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 21,692 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This study covers patients seen at a Wilson disease clinic between 1960 and 2002. The diagnosis of Wilson disease was confirmed in 316. Of 40 patients with a neurological presentation who were found not to have the disease, caeruloplasmin concentration was found to be reduced in 19. These 19 patients comprised 17 men and 2 women. The various diagnoses included Huntington's disease, multiple sclerosis, subacute sclerosing panencephalitis (SSPE), Hallervorden Spatz syndrome, and acaeruloplasminemia; in 9 no definite diagnosis was made. In view of the marked male preponderance, the lower limit of caeruloplasmin is calculated as for males. In an earlier study this was found to be 33.3 mg/dl (standard deviation, 6.1 mg/dl); therefore, 21.1 mg/dl is taken as the lower limit of normal. Particular attention is paid to 3 patients, 2 of whom had zero concentrations of caeruloplasmin and 1 had a very low level of the protein. Only 1 of these patients conformed to the classical picture of acaeruloplasminemia. The significance of a low caeruloplasmin concentration in patients with a variety of neurological syndromes is not clear but can lead to diagnostic confusion. When the concentration of this protein is very low or absent the diagnosis of acaeruloplasminemia must be considered.
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PMID:Diagnostic significance of reduced serum caeruloplasmin concentration in neurological disease. 1609 17

In the years following the hepatitis B vaccination/multiple sclerosis controversy, a number of new issues regarding vaccine safety have been raised, in some cases leading to more debate and confusion. Against this background, an international group of experts was convened to review the current points of view concerning the use of thimerosal as a preservative and its potential risks; the suggested link between thimerosal-containing vaccines and acute lymphoblastic leukemia; the alleged association between aluminum-containing vaccines/macrophagic myofasciitis and general systemic complaints; a possible link between vaccination and autoimmune pathology; and a hypothetical link between measles-mumps-rubella vaccination and autism. At present, there are no data to conclude that childhood vaccines, and in particular hepatitis B vaccine, pose a serious health risk or justify a change in current immunization practice. However, vaccine "scares" continue to have an international impact on immunization coverage. Creating a positive environment for immunization can be achieved by repositioning the value of vaccines and vaccination, supported by evidence-based information. The role of international organizations, the media, and the industry in the implementation of communication strategies was discussed and the impact of litigation issues on vaccination was evaluated. The Viral Hepatitis Prevention Board confirms its commitment to current recommendations for universal and risk group hepatitis B vaccination and further encourages the conduct of vaccine safety studies and the dissemination of their results.
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PMID:Vaccine safety controversies and the future of vaccination programs. 1628 28

Multiple sclerosis (MS) is an inflammatory demyelinating autoimmune disease of the central nervous system. The disorder displays marked clinical heterogeneity. In certain cases, making diagnosis can be challenging. Diagnosis of MS has become more important in the era of treatments that change the natural history of the disease. Several general diagnostic principles are useful to guide the diagnostic approach to MS. Clinically, MS requires neurological problems associated with objective abnormalities. Certain basic principles, first outlined by Schumacher et al. (1965) are still pertinent. Poser et al. (1983) have further modified the criteria using data derived from clinical evaluation and laboratory studies, including cerebrospinal fluid analysis, evoked potentials, and imaging studies. Poser criteria have long been familial for most neurologists. The most recent addition to our diagnostic armamentarium are the McDonald criteria (2001), which are the first attempt to incorporate standardized MRI criteria into the MS diagnostic process. The most innovative use of MRI to support an MS diagnosis is dissemination of demyelination can be demonstrated by MRI alone, in the absence of any new clinical attacks. Diagnosing MS by such sensitive MRI criteria will occur more quickly than waiting for a second clinical event. This has added some sensitivity, some controversy, and a lot of confusion. The application of the new criteria on Asian MS patients remains to be validated. Each of the criteria will be discussed, with major emphasis on the McDonald criteria.
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PMID:[Diagnosis of multiple sclerosis]. 1642 50

