syndrome with basal ganglia calcification diagnosed in life by computed axial tomography. This case illustrates that dense basal ganglia calcification can exist with no signs or symptoms of a movement disorder.

Adolescent↗
PubMed2025

Genetic background of neurological disorders with basal ganglia calcification.

BACKGROUND: Bilateral basal ganglia calcifications (BGCs), if severe, are known hallmarks for idiopathic BGC disease (IBGC), but if milder, are often considered radiological findings of unknown significance. In previous studies, only a minority of patients with BGC had monogenic forms of IBGC. METHODS: We studied consecutive patients from a tertiary neurology clinic with bilateral BGCs of variable severity, and their families. We analyzed known IBGC genes, and an extended panel of genes linked to monogenic stroke and metabolic conditions. Clinical, radiological, and genetic data were collected, including vascular risk factors, cerebrovascular events, imaging findings (total calcification score, white matter hyperintensities, ischemic/hemorrhagic lesions), and relevant family history. RESULTS: Twenty-four families with BGCs and neurological symptoms were analyzed. Disease-causing variants were identified in 14 families (58.3%). Eight patients had IBGC (variants in SLC20A2, PDGFB, MYORG), 4 had mitochondrial disease (MT-TL1), and 2 had monogenic vascular conditions (GAL, MAP3K6). Three variants were novel. BGC severity was highest in IBGC cases, while vascular and mitochondrial cases had milder calcifications. White matter hyperintensities were seen in 94.7% of cases and correlated highly with the total calcification score. Clinical vascular events had occurred in 41.7% cases. No monogenic cause was found in 10 patients, although many of these showed clinical or radiological features suggestive of monogenic disease. CONCLUSIONS: Bilateral BGCs can occur in many neurogenetic disorders apart from IBGCs, and a broader genetic search increases the diagnostic yield. Patients with BGCs frequently had clinical cerebrovascular events, which emphasizes the role of cerebrovascular pathology in BGCs.

Humans↗
PubMed1979

Calcification of the basal ganglia: computerized tomography and clinical correlation.

During a 1-year period, 4219 consecutive computerized tomograms (CT) were reviewed for basal ganglia calcification; 14 patients with such calcification were identified. Calcifications on CT scan were bilateral in 12 of these cases and unilateral in 2. All bilateral calcifications were symmetric. The globus pallidus was the site of calcification in 13 of the 14 patients. Bilateral dentate nucleus calcification was seen in one patient. Skull radiograms were normal in all but one. Patients had diverse symptoms that were often explained by other findings, suggesting that calcifications may be coincidental and that basal ganglia calcification may not be a nosologic entity. Disturbances of calcium metabolism were not found in these patients, minimizing the pathophysiologic significance of altered calcium metabolism and the need for extensive endocrinologic evaluation. The finding of basal ganglia calcification alone does not justify invasive diagnostic procedures. Extrapyramidal signs may be associated with basal ganglia calcification; parkinsonism associated with basal ganglia calcification differs from idiopathic parkinsonism in being resistant to levodopa therapy.

Adult↗
PubMed1979

Clinical correlations of CT scan-detected calcifications of the basal ganglia.

A review of CT scans of 7,081 patients demonstrated calcifications of the basal ganglia in 53. The calcifications were evident in the skull roentgenograms of only 4 patients out of 40 in whom both CT scans and plain roentgenograms were available, demonstrating the superior resolution of this new method. Seventy-five percent of the patients were older than 50 years of age. Of the younger patients, 5 had had prior cranial irradiation; 1 had received cranial irradiation and intrathecal methotrexate therapy for meningeal leukemia; and 2 others had deep-seated arteriovenous malformations. Serum concentrations of calcium and phosphorus were normal in all 46 patients in whom they were measured. We conclude that the detection of small calcifications of the basal ganglia in persons above 50 years of age is infrequently associated with either clinical signs of basal ganglia dysfunction or calcium and phosphorus abnormalities. Calcium deposition in these patients may be related to vascular changes associated with aging. In younger patients a specific pathogenetic factor or underlying process is infrequently found.

Adolescent↗
PubMed1977

The biochemistry of the basal ganglia and Parkinson's disease.

The metabolic pathways for five transmitters in the basal ganglia are briefly described; the results of determinations of their concentrations, of their rate-limiting enzymes and of their degradation products are summarized. The changes found in Parkinson's disease are described. While dopamine synthesis in the basal ganglia is defective in this condition, abnormalities of other transmitters occur, and their possible significance is discussed.

