Nine (15%) of 60 patients with repaired myelomeningocele exhibited late deterioration of neurological function with a tethered cord syndrome. Dense adhesions at the lowest laminae and at the site of previous repair were the most common findings at surgery. Postoperatively, 71% of the patients improved. Magnetic resonance (MR) imaging was performed in 29 of the 60 patients. Eight of these 29 patients exhibited a tethered cord syndrome. The MR images in all patients showed a low-lying conus fixed at the site of previous repair, irrespective of the presence or absence of a tethered cord syndrome. The MR images were classified into two groups depending upon the site of adhesions: Group A had potential sites of tethering at the ventral aspect of the last laminae and at the site of previous repair, and Group B showed the adhesion point only at the site of previous repair. Most patients with a tethered cord syndrome were found to be in Group A; conversely, most patients without the syndrome were in Group B. An enlarged low conus was seen in symptomatic patients more commonly than in those without this syndrome. It is concluded that the presence of adhesions specifically at the last laminae as well as a widened low-lying conus may be the cause of tethered cord syndrome in patients with repaired myelomeningoceles. A clear understanding of the tethering process and preoperative evaluation of potential sites of tethering, based on the MR findings, are very important for planning surgery. The release of adhesions at the lowest laminae by laminectomy appeared essential for improvement.
Two cases of germ cell tumors (GCTs) of the basal ganglia are presented and 40 previously reported cases are reviewed. The incidence of GCTs of the basal ganglia and thalamus was estimated as less than 14% of all intracranial GCTs. All patients except for two (95%) were male, aged 7-19 years. The clinical course was usually slow. The major symptoms were hemiparesis, mental deterioration such as dementia or character change, precocious puberty, diabetes insipidus, oculomotor palsy, speech disturbance, and hemianopsia. Signs of intracranial hypertension did not occur until the late stages of the disease. The plain CT finding was characterized by an irregularly defined, slightly high-density area frequently accompanied by central low-density areas without significant mass effect. The tumors showed mild to moderate and nonhomogeneous contrast enhancement. An ipsilateral cerebral hemiatrophy was often found. MR images demonstrated the corresponding findings. GCTs of the basal ganglia had a high possibility of containing components other than germinoma such as choriocarcinoma, endodermal sinus tumor, and embryonal carcinoma. Thus, tumor markers in the serum, CSF, or cyst fluid were frequently positive. With recent refinement of microsurgical techniques as well as immunohistochemical study and measurements of tumor markers of serum, CSF, and cyst fluid, major resections of tumor, accurate pretreatment histologic diagnosis, and early determination of the specific types of this tumor appear to be readily possible. This is essential for effective treatment of patients not only with radiosensitive germinoma, but also those with radioinsensitive nongerminoma variants and a combination of them located in this region.
Eight cases of central neurocytomas were studied by immunohistochemistry and electron microscopy. Seven tumors were located in the lateral ventricles and one in the subependymal region. All but one patient had a favorable postoperative course. The tumors were composed of small uniform cells possessing amitotic round nuclei with frequent perinuclear halos, a few Homer Wright rosettes and no ganglion cells; an appearance resembling that of oligodendroglioma. Immunohistochemical studies disclosed neuron-specific enolase and Leu-7 positivity in all tumors, S-100 protein-positive cells were found in six, while glial fibrillary acidic protein--and vimentin-positive cells were confined to the blood vessels. Myelin basic protein as well as neurofilament were not detected in the tumors. Synaptophysin-positive areas were seen in one tumor. Ultrastructural examination showed distinctive neuronal tumor cells which had a cytoplasm with sparse dense-core vesicles and thin cell processes containing parallel microtubules. They were classified into three different types of tumor cells according to the extent of differentiation. The most consistent finding for histological diagnosis was the presence of typical or abortive synapses with clear and dense-core vesicles. Additionally, synaptophysin may be a specific marker for some central neurocytomas.
A 67-year-old man with generalized fatigue and weight loss developed hyponatremia. Endocrinologic examination demonstrated panhypopituitarism. Magnetic resonance imaging showed a pituitary mass extending slightly to the suprasellar region. Transsphenoidal resection of the tumor was performed. Histological examination found exclusively granulomatous tissue with cholesterol clefts, and no epithelial component. This cholesterol granuloma may be classified as xanthogranuloma of the sellar region.
Three cases of intraspinal tumors associated with hydrocephalus were encountered and treated. This review includes 67 cases, including these 3 cases, and discusses the pitfalls associated with intraspinal tumors presenting with hydrocephalus. These tumors are not easily diagnosed, especially in cases where the chief complaint, due to hydrocephalus, is headache or dementia, and does not include back pain. Among the 67 cases reviewed, symptoms suggesting normal pressure hydrocephalus were particularly noticed in patients over 40 years old. In cases presenting with headaches or dementia, back pain corresponding to the tumor location may appear after a shunt operation or external ventricular drainage. This postoperative back pain is important in indicating the presence of an intraspinal tumor.
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