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Journal ArticleDOI

Limb ataxia originating from peri-central sulcus demyelinating lesion in multiple sclerosis.

15 Sep 2012-Journal of the Neurological Sciences (Elsevier)-Vol. 320, Iss: 1, pp 136-140
TL;DR: Limb ataxia/intention tremor can be associated with a demyelinating lesion involving cortical and adjacent subcortical white matter, in the absence of rubro-cerebellar lesions, and aggressive therapeutic intervention to control the cortical inflammatory process is recommended.
About: This article is published in Journal of the Neurological Sciences.The article was published on 2012-09-15. It has received 6 citations till now. The article focuses on the topics: Intention tremor & Limb ataxia.
Citations
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Journal ArticleDOI
TL;DR: This paper reviews the literature with regard to MS and corresponding mitochondrial DNA variations and concludes that not only may MS stem from nuclear gene variations/mutations, but also it may arise from mitochondrial gene variations /mutations.

36 citations


Cites background from "Limb ataxia originating from peri-c..."

  • ...Patients with MS present with a broad spectrum of neurological symptoms, involving hypoesthesia [5], fatigue, tiredness, lack of energy and sleepiness [6], depression [7], cognitive impairment [8,9], termer [10], spasticity [7], dysarthria [11], dysphagia [12], bowel problems [13], nystagmus [14], and urinary system failures [15]....

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Journal ArticleDOI
TL;DR: An overview of tremor in MS and future perspectives is provided to help overcome the unmet needs and guide future therapeutic and diagnostic studies in this complex disorder.
Abstract: Tremor is an important and common symptom in patients with multiple sclerosis (MS). It constituted one of the three core features of MS triad described by Charcot in the last century. Tremor could have a drastic impact on patients’ quality of life. This paper provides an overview of tremor in MS and future perspectives with a particular emphasis on its epidemiology (prevalence: 25–58%), clinical characteristics (i.e., large amplitude 2.5–7 Hz predominantly postural or intention tremor vs. exaggerated physiological tremor vs. pseudo-rhythmic activity arising from cerebellar dysfunction vs. psychogenic tremor), pathophysiological mechanisms (potential implication of cerebellum, cerebello-thalamo-cortical pathways, basal ganglia, and brainstem), assessment modalities (e.g., tremor rating scales, Stewart–Holmes maneuver, visual tracking, digitized spirography and accelerometric techniques, accelerometry–electromyography coupling), and therapeutic options (i.e., including pharmacological agents, botulinum toxin A injections; deep brain stimulation or thalamotomy reserved for severe, disabling, or pharmaco-resistant tremors). Some suggestions are provided to help overcome the unmet needs and guide future therapeutic and diagnostic studies in this complex disorder.

13 citations

Journal ArticleDOI
TL;DR: The terminology used to describe movement disorders is reviewed, individual movement disorders and their occurrence in patients with multiple sclerosis are discussed, and treatment options are reviewed.

11 citations

Journal ArticleDOI
TL;DR: More studies are required to determine mechanism of action and to provide more suitable and sustainable interventions to decrease upper limb intention tremor and improve quality of life of individuals with MS.
Abstract: Background: To present the current knowledge on the characteristics, assessment, and treatment of upper limb intention tremor to inform and improve future intervention studies in patients with multiple sclerosis (MS), we conducted a literature review for articles on upper limb intention tremor in patients with MS. Methods: Two reviewers conducted searches in PubMed, Web of Science, and MEDLINE (Ovid). Relevant articles, sorted on inclusion criteria, were examined for descriptions and assessments of upper limb intention tremor, and intervention studies were evaluated based on treatment type. Results: Eight descriptive studies were found reporting on the incidence and severity of tremor, impairments, and lesion load. Ten studies focused on measurement of tremor using various assessments. Intervention studies included eight articles using a diverse set of noninvasive techniques mainly showing transient reduction in tremor amplitude and temporary increase in function. Eighteen studies on pharmacologi...