Acetylcholine↗
PubMed1979

[Electron microscopic and histochemical study of the localization of adenylate cylase and acetylcholinesterase in the synapses of the rat cerebral cortex and basal ganglia].

Synapses of the brain cortex and basal ganglia of the rat were studied by means of electron histochemical reactions to adenylate cyclase and acetylcholinesterase. Three types of synapses, viz. cholinergic, adrenergic and mixed, were identified. Typification was carried out on the basis of presynaptic terminal vesicle characteristics and localization of the mentioned enzymes of the synapse receptor area.

Acetylcholinesterase↗
PubMed1978

[Radiological diagnosis and differential diagnosis of calcifications of the basal ganglia (author's transl)].

The radiological appearance and main features of calcifications of the basal ganglia in metabolic, toxic and hypoxemic disorders, degenerative and systemic diseases, tumors as well as infectious and parasitic lesions are described and demonstrated by typical radiographs. The roentgenological differential diagnosis and etiological classification are debated on occurence, incidence, localization, and topography with regard to individual structures of the basal ganglia, distribution pattern and according to the dimension, shape, margin and density pattern of these calcifications.

Basal Ganglia↗
PubMed1979

Disturbance of delayed match-to-sample in macaques by tetanization of anterior commissure versus limbic system or basal ganglia.

Three pig-tailed macaques were trained to select ("match") from a pair of colored images that which they had seen ("sample") and responded to 5--15 s previously. The anterior commissure (AC) and/or its radiation, various loci in basal ganglia, hippocampal formation and "control" areas, (splenium of corpus callosum, precentral gyrus, insular cortex), totalling 40 loci, were each tetanized for 4 s during presentation of the "sample" image, during the delay period, or when the monkey was required to select the "matching" image. For several loci in the hippocampal formation tetanization at any phase of the task reduced "matching" to chance levels and gave evidence of electrical after-discharge; but other comparable hippocampal loci had little or no effect. Response to "sample" or "match" stimuli were absent during tetanization of basal ganglia or anterior commissure. When finally made, upon cessation of tetanization, responses were equally correct for basal ganglia and "control" sites, but for AC were at chance levels.

Animals↗
PubMed1975

Single unit activity in basal ganglia of monkeys during performance of a delayed response task.

Single unit activity was recorded from the basal ganglia (caudate, putamen and globus pallidus) of monkeys during the performance of a delayed-response task. The task was divided into five epochs: stimulus onset, delay, pre-response, post-response and reqard. A high percentage of units recorded from the basal ganglia were found to show significant changes in activity during one or more epochs. Examination of the proportion of units excited or inhibited during a particular epoch indicated that brief increases or decreases in unit firing rates occurred "in phase" in both pallidum and caudate. Longer lasting firing rate changes, however, tended to occur in opposite directions in these two structures. This latter finding is interpreted as representing the consequence of persistent increases or decreases in activity of inhibitory interneurons in the caudate nucleus.

Animals↗
PubMedDate not supplied

Large basal ganglia cyst in site of previously radiated glioma. Case report.

A 3 1/2-year-old boy developed a mass lesion in the right basal ganglia and midbrain, compatible with glioma. During radiation therapy, aqueduct obstruction developed, necessitating a ventriculoatrial (VA) shunt. The child improved and remained well for 6 years, when he developed recurrent symptoms. A computerized tomographic (CT) scan and ventriculogram revealed a large cyst arising from the region of the right basal ganglia, the site of the previous tumor. The VA shunt was converted to a cyst atrial shunt. Subsequently, the cyst decreased in size, but hydrocephalus recurred, as demonstrated by a second CT scan. A Y-tube shunt (one catheter in the cyst, one in the ventricle) has controlled symptoms and signs since that time.

Basal Ganglia↗
PubMed1978

Intracerebral displacement of basal ganglia following septal cannulation.

The effects of septal cannulation on the displacement of basal ganglia form the basis of the present communication. In experimental rats, in which a cannula is chronically implanted for 30 days between the medial and lateral septal nuclei by stereotaxic procedures, considerable shift of the basal ganglion is always observed on the operated side. The displacement takes place along the long axis of the ganglion either rostrally or caudally. Besides gross asymmetry in their relative sizes, no histological differences are observed between the ganglia of the two sides. Control animals, in which the cannula is withdrawn immediately after the septal lesion, manifest similar, but less pronounced movements of the basal ganglia. The probable underlying mechanisms for such intracerebral shifts are discussed and the role of indirect mechanical forces is outlined. The extraordinary degree of plasticity of the subcortical nuclear masses in terms of mobility is also emphasized.

Animals↗
PubMed1978

Cortex, basal ganglia and cerebellum in motor control.

