1/42. Extraventricular neurocytoma with ganglionic differentiation associated with complex partial seizures.We report an unusual case of extraventricular ("cerebral") neurocytoma with ganglion cells located in the right temporal lobe in a 9-year-old girl with complex partial seizures and precocious puberty. CT showed a calcified mass with central cystic zones. MR imaging showed a markedly hyperintense predominately solid tumor on both T1- and T2-weighted images, without appreciable contrast enhancement. Cerebral neurocytomas are histologically benign and radical surgery is curative; they should be included in the differential diagnosis of temporal lobe tumors in children.- - - - - - - - - - ranking = 1keywords = ganglion (Clic here for more details about this article) |
2/42. Exuberant transverse ligament degeneration causing high cervical myelopathy.Two patients with cervical myelopathy and C1-C2 retro-odontoid masses were examined. Preoperative magnetic resonance imaging studies suggested soft tissue pannus, as might be seen in rheumatoid arthritis; however, the results of serologic testing for rheumatoid factor were negative in both patients. Intraoperative findings and pathologic examination revealed degenerative fibrocartilage without inflammation or neoplasia. Similar lesions reported in the literature have been described as retro-odontoid disk hernia, damaged transverse ligaments, transverse ligament degeneration, synovial cysts, ganglion cysts, and degenerative articular cysts. These lesions may share a common pathophysiologic origin and represent a single disease process, namely exuberant degeneration of the transverse ligament.- - - - - - - - - - ranking = 0.2keywords = ganglion (Clic here for more details about this article) |
3/42. natural history of paediatric intervertebral disc calcification.This case report concerns a 5-year-old boy who had intervertebral disc calcification with involvement of two disc spaces and herniation of nucleus pulposus in one. The patient's symptoms resolved completely in a week with conservative measures. At the 4-year follow-up, the child was symptom-free and in full health, the herniation of nucleus pulposus had resolved completely, and calcification had disappeared in one of the disc spaces. Although the cause of this disorder is uncertain, the course is benign and self-limiting, it seldom requires surgical intervention, and the natural history is one of resolution and complete resorption of the calcification.- - - - - - - - - - ranking = 0.0026072414783409keywords = nucleus (Clic here for more details about this article) |
4/42. Neuropathology of Raine syndrome.We present three cases of Raine syndrome occurring in siblings of consanguineous parents. Raine syndrome is characterised by generalised osteosclerosis with craniofacial anomalies and intracranial calcifications. So far, only nine cases have been reported, and no evaluation of the distribution and extent of the cerebral mineralisations, as well as their impact on the surrounding tissue, has been undertaken yet. In our cases, calcifications were unevenly distributed throughout the central nervous system, not associated with neuronal loss or dystrophic events and appeared mostly as single calcospherites within the neuropil with occasional confluent deposits at advanced gestational age. There was intense perifocal microgliosis around single immature calcospherites, as well as mild astrogliosis around and within the confluent lesions, in which occasional macrophages could be found. Rarely, mineralisations occurred in blood-vessel walls, mainly affecting basal ganglia. Preferential sites of calcification were parietal and occipital periventricular white matter and corpus callosum, while frontal lobes were mildly affected. The cortex, temporal lobes as well as internal capsule, brain stem, cerebellum, leptomeninges, pituitary gland and choroid plexus were devoid of mineralisations. The subcortical grey matter was moderately involved in the putamen and pallidum, mildly in the caudate nucleus and subependymal germ cell matrix and not at all in the thalamus, Ammon's horn, amygdala and substantia nigra. The distribution of mineral deposits was thus inversely correlated to regional blood circulation and capillary density, with calcifications being concentrated in more sparsely perfused areas but lacking in highly vascularised tissue. This inverse relationship between mineralisation and regional blood flow was reflected in the varying distribution of calcospherites in grey and white matter as well as in the white matter of different lobes.- - - - - - - - - - ranking = 0.0013036207391704keywords = nucleus (Clic here for more details about this article) |
5/42. Distribution of basal ganglia lesions in diffuse neurofibrillary tangles with calcification: a clinicopathological study of five autopsy cases.We investigated five Japanese autopsy cases of diffuse neurofibrillary tangles with calcification (DNTC), both clinically and pathologically, and examined the degree and distribution of the basal ganglia lesions, especially in the amygdala, striatum, pallidum, and substantia nigra. The lesions in the amygdala, striatum, and pallidum were classified into three categories (mild, moderate, and severe). The lesions in the substantia nigra were qualitatively judged, compared with normal controls. Severe dementia was observed in four cases neuropathologically showing pronounced neuronal loss in the cerebral cortex, but one case without neuronal loss in the cerebral cortex showed mild memory disturbance. Extrapyramidal signs were evident in three cases. Obvious neuronal loss in the substantia nigra with the presence of lewy bodies was noticed in four cases. basal ganglia lesions in all five cases were uniform: the amygdala showed severe to moderate lesions, the caudate nucleus moderate to slight lesions, and the putamen and pallidum slight lesions to normal. Furthermore, the lesions in the amygdala were more prominent in the basolateral group than in the corticomedial group, inconsistent with those in the amygdala of Alzheimer's disease. Moderate lesions were evident in the basolateral group of the amygdala in the case without neuronal loss in the cerebral cortex. In DNTC, the degree and distribution of the basal ganglia lesions, except for nigral lesions, were analogous to those found in Pick's disease with Pick bodies. These clinicopathological findings may contribute to the elucidation of the clinicopathological hallmarks in this disorder.- - - - - - - - - - ranking = 0.0013036207391704keywords = nucleus (Clic here for more details about this article) |
