1/627. 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 = deficiency (Clic here for more details about this article) |
2/627. Neuro-ophthalmic, radiographic, and pathologic manifestations of adult-onset alexander disease.A 61-year-old woman had a 3-year history of imbalance. Eye movement studies revealed square-wave jerks, gaze paretic nystagmus, rebound nystagmus, impaired smooth pursuit, impaired optokinetic nystagmus, and abnormal fixation suppression of vestibular nystagmus. A brain magnetic resonance imaging study showed extensive areas of increased signal from the middle cerebellar peduncles and dentate nuclei, which enhanced with gadolinium. Histopathological analysis of a needle biopsy specimen of the left cerebellar peduncle revealed diffuse gliosis in the presence of symmetrically distributed areas of demyelination. There were associated Rosenthal fibers. Clinicopathologic correlation supported a diagnosis of alexander disease. An adult patient with a history of progressive imbalance, ocular motility abnormalities consistent with cerebellar and/or brainstem dysfunction, and diffuse, symmetric hyperintense magnetic resonance imaging signals in brainstem and cerebellar white matter should suggest a diagnosis of adult-onset alexander disease.- - - - - - - - - - ranking = 198.71239822537keywords = smooth pursuit, pursuit, movement, ocular, motility, smooth (Clic here for more details about this article) |
3/627. Osmotic demyelination syndrome with two-phase movement disorders: case report.Osmotic demyelination syndrome (ODS) is characterized by regions of demyelination throughout the brain, which are most prominent in the pons. This demyelinating disease is associated with electrolyte disturbances and typically occurs in patients who are alcoholic or malnourished. movement disorders are not frequently recognized in patients with ODS. This report describes a 22-year-old woman with ODS after correction of profound hyponatremia. The main neurologic symptom was two-phase movement disorder. First, she had acute onset dystonia, then the movement disorder transformed to generalized rigidity and tremors in the delayed second phase. magnetic resonance imaging in the first phase revealed demyelinating lesions in the central pons, bilateral thalami and basal ganglia. In the second phase, the previous myelinolysis had been partially resolved. The clinical course of the two-phase movement disorder did not correlate with the resolving feature of neuroradiologic findings. During the second-phase movement disorder, the patient had a good response to propranolol and trihexyphenidyl.- - - - - - - - - - ranking = 62.116171200301keywords = movement disorder, movement (Clic here for more details about this article) |
4/627. Intracranial calcification mimicking the sturge-weber syndrome: a consequence of cerebral folic acid deficiency?Cerebral cortical calcification identical to that of the sturge-weber syndrome was observed in two children. In one child the calcification appeared after intrathecal administration of methotrexate and skull irradiation because of leukemia involving the central nervous system. In the other child, who had coeliac disease and epilepsy, the calcification appeared after treatment with anticonvulsants. This treatment was also contributing to the development of profound megaloblastic anemia. The unspecificity of the Sturge-Weber calcification is stressed and the hypothesis is put forward that the calcification may be secondary to folic acid deficiency interfering with the matabolism in the central nervous system.- - - - - - - - - - ranking = 5keywords = deficiency (Clic here for more details about this article) |
5/627. 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 = 0.12490834097467keywords = eye (Clic here for more details about this article) |
6/627. Growing skull fracture of the orbital roof. Case report.Growing skull fractures are rare complications of head trauma and very rarely arise in the skull base. The clinical and radiological finding and treatment of a growing fracture of the orbital roof in a 5-year-old boy are reported, and the relevant literature is reviewed. The clinical picture was eyelid swelling. Computed tomography (CT) scan was excellent for demonstrating the bony defect in the orbital roof. Frontobasal brain injury seems to play an important role in the pathogenesis of the fracture growth. Growing skull fracture of the orbital roof should be considered in the differential diagnosis in cases of persistent ocular symptoms. craniotomy with excision of gliotic brain and granulation tissue, dural repair and cranioplasty is the treatment of choice.- - - - - - - - - - ranking = 0.61632416144683keywords = ocular, eye (Clic here for more details about this article) |
7/627. The pitfall of silent neurosarcoidosis.sarcoidosis in childhood is seldom reported. Most cases are observed in older children and preadolescents as bilateral pulmonary disease and eye lesions. Arthritic features are more likely to be observed in infants and children younger than 4 years of age who do not develop pulmonary disease. Neurosarcoidosis is exceptional in this age group and seldom suspected when the neurologic symptoms are present. The authors report a pediatric patient with systemic sarcoidosis who developed a severe but silent neurologic involvement. Numerous masslike lesions were discovered on systematic cranial magnetic resonance imaging. The authors recommend a complete screening of extrapulmonary manifestations in children with sarcoidosis. The proper management of patients with incidentally discovered neurosarcoidosis has yet to be established.- - - - - - - - - - ranking = 0.062454170487334keywords = eye (Clic here for more details about this article) |
