1/220. Central nervous system T-cell lymphoproliferative disorder in a patient with chronic active Epstein-Barr virus infection.PURPOSE: Central nervous system (CNS)-T cell lymphoproliferative disorder (T-LPD) developing during the course of chronic active Epstein-Barr virus (CAEBV) infection is reported. patients AND methods: CAEBV was diagnosed in a 14-month-old boy with fever, cytopenia, hepatosplenomegaly, and abnormal high titers of anti-Epstein-Barr virus (EBV) antibodies. At 8 years of age, he had a splenectomy because of progressive disease. RESULTS: After 27 months of clinical remission, muscle weakness and paresthesia developed. magnetic resonance imaging of his brain showed spotty T2 prolongation in left parietal, bilateral frontal, and temporal white matter with meningeal enhancement. brain biopsy revealed the cerebral infiltration of CD3 , CD4 , CD8-, CD45RO , CD56-, and EBV-encoded rna 1 cells. CONCLUSIONS: The CNS involvement of EBV-associated T-LPD is a rare but serious complication in CAEBV without known underlying immunodeficiency.- - - - - - - - - - ranking = 1keywords = muscle (Clic here for more details about this article) |
2/220. Subacute encephalopathy after combination chemotherapy including moderate-dose methotrexate in a patient with gastric cancer.An episode of subacute encephalopathy after the infusion of a moderate dose of methotrexate (1500 mg/m2) (MTX) is reported in a young adult with metastastic gastric cancer. Weakness of the right arm, focal seizures, lethargy and confusion appeared on day 10. High signal intensity in periventricular white matter was observed on T2-weighted magnetic resonance imaging. Symptoms resolved spontaneously and completely after 48 h. We believe that this represents an unusual case of moderate-dose MTX-induced neurotoxicity in a patient with gastric cancer, which has not previously been reported.- - - - - - - - - - ranking = 0.25874994002134keywords = cancer (Clic here for more details about this article) |
3/220. Intracranial extracerebral neuroglial heterotopia: A case report and review of the literature.Heterotopic masses of neuroglial tissue are uncommon and most frequently involve extracranial midline structures. We report an unusual case of an intracranial, extracerebral neuroglial heterotopia involving the middle and anterior cranial fossae of a 5-year-old girl who presented with facial asymmetry. The lesion was composed of mature but disorganized gray and white matter admixed with surrounding soft tissues and exhibited histologic features reminiscent of cortical dysplasia. These rare lesions have been postulated to arise from a protrusion of tissue from the neuraxis through a pial defect, from abnormalities in the migration of embryonic neuroepithelial tissue, or from an accessory evagination of the neural tube inferior to the telencephalic vesicles. Regardless of the underlying pathogenic mechanism, these lesions must be histologically distinguished from both teratomas and primary central nervous system neoplasms.- - - - - - - - - - ranking = 0.070027118741635keywords = neoplasm (Clic here for more details about this article) |
4/220. Apneustic breathing in children with brainstem damage due to hypoxic-ischemic encephalopathy.To confirm the presence of apneusis in patients with hypoxic-ischemic encephalopathy and to clarify which factors influence their respiratory patterns, polygraphic studies were performed on two patients. Apneusis was clinically suspected in both patients who had severe brainstem damage. In one subject, inputs of vagal afferents from the gastrointestinal tract and the urinary bladder often resulted in extreme tachypnea instead of apneusis. lung inflation facilitated expiration during inspiratory arrest. Expiration preceded a periodic inhibition of rigospastic discharge in the right biceps muscle. In the other subject, prolonged inspiratory pauses with cyanosis occurred with or without preceding epileptic seizure. Both phenytoin dose reduction and treatment with tandospirone, a serotonin-1A agonist, were effective in improving the respiratory distress in this subject.- - - - - - - - - - ranking = 1keywords = muscle (Clic here for more details about this article) |
5/220. Intracranial papillary endothelial hyperplasia: occurrence of a case after surgery and radiosurgery.Papillary endothelial hyperplasia (PEH) is considered a form of endothelial proliferation rather than a true neoplasm and is usually located in the skin or subcutis. We report a case of intracranial PEH that occurred after surgery for glioma and subsequent radiosurgery. CT and MR revealed an enhancing extra-axial mass located left posterolateral to the brainstem. Intracranial PEH is rare; to our knowledge, development of an intracranial PEH after surgery and radiosurgery has not been previously reported.- - - - - - - - - - ranking = 0.070027118741635keywords = neoplasm (Clic here for more details about this article) |
