|
| ||
|
|
||
Brain Involvement (brain + involvement)
Selected AbstractsVisceral Kaposi's sarcoma with intracranial metastasis: A rare complication of renal transplantationPEDIATRIC TRANSPLANTATION, Issue 6 2002Elif Bahat Abstract: The incidence of Kaposi's sarcoma (KS) has increased in solid organ transplantation recipients. This type of KS tends to be aggressive, involving lymph nodes, mucosa and visceral organs in about half of patients, sometimes in the absence of skin lesions. Brain involvement of KS has rarely been reported. A 16-yr-old Turkish boy underwent renal transplantation from his mother. The immunosuppressive regimen included prednisolone, cyclosporin A and azathioprine. Fourteen months later the azathioprine was changed to cyclophosphamide (3 mg/kg/day) because of the development of a nephrotic syndrome. After 12 weeks, the cyclophosphamide was changed to mycophenolate mofetil (MMF) to control the nephrotic syndrome. At this time his serum creatinine level rose to 2.1 mg/dL. Polyclonal or monoclonal antibodies were never given. Multiple intra-abdominal lymphadenopathy was detected on abdominal tomography at the 32nd month after renal transplantation. Kaposi's sarcoma was diagnosed via laparotomy and biopsy. He had a generalized tonic and clonic seizure and contrast enhanced cranial tomography showed two intracranial masses which had an abundant vascular component which caused a mild shift. One of the masses was removed via a burr-hole with the aim of diagnosis and treatment of the shift. A pathologic examination of the intracranial lesion was also reported as Kaposi's sarcoma. Herpes virus-8 DNA was detected by PCR in the intracranial lesion. [source] Brain involvement in muscular dystrophies with defective dystroglycan glycosylation,ANNALS OF NEUROLOGY, Issue 5 2008Emma Clement MBChB Objective To assess the range and severity of brain involvement, as assessed by magnetic resonance imaging, in 27 patients with mutations in POMT1 (4), POMT2 (9), POMGnT1 (7), Fukutin (4), or LARGE (3), responsible for muscular dystrophies with abnormal glycosylation of dystroglycan (dystroglycanopathies). Methods Blinded review of magnetic resonance imaging brain scans from 27 patients with mutations in 1 of these 5 genes. Results Brain magnetic resonance images were normal in 3 of 27 patients; in another 5, only nonspecific abnormalities (ventricular dilatation, periventricular white matter abnormalities, or both) were seen. The remaining 19 patients had a spectrum of structural defects, ranging from complete lissencephaly in patients with Walker,Warburg syndrome to isolated cerebellar involvement. Cerebellar cysts and/or dysplasia and hypoplasia were the predominant features in four patients. Polymicrogyria (11/27) was more severe in the frontoparietal regions in 6, and had an occipitofrontal gradient in 2. Pontine clefts, with an unusual appearance to the corticospinal tracts, were seen in five patients with a muscle-eye-brain,like phenotype, three patients with POMGnT1, one with LARGE, and one with POMT2 mutations. Prominent cerebellar cysts were always seen with POMGnT1 mutations, but rarely seen in POMT1 and POMT2. Brainstem and pontine abnormalities were common in patients with POMT2, POMGnT1, and LARGE mutations. Interpretation Our results expand the spectrum of brain involvement associated with mutations in LARGE, POMGnT1, POMT1, and POMT2. Pontine clefts were visible in some dystroglycanopathy patients. Infratentorial structures were often affected in isolation, highlighting their susceptibility to involvement in these conditions. Ann Neurol 2008;64:573,582 [source] Brain involvement in rheumatoid arthritis: A magnetic resonance spectroscopy studyARTHRITIS & RHEUMATISM, Issue 11 2009Bart J. Emmer Objective Tumor necrosis factor , was recently implicated as an important mediator of communication between the peripheral and cerebral immune systems in an animal model of chronic inflammation. The purpose of this study was to examine by proton magnetic resonance spectroscopy (1H-MRS) the influence of inflammation on cerebral metabolism in patients with rheumatoid arthritis (RA). Methods Single-voxel 1H-MRS of the centrum semiovale was performed on 35 RA patients (6 men and 29 women; mean ± SD age 51.8 ± 14.6 years) and 28 healthy age- and sex-matched control subjects (9 men and 19 women; mean ± SD age 50.2 ± 10.4 years). None of the study subjects had any neurologic signs or symptoms. Clinical markers of disease activity were correlated with the 1H-MRS findings. Results Patients with active RA, as reflected by an elevated erythrocyte sedimentation rate (ESR), had a significantly higher ratio of choline to creatine and a significantly lower ratio of N -acetylaspartate to choline than did patients with inactive RA, as reflected by a normal ESR. Moreover, the ratios of choline to creatine and NAA to choline were significantly correlated with the ESR after correction for age, sex, smoking status, handedness, alcohol consumption, medication use, and disease duration. Medication use had no additional effect on these associations. Conclusion Our data show that systemic inflammation in RA is associated with metabolic changes in the brain. [source] Quantitative EEG Asymmetry Correlates with Clinical Severity in Unilateral Sturge-Weber SyndromeEPILEPSIA, Issue 1 2007Laura A. Hatfield Summary:,Purpose: Sturge-Weber syndrome (SWS) is a neurocutaneous disorder with vascular malformations of the skin, brain, and eye. SWS results in ischemic brain injury, seizures, and neurologic deficits. We hypothesized that a decrease in quantitative EEG (qEEG) power, on the affected side, correlates with clinical severity in subjects with SWS. Methods: Fourteen subjects had 16-channel scalp EEG recordings. Data were analyzed using fast Fourier transform and calculation of power asymmetry. Blinded investigators assigned scores for clinical neurological status and qualitative assessment of MRI and EEG asymmetry. Results: The majority of subjects demonstrated lower total power on the affected side, usually involving all four frequency bands (delta, theta, alpha, and beta). qEEG asymmetry correlated strongly with neurologic clinical severity scores and MRI asymmetry scores. qEEG data generally agreed with the MRI evidence of regional brain involvement. In MRI-qEEG comparisons that did not agree, decreased power on qEEG in a brain region not affected on MRI was more likely to occur in subjects with more severe neurologic deficits. Conclusions: qEEG provides an objective measure of EEG asymmetry that correlates with clinical status and brain asymmetry seen on MRI. These findings support the conclusion that qEEG reflects the degree and extent of brain involvement and dysfunction in SWS. qEEG may potentially be a useful tool for early diagnosis and monitoring of disease progression in SWS. qEEG may prove useful, in severely affected individuals with SWS, for determining regions of brain dysfunction. [source] Viral encephalitis: a review of diagnostic methods and guidelines for managementEUROPEAN JOURNAL OF NEUROLOGY, Issue 5 2005I. Steiner Viral encephalitis is a medical emergency. The spectrum of brain involvement and the prognosis are dependent mainly on the specific pathogen and the immunological state of the host. Although specific therapy is limited to only several viral agents, correct immediate diagnosis and introduction of symptomatic and specific therapy has a dramatic influence upon survival and reduces the extent of permanent brain injury in survivors. We searched MEDLINE (National Library of Medicine) for relevant literature from 1966 to May 2004. Review articles and book chapters were also included. Recommendations are based on this literature based on our judgment of the relevance of the references to the subject. Recommendations were reached by consensus. Where there was lack of evidence but consensus was clear we have stated our opinion as good practice points. Diagnosis should be based on medical history, examination followed by analysis of cerebrospinal fluid for protein and glucose contents, cellular analysis and identification of the pathogen by polymerase chain reaction (PCR) amplification (recommendation level A) and serology (recommendation level B). Neuroimaging, preferably by magnetic resonance imaging, is an essential aspect of evaluation (recommendation level B). Lumbar puncture can follow neuroimaging when immediately available, but if this cannot be obtained at the shortest span of time it should be delayed only in the presence of strict contraindications. Brain biopsy should be reserved only for unusual and diagnostically difficult