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Neurological Manifestations (neurological + manifestation)
Selected AbstractsHow hypoglycaemia can affect the life of a person with diabetesDIABETES/METABOLISM: RESEARCH AND REVIEWS, Issue 2 2008Brian M. Frier Abstract Hypoglycaemia is the commonest side-effect of insulin treatment for diabetes, and is the single greatest barrier to achieving and maintaining good glycaemic control. Severe hypoglycaemia (requiring assistance for recovery) is associated with significant morbidity and is feared by most people with type 1 diabetes and their families. It causes stress and anxiety and may influence self-management and glycaemic control. The annual prevalence of severe hypoglycaemia is around 30% in people with type 1 diabetes, and is higher in those with risk factors such as strict glycaemic control, impaired awareness of hypoglycaemia and increasing duration of diabetes. It is also common during sleep (nocturnal hypoglycaemia). Neurological manifestations include coma, convulsions, transient hemiparesis and stroke, while reduced consciousness and cognitive dysfunction may cause accidents and injuries. Cardiac events may be precipitated such as arrhythmias, myocardial ischaemia and cardiac failure. Hypoglycaemia can affect all aspects of life, including employment, driving, recreational activities involving exercise, and travel, and measures should be taken in all of these situations to avoid this potentially dangerous side-effect of insulin therapy. Copyright © 2007 John Wiley & Sons, Ltd. [source] Neurological manifestations of antiphospholipid syndromeEUROPEAN JOURNAL OF CLINICAL INVESTIGATION, Issue 4 2010Carlos E. M. Rodrigues Eur J Clin Invest 2010; 40 (4): 350,359 Abstract Background, Neurologic disorders are among the most common and important clinical manifestations associated with the antiphospholipid syndrome (APS). It is characterized by diverse neurological manifestations. These include stroke, transient ischaemic attack, Sneddon's syndrome, convulsions/epilepsy, dementia, cognitive deficits, headaches/migraine, chorea, multiple sclerosis-like, transverse myelitis, ocular symptoms and Guillain,Barré syndrome. Material and methods, We review the latest data about neurologic disorders and APS. Results, In patients under 45 years of age, 20% of strokes are potentially associated with APS. Our study group recently reported a correlation between primary APS and peripheral neuropathy. Only one study investigated the occurrence of peripheral neuropathy in patients diagnosed with PAPS through electrophysiological study and showed alterations in 35% of patients. The mechanism of nervous system involvement in APS is considered to be primarily thrombotic. However, other mechanisms have been described, such as antiphospholipid antibodies that bind to the neural tissue, deregulating their functions and having an immediate pathogenic effect. Conclusions, This review summarizes the latest data regarding the clinical aspects, radiological and therapeutic of major neurologic manifestations associated with antiphospholipid antibodies. [source] How to deal with Behcet's disease in daily practiceINTERNATIONAL JOURNAL OF RHEUMATIC DISEASES, Issue 2 2010Fereydoun DAVATCHI Abstract Introduction:, Behcet's Disease (BD) is classified as a vasculitis, and progresses via attacks and remissions. BD is mainly seen around the Silk Road. The picture varies in different reports. For clinical descriptions, the data from the international cohort of patients (27 countries), will be used. Clinical manifestations:, Mucous membrane manifestations were oral aphthosis seen in 98.1%, and genital aphthosis in 76.9% of patients. Skin manifestations were seen in 71.9% (pseudofolliculitis in 53.6% and erythema nodosum in 33.6%). Ocular manifestations were seen in 53.7% (anterior uveitis 38.8%, posterior uveitis 36.9%, retinal vasculitis 23.5%). Joint manifestations were seen in 50.5% (arthralgia, monoarthritis, oligo/polyarthritis, ankylosing spondylitis). Neurological manifestations were seen in 15.5% of patients (central 11.5%, peripheral 4.4%). Gastrointestinal manifestations were seen in 6.3% of patients. Vascular involvement was seen in 18.2% of patients and arterial involvement in 3% (thrombosis, aneurysm, pulse weakness). Deep vein thrombosis was seen in 8%, large vein thrombosis in 6.5%, and superficial phlebitis in 5.8%. Orchitis and epididymitis were seen in 7.2%. Pathergy test was positive in 49.3% and HLA-B51 in 49.1% of patients. Diagnosis:, Diagnosis is based on clinical manifestations. The