Gangliogliomas are rare benign tumors of the central nervous system containing neoplastic ganglion and low grade glial cells. In studying 10 surgically treated cases, we evaluated the clinical, pathological, radiological, and immunocytochemical features, with follow-up. Ranging from 18 to 58 years in age, 7 patients were women, and 3 were men. The most common presenting symptom was seizure. Computed tomographic scan showed a low density enhancing mass in 8 and calcification in 5. Six had minimally abnormal vascularity on angiography. Seven patients had total and 3 had subtotal resections of the tumor. The temporal lobe was the location of the tumor in 6 cases. All of the cases met the histological criteria of Russell and Rubinstein for ganglioglioma. Four patients received postoperative radiotherapy because of subtotal resection or aggressive histological makeup. On follow-up, from 2.5 to 7 years, 8 patients are alive and tumor-free, and 7 are also seizure-free. Two died after operation: one immediately and the other of a glioblastoma that developed 5 years later. Our study confirms that ganglioglioma is a distinct histological entity, anatomically localized, with characteristic clinical and radiological findings and long term survival. Aggressive histological makeup is not a definite indication of malignant potential. The definitive role of follow-up radiotherapy for this tumor needs further study. Malignant evolution is rare, but warrants follow-up.

Ten cases of sporadic cerebral amyloid angiopathy (CAA) were seen as intracranial hemorrhage. CAA was seen as a stroke or catastrophic intracranial hemorrhage in all cases. CAA complicated by intracranial hemorrhage carried a high mortality; 9 of 10 patients died in spite of aggressive medical or surgical treatment. Senile dementia of Alzheimer's type was an associated finding in only 1 patient. Computed tomographic scanning in 4 patients initially was not diagnostic for hemorrhage. Lambda chains and amyloid P protein were demonstrated in the areas of amyloid angiopathy immunocytochemically. The pathogenesis of hemorrhage in CAA could be either increased fragility of amyloid-laden blood vessels or rupture of microaneurysms. Surgical treatment of intracranial hemorrhage in CAA should therefore be undertaken with caution.

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Giant-celled glioblastoma multiforme is characterized by bizarre multinucleated giant cells with abundant eosinophilic cytoplasm, paucity of vascular endothelial proliferation, and increased reticulin fibers. It is considered by Rubinstein to be a variant of glioblastoma multiforme and by Zülch to be of mesenchymal origin. We studied retrospectively ten cases of giant-celled glioblastoma to correlate clinical and pathological features including immunohistochemistry and ultrastructure, radiology and survival time. Seven men and three women ranging in age from 23-75 years had variable clinical presentations and well defined lesions on computed tomography (eight patients). Well-defined at surgery, the tumor was located in temporal (six patients), frontal (three patients), and parieto occipital (one patient) lobes. Besides characteristic histology, glial-fibrillary acidic protein was positive in all, along with intracytoplasmic fibrils on electron microscopy. Three patients died within 3 days of surgery. Of the surviving seven treated with radiation and/or chemotherapy, three had a survival time of more than 36 months and four less than 15 months. Our study identifies this tumor to be of astrocytic lineage, with temporal lobe predilection, and overall a longer survival rate than glioblastoma multiforme.

Magnetic resonance imaging (MRI) has improved the diagnosis of several pathological entities of the brain. MRI especially has been credited with distinguishing demyelinating diseases of the central nervous system from other diseases. The presence of a mass effect in a demyelinating disorder, however, makes difficult the distinction between tumor and a demyelinating disease. We report a case of a demyelinating disorder simulating an infiltrating glioma of the corpus callosum on MRI scan, resulting in surgical intervention. To our knowledge, this is the first case to be reported with MRI and documented pathological findings.

A 60-year-old woman presented with stiff-person syndrome (SPS). Treatment with diazepam controlled her painful spasms initially. Two and one-half years after the onset of SPS, new spells of paroxysmal leg jerking and apnea developed. A spell was recorded with simultaneous video and polygraphic techniques that revealed simultaneous firing of motor unit potentials in several muscles (paraspinal, internal hamstring, and abdominal muscles). Apnea was associated with arterial oxygen desaturation. An increase in the dose of diazepam decreased the number and severity of these episodes. Seventeen months later, the patient began to taper the diazepam dose. Shortly thereafter, she had a cardiorespiratory arrest and subsequently died. Autopsy showed small chronic inflammatory foci in the pancreas (some associated with islets) and findings of diffuse encephalomyelitis characterized by perivascular cuffing in the spinal cord, brainstem, thalamus, hippocampus, and amygdala and a dense mononuclear infiltrate in the anterior horns of the lumbar and cervical cord, with relative preservation of axons and myelin. Cell typing showed this infiltrate was polyclonal and reactive. There have been rare cases of SPS associated with encephalomyelitis reported previously. Although the prolonged course in our patient suggested that SPS may have preceded encephalomyelitis, the more likely explanation is that the patient had an unusually long course of encephalomyelitis alone.

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