Cases reported "Multiple Sclerosis"

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1/18. Botulinum toxin injection in the treatment of vocal fold paralysis associated with multiple sclerosis: a case report.

    Botulinum toxin has been demonstrated clinically to be an effective treatment for a variety of laryngeal problems, most notably spasmodic dysphonia. As in other movement disorders, the theory behind the injection of this substance in the larynx has been a weakening of the vocal fold musculature to relieve uncoordinated and spasmodic movement of the vocal folds, presumably rebalancing the forces within the intralaryngeal musculature. Recently, this concept was applied to help reposition the arytenoid cartilage in acute and longstanding anteromedial cricoarytenoid dislocations. This same concept may apply to the paralyzed vocal fold. In support of this idea, a number of investigators have shown that immobile, clinically paralyzed vocal folds may still have partial voluntary motor unit activity. This voluntary activation may not produce clinically evident movement but may be sufficient to produce tone within the fold. If the voluntary motor units in the abductor musculature of the paralyzed fold are weakened with botulinum toxin, the continued pull of the functioning adductor musculature may be sufficient to medialize the paralyzed fold. This idea has been supported by animal experiments, which have shown that botulinum toxin may affect the ability of the fold to rebalance itself. With this evidence in mind, a patient with fold immobility secondary to multiple sclerosis was treated in an attempt at laryngeal rebalancing, using botulinum toxin to medialize the fold. However, instead of simply having the fold return fixed to the midline, the patient regained normal laryngeal mobility and voice. While it is unclear whether the botulinum toxin alone was responsible, the coincidence of this occurrence certainly requires reporting. This paper is a report of the first successful treatment of vocal fold paralysis using botulinum toxin to treat vocal fold fixation in a patient with multiple sclerosis.
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2/18. New therapeutic perspectives for demyelinating retrobulbar optic neuritis.

    In patients with demyelinating retrobulbar optic neuritis (RON), a spontaneous or corticosteroid-induced improvement is generally observed within the first month, but this is clinically insignificant in 5%-7% of patients. We report the case histories of four patients who were considered to be "non-responders" to corticosteroids because their visus remained unchanged or had improved by only 1/10 after one month from intravenous corticoid therapy begun 2-7 days after disease onset, and who were therefore subsequently administered high intravenous doses of immunoglobulin. Three of these patients completely recovered in a period of 3-9 months; the fourth showed only a partial improvement, but this was consolidated after long-term continuation of the same therapy. These cases suggest the possible efficacy of early administration of intravenous immunoglobulin in RON patients who fail to respond to cortisone therapy. As recently demonstrated in animal models, it can be hypothesised that the result is due to immuno-mediated mechanisms of action that reduce autoimmune responses in the short- and medium-term, and in the long-term favour remyelination.
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3/18. Linear pontine trigeminal root lesions in multiple sclerosis: clinical and magnetic resonance imaging studies in 5 cases.

    BACKGROUND: magnetic resonance imaging (MRI) is useful for demonstrating demyelinating lesions in patients with multiple sclerosis (MS). magnetic resonance imaging studies show that MS lesions are generally not uniform in shape, size, or distribution. Linearly shaped lesions at the trigeminal root entry zone have been occasionally reported in single cases of MS, but, to our knowlege, the frequency and the clinical features of such patients have not been comprehensively characterized. OBJECTIVE: To describe the frequency and the clinical and laboratory features of patients with MS who had linearly shaped lesions at the trigeminal root as seen on MRI. DESIGN AND SETTING: A retrospective review of medical records and MRI films of Japanese patients with MS admitted to a university hospital and its affiliated hospital in Sendai, japan. patients AND methods: brain MRI films of 74 consecutive Japanese patients with MS (51 females and 23 males) were studied retrospectively and the clinical and laboratory features of the patients with linearly shaped lesions at the trigeminal root were also investigated retrospectively. RESULTS: Five patients (6.8%) were shown to have T1-weighted-hypointense, T2-weighted-hyperintense, nonenhanced linear lesions in the pons on MRI, and these were uniformly localized in the intramedullary portion of the trigeminal root. All of these patients had clinically definite MS and had various types of facial sensory disturbances, such as neuralgia (1 patient), hypesthesia (2 patients), or paresthesia (3 patients). No other clinical or laboratory feature was characteristic in these 5 patients. CONCLUSIONS: Linear pontine trigeminal root lesions were common in our patients with MS. They were associated with various facial sensory symptoms. Since similar lesions are formed in animal models of herpes simplex virus infection, further study is needed to clarify whether these MS lesions are virally induced.
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4/18. antibodies against myelin oligodendrocyte glycoprotein in the cerebrospinal fluid of multiple sclerosis patients.

