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Tarnacka B - - 2009
Wilson's Disease (WD) is a rare autosomal recessive disorder. The literature about proton MR spectroscopy (MRS) in WD is based mostly on data derived from patients undergoing treatment. The aim of this study was to identify brain metabolic changes in newly diagnosed WD patients using MRS to elucidate the pathomechanism ...
Hsieh Te-Chun - - 2009
Chronic subdural hematoma (SDH) is difficult to diagnose by clinical manifestations only. Nonspecific neurologic symptoms and signs may lead physicians to make other diagnoses. Although head trauma is the most common cause, it may be considered insignificant or omitted due to its minor initial manifestations. We present a patient with ...
Tunes Roberto Santos - - 2009
OBJECTIVES: The aim of this study was to describe the clinical characteristics of a series of patients with Behçet's syndrome (BS) seen at a referral centre for connective tissue diseases in Bahia, Brazil. MATERIALS AND METHODS: All the patients diagnosed as having BS by the criteria of the International Study ...
Yamamoto Takehisa - - 2009
BACKGROUND: The aim of the present study was to investigate the predictive parameters for encephalopathy in complete hemolytic uremic syndrome (HUS) in a large outbreak of O157: H7 infection in 1996. METHODS: A total of 182 inpatients, 71 of whom had complete HUS, including 12 patients with neurological complications, and ...
Wiśniewski Marek - - 2009
The clinical manifestations of acute topiramate toxicity are described. Seven cases of acute and acute-on-chronic topiramate toxicity observed in two clinical units of Polish Poison Control Centers in 2004-2005 were analyzed. The patients were 4 women and 2 men aged between 16 and 38 (mean 21.0 +/- 8.4) years. The ...
Liu Hsin-Tzu - - 2009
OBJECTIVE: To measure urinary nerve growth factor (uNGF, essential in nerve growth and regeneration) levels in patients with a cerebrovascular accident (CVA), to determine whether uNGF could be a biomarker for predicting the neurological deficits in CVA, as the level of uNGF increases in patients with idiopathic detrusor overactivity (DO) ...
Cosson M A - - 2009
OBJECTIVE: To better delineate the natural history of patients with methylmalonic aciduria (MMA). STUDY DESIGN: Thirty patients with vitamin-B12-unresponsive MMA (25 aged 1.5 to 22.0 years (y) at the end of the study and 5 who died during a metabolic crisis) were managed following standardized guidelines and studied retrospectively. The ...
Avenel Gilles - - 2009
Syphilis is a sexually transmitted disease caused by the spirochete Treponema pallidum. A chancre usually develops initially. Organ involvement and neurological complications may occur, sometimes several years after the initial exposure. We managed two patients with syphilis responsible for joint or neurological manifestations, diagnosed in 2008. One patient presented with ...
Kyprianou N - - 2009
Phenylketonuria (PKU) is an autosomal recessive disorder resulting in neurological and intellectual disability when untreated. However, even in treated patients there may be residual neurological impairment such as tremor. It has been suggested that the hyperphenylalaninaemia in patients with PKU reduces complex I (NADH:ubiquinone reductase) activity of the mitochondrial respiratory ...
Millichap John J - - 2009
Respiratory syncytial virus is a common cause of infection in children. The authors summarize the clinical and diagnostic features of 9 patients admitted to the pediatric intensive care unit with neurological consultation. Patients were aged 5 weeks to 3 years. Four had seizures, 4 had cardiac arrest, and 1 had ...
Joffe Aaron M - - 2009
The differential diagnosis of new or worsening focal neurologic deficits on emergence from anesthesia is broad. Cerebral ischemia or hemorrhage, focal seizures, and acute metabolic abnormalities can all result in similar neurologic findings. Intravenously administered anesthetic agents also have been reported to cause new or worsening focal neurologic deficits in ...
Topakian Raffi - - 2009
Even with mild neurological signs, patients with botulism frequently complain of autonomic symptoms. This study aimed at the evaluation of sudomotor and cardiovascular reflex functions by quantitative autonomic testing (QAT), which may identify patients with autonomic involvement but otherwise benign clinical presentation. Five patients with food-borne botulism were subjected to ...
Fries Michael - - 2009
PURPOSE: Although animal studies document conflicting data on the influence of hypothermia on cytokine release in various settings, no data exist if hypothermia affects the inflammatory response after successful cardiopulmonary resuscitation. MATERIALS AND METHODS: Arrest- and treatment-related variables of 71 patients were documented, and serum samples were analyzed for levels ...
