Cases reported "Multiple Sclerosis"

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1/24. Isolated trochlear nerve palsy in patients with multiple sclerosis.

    The authors describe five patients with trochlear nerve palsy and MS to characterize this rare association. In two patients, trochlear nerve palsy was the initial clinical manifestation of MS. In the other three patients, this sign occurred after previous neurologic events. MRI did not identify a lesion of the fourth nerve nucleus or fascicle. ophthalmoplegia resolved within 2 months in four of the five patients. A reason this association is rare is that the fascicular course of the trochlear nerve is exposed to little myelin.
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2/24. intention myoclonus of multiple sclerosis, its patho-anatomical basis and its stereotactic relief.

    The typical multiple sclerosis case considered here is especially informative from both the standpoint of its clinical course and on the basis of the autopsy findings. The foci responsible for the severe bilateral intention myoclonus of the trunk and limbs are the nerve cell losses in both red nuclei due to extensive and almost complete demyelination. Thereby the triangle of Mollaret between the red nucleus, inferior olives and dentate nucleus is involved as the patho-physiological circuit responsible for myoclonus. Stereotactic coagulation of dentato-thalamic fibres resulted in complete relief of intention myoclonus. With regard to the triggering of fresh demyelinating foci by stereotactic interventions, our point of view is as follows: Although a stereotactic operation introduces the possibility of triggering new demyelinating foci in less than 10% of the cases, such a possibility does not represent an absolute contra-indication to the stereotactic treatment of action myoclonus in multiple sclerosis, if the patient is informed accordingly.
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3/24. multiple sclerosis with caudate lesions on MRI.

    A 31-year-old woman displayed sleepiness and impairment of recent memory. T2-weighted MRI revealed high signal intensity lesions in the bilateral basal ganglia, thalamus, and brainstem. Although remission was achieved with corticosteroid therapy, she again displayed memory dysfunction and emotional disturbance one year later, at which time MRI disclosed new lesions in the right caudate nucleus and left frontal white matter. Corticosteroid therapy lead to improvement, and she suffered no recurrence on maintenance steroid therapy. These findings suggest that caudate lesions do occur in multiple sclerosis, the manifestations of which can be abulia and memory dysfunction, as in the present case.
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4/24. Perverted head-shaking nystagmus: a possible mechanism.

    The authors describe a patient with acute MS who developed vertigo (tumbling) and downbeat nystagmus upon horizontal head oscillation (perverted head-shaking nystagmus). The only abnormality on brain MRI was a hyperintense signal in the caudal medulla that contains the nucleus Roller and nucleus intercalatus. These nuclei project to structures involved in the velocity storage system for horizontal vestibulocular reflex (VOR) and vertical VOR, and also to the vestibular cerebellum. The authors offer possible mechanisms for perverted nystagmus in this patient.
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5/24. Electrophysiological confirmation of the zona incerta as a target for surgical treatment of disabling involuntary arm movements in multiple sclerosis: use of local field potentials.

    Lesioning or chronic deep brain stimulation (DBS) of the nucleus ventralis intermedius results in abolition of tremor in the contralateral limbs in Parkinson's disease (PD) and also in essential tremor. Recently, chronic DBS of the subthalamic nucleus has also proved to be very effective in reducing contralateral limb tremor in PD. These targets have been less effective in controlling the complex limb tremor often seen in multiple sclerosis (MS). Consequently, other targets have been sought in cases of MS with tremor. We describe a patient with MS with disabling proximal and distal involuntary arm movements in whom we were able to obtain sustained control of contralateral arm tremor and achieve functional improvement of the affected arm by chronic DBS of the region of the zona incerta. We also highlight the important role played by local field potentials recorded from the brain, with simultaneous recording of corresponding EMGs, in target localisation.
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6/24. Acquired sexual paraphilia in patients with multiple sclerosis.