6/220. Histiocytic lesion mimicking intrinsic brainstem neoplasm. Case report.This 10-year-old girl presented with a 1-month history of progressive bulbar palsy and a solitary enhancing mass originating within the floor of the fourth ventricle. Results of initial imaging studies and presentation were suggestive of neoplasia. Subtotal resection was performed and pathological examination revealed the mass to be a histiocytic lesion, with no evidence of a glioma. The patient had no other stigmata of histiocytosis and was treated with steroid medications, resulting in prolonged resolution of the lesion. This case demonstrates that for discrete brainstem lesions the differential diagnosis includes entities other than glioma for which treatment is available. biopsy sampling should be considered when technically feasible.- - - - - - - - - - ranking = 0.28010847496654keywords = neoplasm (Clic here for more details about this article) |
7/220. Meningioangiomatosis: a case report and review of the literature.A case of cerebral meningioangiomatosis in an adult man without any stigmata of neurofibromatosis is reported. A 22 year-old man with no previous neurological history, presented with adversive seizures; clinical examination and electro-encephalogram were normal. CT showed nodular calcified masses in the left frontal lobe and in the left posterior parietal area. A craniotomy was performed and the frontal lesion was excised. Histological examination showed a predominantly intracerebral tumour involving both grey and white matter, with a complex picture of variable cellularity, dense calcification and prominent perivascular arrangements of reticulin-rich spindle cell fascicles with palisade formation widely involving the brain tissue. immunohistochemistry for S100 protein varied between different parts of the tumour; there was focal positivity for S100 protein and smooth muscle actin in the lesion with no reaction for GFAP or EMA. Immunostaining for Factor 8-related antigen highlighted the perivascular arrangement of lesional cells and demonstrated an increase in the number of small vessels in other areas. Electron microscopy of the main mass showed elongated spindle cells with formation of pericellular basal lamina. The literature on meningioangiomatosis was reviewed. The evidence for meningeal, perivascular neural plexus or pericyte origin does not appear to be well founded. The present case further illustrates the difficulty in identifying an exact histogenetic cell and probably reflects an origin from a primitive perivascular mesenchymal cell.- - - - - - - - - - ranking = 1keywords = muscle (Clic here for more details about this article) |
8/220. Congenital encephalomyopathy with epilepsy, chorioretinitis, basal ganglia involvement, and muscle minicores.A woman had severe psychomotor retardation, epilepsy, rigidity, and chorioretinitis. magnetic resonance imaging showed cerebellar and cerebral atrophy and hypointensities in T2-weighted images of the thalami and basal ganglia. Muscle biopsy documented size variations in rounded muscle fibers, fibrosis, and minicores on electron microscopy. Merosin staining was normal. These hitherto unreported features do not permit classification of our patient within the current types of encephalomyopathy and congenital muscular dystrophies.- - - - - - - - - - ranking = 5keywords = muscle (Clic here for more details about this article) |
9/220. Pleomorphic xanthoastrocytoma derived from glioneuronal malformation in a child with intractable epilepsy.Malformative lesions as well as neoplasms can cause intractable epilepsy in childhood. Even though the neoplastic nature of a lesion is evident in most cases, the distinction can be difficult in some patients. We present the case of a child with intractable epilepsy caused primarily by a glioneuronal malformation. Years after the first surgical intervention, a pleomorphic xanthoastrocytoma evolved from remnants of this lesion. This case suggests that glioneuronal malformations might be precursor lesions of pleomorphic xanthoastrocytomas.- - - - - - - - - - ranking = 0.070027118741635keywords = neoplasm (Clic here for more details about this article) |
10/220. Partial laminin alpha2 chain deficiency in a patient with myopathy resembling inclusion body myositis.It is becoming evident that clinical phenotypes associated with partial laminin alpha2 chain deficiency are variable. We recently observed a 29-year-old man with leukoencephalopathy and vacuolar myopathy resembling inclusion body myositis. laminin alpha2 immunohistochemical analysis showed reduction of the protein on muscle fiber surfaces. Molecular analysis revealed two novel compound heterozygous mutations in the LAMA2 gene. This is the first report linking a mutation in the LaMA2 gene with leukoencephalopathy and inclusion body-like myositis.- - - - - - - - - - ranking = 1keywords = muscle (Clic here for more details about this article) |
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