Nuclear Magnetic Resonance (NMR) Imaging in Wilson Disease

Ga, Lawler; Jm, Pennock; Steiner, R. E.; Wj, Jenkins; Sherlock, Sheila; Ir, Young

doi:10.1097/00004728-198302000-00001

Cited by 76 publications

(22 citation statements)

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“…Numerous reports exist where focal lesions in the thalamus, the brain stem, the dentate nuclei, the cerebral white matter and the basal ganglia of patients with WD are found using magnetic resonance imaging, and their pathogenesis is extensively discussed (Aisen et al, 1985;De Haan et al, 1987;Grimm et al, 1991;Lawler et al, 1983;Magalhaes et al, 1994;Metzer and Angtuaco, 1986;Prayer et al, 1990;Roh et al, 1994). Focal edema, nerve cell loss, demyelination, gliosis, intracellular deposition of haemosiderin and cavitation have been surmised to represent the morphological changes correlating to the magnetic resonance findings in WD.…”

Section: Discussionmentioning

confidence: 99%

Dopamine D2 receptor binding is reduced in Wilson's disease: Correlation of neurological deficits with striatal123I-Iodobenzamide binding

Oder

Brücke

Kollegger

et al. 1996

J. Neural Transmission

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To visualise and quantify dopamine D2 receptor binding in the corpus striatum of patients with neurological Wilson's disease (WD) 123I-Iodobenzamide (IBZM) binding was measured using single photon emission computer tomography (SPECT). Ratios of striatal to frontal countrates were calculated in 8 patients and in 21 healthy control subjects. We found reduced IBZM binding ratios in all patients with WD in comparison to those in controls (1.48 +/- 0.13 vs. 1.73 +/- 0.09). The reduction in IBZM binding was correlated with the overall severity of neurological deficits and the severity of dysarthria (correlation coefficients -0.86 [p < 0.01] and -0.79 [p < 0.01], respectively). When patients of three different subgroups of neurological WD were compared no differences in IBZM binding were found. We conclude that assessing basal ganglia function in vivo using IBZM-SPECT is a valuable diagnostic tool in WD.

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Section: Discussionmentioning

confidence: 99%

Dopamine D2 receptor binding is reduced in Wilson's disease: Correlation of neurological deficits with striatal123I-Iodobenzamide binding

Oder

Brücke

Kollegger

et al. 1996

J. Neural Transmission

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“…MAI has been used successfully in children to show brain pathology [1 -3], lymphoma and osteogenic sarcoma [3], and Wilson disease [4]. Our study indicates that MRI may have a role in evaluating children with neuroblastoma.…”

Section: Discussionmentioning

confidence: 68%

Magnetic resonance imaging of neuroblastoma with a 0.15-T magnet

Cohen¹,

Weetman²,

Provisor³

et al. 1984

American Journal of Roentgenology

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Magneticexperience with MRI of nine children with neuroblastoma. Subjects and MethodsFrom January 1983 to April 1984, nine consecutive new patients with neuroblastoma were studied with MRI. In all cases, the area of the primary tumor was imaged. In addition, the liver, upper abdominal lymph nodes, and the femur bone marrow were scanned in some cases. In cases 1 , 3, 4, 6, and 7, in whom the primary tumor was not resected, repeat MRI scans were used to evaluate the tumor response to chemotherapy and radiation therapy.Eight follow-up studies were performed on these children at periods ranging from 2 weeks to 10 months from the initial diagnosis. Patients were not routinely sedated; when needed, chloral hydrate was used for sedation. Patients were fed before scanning, and those old enough to cooperate emptied their bladders. Care was taken to ensure patients were warm and comfortable when lying in the scanner. With these simple precautions, patients tolerated scanning well, with most falling asleep. In addition to the MRI, all patients had computed tomographic (CT) scans done on initial presentation. CT scans were also done with six of the eight follow-up MRI scans. In all cases, corresponding CT and MRI scans were done within 4 days of each other. MRI was performed with a Technicare 1 .5-kG scanner. Anatomic regions of interest were initially completely scanned using contiguous slices and a spin-echo (SE) technique with a pulse repetition time (TA) of either 250 or 500 msec and an echo time (TE) of 30 msec. Planes of interest were then rescanned using different pulse sequences. These pulse sequences were inversion recovery (IA) with 1400-1 500 msec TA and an inversion time (TI) of 400 or 450 msec and an additional SE sequence with 1 000 msec TA and 60 msec TE. The matrix size was 256 x 256 and slice thickness about 1 cm. Most studies were complete in 60 mm. CT scans were done on a Picker Synderdyne 600 or a Philips Tomoscan 31 0 scanner.The intensity of the tumors also was compared with the intensity of liver and muscle for each pulse sequence used. Downloaded from www.ajronline.org by 54.191.190.102 on 05/11/18 from IP address 54.191.190.102.

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“…Contrastenhanced MRI confirmed bilateral blood-brain barrier disruption on the putamina. We assumed that putaminal necrosis was associated with alcoholic intoxication such as methanol poisoning, but this finding is not specific and was also seen in a variety of conditions including Wilson and Leigh disease, Kearns-Sayre syndrome and various other neurodegenerative disorders [14][15][16][17][18] . The mechanism underlying selective putaminal necrosis is unknown.…”

Section: Discussionmentioning

confidence: 99%