1/12. An intrasylvian "fibroma" in a child with cystic fibrosis: case report.OBJECTIVE AND IMPORTANCE: Intracranial fibrous tumors are uncommon during childhood. An unusual case of benign intrasylvian "fibroma" that has remained clinically and radiographically stable more than 3 years after a subtotal resection is described. CLINICAL PRESENTATION: A 9-year-old girl with cystic fibrosis presented with new-onset focal seizures referable to a large calcified left sylvian fissure mass. INTERVENTION: An open biopsy with subtotal resection of the lesion revealed a benign process characterized by exuberant fibrocollagenous tissue intermeshed with chronic inflammatory cells and foreign body giant cells, encompassing islands of gliotic brain tissue. Immunohistochemical analysis showed staining for epithelial membrane antigen and reticulin within some of the spindle cells, although the majority were nonreactive. The majority of tumor cells exhibited staining for laminin; CD34 staining was absent. Ultrastructural studies were also suggestive of a fibroblastic rather than a meningothelial origin of the lesion, with elongated cells separated by abundant extracellular collagen. Although dense adherence of the mass to the pial surface and the middle cerebral artery vessels precluded a complete resection, the patient remains seizure-free without anticonvulsant therapy more than 3 years postoperatively with no evidence of growth of the lesion. CONCLUSION: The lesion in this patient bears morphological similarity to a rare group of tumors referred to as "intracerebral fibromas," although a variety of other rare mesenchymal neoplasms were also considered within the differential diagnosis. However, the absence of any definite neoplastic features, the finding of chronic inflammatory changes, and the lack of growth of the residual tumor during an extended follow-up interval indicate that the mass may represent either an extremely indolent neoplasm or a nonneoplastic process. The differential diagnosis of intracranial fibrous tumors is contrasted with that of the reported case.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
2/12. A giant intracranial mucocele associated with an orbitoethmoidal osteoma. Case report.The authors present a rare case of a giant intracranial mucocele associated with an orbitoethmoidal osteoma in a patient suffering from a generalized convulsive disorder. The broad pedicle of the osteoma had penetrated the cribriform plate and extended intracranially to form a nodular mass in the olfactory groove. The intracranial portion of the osteoma was surrounded by a mucocele. Both the cyst wall and multilayered intracystic septations of the mucocele were indented by layers of the osteoma. Although the extracranial portion adhered to the mucosa of the ethmoidal sinus, there were no signs of sinus obstruction. No direct communication other than the osteoma was identified between the mucocele and the ethmoidal mucosa. The large cerebral defect, which the mucocele occupied, communicated directly with the lateral ventricle without any intervening membranous structures. A frontal craniotomy is recommended for exposure of the lesion and plastic repair of the dural defect.- - - - - - - - - - ranking = 5keywords = giant (Clic here for more details about this article) |
3/12. Pleomorphic xanthoastrocytoma--a clinicopathological study.Six cases of Pleomorphic Xanthoastrocytoma of central nervous system are presented. One case presented with recurrent refractory seizures and others as intracranial space occupying lesions. They were located in temporal/parietal lobes, were partly cystic and partly solid on CT scan, enhancing with contrast. Histopathology was characterized by pleomorphic giant cells and cells with vacuolated cytoplasm. glial fibrillary acidic protein was strongly positive in all the cases.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
4/12. Giant ependymal cyst of the temporal horn -- an unusual presentation. Case report with review of the literature.Primary benign cystic lesions in the brain are uncommon. However, extracerebral cysts like arachnoid cyst, epidermoid cyst and craniopharyngiomas are fairly common lesions. Also, colloid cyst in the third ventricle, dermoid cyst and endodermal cyst in the extracerebral location are not uncommon. On the contrary, intraventricular ependymal and choroidal cysts in the intraventricular location are infrequent. Surgical intervention is warranted in cysts, which produce a mass effect and raised intracranial pressure. We present an interesting case of a giant intraventricular ependymal cyst in the temporal horn in a 14-year-old boy, who presented with recent onset of headaches and epilepsy. He also had long-standing progressive proptosis of the left eye and left temporal bossing. Excision of this cystic lesion was curative. Interesting clinical and neuroimaging features are presented.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
5/12. giant axonal neuropathy with predominant central nervous system manifestations.The authors describe a 25-year-old woman with giant axonal neuropathy (GAN) and severe CNS involvement. She had been admitted to hospital with generalized seizures, and had gait disturbances followed by progressive mental deterioration since childhood. Neurological examination revealed mental retardation, scanning speech, cerebellar dysfunction, pyramidal signs, mainly in the lower extremities, and peripheral sensory neuropathy. Sensory nerve conduction velocity was decreased; brain CT and MRI showed diffuse demyelination. sural nerve biopsy revealed characteristic signs of GAN. The patient's older sister had died at the age of 23, after having had similar neurological disturbances since childhood. This case illustrates an unfamiliar presentation of GAN, characterized by mild sensory neuropathy and serve CNS involvement, including seizures.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
