Cases reported "Basal Ganglia Diseases"

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11/34. hyperglycemia-induced unilateral basal ganglion lesions with and without hemichorea. A PET study.

    hyperglycemia-induced unilateral basal ganglion lesions occur mostly in Asian patients. A signal abnormality in the basal ganglion region is evident on these patients' neuroimaging. Despite characteristic imaging findings and clinical manifestations, the underlying mechanism is still unclear. To clarify the underlying pathophysiology of unilateral basal ganglion lesions, we examined the [18F]-fluorodeoxyglucose (FDG) positron emission tomography (PET) findings in 3 patients with hyperglycemia. The PET studies were performed at 3 weeks, 5 weeks, and 7 months after clinical onset. The markedly reduced rates of cerebral glucose metabolism in the corresponding lesions on T1-weighted magnetic resonance images provided direct evidence of regional metabolic failure. We suggest that the metabolic derangements associated with hyperglycemia and vascular insufficiency contribute to regional metabolic failure in patients with poorly controlled diabetes mellitus.
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12/34. Parkinsonism caused by cavernoma located in basal ganglion.

    Deep-seated cavernoma or cavernous angioma is a very rare clinical entity, as is basal ganglia cavernoma presenting with Parkinsonism. The authors demonstrate a 56-year-old man with a cavernoma located in basal ganglion, who subsequently developed Parkinsonism. The patient refused the surgical intervention, and received L-dopa trial; however, no change in the tremor and bradykinesia was observed in spite of high doses of L-dopa. Our case indicates that chronic compression and continuous hemorrhage could cause Parkinsonism, which is uncommon symptom of cavernous angioma.
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13/34. Novel mitochondrial mutation in the ND4 gene associated with Leigh syndrome.

    We analyzed the complete mitochondrial genome of a 3-month-old female child with basal ganglionic lesions and other clinical features suggestive of Leigh syndrome, which is caused by variations in mitochondrial and nuclear genes. Our study revealed a novel, homoplasmic T11984C missense mutation in ND4 gene, which replaces a highly conserved amino acid tyrosine with histidine. Computational analysis showed that this mutation alters the secondary structure of ND4 subunit. As the mutation observed in this study was novel and homoplasmic, we speculate that there could be interplay of this mitochondrial mutation along with nuclear gene(s) in the pathogenesis.
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14/34. The metabolic landscape of cortico-basal ganglionic degeneration: regional asymmetries studied with positron emission tomography.

    Regional metabolic rate for glucose (rCMRGlc) was estimated using [18F]fluorodeoxyglucose (FDG) and positron emission tomography (PET) in five patients (four men, one woman; mean age 68; mean disease duration 2.4 years) with clinical findings consistent with the syndrome of cortico-basal ganglionic degeneration (CBGD). Left-right rCMRGlc asymmetry, (L-R)/(L R) x 100, was calculated for 13 grey matter regions and compared with regional metabolic data from 18 normal volunteers and nine patients with asymmetrical Parkinson's disease (PD). In the CBGD group mean metabolic asymmetry values in the thalamus, inferior parietal lobule and hippocampus were greater than those measured in normal control subjects and patients with asymmetrical PD (p less than 0.02). parietal lobe asymmetry of 5% or more was evident in all CBGD patients, whereas in PD patients and normal controls, all regional asymmetry measures were less than 5% in absolute value. Measures of frontal, parietal and hemispheric metabolic asymmetry were found to be positively correlated with asymmetries in thalamic rCMRGlc (p less than 0.05). The presence of cortico-thalamic metabolic asymmetry is consistent with the focal neuropathological changes reported in CBGD brains. Our findings suggest that metabolic asymmetries detected with FDG/PET may support a diagnosis of CBGD in life.
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15/34. Cortical-basal ganglionic degeneration.

