11/20. ataxia, chorea, seizures, and dementia. Pathologic features of a newly defined familial disorder.Five generations of a family exhibit a unique autosomal dominant neurologic disorder characterized by the development (usually between 15 and 30 years of age) of ataxia, seizures, choreiform movements, progressive dementia, and death after 15 to 25 years of illness. Neuropathologic findings in two deceased family members revealed remarkably similar findings, including marked neuronal loss of the dentate nucleus, microcalcification of the globus pallidus, neuroaxonal dystrophy of the nucleus gracilis, and demyelination of the centrum semiovale. The clinical and pathologic findings are closely correlated. ataxia and chorea are related to severe neuronal loss in the dentate nucleus with calcification in the globus pallidus. dementia occurs with progressive demyelination of the centrum semiovale, and loss of posterior column function occurs with neuroaxonal dystrophy of the nucleus gracilis and nucleus cuneatus.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
12/20. Porencephaly and hydranencephaly: a neuropathological study of four autopsy cases.Four autopsy cases of porencephaly and hydranencephaly were evaluated clinico-pathologically. The patients showed profound mental retardation, convulsive seizures and spastic quadriplegia. The life spans were much shorter in the hydranencephalic cases. Pathologically, the cerebral cortex in the vicinity of the parenchymal defects often showed an abnormal cytoarchitecture, which appeared to be closely correlated with superficial glial proliferation. Several neurofibrillary tangles were found in the nucleus basalis of Meynert and the locus ceruleus in a 23-year-old porencephalic patient, and so-called grumose degeneration of the dentate nucleus in three patients, including a 2-month-old hydranencephalic boy. It is suggested that extensive cerebral defects may cause neuronal degeneration of the subcortical nuclei.- - - - - - - - - - ranking = 0.4keywords = nucleus (Clic here for more details about this article) |
13/20. Podiatric implications of psychogenic seizures.Psychogenic seizures can mimic epileptic conditions and are often difficult to diagnose. The present study reviews one such unusual occurrence in a patient having a complex psychiatric history who required surgery for the removal of a ganglionic cyst in her foot. The differential diagnosis and treatment of psychogenic seizures is discussed.- - - - - - - - - - ranking = 3.9074521842785keywords = ganglion (Clic here for more details about this article) |
14/20. Percutaneous glossopharyngeal thermocoagulation complicated by syncope and seizures.We describe the case of a patient who, during the percutaneous thermocoagulation of the petrous ganglion of Andersch, suffered a serious vagal response resulting in cardiac arrest, collapse, and generalized convulsions. Notwithstanding such major symptoms, the postoperative examination revealed no lesions of the vagus nerve and the glossopharyngeal lesion was shown to be isolated and selective.- - - - - - - - - - ranking = 3.9074521842785keywords = ganglion (Clic here for more details about this article) |
15/20. primidone in the treatment of the long qt syndrome: QT shortening and ventricular arrhythmia suppression.We studied a family with the long qt syndrome. The initial case was in a patient who presented with seizures, ventricular fibrillation, and syncope. After a trial of conventional modalities of treatment including left stellate ganglionectomy, primidone (Mysoline) therapy was initiated. This drug has been successful in suppressing ventricular arrhythmias and shortening the QT interval over a 2-year follow-up period. Two additional family members with QT prolongation have been treated with primidone. During an 8-month follow-up, electrocardiographic and Holter recordings showed shortening of the QT interval.- - - - - - - - - - ranking = 3.9074521842785keywords = ganglion (Clic here for more details about this article) |
16/20. pyridoxine dependent epilepsy with iatrogenic sensory neuronopathy.An 18-year-old man was treated from birth with chronic high dose pyridoxine (vitamin B6) up to 2000 mg per day for pyridoxine-dependent seizures. Within two years of onset of treatment, he developed a sensory neuropathy which did not progress over the following 16 years. Electrophysiological studies were consistent with a pure sensory neuronopathy expressed as centripetal degeneration of processes of the dorsal root ganglion cells.- - - - - - - - - - ranking = 3.9074521842785keywords = ganglion (Clic here for more details about this article) |
17/20. Dysembryoplastic neuroepithelial tumor. A tumor with small neuronal cells resembling oligodendroglioma.Dysembryoplastic neuroepithelial tumor (DNT) is a clinicopathologically unique group of tumors, mostly located in the temporal lobe, associated with intractable complex partial seizure in young patients. We report two unusual cases with multifocal involvement of diverse sites in the central nervous system. Case 1 is that of a 50-year-old man with 9-year history of grand mal seizures, who died of acute myocardial infarction. Case 2 is that of a 10-year-old girl with intractable complex partial seizures and behavioral disorder. Postmortem examination in case 1 showed multifocal tumor in the left temporal lobe, third ventricle, and basal ganglia. magnetic resonance imaging in case 2 showed tumor in the right temporal lobe, both thalami, right cerebellar hemisphere, and pons. Histologically, both tumors were characterized by a multinodular appearance with a predominant component of alveolar arrangement of oligodendroglial-like cells around delicate capillaries, with mucoid matrix containing floating ganglion cells. There were also astrocytic nodules resembling pilocytic astrocytoma in case 1, and a gangliocytoma-like area merging with surrounding cortical dysplasia in case 2. Ultrastructural examination showed ganglionic differentiation in the oligodendroglial-like cells in case 2. They possessed dense core neurosecretory granules and many slender neuritic processes with microtubules arranged in parallel and terminating in synaptic junctions. The periventricularly located tumor with nodular extension to the periphery suggests an origin from subependymal germinal matrix with nests of primitive neuroblasts arrested in their embryonal migration. DNTs are related to ganglioglioma based on their common location and clinical behavior and on the presence of both ganglionic and astrocytic cells. They are also related to pilocytic astrocytoma by morphological and behavioral similarity. Together with cerebral neuroblastoma and central neurocytoma, they form a spectrum of tumors harboring small neuronal cells. The differentiation of DNT from oligodendroglioma is important so as to avoid unnecessarily aggressive therapy.- - - - - - - - - - ranking = 11.722356552835keywords = ganglion (Clic here for more details about this article) |
