1/31. Cranial polyneuropathies in multiple sclerosis: case report and literature review.Although cranial neuropathies are common disorders in multiple sclerosis patients, multiple cranial nerve involvement is an unusual occurrence. Correlation of clinical symptoms with magnetic resonance imaging evidence of demyelinating central nervous system lesions can confirm the diagnosis. The authors report on the case of a 43-year-old woman who initially was thought to have suffered a brainstem infarct but, in fact, had developed multiple cranial nerve functional deficits. Treatment of multiple sclerosis remains primarily supportive in nature, with corticosteroids used for acute exacerbations and chronic progression.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/31. A longitudinal MR study of the presymptomatic phase in a patient with clinically definite multiple sclerosis.We describe the dynamics and the nature of the presymptomatic phase of multiple sclerosis (MS) in a patient for whom MR abnormalities suggestive of MS were found before the development of clinical symptoms. The patient was monitored with serial monthly MR imaging of the brain and spinal cord for 5 months. Disease activity during the presymptomatic phase showed imaging characteristics comparable to that of early relapsing-remitting MS in terms of enhancing lesions, duration of enhancement, and new lesions depicted by T2-weighted imaging. Measurements derived from magnetization transfer imaging suggested that the amount and degree of tissue destruction within and outside the lesions revealed by T2-weighted imaging were mild. This, together with the fact that only one of the 43 new lesions that developed during the presymptomatic phase was located in a neurologically eloquent area, may be the reason why, for a relatively long period, the patient had no clinical manifestations of MS despite the marked MR findings of disease activity.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/31. Confabulation and multiple sclerosis: a rare association.BACKGROUND: Fantastic confabulation in the context of multiple sclerosis (MS) has not previously been reported in the literature. The association is of interest because clearly demonstrable brain pathology in MS together with other cognitive and behavioural correlates may further our understanding of the neural basis underlying confabulation. methods: A single case report with magnetic resonance imaging of the brain and detailed neuropsychological evaluation. RESULTS: Confabulation occurred together with disinhibited and stimulus bound behaviour. While the patient's physical and emotional state limited the range of psychometric tests administered, the results revealed an inability to maintain focused, regulated information processing. Although memory difficulties were present, they were not in the nature of a severe amnesic disorder. The patient appeared to have a broad fund of knowledge, but the associations binding the information together and putting it into context were loose. All three features of a triad of responses previously described in confabulating patients were present, namely an inability to withhold answers, to monitor one's own responses and provide verbal self corrections. MRI of the brain showed bilateral periventricular lesions and discrete frontal lesions with 53% of the total lesion volume distributed in frontal areas. Cortical atrophy, most marked in frontal regions also was conspicuous. CONCLUSIONS: Confabulation linked to frontal lobe involvement may occur as part of the changes in mentation found in MS. It is, however, rare and although associated with impaired memory, may be found in the absence of a severe amnesic disorder. This conclusion is discussed in the light of observations from the literature suggesting that frontal involvement is a prerequisite before fantastic confabulation occurs.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/31. Bilateral trigeminal neuralgia: a therapeutic dilemma.In order to illustrate the inherent problems of managing bilateral trigeminal neuralgia a retrospective study of the 16 cases of bilateral trigeminal neuralgia, out of just over 300 cases of trigeminal neuralgia, treated over a 14-year period, has been performed. All the patients, presented with a typical history of trigeminal neuralgia and underwent surgical exploration. pain relief was initially achieved in all cases; however, only four remained cured, three have become pain free after additional rhizotomy, a further one after peripheral cryotherapy and four with medical treatment. Four patients have had bilateral operations for trigeminal neuralgia, but in two cases the pain was relieved on one side only. Bilateral trigeminal neuralgia presents special problems of management with respect to underlying neuropathology (e.g. multiple sclerosis), the need for the limitation of the use of ablative techniques in order to minimise the disability of bilateral sensory and motor dysfunction, and the relatively poor response to microvascular decompression. These factors emphasize the multifactorial nature of the cause of trigeminal neuralgia. Magnetic resonance tomographic angiography is now available and is important in determining the range of therapeutic options for this group of patients.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/31. glioma and multiple sclerosis: case report.We report a case of a 44-years-old woman with relapsing-remitting and secondarily progressive form of multiple sclerosis (MS) since aged 24 years, who developed an anaplastic astrocytoma. The neurological manifestations of the tumor were misinterpreted as resulting from MS. Sequential MRI examination and seizures raised the possibility of another nature of her symptoms, besides MS. Her initial good response to high doses corticosteroids led to the initial assumption her symptoms were only exclusively due to the demyelinating process. She underwent craniotomy with radical excision of the lesion. Pathological examination disclosed anaplastic astrocytoma. Other cases of coincidental MS and primary CNS tumors are reviewed, as well as their possible relation.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/31. aphasia as a rare presentation of monosymptomatic demyelinating disease: case report and review of the literature.We present a case of sudden-onset aphasia due to a single pathological lesion, which at neuroradiological imaging studies was suggestive of glioma, while on biopsy proved be of demyelinating nature. Every cause of demyelinating lesions of the central nervous system was considered in the differential diagnosis, concluding for a primary demyelinating disease. The clinical and radiological differences between multiple sclerosis and acute disseminated encephalomyelitis are discussed. Although aphasia has already been described in demyelinating diseases, we underline its rarity as onset symptom.