1/34. Cerebellar and basal ganglion involvement in Langerhans cell histiocytosis.Langerhans cell histiocytosis (LCH) is a disease of unknown cause characterised by proliferation of histiocytic granulomas in tissues; the primary cerebral manifestation is diabetes insipidus caused by hypothalamic infiltration. We present a patient in whom, except for the absence of high signal on T1 weighting in the posterior pituitary, consistent with central diabetes insipidus, MRI showed no evidence of hypothalamic involvement by histiocytosis, despite the long duration of the disease. However, there was bilateral, symmetrical involvement of the cerebellum and globus pallidus in addition to a calvarial lesion. High signal in the cerebellar white matter on T2-weighted images may represent demyelination, gliosis and cell loss, as previously reported on pathologic examination.- - - - - - - - - - ranking = 1keywords = ganglion (Clic here for more details about this article) |
2/34. motor neuron disease-inclusion dementia presenting as cortical-basal ganglionic degeneration.The frontotemporal dementias are a group of relatively new and evolving clinical and pathologic entities. The predominant frontal-temporal atrophy causes a variety of clinical syndromes, usually dominated by disturbances in behavior, mood, and speech. The motor neuron disease-inclusion dementia (MNDID) subtype is characterized by the accumulation of specific intraneuronal ubiquitin-immunoreactive inclusions with the complete absence of tau immunoreactivity. We present a patient with the clinical and neuroimaging characteristics of a highly asymmetric neurodegenerative condition distinguished by limb rigidity, bradykinesia, dystonia with an alien limb phenomenon, cortical sensory findings, and limb apraxia. His premorbid diagnosis was cortical-basal ganglionic degeneration but he had the typical histologic features of a frontotemporal dementia of the MNDID subtype.- - - - - - - - - - ranking = 1.25keywords = ganglion (Clic here for more details about this article) |
3/34. Corticobasal ganglionic degeneration and/or frontotemporal dementia? A report of two overlap cases and review of literature.OBJECTIVE: According to the existing viewpoint, Corticobasal degeneration (CBD) is thought of as a predominantly extrapyramidal motor disorder that is distinct and unrelated to frontotemporal dementia (FTD), the most common form of non-Alzheimer dementias. A lack of understanding of the aetiopathogenesis, and poor correlation between the pathology and the clinical syndromes, has resulted in a disparity in the classification of cases of non-Alzheimer dementias. This report intends to highlight the overlap between FTD and CBD in the light of the evolution of these terms, and to discuss the implications of these findings on the nosology of CBD and the classification of non-Alzheimer dementias. methods AND RESULTS: Two cases who presented with cognitive dysfunction, which, on comprehensive neuropsychological testing warranted an antemortem diagnosis of FTD are reported. A detailed necropsy study of their brains, however, favoured a pathological diagnosis of CBD. The literature on the overlap between CBD and FTD is also reviewed. CONCLUSIONS: Firstly, evidence is emerging to suggest that the clear distinction drawn between FTD and CBD by the existing viewpoint, needs revision. Secondly, until such time that a comprehensive classification of non-Alzheimer dementias is evolved, it may be better to distinguish between the clinical and pathological levels of description and to classify cases, in vivo, on the basis of the clinical phenotype.- - - - - - - - - - ranking = 1keywords = ganglion (Clic here for more details about this article) |
4/34. Corticobasal ganglionic degeneration with Balint's syndrome.Corticobasal ganglionic degeneration (CBGD) is a neurodegenerative dementia characterized by asymmetric parkinsonism, ideomotor apraxia, myoclonus, dystonia, and the alien hand syndrome. This report describes a patient with CBGD who developed Balint's syndrome with simultanagnosia, oculomotor apraxia, and optic ataxia.- - - - - - - - - - ranking = 1.25keywords = ganglion (Clic here for more details about this article) |
5/34. Cortical-basal ganglionic degeneration: a clinical, functional and cognitive evaluation (1-year follow-up).We decided to evaluate a patient who was diagnosed with cortical-basal ganglionic degeneration from a clinical, instrumental and neuropsychological perspective. Our aim was to employ a new instrumental tool, functional magnetic resonance, in order to evaluate his cortical damage. We then followed the pathological course for 1 year and tested the patient again: we discuss the results of our evaluation, having an overview of the literature on the topic. In particular, we focused our attention on his apraxia, trying to suggest a dynamic and anatomical model to guarantee a possible explanation of his behavior.- - - - - - - - - - ranking = 1.25keywords = ganglion (Clic here for more details about this article) |
6/34. Involuntary hand levitation associated with parietal damage: another alien hand syndrome.The alien hand syndrome (AHS) usually consists of an autonomous motor activity perceived as an involuntary and purposeful movement, with a feeling of foreignness of the involved limb, commonly associated with a failure to recognise ownership of the limb in the absence of visual clues. It has been described in association to lesions of the frontal lobes and corpus callosum. However, parietal damage can promote an involuntary, but purposeless, hand levitation, which, sometimes, resembles AHS. In the present study, four patients (cortico-basal ganglionic degeneration - n=2; Alzheimer's disease - n=1 and parietal stroke - n=1) who developed alien hand motor behaviour and whose CT, MRI and/or SPECT have disclosed a major contralateral parietal damage or dysfunction are described. These results reinforce the idea that parietal lobe lesions may also play a role in some patients with purposeless involuntary limb levitation, which is different from the classic forms of AHS.- - - - - - - - - - ranking = 0.25keywords = ganglion (Clic here for more details about this article) |