cases. All encephalitis cases must be hospitalized with an access to intensive care units. Supportive therapy is an important basis of management. Specific, evidence-based, anti-viral therapy, acyclovir, is available for herpes encephalitis (recommendation level A). Acyclovir might also be effective for varicella-zoster virus encephalitis, gancyclovir and foscarnet for cytomegalovirus encephalitis and pleconaril for enterovirus encephalitis (IV class of evidence). Corticosteroids as an adjunct treatment for acute viral encephalitis are not generally considered to be effective and their use is controversial. Surgical decompression is indicated for impending uncal herniation or increased intracranial pressure refractory to medical management. [source] Craniofacial resection for tumors of the nasal cavity and paranasal sinuses: A 25-year experienceHEAD & NECK: JOURNAL FOR THE SCIENCES & SPECIALTIES OF THE HEAD AND NECK, Issue 10 2006David J. Howard FRCS, FRCS(Ed) Abstract Background. Craniofacial resection is the established "gold standard" for surgical treatment of tumors affecting the anterior skull base. Methods. This study analyzed 308 patients (220 males, 88 females) who had undergone craniofacial resection for sinonasal neoplasia with up to 25-year follow-up. Results. An overall actuarial survival of 65% at 5 years and 47% at 10 years was found for the cohort as a whole. For patients with malignant tumors, the 5-year actuarial survival was 59%, falling to 40% at 10 years. For patients with benign pathology, the actuarial survival was 92% at 5 years falling to 82% at 10 years. Statistical analysis again identified brain involvement, type of malignancy, and orbital involvement as the 3 most significant prognostic factors. Conclusion. Analysis of one of the largest single institution cohorts over a 25-year period provides a baseline against which other approaches such as an entirely endoscopic skull base resection must be judged. © 2006 Wiley Periodicals, Inc. Head Neck, 2006 [source] Electroencephalographic Abnormalities in Aseptic Meningitis and Noninfectious Headache.HEADACHE, Issue 1 2001A Comparative Study Background.,The finding of abnormalities on electroencephalogram (EEG) during the course of aseptic meningitis is often considered to be indicative of parenchymal brain involvement, even in absence of clinical signs of encephalitis. Objective.,To investigate if patients with aseptic nonherpetic meningitis who have abnormal EEG recordings during the acute stage of the disease differ in clinical characteristics or cerebrospinal fluid findings from patients with aseptic meningitis and normal EEG recordings. Methods.,The EEG records of 82 patients with aseptic meningitis were reviewed. A comparative group consisted of 41 age-matched patients with severe headaches without evidence of meningeal inflammation. Results.,Significantly more patients with aseptic meningitis (28%) demonstrated abnormalities on EEG than controls (12%) (P = .048). Patients with aseptic meningitis and abnormal EEG findings (n = 23) did not differ in age, duration of symptoms, clinical course, cerebrospinal fluid cell count, or protein level from those with normal EEG findings (n = 59). However, all patients with aseptic meningitis who were confused (n = 5) also revealed EEG abnormalities (P<.00012). Patients with headache with normal EEG recordings did not differ from those with abnormal EEGs in age, sex, or duration of symptoms. Nevertheless, patients with common migraine (n = 9) showed abnormalities on EEG (P = .06) more frequently. Conclusions.,The finding of an abnormal EEG in patients with aseptic meningitis, clear mental state and absence of focal neurological signs should not be used as proof of encephalitis. Because pathological examination is usually not performed, it remains unclear if EEG abnormalities in patients with aseptic meningitis indicate a silent parenchymal inflammation, or reflect an infectious encephalopathy. [source] Intravascular lymphoma: a neoplasm of ,homeless' lymphocytes?HEMATOLOGICAL ONCOLOGY, Issue 3 2006Maurilio Ponzoni Abstract Intravascular lymphoma (IVL) is an extremely rare form of non-Hodgkin lymphoma characterized by almost exclusive growth of neoplastic lymphocytes within blood vessel lumen. IVL is morphologically characterized in most instances by