6/42. Lumbar nerve root "walled" by a calcified herniated mass in a young patient.INTRODUCTION: Discal calcification in childhood is rare. Calcifications are occasionally discovered during routine examinations, especially in the cervical spine. Generally, the calcification process is confined to the nucleus pulposus of the intervertebral disc. CASE REPORT: In this report, we describe the case of a 16-year-old girl who presented with acute low back pain, right S1 radiculopathy and a history of increasing paresthesia. The patient underwent a CT scan, which demonstrated a postero-lateral calcified disc hernia at the L5-S1 level. The surgical decompression of the lumbar nerve root was carried out; at the operation it was observed that the nerve root was "walled" into the calcified hernia. DISCUSSION: Only a few cases with evidence of calcification of the herniated portion of the disc have been previously described. In none of them was the phenomenon was so pronounced that it sealed the nerve root completely.- - - - - - - - - - ranking = 0.0013036207391704keywords = nucleus (Clic here for more details about this article) |
7/42. Vertical ophthalmoplegia in a demented patient with striatopallidodentate calcification.A case is presented here of a 73-year-old man who showed signs of dementia, supranuclear vertical ophthalmoplegia, pseudobulbar palsy, axial dystonia, mild rigidity, and parkinsonian gait. Computed tomography of the head revealed symmetrical calcification in the striatum, globus pallidus and dentate nucleus to an extraordinary degree. No metabolic conditions were observed that could explain the intracranial calcification. Oral administration of levodopa improved the patient's motor symptoms to some extent. ophthalmoplegia, parkinsonism and dementia combined are typically seen in patients with progressive supranuclear palsy. However, the present case and a few others that have been reported would seem to indicate that these unique symptoms might also be found in patients with intracranial calcification.- - - - - - - - - - ranking = 0.0013036207391704keywords = nucleus (Clic here for more details about this article) |
8/42. Non-Alzheimer non-Pick dementia with Fahr's syndrome.Five patients with non-Alzheimer non-Pick dementia combined with Fahr's syndrome were studied. Atypical clinical pictures emerged from an evaluation of these cases. Their symptoms and signs could be attributed neither to Alzheimer's disease nor to Pick's disease but to a partial mixture of both. The neuropathological changes were characteristic, and the common findings were as follows: 1) the absence of senile (neuritic) plaques, 2) the widespread presence of numerous neurofibrillary tangles throughout the neocortex, 3) a calcareous deposition of Fahr's type, 4) a circumscribed cerebral atrophy in the temporal or/and frontal lobes, 5) a moderate or severe demyelination and fibrous gliosis in the white matter of the atrophied areas and 6) a mild or moderate neuronal loss in the nucleus basalis of Meynert. These neuropathological changes were not due to Alzheimer's disease nor to Pick's disease. Similar cases reported previously were reviewed.- - - - - - - - - - ranking = 0.0013036207391704keywords = nucleus (Clic here for more details about this article) |
9/42. Clinicopathological experience with intraventricular neurocytomas.Intraventricular neurocytoma is a rare clinicopathological entity that has been recently described. We are reporting our experience with four diagnosed cases and the previously reported cases from the available literature are reviewed. These neoplasms occur mainly in young adults, and their histological diagnosis is difficult on light microscope, because they are almost indistinguishable from oligodendrogliomas. Nevertheless, the presence of tumoral cells arranged around nucleus-free fibrillary zones, resembling the large rosettes of pineocytomas and the immunohistochemical demonstration of synaptophysin are useful data for the pathological diagnosis. This diagnosis is easy on electron microscope, because it demonstrates the neuronal nature of tumoral cells. Regarding prognosis, we have found increasing evidence that these tumors are associated with a favourable course after surgery, and at present there is no clear evidence of the usefulness of radiotherapy.- - - - - - - - - - ranking = 0.0013036207391704keywords = nucleus (Clic here for more details about this article) |
10/42. Cerebellopontine calcification: a new entity of idiopathic intracranial calcification?We report the autopsy case of a 40-year-old woman with severe intellectual and motor disabilities, who showed calcification in the cerebellum and pons but not in the basal ganglia on CT scan, and died of intracranial hemorrhage due to intractable hypertension. At autopsy, numerous calcium deposits were noted in the cerebellar cortex, the dentate nucleus, the cerebellar white matter and the ventral pons. These deposits were distributed both in the neuropil and the white matter, but rarely within the arterial walls or in contact with capillaries. This weak relationship between calcification and the blood vessels, in addition to the paucity of basal ganglia calcification, is in contrast to the findings with other disorders involving intracranial calcification, including Fahr's disease and calcium metabolism disorders. immunohistochemistry revealed intense staining of calbindin-D28K and parvalbumin at sites of calcium deposits both in the present case and in a case of pseudohypoparathyroidism, whereas these proteins were not localized to calcium deposits in the cerebellum of a Fahr's disease brain. We propose that the present case may represent a distinct entity among diseases characterized by idiopathic intracranial calcification. In addition, calcium-binding proteins may be involved in the calcification process in some cases with intracranial calcification.- - - - - - - - - - ranking = 0.0013036207391704keywords = nucleus (Clic here for more details about this article) |
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