10 citations

Journal ArticleDOI
TL;DR: The most prevalent movement disorders described in MS include restless leg syndrome, tremor, ataxia, parkinsonism, paroxysmal dyskinesias, chorea and ballism, facial myokymia, including hemif facial spasm and spastic paretic hemifacial contracture, tics, and tourettism.
Abstract: Background: Multiple sclerosis (MS), a subset of chronic primary inflammatory demyelinating disorders of the central nervous system, is closely associated with various movement disorders. These disorders may be due to MS pathophysiology or be coincidental. This review describes the full spectrum of movement disorders in MS with their possible mechanistic pathways and therapeutic modalities. Methods: The authors conducted a narrative literature review by searching for ‘multiple sclerosis’ and the specific movement disorder on PubMed until October 2021. Relevant articles were screened, selected, and included in the review according to groups of movement disorders. Results: The most prevalent movement disorders described in MS include restless leg syndrome, tremor, ataxia, parkinsonism, paroxysmal dyskinesias, chorea and ballism, facial myokymia, including hemifacial spasm and spastic paretic hemifacial contracture, tics, and tourettism. The anatomical basis of some of these disorders is poorly understood; however, the link between them and MS is supported by clinical and neuroimaging evidence. Treatment options are disorder-specific and often multidisciplinary, including pharmacological, surgical, and physical therapies. Discussion: Movements disorders in MS involve multiple pathophysiological processes and anatomical pathways. Since these disorders can be the presenting symptoms, they may aid in early diagnosis and managing the patient, including monitoring disease progression. Treatment of these disorders is a challenge. Further work needs to be done to understand the prevalence and the pathophysiological mechanisms responsible for movement disorders in MS.

8 citations

References
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Journal ArticleDOI
TL;DR: The classification of tremor is based on the distinction between rest, postural, simple kinetic, and intention tremor (tremor during target-directed movements) as mentioned in this paper.
Abstract: This is a proposal of the Movement Disorder Society for a clinical classification of tremors. The classification is based on the distinction between rest, postural, simple kinetic, and intention tremor (tremor during target-directed movements). Additional data from a medical history and the results of a neurologic examination can be combined into one of the following clinical syndromes defined in this statement: enhanced physiologic tremor, classical essential tremor (ET), primary orthostatic tremor, task- and position-specific tremors, dystonic tremor, tremor in Parkinson's disease (PD), cerebellar tremor, Holmes' tremor, palatal tremor, drug-induced and toxic tremor, tremor in peripheral neuropathies, or psychogenic tremor. Conditions such as asterixis, epilepsia partialis continua, clonus, and rhythmic myoclonus can be misinterpreted as tremor. The features distinguishing these conditions from tremor are described. Controversial issues are outlined in a comment section for each item and thus reflect the open questions that at present cannot be answered on a scientific basis. We hope that this statement provides a basis for better communication among clinicians working in the field and stimulates tremor research.

1,939 citations

Journal ArticleDOI
01 Nov 2005-Brain
TL;DR: Global brain pathology in multiple sclerosis is analysed, focusing on the normal-appearing white matter (NAWM) and the cortex, to suggest that multiple sclerosis starts as a focal inflammatory disease of the CNS, which gives rise to circumscribed demyelinated plaques in the white matter.
Abstract: Focal demyelinated plaques in white matter, which are the hallmark of multiple sclerosis pathology, only partially explain the patient's clinical deficits. We thus analysed global brain pathology in multiple sclerosis, focusing on the normal-appearing white matter (NAWM) and the cortex. Autopsy tissue from 52 multiple sclerosis patients (acute, relapsing-remitting, primary and secondary progressive multiple sclerosis) and from 30 controls was analysed using quantitative morphological techniques. New and active focal inflammatory demyelinating lesions in the white matter were mainly present in patients with acute and relapsing multiple sclerosis, while diffuse injury of the NAWM and cortical demyelination were characteristic hallmarks of primary and secondary progressive multiple sclerosis. Cortical demyelination and injury of the NAWM, reflected by diffuse axonal injury with profound microglia activation, occurred on the background of a global inflammatory response in the whole brain and meninges. There was only a marginal correlation between focal lesion load in the white matter and diffuse white matter injury, or cortical pathology, respectively. Our data suggest that multiple sclerosis starts as a focal inflammatory disease of the CNS, which gives rise to circumscribed demyelinated plaques in the white matter. With chronicity, diffuse inflammation accumulates throughout the whole brain, and is associated with slowly progressive axonal injury in the NAWM and cortical demyelination.

1,652 citations


"Limb ataxia originating from peri-c..." refers background in this paper

  • ...Histopathological studies have shown demyelination in the cortical graymatter (GM) of patients withMS [6], and a pathology based classification with four different GM lesion types was proposed....

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Journal ArticleDOI
TL;DR: In this cohort of patients with early-stage multiple sclerosis, cortical demyelinating lesions were frequent, inflammatory, and strongly associated with meningeal inflammation.
Abstract: Background Cortical disease has emerged as a critical aspect of the pathogenesis of multiple sclerosis, being associated with disease progression and cognitive impairment. Most studies of cortical lesions have focused on autopsy findings in patients with long-standing, chronic, progressive multiple sclerosis, and the noninflammatory nature of these lesions has been emphasized. Magnetic resonance imaging studies indicate that cortical damage occurs early in the disease. Methods We evaluated the prevalence and character of demyelinating cortical lesions in patients with multiple sclerosis. Cortical tissues were obtained in passing during biopsy sampling of white-matter lesions. In most cases, biopsy was done with the use of stereotactic procedures to diagnose suspected tumors. Patients with sufficient cortex (138 of 563 patients screened) were evaluated for cortical demyelination. Using immunohistochemistry, we characterized cortical lesions with respect to demyelinating activity, inflammatory infiltrates, ...

