1/13. Migratory basal ganglia lesions in subacute sclerosing panencephalitis (SSPE): clinical implications of axonal spread.We report a boy with subacute sclerosing panencephalitis (SSPE) who exhibited parkinsonian symptoms four months after onset. The symptoms improved after administration of levodopa. One year after onset, bilateral symmetric lesions appeared in the substantia nigra and the putamen, as observed using magnetic resonance imaging. After a one-year interval, the lesions migrated to the bilateral caudate and the cerebellar dentate nuclei. The series of migratory legions, each of which was connected by axonal pathways originating from the substantia nigra, suggests axonal spread of the sspe virus.- - - - - - - - - - ranking = 1keywords = putamen (Clic here for more details about this article) |
2/13. Magnetic resonance and positron emission tomography changes during the clinical progression of Rasmussen encephalitis.The authors describe serial positron emission tomography (PET) and magnetic resonance imaging (MRI) studies in a patient with pathologically confirmed Rasmussen Encephalitis (RE). Results of initial PET and MRI studies were normal. Subsequent studies showed involvement of the percentral and postcentral gyri and the putamen on PET, and the precentral and postcentral gyri on MRI. Coregistration of PET and MR images showed good correlation between the precentral and postcentral gyri involvement. However, subcortical involvement occurred earlier on PET than on MRI. The authors demonstrate the evolution of changes on PET and MR images in a patient with RE. Despite early pathologic confirmation of RE, there were no definite structural or functional imaging changes on PET or MRI until 3 years after symptom onset. These findings demonstrate the variability of imaging changes in RE, and the need to carefully correlate electro-physiologic and clinical findings to confirm the diagnosis of RE.- - - - - - - - - - ranking = 1keywords = putamen (Clic here for more details about this article) |
3/13. Tourettism as clinical presentation of Huntington's disease with onset in childhood.Infantile Huntington's disease (HD) shows a wide clinical heterogeneity. Here we describe the case of a child affected by HD who showed unusual neurological features consistent with tourettism. The absence of family history and persisting normal magnetic resonance imaging (MRI) results long after the onset of symptoms delayed the diagnosis of the disease. An MRI exam performed 26 months after disease onset disclosed bilateral atrophy in the putamen, suggesting HD. The diagnosis was confirmed by genetic analysis. The present report underlines the need to consider HD in childhood cases of unusual and even unfamiliar progressive movement disorders.- - - - - - - - - - ranking = 1keywords = putamen (Clic here for more details about this article) |
4/13. Simultaneous hypertensive intracerebral hematomas: two case reports.We describe two patients (76- and 54-year-old females) with multiple hypertensive intracerebral hematomas occurring simultaneously. One patient had a right thalamic hematoma extending into the internal capsule and basal ganglia together with an other one in the left putamen. The other patient had two hematomas located ipsilaterally in the left putamen and thalamus. Their neurological examinations showed only unilateral deficits. Their magnetic resonance angiograms revealed no vascular malformations. Neuroradiological procedures are essential for the diagnosis of these multiple brain events.- - - - - - - - - - ranking = 2keywords = putamen (Clic here for more details about this article) |
5/13. A neuropsychological-neuropathological case study of variant Creutzfeldt-Jakob disease.We report the first neuropsychological-neuropathological case study of a patient with variant Creutzfeldt-Jakob disease (vCJD) who was seen at the early stages of the disease, and whose cognitive functioning was monitored in the following months until his death. At presentation, his neuropsychological profile included impaired ability to retain new episodic information, deficits on tests of retrieval from semantic memory, and impairments on tests of memory for public knowledge, such as famous personalities. Tests of executive function were also performed poorly. Picture recognition memory and autobiographical memory were relatively spared, as was performance on tests of face perception and complex copying ability. Neuropsychological testing may be useful in monitoring the progression of vCJD, and we provide evidence to show a steady rate of decline in perceptual and naming tasks over a 4-month period. Post-mortem findings showed neuronal loss in the caudate, putamen, dorsal thalamus, cerebellum and occipital cortex. Spongiform changes were found throughout the brain, including the entorhinal cortex and anterior thalamus. Prion protein accumulation was noted in the hippocampus. We hypothesize that primary subcortical damage to structures in the thalamus and neostriatum, together with secondary frontal lobe dysfunction, combine to yield the pattern of neuropsychological impairment and neuropsychological sparing that was found.- - - - - - - - - - ranking = 1keywords = putamen (Clic here for more details about this article) |