Acute hydrocephalus is rarely encountered in adults without venticuloperitoneal or venticuloatrial shunts. The purpose of this report is to describe a case of acute hydrocephalus due to no identifiable cause. A 40-year-old man developed a typical clinical picture of intracranial hypertension within a 24-hours period. On the morning of hospitalization, the patient consulted his physician for severe headache followed quickly by nausea and vomiting. After cerebral CT-scan, the patient was referred to our department. His condition rapidly worsened, with confusion, then drowsiness. Magnetic resonance imaging (MRI) demonstrated quadri-ventricular hydrocephalus with a Chiari I malformation. In view of his rapidly deteriorating clinical condition, emergency endoscopic third ventriculostormy was performed. The patient recovered rapidly and was discharged 8 days after the procedure. Diagnostic work-up included lumbar puncture showing normal cerebrospinal fluid (thereby eliminating multiple sclerosis, low-grade intracranial hemorrhage, and meningitis (bacterial, viral, fungal)), spinal MRI depicting no medullary lesions, and brain angiography revealing no vascular abnormalities. Follow-up brain MRI carried out at six months after hospitalization demonstrated normal ventricles and complete disappearance of the Chiari I malformation. No conclusion can be drawn as to whether the Chiari I malformation was the consequence or cause of hydrocephalus. The explanation of this acute hydrocephalus is the acute decompensation without identifiable cause of idiopathic stenosis of the foramen of Magendie and Luschka. Treatment with endoscopic third ventriculostomy is effective.
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PMID:[Idiopathic acute obstructive hydrocephalus. Case report]. 1660 59

The ocular following response (OFR) is a measure of motion vision elicited at ultra-short latencies by sudden movement of a large visual stimulus. We compared the OFR to vertical sinusoidal gratings (spatial frequency 0.153 cycles/ degrees or 0.458 cycles/ degrees) of each eye in a subject with evidence of left optic nerve demyelination due to multiple sclerosis (MS). The subject showed substantial differences in vision measured with stationary low-contrast Sloan letters (20/63 OD and 20/200 OS at 2.5% contrast) and the Lanthony Desaturated 15-hue color test (Color Confusion Index 1.11 OD and 2.14 OS). Compared with controls, all of the subject's OFR to increasing contrast showed a higher threshold. The OFR of each of the subject's eyes were similar for the 0.153 cycles/ degrees stimulus, and psychophysical measurements of his ability to detect these moving gratings were also similar for each eye. However, with the 0.458 cycles/ degrees stimulus, the subject's OFR was asymmetric and the affected eye showed decreased responses (smaller slope constant as estimated by the Naka-Rushton equation). These results suggest that, in this case, optic neuritis caused a selective deficit that affected parvocellular pathways mediating higher spatial frequencies, lower-contrast, and color vision, but spared the field-holding mechanism underlying the OFR to lower spatial frequencies. The OFR may provide a useful method to study motion vision in individuals with disorders affecting anterior visual pathways.
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PMID:Contrast sensitivity, first-order motion and Initial ocular following in demyelinating optic neuropathy. 1664 97

Headache and migraine are common features in multiple sclerosis (MS) and can influence the diagnosis, radiological evaluation, treatment, and quality of life of these patients. Similarities in symptoms between patients with migraine and MS presenting with headache can lead to misdiagnosis. Likewise, MRI lesions which may be found in migraine patients without other neurological symptoms or signs may cause diagnostic confusion and patient anxiety. Studies addressing the pathophysiology of these comorbid conditions have not found a clear link, but brainstem lesions and inflammatory processes have been proposed. Management of de novo or treatment-induced headache in the MS patient is fairly straightforward if the physician is aware of the problem.
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PMID:Migraine in multiple sclerosis. 1753 46

Multiple sclerosis (MS) is used as the best example to illustrate the interplay between genetic endowment and environmental effects. The former is widely considered as being the more important one, although there is mounting evidence that environmental factors influence genetic predisposition. The vast majority of environmental epidemiological studies have failed for two major reasons: they have lacked biological plausibility, and they have not considered possible environmental factors operative during the putative period of acquisition of the disease. Lack of diagnostic precision in distinguishing among the inflammatory demyelinating diseases has caused confusion. The role of infectious agents remains controversial: there is little evidence in favor of MS resulting from a specific infection, but the effect of viral infections and vaccinations on the immune system of a genetically vulnerable person remains unresolved.
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PMID:The environment and the nervous system. 1765 66