8/627. posterior leukoencephalopathy syndrome may not be reversible.The association of an acute reversible encephalopathy with transient occipital lobe abnormalities on imaging studies is well known. This condition has been called reversible posterior leukoencephalopathy syndrome. The clinical presentation usually includes seizures, headache, altered mental status, and blindness, often associated with hypertension and immunosuppressants. The authors discuss a two-year-old male with down syndrome who presented 2 months after allogeneic bone marrow transplantation with severe oculogyric crisis, without other complaints. The patient was being treated for hypertension and was receiving cyclosporine for prophylaxis of graft-vs-host disease. A computed tomography scan of the head revealed marked bilateral lucencies mainly involving the white matter of the occipital lobes, with a few foci of punctate hemorrhage. The condition improved when cyclosporine was discontinued, but an area of leukomalacia was identified on follow-up magnetic resonance imaging. To the authors' knowledge, oculogyric crisis as a presentation of reversible posterior leukoencephalopathy has not been previously described. Recognizing this association is important, because patients receiving cyclosporine are often receiving other medications that can potentially cause dystonic eye movements, possibly leading to a delay in diagnosis and treatment, which can result in an irreversible neurologic deficit.- - - - - - - - - - ranking = 9.8677371274287keywords = eye movement, movement, eye (Clic here for more details about this article) |
9/627. movement disorders following nonfunctional neurosurgery.OBJECT: knowledge is scarce about movement disorders that follow neurosurgical operations other than functional stereotactic surgery. The cases of 14 patients who suffered from movement disorders secondary to craniocerebral or spinal surgery are analyzed. None of these patients was initially treated by any of the authors. methods: Twelve patients underwent surgery for cerebral diseases. Nine of these patients harbored tumors and three patients had neurovascular disorders. Two patients underwent spinal surgery for cervicothoracic ependymoma or for multiple cervical disc herniations. Twelve of the 14 patients had immediate postoperative side effects such as hemiparesis, ataxia, and somnolence. In all but two patients, movement disorders became manifest only after a delay. Dystonic movement disorders developed in eight patients, unilateral tremors in three patients, unilateral facial myokymia in one patient, and hemichorea-hemiballism in two patients. The mean delay of onset for tremor was 5 weeks and that for dystonic movement disorders was 5.5 months. movement disorders were transient in three patients; however, they were persistent in 11 patients at a mean follow-up period of 5 years. These movement disorders caused marked persistent disability in four patients. Lesions of the contralateral striatum were identified in patients with dystonic syndromes and lesions of the dentatothalamic outflow in patients with tremors. In three patients who had postoperative basal ganglia lesions after partial removal of astrocytomas, tumor regrowth was later documented. Medical treatment in patients with persistent movement disorders rendered only limited benefit. Two patients improved with botulin injections. In one patient postoperative hemidystonia was alleviated by contralateral thalamotomy. CONCLUSIONS: Dystonic syndromes and tremors are the most common movement disorders that occur after craniocerebral and spinal surgery. Postoperative movement disorders can lead to various degrees of functional disability. The pathoanatomical correlations are similar to those described in other patients with secondary movement disorders.- - - - - - - - - - ranking = 77.645214000376keywords = movement disorder, movement (Clic here for more details about this article) |
10/627. Periventricular brain heterotopias in a child with adrenocortical insufficiency, achalasia, alacrima, and neurologic abnormalities (Allgrove syndrome).We describe a previously unreported finding of periventricular heterotopias in a brain magnetic resonance imaging (MRI) study, in a girl with adrenocortical insufficiency, alacrima, achalasia, and neurologic deterioration (Allgrove syndrome). This finding could indicate that the underlying mechanism in this syndrome can be traced to the first half of fetal life and also might cause abnormal neuronal migration. This disorder recently has been linked to chromosome 12q13. There could be several explanations for the clinical heterogeneity in this syndrome: a contiguous gene syndrome involving multiple genes, including one whose deletion causes heterotopias, or a deficiency of a gene for a neurotrophic factor active during pre- and postnatal life and responsible for both migration and survival of neurons could be the cause. The identification of the responsible gene(s) will lead to further understanding of this multisystem disorder.- - - - - - - - - - ranking = 1.2601240444299keywords = deficiency, insufficiency (Clic here for more details about this article) |
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