Search Results
Results 201 - 250 of 889
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da Silva-Júnior F P - - 2008
Although dysphagia is a common complaint of patients with Wilson's disease (WD) and pneumonia is an important cause of death in these patients, swallowing function remains an underinvestigated field in this condition. The aim of this study was to characterize swallowing dynamics in WD patients. Eight WD patients and 15 ...
Wraith J Edmond - - 2008
Mucopolysaccharidosis type II (MPS II; Hunter syndrome) is a rare X-linked recessive disease caused by deficiency of the lysosomal enzyme iduronate-2-sulphatase, leading to progressive accumulation of glycosaminoglycans in nearly all cell types, tissues and organs. Clinical manifestations include severe airway obstruction, skeletal deformities, cardiomyopathy and, in most patients, neurological decline. ...
Wang Ping - - 2007
PURPOSE: The purpose of this study was to determine the clinical value of applying Tc-99m TRODAT-1 single photon emission computed tomography (SPECT) of the dopamine transporter to evaluate disease severity in young patients with symptomatic or asymptomatic Wilson disease (WD). MATERIALS AND METHODS: The evaluation of Tc-99m TRODAT-1 SPECT was ...
Kudo Hiroshi - - 2007
Treatment for severe cerebellar infarction has been controversial. Clinical outcomes of patients with external ventricular drainage (EVD) and decompression as the first treatment for the infarction were compared. A total of 25 patients with severe cerebellar infarction were subdivided into two groups to compare outcome of the group (group A) ...
Kumar Raj - - 2007
OBJECTIVE: The assessment of response to treatment in pediatric patients with congenital atlantoaxial dislocation (AAD) is performed using a disability grading system but may be better determined by a score based on clinical parameters. This study proposes a scoring system based on a comprehensive neurological examination to assess surgical outcome ...
Salvarani Carlo - - 2007
OBJECTIVE: To analyze the clinical findings, response to therapy, outcome, and incidence of primary central nervous system vasculitis (PCNSV) in a large cohort from a single center. METHODS: We retrospectively studied 101 patients with PCNSV, selected by predetermined diagnostic criteria, who were seen during a 21-year period. This was a ...
López-Laso E - - 2007
Late-onset neurological disease has rarely been reported in patients with glutaryl-CoA dehydrogenase (GCDH) deficiency. We present two siblings with GCDH deficiency. One of them presented with the classic neurological disease (patient 1). Routine investigation of family members revealed that her apparently unharmed 13-year-old sister was also affected (patient 2). Patient ...
Brimioulle Serge - - 2008
OBJECTIVE: To assess whether hyponatremia in acute neurological patients is associated with the syndrome of inappropriate antidiuretic hormone secretion (SIADH) or with the cerebral salt-wasting syndrome (CSWS). DESIGN: Clinical, controlled, prospective study. SETTING: Department of intensive care of a tertiary care academic hospital. PATIENTS: Forty acute neurological patients with hyponatremia ...
García-Cazorla A - - 2007
Neurotransmitters are essential in young children for differentiation and neuronal growth of the developing nervous system. We aimed to identify possible factors related to secondary neurotransmitter abnormalities in pediatric patients with neurological disorders. We analyzed cerebrospinal fluid (CSF) and biogenic amine metabolites in 56 infants (33 males, 23 females; mean ...
Ding Ding - - 2007
We traced 85 Japanese encephalitis (JE) patients, 6-27 years after hospitalizations. The first control group was made up of 73 non-JE encephalitis patients 6-27 years previously, whereas the second control group was made up 78 neighborhood residents, matched to the 78 surviving JE cases by age, sex, and residence. All ...
Westermaier Thomas - - 2007
OBJECTIVE: Spontaneous acute subdural hematoma (aSDH) may be caused by aneurysm rupture. Patients can present in very poor clinical condition with anisocoria or even bilaterally dilated pupils, absent brainstem reflexes, and cardiac insufficiency. For the clinician, the question is how should these patients be treated? Large series on this subject ...
Azevedo José Raimundo A de - - 2007
OBJECTIVE: To compare intensive insulin therapy to conventional glycemic control in patients with acute neurological injury evaluating neurological outcome and morbimortality. METHOD: Patients with two glycemias above 150 mg/dL 12 hours after admission were randomized to receive intensive insulin therapy (G1) or conventional treatment (G2). We evaluated a subgroup of ...