Neuromyelitis optica (NMO) is an inflammatory disease mainly affecting optic and spinal cords, and was originally described by Devic in 1894. There has been long controversy about whether NMO is a subtype of multiple sclerosis (MS) or a distinct disease. In Japan and other Asian countries, relapsing NMO has been called as optic-spinal form of MS (OSMS), but we reported in 2002 that OSMS was heterogeneous and it comprised both typical NMO and MS with optic spinal presentation. Recently, a highly specific serum autoantibody marker, NMO-IgG, was found in the sera of Caucasian NMO and Japanese OSMS cases, and the target antigen was identified as the water channel protein aquaporin (AQP) 4. So in NMO and OSMS, similar autoimmune backgrounds were revealed. In our anti-AQP4 antibody assay using HEK293 cells transfected with human AQP4, we found that the sensitivity and specificity of anti-AQP4 antibody was 91% and 100%, respectively, which was superior to the original immunohistological assay using mouse brain slices (NMO-IgG). The titre of AQP4 antibody correlated with the length of spinal cord lesions and relapse rate. We also studied the expression of AQP4 in autopsied cases of NMO and MS and revealed that AQP4 and GFAP, an astrocytic marker protein, were completely lost at the acute inflammatory lesions surrounding immunoglobulin and complement-deposited dilated vessels, but the myelin basic protein was relatively preserved. Those results suggest that astrocytic damage associated with autoimmunity to AQP4 may be involved in the pathogenesis of NMO, which is distinct from MS, primarily demyelinating disease. After the long history of confusion, NMO became clearly discriminated disease from MS. In this review, we focus on the historical changes of the disease concept and new knowledge gained from the clinical or immunological analyses of NMO.
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PMID:[Neuromyelitis optica and anti-aquaporin 4 antibody--an overview]. 1851 75

A 61-year-old woman with secondary progressive multiple sclerosis presented on six occasions over a 2-year period with severe hypothermia (31-33.5 degrees C). This resulted in numerous multi-system complications comprising acute pancreatitis, hepatitis, gastrointestinal haemorrhage, psychiatric disturbance, bradycardia, paradoxical sweating, thrombocytopenia, anaemia and raised inflammatory markers. Septic screens were consistently normal. On each occasion she was successfully treated with passive external rewarming and made a complete recovery. This is the first reported case of such extensive sequelae in a single patient with recurrent hypothermic episodes. This unusual patient provides an invaluable insight into the natural history and pathophysiology of hypothermia. The case report is followed by a review of dysfunctional thermoregulation and pathophysiology of hypothermia-induced multi-system complications. A key learning point is to recognise that the clinical manifestations of hypothermia may be widespread and serious but are nonetheless reversible. In addition, one should consider the differential diagnosis of covert hypothermia in those patients with episodic confusion, as hypothermia is under-recognised, particularly in older people, who are prone to accidental hypothermia, and in those with common neurological conditions, such as stroke, head injury and multiple sclerosis, that may have suboptimal thermoregulation.
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PMID:Multi-system complications of hypothermia: a case of recurrent episodic hypothermia with a review of the pathophysiology of hypothermia. 1864 17

Central nervous system vasculitides are rare and life-threatening diseases with challenging diagnostics. Their neurological symptom spectrum is multifaceted: the patient may have intense headache, confusion, decreased cognitive function, changes in consciousness, epileptic attacks and symptoms resembling multiple sclerosis. Angiographic investigations, magnetic resonance imaging of the brain and examination of cerebrospinal fluid will clarify the diagnosis, but brain biopsy may be required to confirm the diagnosis. In differential diagnostics, special attention should be paid to cerebrovascular vasoconstriction syndromes. Standard therapy of cerebral vasculitis includes corticosteroids often combined with immunosuppressants.
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PMID:[Central nervous system vasculitis]. 1934 Oct 24


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