    BACKGROUND: Sexual dysfunction in patients with multiple sclerosis is typically characterized by diminished libido, erectile and ejaculatory dysfunction in men, and poor lubrication and anorgasmy in women. In contrast, hypersexual behavior and paraphilias are distinctly uncommon in this population of patients, but have been associated with various focal brain lesions. PATIENT AND methods: We describe a man with clinically definite multiple sclerosis who developed profound and abrupt disinhibition and paraphilic behavior during an exacerbation. RESULTS: neuroimaging revealed a marked increase in the number of enhancing lesions in the right sides of the hypothalamus and mesencephalon and extending into the right sides of the red nucleus, substantia nigra, and internal capsule. The altered sexual behavior was characterized by an obsessive and insatiable desire to touch women's breasts. CONCLUSIONS: Acquired sexual paraphilic behavior is uncommon in patients with multiple sclerosis but may occur when inflammatory demyelination involves the hypothalamic and septal regions of the basal prosencephalon. Our experience with this man illustrates the great difficulty involved in treating such patients when the paraphilic behavior becomes persistent.
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7/24. Analysis of intention tremor.

    A marked effect of stereotaxic thalamotomy on intention tremor is described and a neurophysiological interpretation is offered. Tremor-generating activity seems to start in the ventral intermediate nucleus (VIM) of the thalamus, as revealed by recording of the unitary activity through a microelectrode at the tip of the insertion needle, after diminution of facilitatory input due to pathology of the cerebellum or its efferent pathway to the cerebrum. This secondary change within the VIM and the loss of facilitatory input leads to an intention tremor as one of the cerebellar symptoms seen in various neurological diseases.
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8/24. Chronic deep brain stimulation for the treatment of tremor in multiple sclerosis: review and case reports.

    BACKGROUND: deep brain stimulation (DBS) offers a non-ablative alternative to thalamotomy for the surgical treatment of medically refractory tremor in multiple sclerosis. However, relatively few outcomes have been reported. OBJECTIVE: To provide a systematic review of the published cases of DBS use in multiple sclerosis and to present four additional patients. methods: Quantitative and qualitative review of the published reports and description of a case series from one centre. RESULTS: In the majority of reported cases (n=75), the surgical target for DBS implantation was the ventrointeromedial nucleus of the thalamus. Tremor reduction and improvement in daily functioning were achieved in most patients, with 87.7% experiencing at least some sustained improvement in tremor control postsurgery. Effects on daily functioning were less consistently assessed across studies; in papers reporting relevant data, 76.0% of patients experienced improvement in daily functioning. Adverse effects were similar to those reported for DBS in other patient populations. CONCLUSIONS: Few of the studies reviewed used highly standardised quantitative outcome measures, and follow up periods were generally one year or less. Nonetheless, the data suggest that chronic DBS often produces improved tremor control in multiple sclerosis. Complete cessation of tremor is not necessarily achieved, there are cases in which tremor control decreases over time, and frequent reprogramming appears to be necessary.
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9/24. Automatic-voluntary dissociation: an unusual facial paresis in a patient with probable multiple sclerosis.

    A patient with multiple sclerosis is described who presented with a unilateral loss of voluntary function of his lower face muscles. However, in an emotional situation, there was strong involuntary innervation of these muscles: automatic-voluntary dissociation. The subcortical afferents to the facial motor nucleus are discussed. It is hypothesized that cortical disinhibition of midbrain nuclei underlies the accentuated involuntary innervation.
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10/24. Acute VIth cranial nerve dysfunction in multiple sclerosis. Evaluation by magnetic resonance imaging.

    VIth nerve palsy is not frequently considered a presenting sign of multiple sclerosis (MS); however, MS has been documented as a fairly common cause of VIth nerve dysfunction. In the present study we have evaluated the clinical features and magnetic resonance imaging (MRI) findings in four MS patients with acute VIth nerve palsy. diplopia as a result of acute VIth nerve palsy was the prominent symptom leading to the diagnosis of MS in all of the individuals. Other signs specifically localizing to the ipsilateral brainstem were absent in these patients. Cranial MRI revealed multiple white matter lesions with a periventricular predominance in all four patients and pontine white matter lesions in three of the patients. These lesions were either adjacent to the VIth nerve nucleus or involved the fasciculus of the VIth nerve or both.
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