International Criteria for Behcet's Disease (ICBD) may be helpful. Treatment:, The first line treatment is colchicine (1 mg daily) for mucocutaneous manifestations, non-steroidal anti-inflammatory drugs for joint manifestations, anticoagulation for vascular thrombosis, and cytotoxic drugs for ocular and brain manifestations. If incomplete response or resistance occurs, therapeutic escalation is worthwhile. Conclusion:, Behcet's disease is a systemic disease characterized by mucocutaneous, ocular, vascular and neurologic manifestations, progressing by attacks and remissions. [source] Neurological disorders in complex humanitarian emergencies and natural disastersANNALS OF NEUROLOGY, Issue 3 2010Farrah J. Mateen MD Complex humanitarian emergencies include the relatively acute, severe, and overwhelming health consequences of armed conflict, food scarcity, mass displacement, and political strife. Neurological manifestations of complex humanitarian emergencies are important and underappreciated consequences of emergencies in populations worldwide. This review critically assesses the existing knowledge of the range of neurological disorders that accompany complex humanitarian emergencies and natural disasters in both the acute phase of crisis and the "long shadow" that follows. Ann Neurol 2010;68:282,294 [source] Restoration of central nervous system ,- N -acetylglucosaminidase activity and therapeutic benefits in mucopolysaccharidosis IIIB mice by a single intracisternal recombinant adeno-associated viral type 2 vector deliveryTHE JOURNAL OF GENE MEDICINE, Issue 7 2010Haiyan Fu Abstract Background Finding efficient central nervous system (CNS) delivery approaches has been the major challenge facing therapeutic development for treating diseases with global neurological manifestation, such as mucopolysaccharidosis (MPS) IIIB, a lysosomal storage disease, caused by autosomal recessive defect of ,- N -acetylglucosaminidase (NaGlu). Previously, we developed an approach, intracisternal (i.c.) injection, to deliver recombinant adeno-associated viral (rAAV) vector to the CNS of mice, leading to a widespread periventricular distribution of transduction. Methods In the present study, we delivered rAAV2 vector expressing human NaGlu into the CNS of MPS IIIB mice by an i.c. injection approach, to test its therapeutic efficacy and feasibility for treating the neurological manifestation of the disease. Results We demonstrated significant functional neurological benefits of a single i.c. vector infusion in adult MPS IIIB mice. The treatment slowed the disease progression by mediating widespread recombinant NaGlu expression in the CNS, resulting in the reduction of brain lysosomal storage pathology, significantly improved cognitive function and prolonged survival. However, persisting motor function deficits suggested that pathology in areas outside the CNS contributes to the MPS IIIB behavioral phenotype. The therapeutic benefit of i.c. rAAV2 delivery was dose-dependent and could be attribute solely to the CNS transduction because the procedure did not lead to detectable transduction in somatic tissues. Conclusions A single IC rAAV2 gene delivery is functionally beneficial for treating the CNS disease of MPS IIIB in mice. It is immediately clinically translatable, with the potential of improving the quality of life for patients with MPS IIIB. Copyright © 2010 John Wiley & Sons, Ltd. [source] Seeing the phantom: A functional magnetic resonance imaging study of a supernumerary phantom limb,ANNALS OF NEUROLOGY, Issue 6 2009Asaid Khateb PhD Objective Supernumerary phantom limb (SPL) is a rare neurological manifestation where patients with a severe stroke-induced sensorimotor deficit experience the illusory presence of an extra limb that duplicates a real one. The illusion is most often experienced as a somesthetic phantom, but rarer SPLs may be intentionally triggered or seen. Here, we report the case of a left visual, tactile, and intentional SPL caused by right subcortical damage in a nondeluded woman. Methods Using functional magnetic resonance imaging, we investigated the multimodal nature of this phantom, which the patient claimed to be able see, use, and move intentionally. The patient participated in a series of sensorimotor and motor imagery tasks involving the right, the left plegic, and the SPL's hand. Results Right premotor and motor regions were engaged when she imagined that she was scratching her left cheek with her left plegic hand, whereas when she performed the same task with the SPL, additional left middle occipital areas were