7/34. Bilateral metachronous germinoma of the basal ganglia occurring long after total removal of a mature pineal teratoma: case report.OBJECTIVE AND IMPORTANCE: We report the extremely rare occurrence of a second germ cell tumor at a different site and with different histological types long after total removal of a mature pineal teratoma. CLINICAL PRESENTATION: A 10-year-old boy who presented with headache and vomiting was admitted to our hospital. Neuroradiological studies revealed a tumor in the pineal region. The tumor was totally removed. Histologically, the tumor proved to be a mature teratoma. The patient's postoperative course was uneventful. The patient received no adjuvant therapy and was followed in the outpatient clinic. Three years later, he was readmitted with transient left upper limb weakness and vomiting. Neuroradiological studies showed a tumor in the bilateral basal ganglia. INTERVENTION: The second tumor, which was located in the right basal ganglion, was partially removed for biopsy. Histologically, the tumor proved to be a germinoma. The patient received three cycles of combination chemotherapy consisting of carboplatin and etoposide with radiotherapy. After the second course of chemotherapy, magnetic resonance imaging studies revealed no evidence of the tumor. CONCLUSION: The second tumor was considered to be a de novo metachronous neoplasm rather than a recurrence of the original mature teratoma. We think that if primordial germ cell groups exist along the midline of the brain, more than two primordial germ cell groups could give rise to metachronous neoplasms at different sites and with different histological types.- - - - - - - - - - ranking = 0.25keywords = ganglion (Clic here for more details about this article) |
8/34. The syndrome of acute bilateral basal ganglia lesions in diabetic uremic patients.Acute extrapyramidal movement disorders have rarely been reported in uremic patients. We had previously presented three cases of acute movement disorders with bilateral basal ganglia lesions in uremia, and had proposed that it is not a rare condition. The objective of this study is to establish a more accurate clinical profile of this rarely described clinical syndrome, and to call attention to its common occurrence. We prospectively studied six patients we encountered from March 1996 to June 2001. We also reviewed the clinical records of a large population of uremic patients and identified six more cases. The clinical manifestations, laboratory findings, neuroimages, and clinical outcomes of these 12 patients were analyzed. When possible, each patient was followed up to the present time. Twelve patients had acute onset of movement disorders and bilateral basal ganglia lesions. All of the patients were diabetic. They had acute-onset Parkinsonism or dyskinesias, together with various symptoms such as consciousness disturbance, dysarthria, dysphagia, or ataxia. The main laboratory test results of abnormalities consisted of elevated blood urea nitrogen, creatinine, and metabolic acidosis. They had uniform neuroimaging findings of symmetrical bilateral basal ganglion changes. These changes regressed or disappeared during follow-up. The clinical prognoses were diverse. We believe that this group of patients represents a well-demarcated clinical syndrome, which is not uncommon but has previously been rarely addressed. The underlying mechanism of such lesions may be associated with metabolic, as well as vascular factors.- - - - - - - - - - ranking = 0.25keywords = ganglion (Clic here for more details about this article) |
9/34. Cortical basal ganglionic degeneration.In this case study, we describe the symptoms, neuropsychological testing, and brain pathology of a retired mason's assistant with cortical basal ganglionic degeneration (CBGD). CBGD is an extremely rare neurodegenerative disease that is categorized under both Parkinsonian syndromes and frontal lobe dementias. It affects men and women nearly equally, and the age of onset is usually in the sixth decade of life. CBGD is characterized by Parkinson's-like motor symptoms and by deficits of movement and cognition, indicating focal brain pathology. Neuronal cell loss is ultimately responsible for the neurological symptoms.- - - - - - - - - - ranking = 1.25keywords = ganglion (Clic here for more details about this article) |
10/34. Autonomic failure as the initial presentation of parkinson disease and dementia with lewy bodies.The authors report the clinical and postmortem neuropathologic findings of two patients, one with parkinson disease (PD) and one with dementia with lewy bodies (DLB), both of whom initially sought treatment for isolated autonomic failure. These cases suggest that neurodegeneration in PD and DLB may begin outside the CNS in autonomic postganglionic neurons, a finding with potential diagnostic and therapeutic implications.- - - - - - - - - - ranking = 0.25keywords = ganglion (Clic here for more details about this article) |
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