    We report our experience with 15 patients believed to have cortical-basal ganglionic degeneration. The clinical picture is distinctive, comprising features referable to both cortical and basal ganglionic dysfunction. Characteristic manifestations include cortical sensory loss, focal reflex myoclonus, "alien limb" phenomena, apraxia, rigidity and akinesia, a postural-action tremor, limb dystonia, hyperreflexia, and postural instability. The asymmetry of symptoms and signs is often striking. brain imaging may demonstrate greater abnormalities contralateral to the more affected side. Postmortem studies in 2 patients revealed the characteristic pathologic features of swollen, poorly staining (achromatic) neurons and degeneration of cerebral cortex and substantia nigra. Biochemical analysis of 1 brain showed a severe, diffuse loss of dopamine in the striatum. This condition is more frequent than previously believed, and the diagnosis can be predicted during life on the basis of clinical findings. However, as with other "degenerative" diseases of the nervous system, a definitive diagnosis of cortical-basal ganglionic degeneration requires confirmation by autopsy.
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16/34. Basal ganglion calcification in hyperphenylalaninemia due to deficiency of dihydropteridine reductase.

    The disease course and therapy of a nine-and-a-half-year-old boy with hyperphenylalaninemia due to a dihydropteridine reductase deficiency are reported. Clinically, there is a marked mental retardation and complex basal ganglion symptoms. The cranial computed tomographic investigation shows bilateral, symmetrical, comma-shaped calcifications in the globus pallidus and the putamen of the lentiform nucleus. The cause of these basal ganglion calcifications remains unclear. Lowering of serum and CSF folic acid levels could not be detected, in contrast to cases with the same enzyme defect described previously.
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17/34. Unilateral alpha rhythm and Cheyne-Stokes respirations.

    A case of unilateral basal ganglion hemorrhage with Cheyne-Stokes respirations is presented. During the hyperpneic periods there was rhythmic alpha activity in the hemisphere contralateral to the basal ganglion hemorrhage. We postulate that the hemorrhage interrupted the thalamocortical fibers on the involved side. The implications of this clinical evidence are discussed in the light of the theory of cerebral autorhythmicity.
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18/34. Germ cell tumors (germinoma and yolk sac tumor) in unusual sites in the brain.

    This report presents three cases of primary intracranial germ cell tumor encountered in unusual sites, or essentially non-midline structures. The three provided the opportunity to examine surgically obtained tissues with the electron microscope. The histological diagnosis was initially made by light microscopic observations. The first case was a 10-year-old boy in whom the tumor occupied the right thalamic and basal ganglionic region and was diagnosed as a yolk sac tumor. The second an 11-year-old boy who presented with a mass lesion on the left thalamic and basal ganglionic region, diagnosed as germinoma. The third was a 39-year-old man who presented with multiple tumors in the ventricular system and posterior fossa, also diagnosed as germinoma. Among the three cases, neither diabetes insipidus nor ophthalmologic disorder was manifested. Extensive examination and autopsy findings indicated that these intracranial lesions had not metastasized from primary extracranial tumors, including those of the genital organs.
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19/34. Cerebral subcortical calcification and hypoparathyroidism--a case report and review of the literature.

    Hypoparathyroidism is known to exhibit various neurological manifestations, but most of the neurological disorders seen in this condition are motor ones, and involuntary movement is rarely encountered. Basal ganglion calcification is a common feature in hypoparathyroidism. But calcification of the cerebral cortex is, to our knowledge, extremely rare with only nine previously documented cases reported. We report a case of both involuntary movement and extensive cortical calcification with some reference to the literature. Furthermore, we discuss the relationship between neurological disorders and intracranial calcification.
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20/34. Intracranial calcification in oculodento-osseous dysplasia.

    Oculodento-osseous dysplasia (ODOD) is a rare genetic disorder characterized by microphthalmia, hypoplasia of the dental enamel, dysplastic and sclerotic changes in the skeleton and various digital malformations. The occurrence of basal ganglion calcification in this condition is of considerable interest, particularly as it has some biochemical features in common with hypoparathyroidism and pseudohypoparathyroidism. The 2 patients presented in this article did not show any features of an extrapyramidal disorder, but both experienced progressive spasticity in the limbs. The neurological disturbance was not due to compression of the neuraxis by bony overgrowth, but to an associated neurological lesion of undetermined nature. The biochemical abnormalities are discussed but as yet no explanation for the association between intracranial calcification and ODOD can be offered.
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