recruited. Moreover, comparison of responses induced by left cheek (subjectively feasible) versus right cheek scratching (reportedly unfeasible movement) with the SPL demonstrated significant activation in right somesthetic areas. Interpretation These findings demonstrate that intentional movements of a seen and felt SPL activate premotor and motor areas together with visual and sensory cortex, confirming its multimodal dimension and the reliability of the patient's verbal reports. This observation, interpreted for cortical deafferentation/disconnection caused by subcortical brain damage, constitutes a new but theoretically predictable entity among disorders of bodily awareness. Ann Neurol 2009;65:698,705 [source] Sustained-release bupropion overdose: A new entity for Australian emergency departmentsEMERGENCY MEDICINE AUSTRALASIA, Issue 1 2002Richard Paoloni Abstract Bupropion hydrochloride (Zyban, Glaxo Wellcome Australia, Melbourne, Vic., Australia) was released in Australia in November 2000 as adjunctive therapy to assist with smoking cessation, having previously been used as an antidepressant in the US since 1989. The toxicity profile of bupropion hydrochloride in overdose differs considerably from other antidepressants, with prominent neurological manifestations and little cardiovascular toxicity. A case of bupropion overdose demonstrating the typical toxic syndrome is presented, together with a review of the literature and a discussion of the magnitude of the demand for bupropion and of the potential differences in presentation of overdoses in Australia. [source] Neurological manifestations of antiphospholipid syndromeEUROPEAN JOURNAL OF CLINICAL INVESTIGATION, Issue 4 2010Carlos E. M. Rodrigues Eur J Clin Invest 2010; 40 (4): 350,359 Abstract Background, Neurologic disorders are among the most common and important clinical manifestations associated with the antiphospholipid syndrome (APS). It is characterized by diverse neurological manifestations. These include stroke, transient ischaemic attack, Sneddon's syndrome, convulsions/epilepsy, dementia, cognitive deficits, headaches/migraine, chorea, multiple sclerosis-like, transverse myelitis, ocular symptoms and Guillain,Barré syndrome. Material and methods, We review the latest data about neurologic disorders and APS. Results, In patients under 45 years of age, 20% of strokes are potentially associated with APS. Our study group recently reported a correlation between primary APS and peripheral neuropathy. Only one study investigated the occurrence of peripheral neuropathy in patients diagnosed with PAPS through electrophysiological study and showed alterations in 35% of patients. The mechanism of nervous system involvement in APS is considered to be primarily thrombotic. However, other mechanisms have been described, such as antiphospholipid antibodies that bind to the neural tissue, deregulating their functions and having an immediate pathogenic effect. Conclusions, This review summarizes the latest data regarding the clinical aspects, radiological and therapeutic of major neurologic manifestations associated with antiphospholipid antibodies. [source] PET-CT imaging of combined brachial and lumbosacral neurolymphomatosisEUROPEAN JOURNAL OF HAEMATOLOGY, Issue 1 2005Pazit Kanter Abstract:, Neurolymphomatosis is a rare manifestation of progressive non-Hodgkin's lymphoma. A 44-yr-old man with diffuse large B-cell lymphoma presented with unilateral progressive peripheral sensorimotor neuropathy after the 7th cycle of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone) therapy. No pathology in the nervous system was evident by computerized tomography (CT), magnetic resonance imaging (MRI) of the head, spinal axis and plexuses and by repeated analysis of cerebrospinal fluid. However, the hybrid modality of positron emission tomography (PET) of fluorinated deoxyglucose (FDG) combined with CT scan (PET-CT) showed unilateral involvement of both the brachial and lumbosacral nervous plexuses. A complete recovery of neurological manifestations and normalization of PET-CT followed intensive chemotherapy with autologous stem cell transplantation. The diagnosis and localization of neurolymphomatosis may be supported by PET-CT imaging. [source] Systemic lupus erythematosus complicated with posterior reversible encephalopathy syndrome and intracranial vasculopathyINTERNATIONAL JOURNAL OF RHEUMATIC DISEASES, Issue 4 2010Hung-An CHEN Abstract Posterior reversible encephalopathy syndrome (PRES) is a neurotoxic condition characterized by reversible vasogenic edema on neuroimaging. It