large cells with B-cell lineage. IVL is an aggressive and usually disseminated disease that predominantly affects elderly patients, resulting in poor PS, B-symptoms, anemia, and high lactate dehydrogenase serum level. The brain and skin are the most commonly involved sites; nodal disease is rare. Survival after conventional chemotherapy is disappointing, with a relevant impact of diagnostic delay and lethal complications. Notwithstanding these results, IVL limited to the skin (cutaneous variant) is a favorable presentation with distinctive clinical characteristics. Moreover, differences in clinical presentation with Eastern Countries IVL cases, mostly associated with hemophagocytic syndrome, do exist. Intensive combinations containing drugs with higher central nervous system bioavailability are needed in cases with brain involvement; the role of high-dose chemotherapy with autologous stem cell transplantation should be investigated in younger patients with unfavorable features. The present review will discuss the most recent acquisitions related either to diagnosis and immunophenotypic/biologic characteristics as well as clinical/therapeutic issues of IVL. Copyright © 2006 John Wiley & Sons, Ltd. [source] Two Patients With Imported Acute Neuroschistosomiasis Due to Schistosoma mansoniJOURNAL OF TRAVEL MEDICINE, Issue 4 2010Laure Houdon MD We report the case of two brothers who returned from Madagascar presenting all the acute phase symptoms of a primary invasive Schistosoma mansoni infection, together with brain involvement characterized by acute encephalitis. This rarely described issue should be considered in travelers returning from endemic areas with acute neurological symptoms. [source] Brain involvement in muscular dystrophies with defective dystroglycan glycosylation,ANNALS OF NEUROLOGY, Issue 5 2008Emma Clement MBChB Objective To assess the range and severity of brain involvement, as assessed by magnetic resonance imaging, in 27 patients with mutations in POMT1 (4), POMT2 (9), POMGnT1 (7), Fukutin (4), or LARGE (3), responsible for muscular dystrophies with abnormal glycosylation of dystroglycan (dystroglycanopathies). Methods Blinded review of magnetic resonance imaging brain scans from 27 patients with mutations in 1 of these 5 genes. Results Brain magnetic resonance images were normal in 3 of 27 patients; in another 5, only nonspecific abnormalities (ventricular dilatation, periventricular white matter abnormalities, or both) were seen. The remaining 19 patients had a spectrum of structural defects, ranging from complete lissencephaly in patients with Walker,Warburg syndrome to isolated cerebellar involvement. Cerebellar cysts and/or dysplasia and hypoplasia were the predominant features in four patients. Polymicrogyria (11/27) was more severe in the frontoparietal regions in 6, and had an occipitofrontal gradient in 2. Pontine clefts, with an unusual appearance to the corticospinal tracts, were seen in five patients with a muscle-eye-brain,like phenotype, three patients with POMGnT1, one with LARGE, and one with POMT2 mutations. Prominent cerebellar cysts were always seen with POMGnT1 mutations, but rarely seen in POMT1 and POMT2. Brainstem and pontine abnormalities were common in patients with POMT2, POMGnT1, and LARGE mutations. Interpretation Our results expand the spectrum of brain involvement associated with mutations in LARGE, POMGnT1, POMT1, and POMT2. Pontine clefts were visible in some dystroglycanopathy patients. Infratentorial structures were often affected in isolation, highlighting their susceptibility to involvement in these conditions. Ann Neurol 2008;64:573,582 [source] Fukutin gene mutations in steroid-responsive limb girdle muscular dystrophyANNALS OF NEUROLOGY, Issue 5 2006Caroline Godfrey BSc Objective Defects in glycosylation of ,-dystroglycan are associated with several forms of muscular dystrophy, often characterized by congenital onset and severe structural brain involvement, collectively known as dystroglycanopathies. Six causative genes have been identified in these disorders including fukutin. Mutations in fukutin cause Fukuyama congenital muscular dystrophy. This is the second most common form of muscular dystrophy in Japan and is invariably associated with mental retardation and structural brain defects. The aim of this study