Rasmussen Keith G - - 2007
There are relatively few case reports of electroconvulsive therapy (ECT) in patients with multiple sclerosis. We present 3 such patients, all of whom received safe, effective ECT without evidence of acute neurological deterioration. We conclude that patients with multiple sclerosis being considered for ECT should have a thorough neurological evaluation, ...
Erguven M - - 2007
OBJECTIVE: We aimed to review characteristics of mushrooms and mushroom poisoning and compare clinical picture, laboratory data, treatment modalities and prognostic factors in children with amanita intoxication and non-amanita mushroom poisoning. METHODS: We analyzed 39 pediatric patients through 1994-2004, retrospectively from the patient files and evaluated the patients in two ...
Capablo J L - - 2008
Type 1 Gaucher disease (GD1) is characterised by lack of central nervous system involvement; however, there are several reports of associated neurological manifestations. The aim of this study was to systematically evaluate neurological manifestations in 31 patients with GD1 (12 males and 19 females; mean age 39.4 (range 5-77) years). ...
Lovrić Mila - - 2007
A 40-year-old man was admitted to the emergency department after a suicide attempt. The patient was found at home unconscious, with an open bottle of antifreeze near him. The patient was in a coma on admission, but neurological examination excluded intracranial changes. Results of initial urine and serum toxicological screening ...
Prikryl Radovan - - 2007
OBJECTIVES: To examine the relationship between the severity of neurological soft signs at onset and at the 1-year follow-up of patients with schizophrenia, and to investigate temporal stability of neurological soft signs within 1year from the onset of the first episode schizophrenia. METHODS: The study included 92 first-episode male schizophrenic ...
Talaie H - - 2007
Rhabdomyolysis is a clinical and biochemical syndrome occurring when skeletal muscle cells erupt and result in release of creatine phosphokinase (CPK), lactate dehydrogenase (LDH) and myoglobin into the interstitial space and plasma. Mechanical trauma, compression, excessive muscle activity and ischemia are frequent causes, but non-traumatic rhabdomyolysis is usually caused by ...
Wallace Patricia A - - 2007
OBJECTIVE: Sacral nerve neuromodulation (SNS) is an effective treatment for lower urinary tract dysfunction. Many underlying neurologic processes affect lower urinary tract function. We present results of SNS in patients with underlying neurologic dysfunction. STUDY DESIGN: This is a retrospective case series of 33 patients with neurologic disease and lower ...
Wellons John C JC - - 2007
Patients with Chiari malformation Type I (CM-I) most commonly present with chronic symptoms. A search of the current medical literature revealed scant information regarding acute presentations of CM-I in either pediatric or adult patients. The authors report on two children who presented with rapidly worsening neurological symptoms attributable to a ...
Low M - - 2007
BACKGROUND: Fabry disease has diverse neurological manifestations, many of which influence morbidity and quality of life. AIMS: The aim of the study was to document the clinical and subclinical neurological manifestations in a cohort of Australian patients with Fabry disease, using multiple clinical tools and a multidisciplinary approach. METHODS: Participants ...
Chien Y-H - - 2007
Niemann-Pick disease type C (NP-C) is a lipid storage disorder characterized by the accumulation of unesterified cholesterol and glycolipids in the lysosomal/late endosomal system of certain cells in the central nervous system (CNS) and visceral organs. Clinical symptoms include progressive neurological deterioration and visceral organomegaly. Miglustat, a small iminosugar molecule ...
Chamberlain Marc C - - 2007
BACKGROUND: Acute binocular blindness is an uncommon cause for neurologic consultation. METHODS: Over the last 20 months 10 patients with acute onset blindness were seen at a tertiary cancer center. Probable cause was identified in all patients. RESULTS: Treatment varied and was determined by etiology of visual loss. CONCLUSIONS: Six ...
Matsumoto Lumine - - 2007
We report a 40-year-old man with severe hypokinesis as paraneoplastic manifestation of a microscopic "carcinoma in situ" of the testis. The young age of the patient, along with progressive neurologic deterioration, detection of anti-Ma2 antibodies, and ultrasound findings of bilateral microcalcifications, led to bilateral orchiectomy, revealing the tumor in both ...
Gomceli Y B - - 2007
Valproate is an effective anticonvulsant. Although it is usually well tolerated, it has been associated with many neurological, hematopoietic, hepatic, and digestive system side effects. Among these side effects, hyperammonemia without clinical or laboratory evidence of hepatotoxicity is rare and is an important clinical consideration. The aim of this article ...
