1/446. Chronic steadily progressive central and peripheral predominantly motor demyelination, involving the cranial nerves, responsive to immunoglobulins.The association of central and peripheral demyelination was reported previously. Most of the cases refer to central chronic relapsing demyelination with clinical criteria for multiple sclerosis associated with later signs of peripheral nerve involvement. Other authors, described central lesions in patients with chronic inflammatory demyelinating polyneuropathy and in guillain-barre syndrome, as a seldom occurrence. We report a patient in which a chronic steadily progressive central and peripheral predominantly motor nervous system demyelination, involving the cranial nerves, was identified. The patient improved after intravenous immunoglobulin suggesting an immune-mediated mechanism. To our knowledge this presentation was not described before.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
2/446. Visual loss with Langerhans cell histiocytosis: multifocal central nervous system involvement.A 42-year-old woman with a 6-year history of diabetes insipidus and progressive hypersomnolence presented with visual loss. neuroimaging showed infiltration in the hypothalamus, the optic nerve, and the chiasm, as well as multiple lesions in other areas of the brain parenchyma. biopsy showed Langerhans cell histiocytosis. This is an unusual presentation of Langerhans cell histiocytosis, involving the visual pathways without manifestations outside of the central nervous system. The differential diagnosis and the magnetic resonance imaging findings will be discussed.- - - - - - - - - - ranking = 0.16666666666667keywords = nerve (Clic here for more details about this article) |
3/446. Discourse deficits following right hemisphere damage in deaf signers.Previous findings have demonstrated that hemispheric organization in deaf users of American sign language (ASL) parallels that of the hearing population, with the left hemisphere showing dominance for grammatical linguistic functions and the right hemisphere showing specialization for non-linguistic spatial functions. The present study addresses two further questions: first, do extra-grammatical discourse functions in deaf signers show the same right-hemisphere dominance observed for discourse functions in hearing subjects; and second, do discourse functions in ASL that employ spatial relations depend upon more general intact spatial cognitive abilities? We report findings from two right-hemisphere damaged deaf signers, both of whom show disruption of discourse functions in absence of any disruption of grammatical functions. The exact nature of the disruption differs for the two subjects, however. Subject AR shows difficulty in maintaining topical coherence, while SJ shows difficulty in employing spatial discourse devices. Further, the two subjects are equally impaired on non-linguistic spatial tasks, indicating that spared spatial discourse functions can occur even when more general spatial cognition is disrupted. We conclude that, as in the hearing population, discourse functions involve the right hemisphere; that distinct discourse functions can be dissociated from one another in ASL; and that brain organization for linguistic spatial devices is driven by its functional role in language processing, rather than by its surface, spatial characteristics.- - - - - - - - - - ranking = 0.056484548067521keywords = organ (Clic here for more details about this article) |
4/446. Successful use of alternate waste nitrogen agents and hemodialysis in a patient with hyperammonemic coma after heart-lung transplantation.BACKGROUND: Lethal hyperammonemic coma has been reported in 2 adults after lung transplantation. It was associated with a massive elevation of brain glutamine levels, while plasma glutamine levels were normal or only slightly elevated. In liver tissue, glutamine synthetase activity was markedly reduced, and the histologic findings resembled those of reye syndrome. The adequacy of therapy commonly used for inherited disorders of the urea cycle has not been adequately evaluated in patients with this form of secondary hyperammonemia. OBJECTIVE: To determine whether hemodialysis, in conjunction with intravenous sodium phenylacetate, sodium benzoate, and arginine hydrochloride therapy, would be efficacious in a patient with hyperammonemic coma after solid-organ transplantation. DESIGN: Case report. SETTING: A children's hospital. PATIENT: A 41-year-old woman with congenital heart disease developed a hyperammonemic coma with brain edema 19 days after undergoing a combined heart and lung transplantation. methods: Ammonium was measured in plasma. amino acids were quantitated in plasma and cerebrospinal fluid by column chromatography. The effectiveness of therapy was assessed by measuring plasma ammonium levels and intracranial pressure and performing sequential neurological examinations. RESULTS: The patient had the anomalous combination of increased cerebrospinal fluid and decreased plasma glutamine levels. To our knowledge, she is the first patient with this complication after solid-organ transplantation to survive after combined therapy with sodium phenylacetate, sodium benzoate, arginine hydrochloride, and hemodialysis. Complications of the acute coma included focal motor seizures, which were controlled with carbamazepine, and difficulty with short-term memory. CONCLUSIONS: The aggressive use of hemodialysis in conjunction with intravenous sodium phenylacetate, sodium benzoate, and arginine hydrochloride therapy may allow survival in patients after solid-organ transplantation. An acute acquired derangement in extra-central nervous system glutamine metabolism may play a role in the production of hyperammonemia in this illness that resembles reye syndrome, and, as in other hyperammonemic disorders, the duration and degree of elevation of brain glutamine levels may be the important determining factors in responsiveness to therapy.- - - - - - - - - - ranking = 0.084726822101282keywords = organ (Clic here for more details about this article) |