6/12. erdheim-chester disease mimicking a primary brain tumor. Case report.erdheim-chester disease (ECD) is a rare systemic histiocytic disease. The authors present a case report detailing the presentation and treatment of a 26-year-old man diagnosed with seizures and a well-circumscribed temporoparietal mass that had been demonstrated on imaging studies. Both preoperative and intraoperative diagnoses were consistent with a low-grade astrocytic neoplasm. Subsequent pathological examination indicated a histiocytic proliferation positive for CD68 and factor viii, and negative for CD1a and S100, with Touton giant cells characteristic of ECD. This case represents the first isolated occurrence of intracranial ECD and its potential to mimic glial neoplasms.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
7/12. Giant unruptured anterior communicating artery aneurysm presenting with seizure.Giant aneurysms of the anterior communicating artery (AComA) are rare. The clinical presentation of giant AComA aneurysms is usually associated with the mass effect of the space-occupying lesion or with subarachnoid haemorrhage. A giant AComA aneurysm presenting with a seizure has only been reported twice previously. We report a 70-year-old female patient, in whom a single seizure was the only symptom of a giant AcomA aneurysm, with no neurological deficit. The diagnosis of unruptured giant AComA aneurysm was made with cranial CT, MRI and angiography. The patient refused surgical intervention, was treated with anti-epileptic therapy and has been asymptomatic for 7 months. We suggest that elderly patients presenting with a first seizure need detailed evaluation and giant aneurysms, which may be confused with other intracranial space-occupying lesions, need to be considered in the differential diagnosis.- - - - - - - - - - ranking = 5keywords = giant (Clic here for more details about this article) |
8/12. Hypomelanosis of Ito (incontinentia pigmenti achromians)--a clinicopathologic study: macrocephaly and gray matter heterotopias.We studied a boy with macrocephaly, hypotonia, pigmentary retinopathy, unilateral whorled hypopigmented skin lesions, and seizures. skin biopsy confirmed the clinical diagnosis of hypomelanosis of Ito. Postmortem examination at age 22 months revealed a severe neuronal migrational defect that altered the cerebral cortex architecture of white matter. There were many gray matter heterotopias characterized by altered neurons and giant cells. Electronmicroscopy revealed the astrocytic nature of the giant cells. Embryologic migration of both melanoblasts from neural crest and cortical neurons occurs in the second trimester, suggesting a common mechanism for the developmental pathology of skin and brain.- - - - - - - - - - ranking = 2keywords = giant (Clic here for more details about this article) |
9/12. Transient giant R waves in the early phase of acute myocardial infarction: association with ventricular fibrillation.An unusual electrocardiographic (ECG) pattern was observed in five patients who suffered an acute anterior myocardial infarction. Early in their illness and following resuscitation from ventricular fibrillation (three patients), in the midst of recurrent ventricular irritability prior to development of ventricular fibrillation (one patient), and following a period of seizures (one patient), the ECG showed ST-segment elevation, marked increase in the R-wave amplitude, disappearance of S waves and merging of QRS complexes with the elevated ST segments, ECG patterns noted in these patients were similar to the ones recorded from dogs immediately after ligation of a large coronary artery, and from patients with severe episodes of variant angina. Although these early electrocardiographic changes probably reflect either marked regional transmural blood flow deprivation or its aftermath, they could not be taken as indices of eventual massive myocardial necrosis since in most of these patients the alterations were followed by development of nontransmural myocardial infarction. The possible mechanisms and the implications of such discrepancy between early and late electrocardiographic indicators of injury or necrosis is discussed.- - - - - - - - - - ranking = 4keywords = giant (Clic here for more details about this article) |
10/12. myoclonus and seizures in a patient with parkinsonism: induction by levodopa and its confirmation on SEPs.A 68-year-old woman with parkinsonism showed cortical myoclonus and seizures under antiparkinsonian medication. myoclonus was induced and enhanced by L-dopa, developing into generalized seizures. EEG was abnormal and somatosensory-evoked potentials (SEPs) showed giant SEPs, transcortical reflex (C reflex) and jerk locked potentials. myoclonus and seizures disappeared after discontinuation of L-dopa and the introduction of valproate sodium (VPA). We described the occurrence of L-dopa-induced myoclonus and seizures in a case of parkinsonism with its SEPs findings.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
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