was to determine the genetic defect in two white families with a dystroglycanopathy. Methods The six genes responsible for dystroglycanopathies were studied in three children with a severe reduction of ,-dystroglycan in skeletal muscle. Results We identified pathogenic fukutin mutations in these two families. Affected children had normal intelligence and brain structure and shared a limb girdle muscular dystrophy (LGMD) phenotype, had marked elevation of serum creatine kinase, and were all ambulant with remarkable steroid responsiveness. Interpretation Our data suggest that fukutin mutations occur outside Japan and can be associated with much milder phenotypes than Fukuyama congenital muscular dystrophy. These findings significantly expand the spectrum of phenotypes associated with fukutin mutations to include this novel form of limb girdle muscular dystrophy that we propose to name LGMD2L. Ann Neurol 2006 [source] The prognostic value of ultrasound abnormalities and biological parameters in blood of fetuses infected with cytomegalovirusBJOG : AN INTERNATIONAL JOURNAL OF OBSTETRICS & GYNAECOLOGY, Issue 7 2008G Benoist Objective, To evaluate the prognostic value of ultrasound abnormalities and of selected biological parameters in blood of fetuses infected with cytomegalovirus (CMV). Design, Retrospective observational study. Setting, Two fetal medicine units in Paris, France. Population, All fetuses infected with CMV referred between 1998 and 2006. Methods, We retrospectively analysed data collected prospectively in 73 fetuses infected by CMV with a positive CMV polymerase chain reaction in amniotic fluid. Fetal blood sampling (FBS) was performed for evaluation of platelet count, plasma levels of aminotransferases and gamma-glutamyl transpeptidases (GGT), presence of viraemia and specific fetal immunoglobulin M. Targeted ultrasound examination was performed every fortnight. Ultrasound findings were categorised into normal examination and any ultrasound abnormality, which was further grouped as ultrasound abnormality of the fetal brain and noncerebral ultrasound abnormality. Main outcome measures, A combination of histological findings after termination of pregnancy and evidence of cytomegalic inclusion disease at birth when pregnancies were continued. Clinical symptoms at birth or histological lesions attributable to CMV were considered as poor outcome. Statistical analysis was conducted to determine the value of each parameter to predict outcome. Logistic regression was used to build up a multivariate model combining the relevant parameters. Results, In univariate analysis, only thrombocytopenia and the presence of any ultrasound abnormality were associated with a poor outcome (P < 10,4 for both abnormalities). In the multivariate analysis, both thrombocytopenia and the presence of ultrasound abnormalities remained significant independent predictors of a poor outcome. Based on univariate logistic regression, odds ratio for a poor outcome were 1.24, 7.2, 22.5 and 25.5 for each 10 000/mm3 decrease in platelet count, the presence of noncerebral, any ultrasound and cerebral ultrasound abnormalities, respectively. Conclusions, The prognosis of CMV-infected fetuses relies independently on both targeted ultrasound examination and fetal platelet count. FBS for platelet count may therefore justify FBS in infected fetuses even in the absence of ultrasound. features of brain involvement. [source] Partial cerebellar hypoplasia in a patient with Prader-Willi syndromeACTA PAEDIATRICA, Issue 7 2006Luigi Titomanlio Abstract We report a 3-y-old male infant with Prader-Willi syndrome (PWS) caused by a de novo interstitial deletion of 15q11-q13. Additional features included a right cerebellar hemisphere hypoplasia. The extent of deletion was determined by FISH analysis using an SNRPN PW/AS probe that maps in the PWS/AS critical region (CR) and with specific 15q BACs. We unravelled an interstitial 15q11.2-q13.1 deletion spanning about 3 Mb. Conclusion: To date only a few other PWS patients,including autopsy cases,with CNS structural anomalies have been described. Our case report adds knowledge to the issue of brain involvement in Prader-Willi syndrome. Further MRI studies of PWS patients will be helpful to clarify a correlation between PWS and brain abnormalities. [source] | ||||||||||