1/227. Disorders in cerebellar ocular motor control. II. Macrosaccadic oscillation. An oculographic, control system and clinico-anatomical analysis.A distinctive cerebellar ocular motor disorder, macrosaccadic oscillation, evolved simultaneously with an acute cerebellar syndrome in 4 patients, 2 with haemorrhagic metastatic melanoma deep in the vermis, a third with a presumed cerebellar haematoma and a fourth with focal demyelinating disease. Ocular oscillations were conjugate, horizontal, symmetrical, occurred in bursts of several seconds duration, had amplitudes of 30 degrees to 50 degrees, and were evoked whenever the patient attempted to shift visual fixation or pursue a moving target. Photo-electric recordings in one patient with tumour defined features of this disorder of saccadic eye movement: (i) oscillation was composed of saccades, (ii) frequency was 2 Hz, (iii) bursts occurred with amplitude first increasing and then decreasing, (iv) intervals between beginnings of saccades averaged 260 ms and (v) eye position did not exhibit systematic drift during the intersaccadic period. These features documented the inreased gain and instability of the visually guided saccadic system. By using increased feed-forward gain in a sampled-data control model we simulated the pattern of macrosaccadic oscillation. We belive that the acute loss of the calibrator function of the cerebellum accounts for the gain abnormality underlying macrosaccadic oscillation.- - - - - - - - - - ranking = 1keywords = demyelinating (Clic here for more details about this article) |
2/227. Chronic steadily progressive central and peripheral predominantly motor demyelination, involving the cranial nerves, responsive to immunoglobulins.The association of central and peripheral demyelination was reported previously. Most of the cases refer to central chronic relapsing demyelination with clinical criteria for multiple sclerosis associated with later signs of peripheral nerve involvement. Other authors, described central lesions in patients with chronic inflammatory demyelinating polyneuropathy and in guillain-barre syndrome, as a seldom occurrence. We report a patient in which a chronic steadily progressive central and peripheral predominantly motor nervous system demyelination, involving the cranial nerves, was identified. The patient improved after intravenous immunoglobulin suggesting an immune-mediated mechanism. To our knowledge this presentation was not described before.- - - - - - - - - - ranking = 1.5339499562316keywords = demyelinating, inflammatory demyelinating, neuropathy, polyneuropathy (Clic here for more details about this article) |
3/227. 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 = demyelinating (Clic here for more details about this article) |
4/227. A multiple sclerosis-like illness in a man harboring the mtDNA 14484 mutation.In most cases of Leber's hereditary optic neuropathy (LHON) the only clinical manifestation is visual loss. A multiple sclerosis-like illness has been infrequently reported in association with LHON. Most patients are women harboring the mtDNA 11778 mutation. We present a young man with clinical and paraclinical evidence of a demyelinating process with profound bilateral visual loss who harbored the mtDNA 14484 mutation associated with LHON.- - - - - - - - - - ranking = 1.0027195740209keywords = demyelinating, neuropathy (Clic here for more details about this article) |
5/227. Acquired convergence-evoked pendular nystagmus in multiple sclerosis.Nystagmus seen only with convergence is unusual. We describe four cases of acquired convergence-evoked pendular nystagmus in patients with multiple sclerosis. The nystagmus was horizontal and asymmetric in all patients. eye movement recordings in one subject showed a conjugate rather than a convergent-divergent relationship of the phase of movement between the two eyes. All patients had evidence of optic neuropathy and cerebellar dysfunction. Occlusion of either eye during fixation of near targets led to divergent drift of the covered eye and a decrease in nystagmus. Intravenous scopolamine reduced nystagmus in one patient. Base-in prisms alleviated symptoms of oscillopsia at near and improving reading visual acuity. Convergence-evoked pendular nystagmus may be more common than currently appreciated, particularly among patients with multiple sclerosis.- - - - - - - - - - ranking = 0.0027195740209231keywords = neuropathy (Clic here for more details about this article) |
6/227. trigeminal neuralgia triggered by auditory stimuli in multiple sclerosis.OBJECTIVES: To describe a patient with a demyelinating brainstem lesion who developed right-sided trigeminal neuralgia triggered by auditory stimuli and to discuss the pathophysiological mechanisms underlying this unusual phenomenon. DESIGN: Case report. SETTING: Referral center. PATIENT: A 27-year-old man who presented with clinical signs of a brainstem lesion developed right-sided trigeminal neuralgia triggered by auditory stimuli to the right ear. magnetic resonance imaging and electrophysiological studies demonstrated a demyelinating lesion in the pons affecting the right lateral lemniscus and the right trigeminal pathway. This phenomenon completely subsided within 4 days. After a relapse, the diagnosis of clinically definite multiple sclerosis was made. CONCLUSION: Lateral spread of impulse activity within the demyelinating pontine lesion is the likely explanation for the unusual phenomenon of trigeminal neuralgia triggered by auditory stimuli.- - - - - - - - - - ranking = 3keywords = demyelinating (Clic here for more details about this article) |