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/31. brain-restricted amyloidoma of immunoglobulin lambda-light chain origin clinically resembling multiple sclerosis.Cerebral amyloid deposits restricted to the white matter and associated with intracerebral lymphoma were biochemically identified. The patient died at 58 years of age after 37 years of illness with progressive neurological symptoms clinically indicative of multiple sclerosis. Pathomorphologically, spongiform alteration and demyelinization of the white matter in the vicinity of the amyloid deposits was detected and systemic amyloidosis excluded. Immunohistochemically, the amyloid was found to be of immunoglobulin lambda-light chain origin. To establish the nature of this amyloid, its fibrils were extracted and the amyloid fibril proteins isolated by size exclusion chromatography. Immunochemically, the purified amyloid fibril proteins were shown to be of immunoglobulin lambda-light chain origin. This finding was substantiated chemically. Since the N-terminal amino acid was blocked, tryptic peptides were isolated by reversed phase HPLC. The amino-acid sequence of two major peptides revealed homology with the variable region of the immunoglobulin lambda-light chain. This report defines a novel local A lambda-amyloid disease restricted to the white matter of the brain.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
8/31. scleromyxedema in a patient with multiple sclerosis and monoclonal gammopathy on interferon beta-1a.BACKGROUND: Animal studies have shown that some human monoclonal antibodies promote myelin repair in models of demyelinating disease. scleromyxedema is a dermatologic disorder associated with a monoclonal gammopathy and neurologic manifestations. The reason for occurrence of cutaneous reactions in interferon treated patients is unknown. CASE DESCRIPTION: A 37-year-old woman was started on weekly interferon beta-1a (IFN beta-1a) following a diagnosis of multiple sclerosis (MS). After having been on interferon therapy for three years, she developed skin lesions secondary to scleromyxedema. Her IFN beta-1a was discontinued and intravenous immunoglobulin therapy was started for her scleromyxedema. At a six-month follow up, her skin lesions improved and there was no recurrence of neurologic symptoms. CONCLUSIONS: This is the first report of occurrence of scleromyxedema in a patient with MS. While this could be a chance association, it does raise the question if her neurologic manifestations could be secondary to scleromyxedema. Further research into the mechanism of IFN related cutaneous side effects is needed. Evidence regarding the remyelinating nature of human monoclonal antibodies raises interest in the potential therapeutic role these antibodies may have.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
9/31. Evidence for shutter-speed variation in CR bolus-tracking studies of human pathology.The standard pharmacokinetic model for the analysis of MRI contrast reagent (CR) bolus-tracking (B-T) data assumes that the mean intracellular water molecule lifetime (tau(i)) is effectively zero. This assertion is inconsistent with a considerable body of physiological measurements. Furthermore, theory and simulation show the B-T time-course shape to be very sensitive to the tau(i) magnitude in the physiological range (hundreds of milliseconds to several seconds). Consequently, this standard model aspect can cause significant underestimations (factors of 2 or 3) of the two parameters usually determined: K(trans), the vascular wall CR transfer rate constant, and v(e), the CR distribution volume (the extracellular, extravascular space fraction). Analyses of animal model data confirmed two predicted behaviors indicative of this standard model inadequacy: (1) a specific temporal pattern for the mismatch between the best-fitted curve and data; and (2) an inverse dependence of the curve's K(trans) and v(e) magnitudes on the CR dose. These parameters should be CR dose-independent. The most parsimonious analysis allowing for realistic tau(i) values is the 'shutter-speed' model. Its application to the experimental animal data essentially eliminated the two standard model signature inadequacies. This paper reports the first survey for the extent of this 'shutter-speed effect' in human data. Retrospective analyses are made of clinical data chosen from a range of pathology (the active multiple sclerosis lesion, the invasive ductal carcinoma breast tumor, and osteosarcoma in the leg) that provides a wide variation, particularly of K(trans). The signature temporal mismatch of the standard model is observed in all cases, and is essentially eliminated by use of the shutter-speed model. Pixel-by-pixel maps show that parameter values from the shutter-speed analysis are increased by more than a factor of 3 for some lesion regions. This endows the lesions with very high contrast, and reveals heterogeneities that are often not seen in the standard model maps. Normal muscle regions in the leg allow validation of the shutter-speed model K(trans), v(e), and tau(i) magnitudes, by comparison with results of previous careful rat leg studies not possible for human subjects.- - - - - - - - - - ranking = 2keywords = nature (Clic here for more details about this article) |
10/31. Monofocal acute large demyelinating lesion mimicking brain glioma.We report the case of a 34-year-old woman with clinical, neuroradiological and intraoperative histological findings, suggesting a low-grade astrocytic tumour. The demyelinating nature of the lesion was established through biopsy only after neurosurgery. The lesion size, in fact, greatly exceeded that of the perivenous demyelination seen in typical multiple sclerosis (MS) and tended to present as a space-occupying mass. This case underlines the importance of considering demyelinating isolated lesions in the differential diagnosis of a brain mass. Since misdiagnosis can result in unwarranted and aggressive therapy, it is critical for the neurologist to be aware of this serious diagnostic pitfall.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
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