Vestibular evoked myogenic potentials in multiple sclerosis: clinical and imaging correlations

2004 ◽  
Vol 10 (3) ◽  
pp. 316-321 ◽  
Author(s):  
D Alpini ◽  
L Pugnetti ◽  
D Caputo ◽  
F Cornelio ◽  
S Capobianco ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Clinical Signs ◽  
Clinical Findings ◽  
Vestibular Evoked Myogenic Potentials ◽  
Vestibular Disorders ◽  
Mri Findings ◽  
Mean Delay ◽  
Stance Control ◽  
Specific Skill ◽  
Prolonged Latency

Patients with multiple sclerosis (MS) frequently report symptoms related to vestibular disorders in the course of their disease. At present, the fundamental tests assessing vestibulospinal involvement are posturography and vestibular evoked myogenic potentials (VEMPs). While posturography cannot be performed in every subject requiring minimal stance control, VEMPs do not require any specific skill on the part of the subjects and they may be investigated in all patients able to sit. VEMPs were recorded for 40 patients (17 men, 23 women; mean age 38 years, range 17-71 years) fulfilling diagnostic criteria of clinically defined MS, by means of rarefaction clicks, recording modulation of sterno-cleido-mastoideus tonic contraction saccule-mediated modulation. VEMPs were found to be abnormal in 28 of 40 patients. In 18 of the cases the VEMPs were asymmetric, i.e., had a prolonged latency on one side. In six cases latency was increased on both sides (mean delay 4.1 ms). In four subjects VEMPs were absent on one side. C oncordance with clinical findings of presence/absence of brainstem involvement was found in 55% and with MRI findings in 65% of the cases. A bnormal VEMPs indicated brainstem dysfunction in four patients (10%) with normal MRI and no specific clinical signs.

Discrepancies in the interpretation of clinical symptoms and signs in the diagnosis of multiple sclerosis. A proposal for standardization

2005 ◽  
Vol 11 (2) ◽  
pp. 227-231 ◽  
Author(s):  
Bernard MJ Uitdehaag ◽  
Ludwig Kappos ◽  
Lars Bauer ◽  
Mark S Freedman ◽  
David Miller ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Demyelinating Disease ◽  
Clinical Symptoms ◽  
Clinical Findings ◽  
Large Trial ◽  
Mri Findings ◽  
Mcdonald Criteria ◽  
Magnetic Resonance Imaging Mri ◽  
Eligibility Assessment

The new McDonald diagnostic criteria for multiple sclerosis (MS) incorporate detailed criteria for the interpretation and classification of magnetic resonance imaging (MRI) findings, but, in contrast, provide no instructions for the interpretation of clinical findings. Because MS according to the McDonald criteria is one of the primary endpoints in a large trial enrolling patients after the first manifestation suggestive for a demyelinating disease (BENEFIT study), it was decided to organize a centralized eligibility assessment for this trial. During this eligibility assessment it was observed that there were marked inconsistencies in the decisions of participating neurologists with respect to the classification of clinical symptoms as being caused by one or more lesions provoking discussions in about one in every five patients. This paper describes these inconsistencies and their sources, and recommends a systematic approach that attempts to reduce the variability in interpreting clinical findings.

scholarly journals 164 Dementia, a Familial Affair

2019 ◽  
Vol 48 (Supplement_3) ◽  
pp. iii17-iii65
Author(s):  
Aqueel Qureshi ◽  
Ahmed Gabr ◽  
Daniel Zulkifli ◽  
Elijah Chaila ◽  
Margaret O'Connor
Keyword(s):  
Family History ◽  
Rare Case ◽  
Clinical Signs ◽  
Final Diagnosis ◽  
Clinical Findings ◽  
Post Mortem ◽  
Mri Findings ◽  
Progressive Dementia ◽  
Familial Predisposition ◽  
Rasmussen’S Encephalitis

Abstract Background In Ireland, there are approximately 55,000 cases of dementia. One of the rare causes of dementia is Creutzfeldt-Jakob disease (CJD), affecting one person per million each year worldwide. It is a rapidly progressive degenerative fatal disorder with an estimated mortality of 70% within one year. In this case report we present a rare case of possible familial-CJD which presented initially as a stroke mimic. Methods A 64-year old female referred via GP with abnormal left arm athetoid movements and in-coordination. MRI brain demonstrated a small sub-acute hyper-intense lesion in the right basal ganglia on diffusion weighted imaging. Her symptoms were atypical for acute stroke, however an alternative cause was not evident. She was monitored closely with early supported discharge. However the left sided athetoid movements worsened and were associated with intermittent myoclonic jerks and dystonic posturing. She was reassessed with a wider differential including focal impaired seizures, Rasmussen’s encephalitis, and CJD. EEG showed periodic lateralizing epileptic discharges, however patient failed to show any response to anti-epileptic treatment and her clinical course was one of rapid deterioration. Clinical findings and subsequent MRI findings showed new areas of hyperintensity supporting CJD. Results Ultimately our patient deteriorated rapidly resulting in an akinetic and abulic state, resulting in death. A final diagnosis of sporadic-CJD was made based on rapid progressive deterioration and findings on MRI as well as confirmation on post-mortem brain pathology. A deeper review of family history revealed a sister who had passed away years prior with rapid progressive neurological illness. Her work-up showed clinical signs and EEG findings supporting CJD, however there was no post mortem to confirm her diagnosis. Conclusion This case highlights the importance of genetics shaping phenotypes and that consideration should always be given to a full relevant family history. It also shows a rare case of rapidly progressive dementia confirmed due to CJD with a likely underlying familial predisposition.

