1/4. Pallido-Luysio-Nigral atrophy revealed by rapidly progressive hemidystonia: a clinical, radiologic, functional, and neuropathologic study.Pallido-luysio-nigral atrophy (PLNA) is a rare neurodegenerative disease in which the clinical and radiologic correlates have not yet been clearly established. A 62-year-old man insidiously developed dystonic postures, choreoathetoid movements, slowness, and stiffness, which initially affected the right hand and foot and progressively spread to the entire right side. T2-weighted magnetic resonance imaging showed increased signal intensity in both left and right medial pallida and in the left substantia nigra. Tests using HMPAO-SPECT and FDG-PET demonstrated left cortical hyperperfusion and hypermetabolism, whereas the left lenticular nucleus was slightly hypometabolic. At age 65, abnormal movements and postures involved all four limbs and the axis causing major gait disturbances, and facial and bulbar muscles atrophied resulting in dysarthria, dysphagia, and impaired breathing. Diffuse amyotrophy and fasciculations also appeared. death occurred at age 66, 4 years after onset. At autopsy, severe bilateral neuronal loss and gliosis restricted to the pallidum, the subthalamic nucleus, the substantia nigra, and the hypoglossal nucleus were noted, accounting for the diagnosis of PLNA with lower motor neuron involvement. Progressive hemidystonia with adult onset represents an unusual clinical presentation for this disorder. Moreover, this observation indicates that a diagnosis of PLNA should be considered for specific magnetic resonance imaging, SPECT, and/or PET data, and suggests that in PLNA, pallidal dysfunction might play a key role in the dystonic presentation.- - - - - - - - - - ranking = 1keywords = subthalamic nucleus, subthalamic, nucleus (Clic here for more details about this article) |
2/4. Thalamic hand dystonia: an MRI anatomoclinical study.Focal dystonia has been attributed to lesions involving the basal ganglia and/or thalamus. hand dystonia was studied in a patient with a unilateral thalamic infarction documented by MRI. A 18-year-old girl presented with severe isolated dystonia of the right hand as a sequel of perinatal infarction. MRI scan revealed infarction affecting part of the dorsomedian, lateral posterior, ventral lateral, ventral posterior lateral nuclei, and centromedian-parafascicular nucleus of the contralateral thalamus. The unique MRI anatomoclinical presentation of this case, taken together with the literature data, could provide evidence that a lesion affecting one or several thalamic nuclei, including the centromedian nucleus, can induce hand dystonia.- - - - - - - - - - ranking = 0.009648143138969keywords = nucleus (Clic here for more details about this article) |
3/4. Neurostimulation of the ventral intermediate thalamic nucleus in inherited myoclonus-dystonia syndrome.We report on the effects of bilateral neurostimulation of the ventral intermediate thalamic nucleus (VIM) in a patient with medically intractable and progressing inherited myoclonus dystonia syndrome (IMDS). Postoperatively, the patient improved by approximately 80% on the modified version of a myoclonus score without any significant change in the dystonic symptoms. This suggests that neurostimulation of the VIM may be an effective treatment for myoclonus in pharmacologically intractable IMDS.- - - - - - - - - - ranking = 0.024120357847422keywords = nucleus (Clic here for more details about this article) |
4/4. Micrographia secondary to lenticular lesions.Four patients with a stroke developed micrographia. In two patients, the condition was pure and in the two other patients it was associated with signs of writer's cramp. We conclude that infarct of the left lenticular nucleus could either mimic pure micrographia similar to that of Parkinson's disease or micrographia associated with dystonia.- - - - - - - - - - ranking = 0.0048240715694845keywords = nucleus (Clic here for more details about this article) |