924 citations


"Limb ataxia originating from peri-c..." refers result in this paper

  • ...The MRI findings although atypical can be supported by the recently published pathological study [9], demonstrating that a significant percentage of patients with early MS (38% of MS patients in the study), that underwent brain biopsy for large white matter lesions, had additional cortical demyelination with associated leptomeningeal inflammatory infiltrates....

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Journal ArticleDOI
01 Sep 2011-Brain
TL;DR: The findings suggest that meningeal infiltrates may play a contributory role in the underlying subpial grey matter pathology and accelerated clinical course, which is exacerbated in a significant proportion of cases by the presence of B cell follicle-like structures.
Abstract: Meningeal inflammation in the form of ectopic lymphoid-like structures has been suggested to play a prominent role in the development of cerebral cortical grey matter pathology in multiple sclerosis. The aim of this study was to analyse the incidence and distribution of B cell follicle-like structures in an extensive collection of cases with secondary progressive multiple sclerosis with a wide age range and to determine their relationship to diffuse meningeal inflammation, white matter perivascular infiltrates and microglial activation. One hundred and twenty three cases with secondary progressive multiple sclerosis were examined for the presence of meningeal and perivascular immune cell infiltrates in tissue blocks and/or whole coronal macrosections encompassing a wide array of brain areas. Large, dense, B cell-rich lymphocytic aggregates were screened for the presence of follicular dendritic cells, proliferating B cells and plasma cells. Ectopic B cell follicle-like structures were found, with variable frequency, in 49 cases (40%) and were distributed throughout the forebrain, where they were most frequently located in the deep sulci of the temporal, cingulate, insula and frontal cortex. Subpial grey matter demyelinated lesions were located both adjacent to, and some distance from such structures. The presence of B cell follicle-like structures was associated with an accompanying quantitative increase in diffuse meningeal inflammation that correlated with the degree of microglial activation and grey matter cortical demyelination. The median age of disease onset, time to disease progression, time to wheelchair dependence and age at death all differed significantly in these cases when compared with those without B cell follicle-like structures. Our findings suggest that meningeal infiltrates may play a contributory role in the underlying subpial grey matter pathology and accelerated clinical course, which is exacerbated in a significant proportion of cases by the presence of B cell follicle-like structures.

672 citations


"Limb ataxia originating from peri-c..." refers background in this paper

  • ...Despite the underlying inflammatory process [7], cortical lesions are difficult to detect on conventional MRI due to their small size and in general limited gadolinium lesion contrast [8]....

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Journal Article
TL;DR: In contrast to WM lesions and mixed GM-WM lesions, intracortical lesions remain largely undetected with current MR imaging resolution.
Abstract: BACKGROUND AND PURPOSE: Cortical lesions constitute a substantial part of the total lesion load in multiple sclerosis (MS) brain. They have been related to neuropsychological deficits, epilepsy, and depression. However, the proportion of purely cortical lesions visible on MR images is unknown. The aim of this study was to determine the proportion of intracortical and mixed gray matter (GM)-white matter (WM) lesions that can be visualized with postmortem MR imaging. METHODS: We studied 49 brain samples from nine cases of chronic MS. Tissue sections were matched to dual-echo T2-weighted spin-echo (T2SE) MR images. MS lesions were identified by means of myelin basic protein immunostaining, and lesions were classified as intracortical, mixed GM-WM, deep GM, or WM. Investigators blinded to the histopathologic results scored postmortem T2SE and 3D fluid-attenuated inversion recovery (FLAIR) images. RESULTS: Immunohistochemistry confirmed 70 WM, eight deep GM, 27 mixed GM-WM, and 63 purely cortical lesions. T2SE images depicted only 3% of the intracortical lesions, and 3D FLAIR imaging showed 5%. Mixed GM-WM lesions were most frequently detectable on T2SE and 3D FLAIR images (22% and 41%, respectively). T2SE imaging showed 13% of deep GM lesions versus 38% on 3D FLAIR. T2SE images depicted 63% of the WM lesions, whereas 3D FLAIR images depicted 71%. Even after side-by-side review of the MR imaging and histopathologic results, many of the intracortical lesions could not be identified retrospectively. CONCLUSION: In contrast to WM lesions and mixed GM-WM lesions, intracortical lesions remain largely undetected with current MR imaging resolution.

420 citations


"Limb ataxia originating from peri-c..." refers background in this paper

  • ...Despite the underlying inflammatory process [7], cortical lesions are difficult to detect on conventional MRI due to their small size and in general limited gadolinium lesion contrast [8]....

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