5/446. Pontine cryptococcoma in a nonimmunocompromised individual: MRI characteristics.The case of a pontine cryptococcoma in a nonimmunocompromised, previously healthy 16-year-old boy is presented. The patient had slowly progressive brainstem signs with right cranial nerves V, VII, and VIII palsies, and contralateral corticospinal and spinothalamic deficits. Magnetic resonance images (MRI) revealed, within the right pons, a 1-cm diameter round mass lesion, hypointense on T1-weighted images, hyperintense on T2-weighted images, and with rim enhancement after infusion of gadopentetate dimeglumine. This is the only report of the MRI findings in an isolated pontine cryptococcoma in an immunocompetent patient. Early recognition of this specific MRI pattern is essential, because complete recovery can be achieved with prompt antifungal treatment.- - - - - - - - - - ranking = 0.16666666666667keywords = nerve (Clic here for more details about this article) |
6/446. Chronic toxic encephalopathy in a painter exposed to mixed solvents.This paper describes symptoms and findings in a 57-year-old painter who had been exposed to various organic solvents for over 30 years. He began to work as a painter at 16 years of age, frequently working in poorly ventilated areas; he used solvents to remove paint from the skin of his arms and hands at the end of each work shift. The patient and his family noticed impaired short-term memory function and changes in affect in his early forties, which progressed until after he stopped working and was thus no longer exposed to paints and solvents. After the patient's exposures had ended, serial neuropsychological testing revealed persistent cognitive deficits without evidence of further progression, and improvement in some domains. magnetic resonance imaging revealed global and symmetrical volume loss, involving more white than gray matter. The findings in this patient are consistent with chronic toxic encephalopathy and are differentiated from other dementing processes such as Alzheimer's disease, multi-infarct (vascular) dementia, and alcoholic dementia. Previous descriptions in the literature of persistent neurobehavioral effects associated with chronic exposure to organic solvents corroborate the findings in this case.- - - - - - - - - - ranking = 0.056484548067521keywords = organ (Clic here for more details about this article) |
7/446. FK506-induced leukoencephalopathy in children with organ transplants.FK506-induced leukoencephalopathy is a well-known entity in adult organ transplant patients. The neurotoxicity of FK506 immunosuppression is frequently reversible, with either reduction or cessation of the drug. This neurologic syndrome is not well documented in children. We report the clinical and radiologic features in four pediatric cases of FK506 leukoencephalopathy. In two of the four patients this syndrome was reversible.- - - - - - - - - - ranking = 0.1412113701688keywords = organ (Clic here for more details about this article) |
8/446. Posterior fossa epithelial cyst: case report and review of the literature.A 49-year old woman with progressive cranial nerve signs and hemiparesis was found at MR imaging and at surgery to have a cyst at the foramen magnum. immunohistochemistry and electron microscopy showed an epithelial cyst of endodermal origin. MR findings were of an extraaxial mass, with short T1 and T2 times. Unless immunohistochemistry and electron microscopy are used in the final diagnosis of such cysts, all posterior fossa cysts lined by a single layer of epithelium should be described simply as epithelial cysts.- - - - - - - - - - ranking = 0.16666666666667keywords = nerve (Clic here for more details about this article) |
9/446. 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 = 0.028242274033761keywords = organ (Clic here for more details about this article) |
10/446. Fatal poisoning from liquid dimethylmercury: a neuropathologic study.Since ancient times, mercury has been recognized as a toxic substance. Dimethylmercury, a volatile liquid organic mercury compound, is used by a small number of chemistry laboratories as a reference material in nuclear magnetic resonance spectroscopy. To our knowledge, dimethylmercury has been reported in only three cases of human poisoning, each proving fatal. Very small amounts of this highly toxic chemical can result in devastating neurological damage and death. We report the neuropathologic findings in a fatal case of dimethylmercury intoxication occurring in a laboratory researcher that resulted from a small accidental spill. We compare these findings to those reported in one previously reported fatal case of dimethylmercury poisoning, and to earlier reports of monomethylmercury poisoning, and discuss the clinicopathologic correlation.- - - - - - - - - - ranking = 0.028242274033761keywords = organ (Clic here for more details about this article) |
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