7/227. Impairment of depth perception in multiple sclerosis is improved by treatment with AC pulsed electromagnetic fields.multiple sclerosis (MS) is associated with postural instability and an increased risk of falling which is facilitated by a variety of factors including diminished visual acuity, diplopia, ataxia, apraxia of gait, and peripheral neuropathy. Deficient binocular depth perception may also contribute to a higher incidence of postural instability and falling in these patients who, for example, find it an extremely difficult task to walk on uneven ground, over curbs, or up and down steps. I report a 51 year old woman with secondary progressive MS who experienced difficulties with binocular depth perception resulting in frequent falls and injuries. Deficient depth perception was demonstrated also on spontaneous drawing of a cube. Following a series of transcranial treatments with AC pulsed electromagnetic fields (EMFs) of 7,5 picotesla flux density, the patient experienced a major improvement in depth perception which was evident particularly on ascending and descending stairs. These clinical changes were associated with an improvement in spatial organization and depth perception on drawing a cube. These findings suggest that in MS impairment of depth perception, which is encoded in the primary visual cortex (area 17) and visual association cortex (areas 18 and 19), may be improved by administration of AC pulsed EMFs of picotesla flux density. The primary visual cortex is densely innervated by serotonergic neurons which modulate visual information processing. Cerebral serotonin concentrations are diminished in MS patients and at least some aspects of deficient depth perception in MS may be related to dysfunction of serotonergic transmission in the primary visual cortex. It is suggested that transcranial AC pulsed applications of EMFs improve depth perception partly by augmenting serotonergic transmission in the visual cortex.- - - - - - - - - - ranking = 0.0027195740209231keywords = neuropathy (Clic here for more details about this article) |
8/227. Lesion development in Marburg's type of acute multiple sclerosis: from inflammation to demyelination.We report a patient who suffered from acute inflammatory CNS demyelination and underwent two consecutive diagnostic stereotactic brain biopsies during the early disease course. The first lesion was drawn 33 days after the onset of disseminated neurological symptoms. macrophages and T lymphocytes diffusely infiltrated small vessel walls and the white matter. mRNA for tumor necrosis factor alpha (TNFalpha) and inducible nitric oxide synthase (iNOS) was abundantly expressed. Myelin sheaths were entirely preserved. The second biopsy 76 days later showed confluent demyelinating lesions with a diffuse infiltration of macrophages that were positive for myelin debris, activation markers and TNFalpha and iNOS mRNA. IgG and C9neo deposits were found along myelin sheaths. The patient had received intravenous immunoglobulins (IVIG) prior to biopsy. Findings from this single patient affirm that demyelination follows the migration of inflammatory cells from the circulation into the white matter with subsequent inflammation and demyelination. inflammation alone may be sufficient to cause significant clinical deficits without demyelination. Inflammatory mediators such as TNFalpha and NO are involved at very early stages in the pathogenetic process. IVIG treatment may lead to the deposition of immunoglobulins and to the activation of the complement cascade, but the clinical relevance of this particular finding remains uncertain.- - - - - - - - - - ranking = 1keywords = demyelinating (Clic here for more details about this article) |
9/227. tissue plasminogen activator gene expression in multiple sclerosis brain tissue.Recent studies have implicated tissue-type plasminogen activator (tPA) in neurodegeneration. We studied multiple sclerosis (MS) brain tissue for tPA gene and protein expression in comparison with reference tissue, by in situ hybridisation and immunohistochemistry. MS is characterised by demyelination in the central nervous system. In this study, neuronal cell bodies in MS brain showed high expression of tPA mRNA and protein, while in reference brains, staining for protein and mRNA expression were very low in neurons and mostly restricted to blood vessel walls. In MS, there was an additional staining of mononuclear cells within perivascular cuffs and foamy macrophages within demyelinating plaques. In view of evidence that the final process of demyelination in MS is thought to be enzyme-mediated, our work suggests the involvement of tPA and by inference plasmin, in the demyelinating process. Blocking tPA or plasmin activity may be a potentially beneficial therapeutic approach in MS.- - - - - - - - - - ranking = 2keywords = demyelinating (Clic here for more details about this article) |
10/227. syringomyelia and complex regional pain syndrome as complications of multiple sclerosis.OBJECTIVE: To describe a patient from Southeast asia with the optic-spinal phenotype of multiple sclerosis who developed syringomyelia and resultant complex regional pain syndrome (formerly named reflex sympathetic dystrophy). DESIGN: Case report. SETTING: Department of neurology at a tertiary care hospital in the Republic of singapore. PATIENT: A 53-year-old Chinese woman with a history of optic neuritis developed an episode of left hemiparesis leading to a diagnosis of multiple sclerosis. Serial neuroimaging studies revealed an active demyelinating plaque in the cervical area that later progressed into a syrinx. Over a period of 1 year she also developed signs of sympathetic dysfunction including horner syndrome of the left eye and complex regional pain syndrome in the left hand. CONCLUSIONS: A case of the optic-spinal phenotype of multiple sclerosis that is commonly observed in Southeast asia is described. This characteristically tissue-destructive form of multiple sclerosis resulted in syringomyelia complicated by a complex regional pain syndrome. Possible pathogenic mechanisms for these associations are discussed.- - - - - - - - - - ranking = 1keywords = demyelinating (Clic here for more details about this article) |
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