11/20. Hereditary spastic dystonia: a new mitochondrial encephalopathy? Putaminal necrosis as a diagnostic sign.A large kindred, in which either Leber's hereditary optic atrophy, or a hereditary spastic dystonia, or a combination of both manifested over many generations was restudied after the first report on it in 1964. NMR scans revealed bilateral, and, in two patients with hemidystonia, unilateral necrosis with shrinkage of the putamen, in one case associated with total disappearance of the head of the caudate nucleus. Except for age-appropriate cortical atrophy in one instance, no other changes were observed in the brain, brainstem, and cerebellum. The putaminal necrosis appears as typical "striatal slits" on the NMR scans. It is argued that this rare disease, since the princeps description in 1964 only reported in england (1986) and the U.S.A (1986), is most likely a singular type of mitochondrial encephalopathy: it is associated with Leber's optic atrophy, and the NMR changes observed have been signalled in other mitochondrial encephalomyelopathies, such as Leigh's disease and MELAS.- - - - - - - - - - ranking = 1keywords = putamen (Clic here for more details about this article) |
12/20. MRI demonstration of cortical laminar necrosis and delayed white matter injury in anoxic encephalopathy.We performed serial radiological examinations on a patient with anoxic encephalopathy. In the early term after the anoxic insult, T1-weighted MRI revealed high signal intensity areas distributed laminarly in the cerebral cortex and diffusely in the putamen, which were thought to reflect the cortical necrosis and necrosis in the putamen. Single photon emission computed tomography using I-123 isopropylamphetamine showed persistent hypoperfusion in the arterial watershed zones. T2-weighted MRI performed several months after the anoxic episode revealed diffuse high-intensity lesions in the arterial water-shed zones. These delayed-onset white matter lesions continued to extend over several months.- - - - - - - - - - ranking = 2keywords = putamen (Clic here for more details about this article) |
13/20. Kojewnikow's epilepsia partialis continua: two cases associated with striatal necrosis.The authors report two cases of children suffering from epilepsia partialis continua (EPC). The first case concerned a boy primarily affected by abdominal neuroblastoma and secondarily by bilateral EPC; "pallidal posture" was the prominent clinical feature. An acute measles encephalitis was diagnosed and the CT scan showed necrosis of the putamina. The second case concerned a girl suffering from increased intracranial pressure due to suprasellar craniopharyngioma. Seven days after intervention, diabetes insipidus and EPC appeared. Enlargement of rolandic and sylvian spaces and lacunar necrosis of the putamen on the left side were also evident on the CT scan. The authors emphasize the significance of occasional metabolic disturbances, especially natremia, in the development of EPC.- - - - - - - - - - ranking = 1keywords = putamen (Clic here for more details about this article) |
14/20. methanol intoxication. Parkinsonism and decreased Met-enkephalin levels due to putaminal necrosis.Due to methanol intoxication, a 38-year-old male developed permanent parkinsonism with visual impairment. brain computerized tomography (CT) and magnetic resonance imaging (MRI) revealed cystic resorption of the putamen. An alteration of the central noradrenergic activity and the opioid system can be assumed because of increased dopamine beta-hydroxylase (D beta H) activity and decreased methionine-enkephalin (Met-Enk) levels in the cerebrospinal fluid (CSF).- - - - - - - - - - ranking = 1keywords = putamen (Clic here for more details about this article) |
15/20. Infantile bilateral striatal necrosis. Clinical and morphological report of a case and a review of the literature.A case of infantile bilateral striatal necrosis (IBSN) is reported, the first one found in scandinavia. Extensive clinical and laboratory investigations did not reveal any evidence of a neurometabolic disease. By computed tomography (CT) degeneration of putamen of either side was detected. The neuropathologic findings included extensive bilateral destruction of the striatum and pallidum and degeneration of the substantia nigra and tegmental nuclei. In the damaged regions, accumulations of cells containing sudanophilic lipids were found and Alzheimer type II glial cells, which were also seen in the cortex. On the basis of the clinical picture and the destruction of nerve cells in particular areas of the brain, in the present case and previously published cases of IBSN, the possible role of glutamate and other excitotoxic transmitters in the pathogenesis of the disease is discussed.- - - - - - - - - - ranking = 1keywords = putamen (Clic here for more details about this article) |