    antibodies against myelin oligodendrocyte glycoprotein (MOG) mediate demyelination in experimental autoimmune encephalomyelitis (EAE) in different animal species and are implicated in the immunopathogenesis of multiple sclerosis (MS). In order to evaluate the anti-MOG response, we have analyzed the cerebrospinal fluids (CSFs) from 44 MS patients and 51 controls, 11 with other inflammatory neurological disorders (OIND) and 40 with non-inflammatory neurological disorders (NIND). The frequency of anti-MOG antibodies positive patients in the MS group (30%) was significantly higher compared to the NIND (8%, p=0.02), but not compared to the OIND group (55%, p=0.228). Interestingly, all six patients with neurosarcoidosis had MOG-specific antibodies in their CSF. Frequency of anti-MOG antibodies was similar in patients with clinically active and stable MS (32% and 26%, respectively; p=0.921). However, in clinically active MS patients, antibody titers were higher in comparison with patients with stable disease, although the difference did not reach the level of statistical significance (p=0.06). These results further support the potential role of anti-MOG antibodies in the immunopathology of MS in the subset of patients with this disease. Furthermore, our findings suggest for the first time that anti-MOG antibodies could be an accessory diagnostic tool in neurosarcoidosis.
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5/18. magnetic fields mimic the behavioral effects of REM sleep deprivation in humans.

    The discovery of rapid eye movement (REM) sleep by Aserinsky and Kleitman in 1953 initiated the impetus for sleep research and specifically the investigations of the effects of REM sleep deprivation (RSD) on animal and human behavior. The behavioral effects of RSD include the enhancement of motivational and "drive"-related behaviors. In laboratory animals, RSD has been reported to increase appetite, sexual behavior, aggressiveness, and locomotor activity. Moreover, RSD reportedly improves mood in patients with endogenous depression and heightens appetite and sexual interest in normal subjects. Since "drive"-related behaviors are thought to involve activation of limbic dopaminergic reward sites, RSD may enhance motivational behaviors through an action on limbic dopaminergic functions. In the present communication, we present two patients (one with multiple sclerosis and the other with Parkinson's disease) in whom treatment with magnetic fields produced behavioral effects which paralleled those observed in REM-sleep-deprived animals and humans. We propose, therefore, that the behavioral and mental effects of treatment with magnetic fields may be mediated via RSD and, by inference, involve activation of limbic dopaminergic reward sites.
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6/18. multiple sclerosis following treatment with a cannabinoid receptor-1 antagonist.

    Laboratory research including animal models of human disease suggests that cannabinoids might have therapeutic potential in multiple sclerosis (MS). We have recently seen a 46-year-old woman who developed MS after starting treatment with a cannabinoid receptor antagonist for obesity. The occurrence of MS several months after starting a cannabinoid receptor antagonist suggests that the cannabinoid system might indeed be relevant to disease pathogenesis in MS.
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7/18. 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.
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8/18. A 71-year-old male with 4 decades of symptoms referable to both central and peripheral nervous system.

    May 2005. Combined demyelination of the central and peripheral nervous system is an uncommon disorder and has been referred to by many appellations. We present the case of a 71-year-old man with a progressive nervous system disorder beginning in his 30s. The diagnosis in life was unclear, but had over the years been variously considered to be guillain-barre syndrome (GBS), friedreich ataxia, or multiple sclerosis (MS). At autopsy old CNS demyelination consistent with MS was found as well as chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with onion bulb formation in hypertrophic nerves. Microscopic examination showed some onion bulb formation in the CNS as well as in peripheral nerves. The nosology of these disorders is discussed and relevant animal models briefly reviewed.
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9/18. Fetal outcome following intrauterine amantadine exposure.

    amantadine hydrochloride is a well-known antiviral agent that has been used for the prevention of influenza A2, the treatment of parkinson disease, and, more recently, multiple sclerosis. However, very few data exist about its use in pregnant women. We report a 34-year-old woman who had used amantadine to prevent relapse of her multiple sclerosis throughout two of her pregnancies who subsequently delivered two normal infants. We review the available animal data and two other human pregnancy exposure reports.
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10/18. Spasmodic torticollis due to a midbrain lesion in a case of multiple sclerosis.

    A case of multiple sclerosis is described in which spasmodic torticollis occurred abruptly and abated after 1 year. magnetic resonance imaging (MRI) demonstrated a lesion in the mesencephalon. Other symptoms and physical signs that developed at the same time as the spasmodic torticollis were compatible with the lesion that had not been present on MRI 18 months previously. There are very few reports of spasmodic torticollis due to an identified focal lesion; there is evidence from experimental work on animals that midbrain lesions may cause spasmodic torticollis but there has been no previous human example.
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