is associated with various neurological manifestations, including headaches, vomiting, seizures, visual loss, altered mental status and focal neurological deficits. PRES mainly occurs in the setting of eclampsia, hypertension, uremia, malignancy, transplantation, autoimmune diseases and/or use of immunosuppressive drugs. This syndrome has been described in patients with systemic lupus erythematosus (SLE). PRES is a potentially reversible clinical,radiological entity; however, it can be complicated with vasculopathy, infarction or hemorrhage. Vasculopathy has been demonstrated to be a common finding in patients with SLE. We report the case of a woman with lupus nephritis and PRES whose diffuse vasculopathy was present on initial neuroimaging. Subsequent brain computed tomography scan demonstrated interval development of intraparenchymal hemorrhage and subarachnoid hemorrhage. To our knowledge, this unique brain image pattern has not been reported in SLE patients. [source] Innate and adaptive immune activation in the brain of MPS IIIB mouse modelJOURNAL OF NEUROSCIENCE RESEARCH, Issue 4 2009Julianne DiRosario Abstract Mucopolysaccharidosis (MPS) IIIB is a lysosomal storage disease with severe neurological manifestations due to ,- N -acetylglucosaminidase (NaGlu) deficiency. The mechanism of neuropathology in MPS IIIB is unclear. This study investigates the role of immune responses in neurological disease of MPS IIIB in mice. By means of gene expression microarrays and real-time quantitative reverse transcriptase,polymerase chain reaction, we demonstrated significant up-regulation of numerous immune-related genes in MPS IIIB mouse brain involving a broad range of immune cells and molecules, including T cells, B cells, microglia/macrophages, complement, major histocompatibility complex class I, immunoglobulin, Toll-like receptors, and molecules essential for antigen presentation. The significantly enlarged spleen and lymph nodes in MPS IIIB mice were due to an increase in splenocytes/lymphocytes, and functional assays indicated that the T cells were activated. An autoimmune component to the disease was further suggested by the presence of putative autoantigen or autoantigens in brain extracts that reacted specifically with serum IgG from MPS IIIB mice. We also demonstrated for the first time that immunosuppression with prednisolone alone can significantly slow the central nervous system disease progression. Our data indicate that immune responses contribute greatly to the neuropathology of MPS IIIB and should be considered as an adjunct treatment in future therapeutic developments for optimal therapeutic effect. © 2008 Wiley-Liss, Inc. [source] The multiform and variable patterns of onset of orofacial granulomatosisJOURNAL OF ORAL PATHOLOGY & MEDICINE, Issue 4 2003Michele D. Mignogna Abstract Background:, The recurrent chronic orofacial swelling caused by orofacial granulomatosis (OFG) can cause significant cosmetic and functional problems but can be prevented if the disease is diagnosed early and promptly treated. Although the enlargement of the lips is described to be the most common presenting complaint, the clinical onset of OFG may be characterized by minor associated mucosal and neurological manifestations, making early diagnosis very difficult or, sometimes, merely presumable. Patients and methods:, We retrospectively analyzed the clinical manifestations of 19 patients with OFG, who were examined at our institution between 1998 and 2002, in order to determine their initial manifestations and presenting symptoms. Results:, A total of 10 patients showed classical recurrent enlargement of the lips (six lower; four upper) as presenting symptom. In the other nine patients, OFG onset was characterized by transient unilateral facial nerve palsy (two cases), intraoral manifestations (two cases), recurrent swelling of the periorbital area (two cases), of the chin (one case), of the zygomatic area (one case), and of the cheeks (one case). Conclusion:, Our data underlined that OFG onset could be frequently characterized by widely variable, multiform, and temporary clinical findings. Involvement of atypical sites of the orofacial region and presence of single minor manifestations may occur as presenting symptoms, often preceding the development of traditional clinical findings. [source] Developmental and benign movement disorders in childhood,MOVEMENT DISORDERS, Issue 10 2010Cecilia Bonnet MD Abstract Developmental and benign movement disorders are a group of movement disorders with onset in the neonatal period, infancy, or