Kennedy April S - - 2009
BACKGROUND: Patients with apparent complete recovery from thrombotic thrombocytopenic purpura (TTP) often complain of problems with memory, concentration, and fatigue. STUDY DESIGN AND METHODS: Twenty-four patients who were enrolled in the Oklahoma TTP-HUS Registry for their initial episode of TTP, 1995-2006, and who had ADAMTS13 activity of less than 10 ...
Longo Nicola - - 2009
Defects in the metabolism or regeneration of tetrahydrobiopterin (BH4) were initially discovered in patients with hyperphenylalaninaemia who had progressive neurological deterioration despite optimal metabolic control (malignant hyperphenylalaninaemia). BH4 is an essential cofactor not only for phenylalanine hydroxylase, but also for tyrosine and two tryptophan hydroxylases, three nitric oxide synthases, and ...
Ben Turkia H - - 2009
The natural history and clinical presentation of the perinatal-lethal Gaucher's disease, a severe variant of acute type 2 Gaucher's disease, is quite different from classic type 2 Gaucher's disease. Rare reported patients had an overlapping phenotype between these two forms confirming that phenotyping may be difficult. Here we report three ...
Manegold C - - 2009
BACKGROUND: Aromatic L-amino acid decarboxylase (AADC) deficiency is a disorder of biogenic amine metabolism resulting in generalized combined deficiency of serotonin, dopamine and catecholamines. Main clinical features are developmental delay, muscular hypotonia, dystonia, oculogyric crises and additional extraneurological symptoms. Response to therapy has been variable and unsatisfactory; the overall prognosis ...
Gunther Peter
<b>Objectives</b> : Fine motor skills disorders belong to the neurological manifestation of Wilson&#x2032;s disease. The aim of this study is to investigate if fine motor performance changes during the course of the disease and with therapy. <b> Methods</b> : In 15 neurological patients with Wilson&#x2032;s disease, severity of neurological symptoms ...
Lepur Dragan - - 2009
The objective of this open cohort study was to assess the association between neurological complications in patients with definite native-valve infective endocarditis (IE) and cerebral microembolism (MES). MES detection was performed with 1-h, bilateral middle cerebral arteries (MCA) insonation using a transcranial Doppler ultrasound (TCD) machine. Thirty patients with definite ...
Abul-Kasim Kasim - - 2009
There is an ongoing controversy about the significance of tonsillar ectopia among patients with idiopathic scoliosis (IS). To find out if tonsillar ectopia occurs more frequently among patients with IS and if it plays any etiological or prognostic role in IS. Retrospective study. Retrospective analysis of 155 consecutive spine MRIs ...
Anand Jacek Sein - - 2009
Four cases, including three adults and one child, suffering from acute poisoning with Tricholoma equestre were described. The patients had eaten from 100 to 400 grams of the mushroom within a few consecutive meals. After consuming about 1000 grams of Tricholoma equestre for 3-4 days, the subjects developed fatigue, muscle ...
Sethi Prahlad - - 2008
The craniovertebral (CV) junction can be involved in many diseases, e.g. rheumatoid arthritis, as well as destructive bone pathologies such as tumour and tuberculosis (craniovertebral Pott's disease). While some of these patients present acutely with neck pain and neurological deficits, in others the signs and symptoms may be more subtle. ...
Lavon Ophir - - 2008
BACKGROUND: Scombroid fish poisoning is an acute illness caused by consumption of fish containing high concentrations of histamine. Improper handling of fish leads to bacterial contamination. Bacterial enzymes convert histidine to histamine. Symptoms develop quickly and resemble an immunoglobulin E-mediated allergic reaction. The diagnosis is often missed. Serious complications (e.g., ...
Schiffmann Raphael - - 2008
To evaluate the efficacy and safety of miglustat, concomitant with enzyme replacement therapy (ERT), in patients with Gaucher's disease type 3 (GD3). This 24-month, phase II, open-label clinical trial of miglustat in GD3 was conducted in two phases. During the initial 12 months, patients were randomized 2:1 to receive miglustat ...
Kuitwaard K - - 2009
BACKGROUND: Guillain-Barré syndrome (GBS) is generally considered to be monophasic, but recurrences do occur in a presently undefined subgroup of patients. OBJECTIVES: To determine which subgroup of patients develops a recurrence and to establish whether preceding infections and neurological symptoms are similar in subsequent episodes. METHODS: A recurrence was defined ...