6/13. A novel grading scale for striatonigral degeneration (multiple system atrophy).striatonigral degeneration (SND) is commonly thought to represent the neuropathological substrate of L-Dopa unresponsive parkinsonism in patients with multiple system atrophy (MSA). Other neuropathological hallmarks of MSA include olivopontocerebellar atrophy (OPCA) and preganglionic sympathetic spinal cord lesions. Clinicopathological evaluation of MSA patients recruited into ongoing natural history studies or neuroprotective intervention trials will require standardized grading of MSA pathology. Based on 25 autopsy cases of MSA, we propose a novel SND grading scale which allows semiquantitative assessment of lesion severity based on neuronal loss, astrogliosis and presence of alpha-synuclein positive glial cytoplasmic inclusions (GCIs) in substantia nigra, putamen, caudate nucleus, and globus pallidus. SND grade I is defined as degeneration of the substantia nigra pars compacta (SNC) with relative preservation of the striatum except for minimal gliosis and GCIs in the posterior putamen ("minimal change MSA"). SND grade II is characterized by neuronal loss, astrogliosis and presence of GCIs in SNC and posterior/dorsolateral putamen. caudate nucleus and external globus pallidus may exhibit slight gliosis. Striatal pathology is severe and extends to anterior ventromedial subregions in SND grade III. There is neuronal loss in caudate nucleus and globus pallidus. GCIs are more abundant in grade II than grade III SNC and putamen. Preliminary clinicopathologic correlation studies suggest milder parkinsonian disability and better initial L-Dopa responsiveness in SND grade I and II cases compared to grade III cases. Prospective clinicopathologic studies are required to validate the proposed SND grading scale and may result in further subdivisions, particularly of SND grade III.- - - - - - - - - - ranking = 4keywords = putamen (Clic here for more details about this article) |
7/13. dysarthria, progressive parkinsonian features and symmetric necrosis of putamen in a family with painful lipomas (Dercum disease variant).We describe painful subcutaneous lipomatosis in four members of a two-generation family. Lipomas appeared in adulthood, were circumscribed, painful on touch and mainly localized to the trunk and proximal parts of the extremities. The disorder was associated with dysarthria, visual pursuit defect and progressive dystonia. MRI showed bilateral increasing cystic lesions in the basal parts of the putamen. No other abnormalities were detected. The lesions corresponded well with the clinical presentation in the patients. Investigation for mitochondrial disease with muscle biopsy and mitochondrial dna gave normal results. No consistent biochemical changes were found. The disorder in this family was considered to differ from MERRF with lipomatous lesions and multiple symmetric lipomatosis but compatible with a Dercum disease variant.- - - - - - - - - - ranking = 5keywords = putamen (Clic here for more details about this article) |
8/13. Extensive cortical damage in a case of Creutzfeldt-Jacob disease: clinicoradiological correlations.MRI demonstrated extensive cortical involvement in a patient with pathologically proven Creutzfeldt-Jacob disease. The whole brain was atrophic; some of the supratentorial cortex, putamen and caudate nucleus gave high signal on T2-weighted images; the changes were more extensive on diffusion-weighted images (DWI). Comparison of the history, and the sites of atrophy and signal change suggested that the latter predominates in regions with long-lasting damage and prevalent gliosis, while high signal on DWI indicate current neuronal loss. This case widens the range of MRI findings in patients with Creutzfeldt-Jacob disease, and suggests that some information about the progression of the disease can be extracted from single MRI study.- - - - - - - - - - ranking = 1keywords = putamen (Clic here for more details about this article) |
9/13. Camptocormia or Pisa syndrome in multiple system atrophy.Although a mild stooped posture is a hallmark of parkinsonism, extreme trunk forward flexion is not common. This phenomenon was described in different etiological entities and called camptocormia. Other similar presentations called Pisa syndrome and antecollis were described mainly in extrapyramidal disorders. Authors present two cases of probable multiple system atrophy (MSA) with predominant parkinsonism and Pisa syndrome (or camptocormia). Both of them were previously misdiagnosed as idiopathic Parkinson's disease (PD) and one was reported 1 year earlier. The typical clinical presentation fulfilling the diagnostic criteria for multiple system atrophy, rapid progression with lack of responsiveness to L-DOPA and apomorphine and typical MRI putaminal pathology observed in both cases allowed us to make a diagnosis. Accuracy of clinical diagnosis in multiple system atrophy is still very poor. Therefore, unusual or rare clinical presentations may support the final diagnosis. The camptocormia, Pisa syndrome and antecollis may represent the continuum of the same motor phenomenon and most of the authors refer them to unusual form of axial dystonia. According to many clinical presentations on different forms of camptocormia/Pisa syndrome authors conclude that not etiology, but the localization of specific lesion, probably within putamen is responsible for that form of dystonia. In cases of parkinsonism and severe forward flexion of trunk multiple system atrophy, diagnosis should be considered.- - - - - - - - - - ranking = 1keywords = putamen (Clic here for more details about this article) |
10/13. Sydenham's chorea: positron emission tomographic (PET) scan studies.Two patients with Sydenham's chorea were evaluated with positron emission tomographic (PET) scans in the active phase of the disease. One patient had repeat scanning in the recovery phase. PET scans showed hypermetabolic changes of the caudate nuclei and putamen in the active phase of Sydenham's chorea. The scan reverted to normal in the recovery phase. These changes can afford a basis for comparing concurrent serum antibody studies in the acute and recovery phases of Sydenham's chorea.- - - - - - - - - - ranking = 1keywords = putamen (Clic here for more details about this article) |
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