Sisillo Erminio - - 2007
OBJECTIVE: Cerebrovascular accidents (CVA) are devastating complications after coronary artery bypass grafting (CABG). The reported incidence of neurological complications after conventional CABG (CCABG) is 3-6%. Off-pump coronary bypass grafting (OPCAB) has been associated in recent studies to a decreased morbidity and risk of perioperative stroke. Nevertheless, uncertainty still surrounds the ...
Debray François-Guillaume - - 2007
OBJECTIVES: We sought to determine the clinical spectrum, survival, and long-term functional outcome of a cohort of pediatric patients with mitochondrial diseases and to identify prognostic factors. METHODS: Medical charts were reviewed for 73 children diagnosed between 1985 and 2005. The functional status of living patients was assessed prospectively by ...
Halperin A - - 2007
BACKGROUND: A previous epidemiological survey from an American referral clinic noted a high incidence of neurological symptoms among patients with type I (non-neuronopathic) Gaucher disease all of whom were treated with specific enzyme replacement. OBJECTIVES: The current study replicates the above in a larger cohort of Ashkenazi Jewish patients with ...
Muen Wisam J - - 2007
PURPOSE: We report a case series of seven patients who presented with strabismus and no apparent known neurological deficit. METHODS: A retrospective review of notes was performed on all patients presenting at the Royal Liverpool Children's Hospital (Alder Hey) with strabismus, in whom subsequent investigation revealed the presence of periventricular ...
Taly Arun B - - 2007
The clinical manifestations of Wilson disease (WD) are varied and challenging. We conducted the current study to present the phenotypic characteristics and follow-up for a large cohort of patients with WD. We reviewed the medical records of 282 cases of WD (male:female ratio, 196:86) for clinical features, investigations, treatment, and ...
Markoula S - - 2007
OBJECTIVES: We present the epidemiological and clinical-laboratory features of Guillain-Barré syndrome (GBS) in northwest Greece over a 9.5-year period. MATERIALS AND METHODS: We studied all the patients with GBS who were admitted to our neurology inpatient service from January 1996 to May 2005 and compared them with previously published series. ...
Haefner Mark D - - 2007
BACKGROUND: We report the case of a patient who experienced a severe neurologic complication after treatment of diffuse large B-cell lymphoma. CASE REPORT: A 62-year old patient was diagnosed with a diffuse large B-cell lymphoma and treated with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone under prophylactic G-CSF substitution. After the ...
Hung Y-M - - 2007
BACKGROUND: Yam bean is a common food in southern Taiwan. However, its seeds are rarely consumed. We describe five patients of yam bean seed poisoning in Taiwan, one of them life-threatening. CLINICAL PRESENTATION: The five patients presented with perioral numbness, nausea and vomiting after eating a same soup made from ...
Minn A Yuriko - - 2007
BACKGROUND: Despite decreases in overall mortality following bone marrow transplantation (BMT), a number of complications such as neurotoxicity have been described and often associated with immunosuppressive agents. The syndrome of reversible posterior leukoencephalopathy has been described in patients receiving cyclosporin and FK-506. We report here a subset of children who ...
Yücesan Canan - - 2007
Therapeutic plasma exchange (TPE) has been used for the treatment of neurologic diseases in which autoimmunity plays a major role. We reviewed the medical records of our patients who had consecutively been treated by TPE between January 1998 and June 2000. Neurological indications included myasthenia gravis (30 patients), multiple sclerosis ...
Mittheisz Edda - - 2007
BACKGROUND: Permanent consequences in Langerhans cell histiocytosis (LCH) are irreversible late sequelae related to the disease that may severely impair the quality of life of survivors. The frequency and pattern of permanent consequences affecting the central nervous system (CNS) remains to be determined. PROCEDURE: In this single center study, 25 ...
Chan Raymond C K - - 2007
BACKGROUND: This study attempted to examine the prevalence and type of neurological signs in Chinese patients with schizophrenia. METHODS: A cross-sectional design was adopted with the use of the Cambridge Neurological Inventory (CNI). The CNI is comprised of 7 subscales, including motor coordination, sensory integration, disinhibition, extrapyramidal signs, dyskinesia, catatonia, ...
Kalita J - - 2007
There is paucity of studies on predictors of long-term sequelae of tuberculous meningitis (TBM). We report the neurological sequelae of TBM at 1 year and their predictors. Patients with TBM who were followed up for 1 year were included. The diagnosis of TBM was based on clinical, cerebrospinal fluid (CSF) ...
