Cases reported "Basal Ganglia Diseases"

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31/36. 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.
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ranking = 1
keywords = nucleus
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32/36. rest tremor and extrapyramidal symptoms after midbrain haemorrhage: clinical and 18F-dopa PET evaluation.

    A 25 year old man had an acute subarachnoid haemorrhage due to the rupture of a right peduncular subthalamic arteriovenous malformation. Seven months later he developed a left rest tremor associated with mild bilateral extrapyramidal symptoms and responsive to levodopa treatment. Surface EMG recording showed synchronous activity of agonist and antagonist muscles in the left limbs. A PET 18F-dopa study showed a large decrease of the Ki value in the right striatum. One year after the stroke a persistent postural component developed in the tremor.
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ranking = 2.4175923141803
keywords = subthalamic
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33/36. Pallidoluysian atrophy: dystonia and basal ganglia functional anatomy.

    The clinical correlates of "pure" pallidoluysian atrophy are not well described. A 59-year-old man presented with 20 years of progressive generalized dystonia, dysarthria, gait disorder, supranuclear vertical gaze palsy, and bradykinesia. At autopsy there was severe bilateral atrophy of the external pallidum and subthalamic nucleus with neuronal loss and marked gliosis. This syndrome may epitomize the consequences of "pure" pallidoluysian atrophy. In this case, dystonia appears to occur in the setting of decreased excitation (increased inhibition) of medial pallidal neurons, a pathophysiologic condition common to several hyperkinetic states.
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ranking = 13.354854757571
keywords = subthalamic nucleus, subthalamic, nucleus
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34/36. reflex blepharospasm associated with bilateral basal ganglia lesion.

    A patient with a bilateral striatal lesion secondary to anoxia presented reflex blepharospasm associated with parkinsonism and dystonia in the limbs. The blink reflex excitability curve was enhanced and the R-2 response prolonged as in patients with essential blepharospasm. The findings in this patient support the notion that blepharospasm may be secondary to basal ganglia dysfunction through abnormal facilitation of reticular formation neurons controlling facial nucleus motoneuron excitability.
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ranking = 1
keywords = nucleus
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35/36. PET study of cerebral glucose metabolism and fluorodopa uptake in patients with corticobasal degeneration.

    We measured cerebral glucose utilization and fluorodopa metabolism in the brain of patients with corticobasal degeneration using position emission tomography. The clinical pictures are distinctive, comprising features referable to both cerebral cortical and basal ganglionic dysfunctions. brain images of glucose metabolism can demonstrate specific abnormalities with a marked asymmetry in the parietal cortex (the primary motor and sensory cortex and the lateral parietal cortex), the thalamus, the caudate nucleus and the putamen of the dominantly affected hemisphere related to clinical symptoms in six patients. [18F]dopa uptake also reduced in an asymmetric pattern, both the caudate nucleus and the putamen in four patients. This unique combination study measuring both cerebral glucose utilization and fluorodopa metabolism in the nigrostriatal system can provide efficient information about the dysfunctions which are correlated with individual clinical symptoms.
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ranking = 2
keywords = nucleus
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36/36. Selective involvement of basal ganglia and occipital cortex in a patient with acute endosulfan poisoning.

    We report a teenage girl with acute endosulfan poisoning who developed psychosis, generalized tonic-clonic seizures, myoclonic jerks, cortical blindness and limb rigidity. Serial magnetic resonance imaging (MRI) showed bilateral reversible lesions localized to caudate nucleus, putamen and occipital cortex; internal capsule and thalamus were spared. This selective involvement may have a bearing on mechanisms underlying endosulfan toxicity.
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ranking = 1
keywords = nucleus
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