childhood. They are characterized by the absence of associated neurological manifestations and by their favorable outcome, although developmental abnormalities can be occasionally observed. Knowledge of the clinical, neurophysiological, and pathogenetic aspects of these disorders is poor. Based on a comprehensive review of the literature and our practical experience, this article summarizes current knowledge in this area. We pay special attention to the recognition and management of these movement disorders in children. © 2010 Movement Disorder Society [source] Reduced rates of axonal and dendritic growth in embryonic hippocampal neurones cultured from a mouse model of Sandhoff diseaseNEUROPATHOLOGY & APPLIED NEUROBIOLOGY, Issue 4 2003D. Pelled Sandhoff disease is a lysosomal storage disease in which ganglioside GM2 accumulates because of a defective ,-subunit of ,-hexosaminidase. This disease is characterized by neurological manifestations, although the pathogenic mechanisms leading from GM2 accumulation to neuropathology are largely unknown. We now examine the viability, development and rates of neurite growth of embryonic hippocampal neurones cultured from a mouse model of Sandhoff disease, the Hexb,/, mouse. GM2 was detected by metabolic labelling at low levels in wild type (Hexb+/+) neurones, and increased by approximately three-fold in Hexb,/, neurones. Hexb,/, hippocampal neurones were as viable as their wild type counterparts and, moreover, their developmental programme was unaltered because the formation of axons and of the minor processes which eventually become dendrites was similar in Hexb,/, and Hexb+/+ neurones. In contrast, once formed, a striking difference in the rate of axonal and minor process growth was observed, with changes becoming apparent after 3 days in culture and highly significant after 5 days in culture. Analysis of various parameters of axonal growth suggested that a key reason for the decreased rate of axonal growth was because of a decrease in the formation of collateral axonal branches, the major mechanism by which hippocampal axons elongate in culture. Thus, although the developmental programme with respect to axon and minor process formation and the viability of hippocampal neurones are unaltered, a significant decrease occurs in the rate of axonal and minor process growth in Hexb,/, neurones. These results appear to be in contrast to dorsal root ganglion neurones cultured from 1-month-old Sandhoff mice, in which cell survival is impaired but normal outgrowth of neurones occurs. The possible reasons for these differences are discussed. [source] Miglustat (Zavesca®) in type 1 Gaucher disease: 5-year results of a post-authorisation safety surveillance programmePHARMACOEPIDEMIOLOGY AND DRUG SAFETY, Issue 9 2009Carla E. M. Hollak MD Abstract Purpose Miglustat (Zavesca®) is an orally-available substrate reduction therapy (SRT) for treatment of mild-to-moderate type 1 Gaucher disease (GD1) in adult patients unsuitable for enzyme replacement therapy (ERT). Miglustat has not been evaluated in children with GD1, and is not used during pregnancy and breast-feeding. A non-interventional, prospective, web-based safety surveillance programme was initiated at the time of the European launch of miglustat in 2003, and is ongoing. We report the first 5 years of collected data, focusing on neurological manifestations. Methods Data were collected on 122 GD1 patients between March 2003 and April 2008, representing 244 patient-years cumulative miglustat post-authorisation experience. The electronically-captured data collected from participating physicians includes patient demographics, prior and current therapies, baseline disease manifestations and concurrent conditions, disease severity, duration of miglustat exposure, and safety-relevant information. Results Mean (SD) age at baseline was 46.1 (16.5) years. At baseline, bone disease and neurological manifestations were reported in 55.6 and 28.6% of patients, respectively; the latter included peripheral neuropathy (7.2%) and a wide variety of neurological symptoms and signs. In addition, 23.2% had other health conditions relevant to neurological status. During the reporting period, new neurological manifestations were reported in 23 (18.9%) patients, principally tremor. Thirty-five (28.7%) patients discontinued treatment, predominantly for gastrointestinal (GI) disturbances (11.5%), two-thirds of which occurred during the first 6 months. Conclusion The safety profile of miglustat in GD1 patients included in the safety surveillance programme is overall consistent