Okumura Akihisa - - 2009
OBJECTIVE: To clarify the features of delirious behavior in patients with acute necrotizing encephalopathy. METHODS: We retrospectively evaluated the clinical course of 38 children with acute necrotizing encephalopathy diagnosed on the basis of neuroradiological findings. The patients were divided into two groups according to the presence or absence of delirious ...
Slobbe L - - 2008
Ciguatera toxicity is a type of seafood poisoning caused by the consumption of ciguatoxic reef fish. We describe two patients with characteristic gastrointestinal and neurological symptoms, both of whom had eaten local seafood. Although mortality is low, morbidity can be considerable due to debilitating symptoms. Most cases originate in the ...
Jain Vidhan - - 2008
A study on the clinicoepidemiology of cerebral malaria (CM) and mild malaria (MM) among adults and children attending NSCB medical college hospital Jabalpur and civil hospital Maihar, Satna, in central India was undertaken. Of 1,633 patients, 401 were Plasmodium falciparum and 18 P. vivax. Of 401, 199 CM patients and ...
Premaratna R - - 2008
The main risk of paraquat poisoning is from deliberate ingestion. Serious accidental or occupational poisoning is comparatively rare. We report two patients who had accidental exposure to paraquat, resulting in scrotal burns in both and systemic poisoning in one, while attending to a patient who had ingested paraquat for deliberate ...
Tarnacka Beata - - 2008
The aim of this study was to determine the effectiveness of brain proton magnetic resonance spectroscopy ((1)H-MRS) for monitoring therapy in Wilson's disease (WD) patients. Voxels were located in the globus pallidus (right, left). We followed 17 newly diagnosed WD cases for 1-year period. During this observation period, 6 neurological ...
Anttinen Anu A Department of Neurology, Turku University Central Hospital, PB 52, 20521 Turku, Finland. - - 2008
We have prospectively followed 16 Finnish xeroderma pigmentosum (XP) patients for up to 23 years. Seven patients were assigned by complementation analysis to the group XP-A, two patients to the XP-C group and one patient to the XP-G group. Six of the seven XP-A patients had the identical mutation (Arg228Ter) ...
Keleş Ayfer - - 2008
INTRODUCTION: Carbon monoxide (CO) is a colorless, odorless and nonirritating gas. CO poisoning is a preventable cause of morbidity and mortality. After the late diagnosis of some CO-poisoned patients, we undertook a chart review to identify characteristics of patients with CO poisoning to help with management of future patients. METHODS: ...
Chee Kerlen J - - 2008
INTRODUCTION: The diagnosis of carbon monoxide poisoning can be difficult because the symptoms are nonspecific and may mimic other illnesses. If carbon monoxide poisoning is suspected, the standard test at this time is venous or arterial carboxyhemoglobin levels. A new device, the Rad-57 pulse CO-oximeter (Masimo Inc.), can measure carboxyhemoglobin ...
Erbetta Alessandra - - 2008
Antitumor and immunosuppressant treatment-related neurotoxicity can determine nonspecific clinical syndromes. Exclusion of other possible causes, among which tumor progression, appearance of paraneoplastic disease, renal or hepatic failure, diabetes or hypertension, is relevant. We report clinical and neuroradiological features in five patients with neurotoxic syndromes due to chemotherapy/radiotherapy or immunosuppression in ...
Aghakhani Nozar - - 2008
OBJECTIVE: Surgery should be considered for patients with intramedullary spinal ependymomas (ISE), particularly those presenting with a neurological deficit preoperatively. In contrast, it is still a debatable matter whether to recommend the same approach for patients with no neurological impairment. To investigate this matter, we analyzed the data of 82 ...
Lin Bing-Liang - - 2008
To analyze the clinical presentation of venous diethylene glycol (DEG) poisoning in patients with preexisting severe liver disease and factors that correlate with DEG poisoning. Retrospective chart review was performed to analyze the epidemiology, clinical presentation, hepatorenal functions, hemodynamics and pathological characteristics of 64 patients with severe liver disease who ...
Hoffman Robert S - - 2008
STUDY OBJECTIVE: Illicit drugs may be adulterated with substances other than the sought-after substance of abuse. Although the true incidence and clinical effects of this practice are unknown, geographically disparate outbreaks of clinically significant adulteration continue to occur. We report on a recent outbreak of clenbuterol-adulterated heroin occurring along the ...