16/20. movement disorders in childhood organic acidurias. Clinical, neuroimaging, and biochemical correlations.Over the last 5 years the Pediatric neurology service at King Faisal Specialist Hospital and research Centre (KFSH&RC) has seen 131 infants and children with movement disorders. Forty-nine (37%) had identifiable biochemical defects, 25 of which were organic acidemias. Nineteen of 29 patients with dystonia had organic acidemias, primarily glutaric aciduria type 1 (7 patients), bilateral striatal necrosis (4 patients), and 3-methyl glutaconic aciduria (3 patients). All patients with parkinsonian rigidity (n = 11) had organic acidemias; again, the greatest number accounted for by glutaric aciduria type 1 (7 patients), who had both parkinsonian rigidity combined with dystonia. myoclonus occurred in only 1 of 25 and chorea in 7 of 25 patients with organic acidemias. At the least all patients had bilateral lesions of putamen and head of caudate, seen best in MRI brain scans as increased T2 signal intensities with normal volume, and later with volume loss.- - - - - - - - - - ranking = 1keywords = putamen (Clic here for more details about this article) |
17/20. Acute basal ganglia infarction in propionic acidemia.An 8-year-old girl with propionic acidemia had acute and rapidly fatal symmetric necrosis of the caudate, globus pallidus, and putamen. Clinical presentation was with acute aphasia, generalized hypotonia, and muscle weakness. There was no evidence of metabolic decompensation, and analysis of the organic acids of the urine indicated good metabolic control. Organic acids in the cerebrospinal fluid were unremarkable. These observations indicate that the pathophysiology of "metabolic stroke" is more complicated than previously thought.- - - - - - - - - - ranking = 1keywords = putamen (Clic here for more details about this article) |
18/20. Serial MRI in infantile bilateral striatal necrosis.A 3-year-old girl with left hemiparesis suffered from bilateral paresis, motor rigidity, gait disturbance, axial hypotonia, dysarthria, apathy, and incontinence. After steroid therapy, mild improvement occurred, but muscle weakness, gait disturbance, and rigidity remained. Leigh encephalopathy was excluded on the basis of muscle biopsy and laboratory findings. Computed tomography and serial magnetic resonance imaging at an early stage revealed right-sided dominant lesions in the putamen and caudate nucleus and later bilateral striatal lesions, appearing as hyperintense signals on T2-weighted images and mixtures of hypo- and hyperintense signals on T1-weighted images. This is the first demonstration of serial magnetic resonance imaging findings in infantile bilateral striatal necrosis.- - - - - - - - - - ranking = 1keywords = putamen (Clic here for more details about this article) |
19/20. MR enhancing brain lesions in methanol intoxication.methanol intoxication can cause necrosis of the putamen and subcortical white matter that is evident on neuroimaging. We report a 47-year-old man with significant methanol intoxication who had enhancing lesions in the caudate nuclei, putamina, hypothalamus, and subcortical white matter by MRI. This case demonstrates that contrast enhancement of brain lesions can be observed after methanol poisoning.- - - - - - - - - - ranking = 1keywords = putamen (Clic here for more details about this article) |
20/20. magnetic resonance imaging and 11C-N-methylspiperone/positron emission tomography studies in a patient with the interval form of carbon monoxide poisoning.Magnetic resonance (MR) and (11)C-N-methylspiperone ((11)C-NMSP)/positron emission tomography (PET) imagings were repeatedly performed in a 50-year-old man with the interval form of carbon monoxide (CO) poisoning. In MR images obtained when delayed neuropsychiatric symptoms developed (two months after poisoning), the inner segments of the bilateral globus pallidus appeared as high signal intensities in the T1-weighted and low signal intensities in the T2-weighted images, suggesting prior focal hemorrhage in these areas. A PET study with (11)C-NMSP performed at that time showed an increase in dopamine D2 receptor binding in the caudate and putamen. Treatment with bromocriptine was very effective and five months after the poisoning, MR and (11)C-NMSP/PET images showed improvement, concomitantly with the disappearance of the neuropsychiatric symptoms.- - - - - - - - - - ranking = 1keywords = putamen (Clic here for more details about this article) |
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