18/20. Aspergilloma in the paracavernous region--two case reports.A 30-year-old male and a 40-year-old female presented with aspergillus fungal granuloma in the cerebral locations involving the gasserian-ganglion and its divisions in one case and was densely adherent to the lateral dural wall of the cavernous sinus in the other. Both patients were otherwise healthy with no evidence of immuno-suppression. The lesions resembled benign tumor on preoperative imaging and intraoperative consistency and vascularity. The lesions were successfully and completely resected. Both patients developed major cerebral arterial territory infarcts in the postoperative phase, remote from the site of operation, leading to crippling neurological deficits in one patient and death in the other. The unusual location and the unusual and similar clinical course suggests that awareness of the possibility of ischemic complications after surgical resection of intracranial aspergillomas is necessary.- - - - - - - - - - ranking = 3.9074521842785keywords = ganglion (Clic here for more details about this article) |
19/20. association of pleomorphic xanthoastrocytoma with cortical dysplasia and neuronal tumors. A report of three cases.BACKGROUND: Several recent publications indicate that pleomorphic xanthoastrocytoma (PXA) may occasionally express ganglionic cell differentiation, linking this type of tumor to other benign, mixed glioneuronal neoplasms. Furthermore, ganglionic tumors and less frequently some benign glial tumors in the central nervous system, could be associated with a variety of mild developmental abnormalities of the cerebral cortex that are classified under the broad term cortical dysplasia. The association of cortical dysplasia with PXA and a neuronal pattern of differentiation has not been reported previously. methods: The authors present clinical, radiologic, immunohistochemical, and ultrastructural findings in three patients with cortical dysplasia. The cortical dysplasia was continuous with PXA, displaying an additional component of neuronal cell differentiation. RESULTS: The ages of the patients were 23, 47, and 52 years. Resection of the tumors was predated by temporal lobe seizures by 6 years in 1 patient and by more than 30 years in the others. In all 3 cases, radiologic studies conducted 3-6 years before surgery showed either no abnormality or lesions that were initially nonprogressing. CONCLUSIONS: The longstanding clinical history of seizures and the existence of radiologically documented dormant lesions several years before tumor removal both suggest that PXA in these patients very likely developed in benign hamartomatous cortical lesions or in preexisting cortical dysplasia. The authors' findings, along with the consistent occurrence of PXA in the gray matter in the majority of the reported cases, indicate that these tumors may originate either from a subclass of astrocytes histogenetically and topographically associated with neurons or from multipotential neuroectodermal precursor cells common to neurons and astrocytes.- - - - - - - - - - ranking = 7.814904368557keywords = ganglion (Clic here for more details about this article) |
20/20. Dysembryoplastic neuroepithelial tumors located in the caudate nucleus area: report of four cases.OBJECTIVE AND IMPORTANCE: Dysembryoplastic neuroepithelial tumors (DNTs) histologically resemble gliomas but behave as stable lesions. These tumors initially were considered to be located exclusively within the supratentorial cortex. The four reported cases demonstrate that DNTs may also arise in the area of the caudate nuclei. Moreover, the peculiar topography of these lesions, which suggests a derivation from the subependymal plate, is in accordance with the putative origin of DNTs from secondary germinal layers. CLINICAL PRESENTATION: The patients experienced partial seizures (two patients), generalized seizures (one patient), or headaches (one patient). All patients were young (17-26 yr) at the onset of symptoms, and all had normal results from their neurological examinations. INTERVENTION: All lesions demonstrated a pseudocystic appearance on computed tomographic scans, were hypointense on T1-weighted magnetic resonance imaging scans, hyperintense on T2-weighted magnetic resonance imaging scans, and did not show contrast enhancement. The four tumors similarly lined the left or right caudate nuclei and expanded within the homolateral ventricle (three patients) or both lateral ventricles (one patient). In one patient, the tumor also involved the adjacent paraolfactory cortex. CONCLUSION: In all patients, stereotactic biopsies helped to identify a specific glioneuronal element of DNTs. None of the tumors was operated on. radiotherapy was performed in only one patient. A long pre- and/or postbiopsy imaging follow-up, which was available in two nontreated patients (3 yr and 16 yr), demonstrated the perfect stability of the lesion. The occurrence of DNTs in this peculiar location needs to be considered to avoid misidentification as "ordinary" gliomas and prevent useless aggressive treatment.- - - - - - - - - - ranking = 0.8keywords = nucleus (Clic here for more details about this article) |
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