with that reported in the registration and other clinical trials, and no new safety finding was identified. Copyright © 2009 John Wiley & Sons, Ltd. [source] A critical evaluation of the Braak staging scheme for Parkinson's disease,ANNALS OF NEUROLOGY, Issue 5 2008Robert E. Burke MD Braak and colleagues have proposed that, within the central nervous system, Parkinson's disease (PD) begins as a synucleinopathy in nondopaminergic structures of the lower brainstem or in the olfactory bulb. The brainstem synucleinopathy is postulated to progress rostrally to affect the substantia nigra and cause parkinsonism at a later stage of the disease. In the context of a diagnosis of PD, made from current clinical criteria, the pattern of lower brainstem involvement accompanying mesencephalic synucleinopathy is often observed. However, outside of that context, the patterns of synucleinopathy that Braak described are often not observed, particularly in dementia with Lewy bodies and when synucleinopathy occurs in the absence of neurological manifestations. The concept that lower brainstem synucleinopathy represents "early PD" rests on the supposition that it has a substantial likelihood of progressing within the human lifetime to involve the mesencephalon, and thereby cause the substantia nigra pathology and clinical parkinsonism that have heretofore defined the disease. However, the predictive validity of this concept is doubtful, based on numerous observations made in populations of aged individuals who, despite the absence of neurological signs, have brain synucleinopathy ranging up to Braak stages 4 to 6 at postmortem. Furthermore, there is no relation between Braak stage and the clinical severity of PD. We conclude that the relation between patterns of abnormal synuclein immunostaining in the human brain and the disease entity now recognized as PD remains to be determined. Ann Neurol 2008;64:485,491 [source] Correlation of neurological manifestations and MR images in a patient with Wilson's disease after liver transplantationACTA NEUROLOGICA SCANDINAVICA, Issue 2 2000J-C. Wu Orthotopic liver transplantation (OLT) has been applied to patients with Wilson's disease (WD) for correction of irreversible liver cirrhosis. However, the neurological outcome and the correlation between clinical manifestations and neuroimage findings after OLT remain uncertain. We present a WD patient who showed an improvement in both liver functions and neurological manifestations after OLT. Serum levels of ceruloplasmin and copper returned to normal rapidly after the operation. His ataxic gait was improved 5 months later and dysmetria and tremor disappeared 11 months later. The high signal intensities on T2-weighted brain magnetic resonance images regressed at bilateral thalami 5 months later and disappeared in bilateral thalami and red nuclei 16 months after OLT. We conclude that the neurological improvement could be expected in WD patients after OLT. The improvement was correlated with the MRI changes in red nuclei and bilateral thalami. [source] Bronze baby syndrome and the risk of kernicterusACTA PAEDIATRICA, Issue 7 2005Giovanna Bertini Abstract Aim: The problem of kernicterus in infants with bronze baby syndrome (BBS) has been reviewed on the basis of cases reported in the literature. In addition, a new case concerning an infant with severe Rh haemolytic disease, who presented with BBS and who has developed neurological manifestations of kernicterus with magnetic resonance images showing basal ganglia abnormalities, is presented. In this patient, the total serum bilirubin (TSB) concentration ranged from 18.0 to 22.8 mg/dl (306 to 388 ,mol/l) and the bilirubin/albumin (B/A) ratio was 6.0 (mg/g) (6.8 is the value at which an exchange transfusion should be considered). The case presented is important due to the fact that kernicterus appeared after an exchange transfusion was performed when the TSB level reached 22.8 mg/dl (388 ,mol/l) on 6th day of life while the haematocrit was 30%. From this case and from other cases reported in the literature, we must stress that, even if the level at which hyperbilirubinemia poses a threat remains undefined, BBS may constitute an additional risk of developing kernicterus. Conclusion: The possible strategies for implementing an approach to the management of hyperbilirubinemia (especially the haemolytic kind) in the presence of BBS may include an exchange transfusion carried out at lower TSB concentration than previously recommended or an early administration of Sn-mesoporphyrin. [source] | |||||||||||||||||||||||||