For voluntary movement, function generators are necessary that are located in the brain-stem, cerebellum and basal ganglia. Following lesions of these generators, voluntary movements are impaired while stimulus-dependent movements are still possible. This discussion also applies to speech production. The motor function generators cooperate with the whole cerebral cortex since both tactical adaptation to the environment and strategic guidance by the motivation system contribute to voluntary action. The motor cortex plays an epicritical role, adding advanced tactile and proprioceptive guidance for those movements that need this kind of regulation, especially the fine finger movements which depend entirely on the motor cortex. The complexity of the cerebral potentials preceding voluntary movement corresponds to the activity of several motor subsystems acting in concert.

Animals↗
PubMed1979

Incidence of basal ganglia calcifications on computed tomography.

In a group of 3,800 computed tomography examinations, 14 patients (0.4%) were found to have high attenuating lesions suggesting calcifications within the basal ganglia. The lesions were bilateral in all patients. A majority of the patients had psychic symptoms and convulsions. Electroencephalographic changes were frequent and extrapyramidal symptoms occurred. Until now only three patients have been endocrinologically examined. One of them had hypoparathyroidism. Since parathyroid dysfunction frequently is associated with calcifications of the basal ganglia, these patients should be referred for endocrinological evaluation.

Adult↗
PubMed1978

[Effect of neuroleptics on acetylcholine metabolism in the basal ganglia of the brain].

Neuroleptics (haloperidol) closapine, pimozid, chlorpromazine) diminished the level of free (functionally active) form of acetylcholine (ACh), and, to some extent, the bound form of ACh; they changed the content of the labile-bound (vesicular) form of ACh and weakly influenced the choline-acetyltranspherase activity in the basal ganglia of the rat brain 5 to 30 min after the injection. In contrast to the inhibitory action on the acetyl-cholinesterase (AChE) activity in vitro, most of the neuroleptics, except closapine, increased the AChE activity in vivo. These results indicate that the neuroleptics activate ACh-metabolism and probably stimulate the cholinergic structure in the basal ganglia of the brain; the AChE activity may serve as a criterion of such stimulating action of neuroleptics.

Acetylcholine↗
PubMedDate not supplied

[Treatment of hemihyperkinesis by stereotaxic surgery on the basal ganglia].

Hemihyperkinesia develops in childhood as a rule as a consequence of meningoencephalitis. The authors operated on 60 patients, performing 76 stereotaxic interventions on the basal ganglia of the brain. It is established that these operations are the only effective method of treatment of the disease. A marked therapeutic effect was noted in over 70 percent of patients, which persists for many years. There were neither fatal outcomes nor stable complications. The best results were produced in one-stage stereotaxic cryodestruction of VL and subthalamic area.

Adolescent↗
PubMed1979

Lactic acidemia, mitochondrial myopathy, and basal ganglia calcification.

A 27-year-old man had limb weakness, short stature, neurosensory hearing loss, seizures, mild peripheral neuropathy, neurogenic bladder, elevated cerebrospinal fluid (CSF) protein content, primary lactic acidemia, and basal ganglia calcification. Muscle biopsy revealed numerous ragged-red fibers. On electronmicroscopy there were mitochondrial alterations, including many intramitochondrial inclusions. The mother of the patient had Vogt-Koyanagi-Harada syndrome, a combination of rare disorders in the same family that has not been previously described.

Adult↗
PubMed1975

Unusual roentgenological findings in cytomegalic inclusion body disease: large and circumscribed calcareous deposits of the basal ganglia and scattered calcifications of the parieto-occipital cortex.

The roentgenological and clinical findings of a five year old girl suffering from cytomegalic inclusion body disease are reported. In this disease unusual circumscript and large calcareous deposits of the basal ganglia and scattered gyriform calcifications of the occipito-parietal cortex were shown on plain skull x-rays and their paraventricular localization demonstrated on pneumoencephalotomogram.

Basal Ganglia↗
PubMed1979

Basal ganglia calcification in Kearns-Sayre syndrome.

The Kearns-Sayre syndrome (KSS) appears to be a distinctive disorder characterized by progressive external ophthalmoplegia, pigmentary degeneration of the retina, heart block, and elevated CSF protein levels. Recent reports have suggested that abnormalities of muscle mitochondria may also be a consistent finding in KSS. We recently examined a patient with KSS whose skeletal muscle contained abnormal mitochondria. In addition, a computerized tomographic scan of the head showed cerebellar and brain stem atrophy, as well as calcification in the region of the basal ganglia.

Basal Ganglia↗