Kuemmerle-Deschner J B - - 2008
OBJECTIVE: To report the successful treatment with recombinant human IFN- alpha 2a (rhIFN-alpha2a) in two male adolescents suffering from severe treatment-resistant Behçet's disease (BD) with central nervous system (CNS) involvement. METHODS: The patients were 14- and 15-yrs old. Both met the International Study Group for Behçet's disease, O'Duffy and the ...
Schlump J-U - - 2008
Neurological crises do not occur in patients with tyrosinaemia type I treated with NTBC. We report an 8 month-old boy with severe neurological crisis after interruption of NTBC treatment including progressive ascending polyneuropathy and diaphragmatic paralysis, arterial hypertension, respiratory distress requiring mechanical ventilation who later also developed impaired liver function and ...
Bailey Benoit - - 2008
OBJECTIVES: To assess the prevalence of neurologic and neuropsychological symptoms in the short-term and 1 year after an electric shock and to explore whether any of these were associated with risk factors. METHODS: Patients presenting to one of 21 EDs between October 2000 and November 2004 were eligible to be ...
Mariconda M - - 2008
We have studied 180 patients (128 men and 52 women) who had undergone lumbar discectomy at a mean of 25.4 years (20 to 32) after operation. Pre-operatively, most patients (70 patients; 38.9%) had abnormal reflexes and/or muscle weakness in the leg (96 patients; 53.3%). At follow-up 42 patients (60%) with ...
Morita Seiji - - 2010
Self-induced water intoxication (SIWI) patients present with various neurological and non-neurological symptoms. However, it is reported that non-neurological manifestations such as rhabdomyolysis are comparatively rare. The mechanism underlying rhabdomyolysis remains controversial. To investigate this further, we evaluated 22 SIWI patients for rhabdomyolysis. We reviewed the records of 22 patients with ...
Lapostolle Frédéric - - 2008
OBJECTIVE: To evaluate the use of antidotal therapy in patients with an elevated digitalis concentration following chronic or acute exposure. DESIGN AND SETTING: Retrospective review of patient records over 2 years in 20 city hospitals in France. PATIENTS: Overall 838 patients with an elevated serum digitalis concentration (digoxin > 1.95ng ...
Miesbach Wolfgang - - 2008
Antiphospholipid antibodies may cause various neurologic diseases by vascular and immune mechanisms. Ischemic stroke and transient ischemic attacks are the most common neurologic complications in patients with antiphospholipid antibodies but migraine, epilepsy, chorea, or multiple sclerosis also may occur. Although the neurologic presentation of patients with the antiphospholipid syndrome may ...
Ravindra B S - - 2008
Celiac disease (CD) has long been known to be associated with neurological and psychiatric manifestations; its in association with myotonic dystrophy however has not yet been reported. We report the case of a 27-year old female patient who presented to us with diarrhoea, weight loss, easy fatigability, irritability and alopecia ...
Rehiman S - - 2008
The aim of the study was to correlate between the clinical score described by Peradenya Organophosphorus Poisoning (POP) scale, serum cholinesterase level at presentation and severity of poisoning. Consecutive patients of organophosphorous poisoning attending Bir Hospital from August 2004 to September 2005 were studied. Patients were grouped into mild, moderate ...
Stepens Ainārs - - 2008
BACKGROUND: A distinctive extrapyramidal syndrome has been observed in intravenous methcathinone (ephedrone) users in Eastern Europe and Russia. METHODS: We studied 23 adults in Latvia who had extrapyramidal symptoms and who had injected methcathinone for a mean (+/-SD) of 6.7+/-5.1 years. The methcathinone was manufactured under home conditions by potassium ...
Karbakhsh Mojgan - - 2008
INTRODUCTION: Poisoning is considered a significant health problem in the elderly. This study aimed to portray the pattern of poisoning in the elderly population of Tehran. METHODS: This cross-sectional study included all patients aged 60 years and older with acute poisoning who attended the emergency department of the Loghman-Hakim hospital ...
Burke Michael J - - 2008
Opsoclonus-myoclonus syndrome (OMS) is a rare paraneoplastic syndrome that occurs in 2%-3% of patients with neuroblastoma. The cause of this syndrome is believed to be immune mediated, but the exact mechanism still remains unclear. There is an urgent need to improve our current strategies for treating patients with OMS, as ...
Wasay Mohammad - - 2008
We report three cases of ciguatera fish poisoning. One patient died secondary to respiratory failure. Two patients showed elevated muscle enzymes and one patients had an abnormal cervical spinal MRI. MRI findings have not been previously described. MRI findings explain the mechanism of the L'hermitte phenomenon (a common complaint) among ...
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