Pae Walter E - - 2007
BACKGROUND: Neurologic events such as thromboembolic and hemorrhagic strokes are common complications of mechanical circulatory support. We report the neurologic events observed in patients treated for end-stage heart failure with the implantable, pulsatile LionHeart left ventricular assist device (LVAD). This sub-study was part of the LionHeart European Clinical Utility Baseline ...
Gaul Charly - - 2007
BACKGROUND AND PURPOSE: Aortic dissection typically presents with severe chest or back pain. Neurological symptoms may occur because of occlusion of supplying vessels or general hypotension. Especially in pain-free dissections diagnosis can be difficult and delayed. The purpose of this study is to analyze the association between type A aortic ...
Barone R - - 2007
CDG Ia (phosphomannomutase deficiency) has a wide clinical spectrum with the most severe affected patients having multisystemic disease in addition to severe nervous system involvement. We report a patient with CDG Ia and an intermediate phenotype due to mild neurological impairment and borderline cognitive abilities despite the occurrence of typical ...
Tunc Tugba, Ortapamuk Hulya, ...
Context: Behηet′s disease (BD) is a multisystem inflammatory disorder with unknown etiology characterized by recurrent oral and genital aphthous ulcers and uveitis. Behçet's disease can affect the central nervous system. Aims: We aimed to investigate subclinical neurological involvement in patients who were suffering from BD and who had no neurological ...
Tunç Tugba - - 2006
CONTEXT: Behçet's disease (BD) is a multisystem inflammatory disorder with unknown etiology characterized by recurrent oral and genital aphthous ulcers and uveitis. Behçet's disease can affect the central nervous system. AIMS: We aimed to investigate subclinical neurological involvement in patients who were suffering from BD and who had no neurological ...
Anderson Steven W - - 2006
OBJECTIVE: Subarachnoid hemorrhage and surgical obliteration of ruptured intracranial aneurysms are frequently associated with neurological and neuropsychological abnormalities. We reported that intraoperative cooling did not improve neurological outcome in good-grade surgical subarachnoid hemorrhage patients, as assessed by the Glasgow Outcome Scale score or other neurological and functional measures (National Institutes ...
Wasserstein Melissa P - - 2006
OBJECTIVE: To document the prevalence of neurologic disease in Niemann-Pick disease (NPD) NPD-B. STUDY DESIGN: Sixty-four patients with NPD-B had detailed neurologic and ophthalmologic evaluations. The presence of neurologic abnormalities was compared with genotype. RESULTS: Nineteen of 64 patients (30%) had neurologic abnormalities, which were minor and nonprogressive in 14 ...
Borhani-Haghighi Afshin - - 2006
OBJECTIVE: To determine the prevalence, clinical manifestations, and laboratory features of Neuro-Behçet's disease. METHODS: This prospective study was carried out in the Behçet's Research Clinic in Shiraz (south-west Iran) and included the patients referred from 1990-1999. The patients' clinical records, images, CSF analyses, and electrodiagnostic studies were reviewed. RESULTS: Eighteen ...
Kumar Neeraj N Department of Neurology, Mayo Clinic College of Medicine, 200 First St SW, Rochester, MN 55905, USA. - - 2006
The hematologic manifestations of copper deficiency are well known and include anemia and neutropenia. In the past few years, the neurological manifestations of acquired copper deficiency in humans has been recognized, the most common being a myelopathy presenting with a spastic gait and prominent sensory ataxia. The known causes of ...
Mueller Antje - - 2006
Wilson's disease is a rare autosomal recessive disorder characterized by the accumulation of copper, mainly in the liver and the brain. As copper accumulation in the brain leads to disturbances in basal ganglia function, neurological-type patients typically present with hypo- and hyperkinetic extrapyramidal symptoms, with Parkinsonism being very common. Although ...
Wittstock Matthias - - 2006
Therapy with intravenous immunoglobulins (IVIg) is considered to be a safe treatment for a number of immune-mediated neurological diseases. Published data about prevalence of adverse effects range from 11 to 81%. The purpose of our study was to preserve a representative view on adverse effects by analysis of a large ...
Yesilot N - - 2006
OBJECTIVE: The aim of this study was to determine the long term clinical course and prognosis of subclinical ('silent') neurological involvement in Behçet's disease (BD). METHODS: We included patients with BD who did not have any neurological complaints other than headache, dizziness or other non-specific complaints, that showed abnormal neurological ...
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