No major association of A poE genotype with disease characteristics and MRI findings in multiple sclerosis

2004 ◽  
Vol 10 (3) ◽  
pp. 272-277 ◽  
Author(s):  
J NP Zwemmer ◽  
T van Veen ◽  
L van Winsen ◽  
G J van Kamp ◽  
F Barkhof ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Disease Severity ◽  
Disease Susceptibility ◽  
Age At Onset ◽  
Clinical Findings ◽  
Minor Role ◽  
Published Data ◽  
Genotype Distribution ◽  
Mri Findings ◽  
A Minor

Background: Whereas a number of studies suggest that the A poE polymorphism is not associated with disease susceptibility in multiple sclerosis (MS), results with regard to disease severity, however, are conflicting. Some studies suggest an unfavourable role of the 4 allele. This study was performed to assess the association of the A poE polymorphism with both disease susceptibility and disease course in a large group of MS patients using clinical and MRI measures. In addition the data were combined with available data from the literature. Methods: In a group of 408 patients with clinically definite MS, genotype distribution was compared with that of 144 healthy controls. C ombined analysis of published data on the association of A poE polymorphism with MS was performed. Demographic and clinical findings were recorded and related to the A poE genotype. In a subgroup, longitudinal MRI findings were available and related to the A poE genotype. Results: No significant differences were found in the distribution of genotypes between MS patients and controls. C ombined analysis of published data showed a slightly increased susceptibility for MS in 2-carriers. Disease character istics (including age at onset and onset type), disease severity (progression index, time to reach EDSS 6) and MRI findings (lesion volumes and atrophy measures) were not associated with carriership o 2 or 4. Conclusions: In this cohort no association of the A poE genotype with disease susceptibility nor clinical and MRI measures could be identified. However, combined analysis of published data could not definitely exclude the possibility of a minor role for 2-carriership in MS.

A CAD system for assessment of MRI findings to track the progression of multiple sclerosis

2007 ◽  
Author(s):  
Alexis Wong ◽  
Arkadiusz Gertych ◽  
Chi-Shing Zee ◽  
Bing Guo ◽  
Brent J. Liu
Keyword(s):  
Multiple Sclerosis ◽  
Mri Findings ◽  
Cad System

scholarly journals Disease phenotype analyses of relapsing-remitting multiple sclerosis in Canada and Saudi Arabia

2015 ◽  
Vol 42 (S1) ◽  
pp. S32-S32
Author(s):  
M Alluqmani ◽  
M Alqermli ◽  
G Blevins ◽  
B Alotibi ◽  
F Giuliani ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Saudi Arabia ◽  
Geographic Location ◽  
Clinical Findings ◽  
Cross Sectional ◽  
Relapsing Remitting ◽  
Similar Disease ◽  
The Mean ◽  
Sectional Analysis ◽  
Relapsing Remitting Multiple Sclerosis

Background: Multiple sclerosis (MS) exhibits a spectrum of clinical findings, especially in relapsing-remitting MS (RR-MS). To assess the effects of geographic location and ethnicity on RR-MS phenotype, we investigated RR-MS patients in Canada and Saudi Arabia. Methods: A retrospective cross-sectional analysis of patients receiving active care in MS Clinics was performed in Medina, Saudi Arabia and Edmonton, Alberta. Demographic and clinical data was collected for each patient. Results: 98 patients with treated RR-MS were recruited (n=51, Medina; n=47, Edmonton); 40 patients were Caucasian (Edmonton) while 46 patients were Bedouin (Medina). Although the disease duration was longer in the Edmonton (5.7+2.3 yr) compared to the Medina group (4.4+1.4 yr) (p<0.05), the mean age of RR-MS onset, relapse rate and EDSS change were similar. The female:male ratio was comparable in Edmonton (35:12) and Medina (32:19), as was the risk of optic neuritis. The likelihood of an infratentorial lesion-associated presentation differed (Edmonton, n=23; Medina; n=13) among groups (p<0.05). Spinal cord lesions on MRI were more frequent in Edmonton (n=18) compared to Medina (n=1) patients (p<0.05). Conclusions: Despite differences in location, ethnicity, and a predominance of infratentorial lesion burden the Edmonton group, the RR-MS phenotype displayed similar disease severity and trajectory in these cohorts.

A second case of Marburg’s variant of multiple sclerosis with vasculitis and extensive demyelination

2011 ◽  
Vol 17 (12) ◽  
pp. 1531-1538 ◽  
Author(s):  
Rania GA Elenein ◽  
Leroy R Sharer ◽  
Stuart D Cook ◽  
Andrew R Pachner ◽  
Jennifer Michaels ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Pathological Finding ◽  
Brain Biopsy ◽  
Original Description ◽  
Clinical Findings ◽  
Polymorphonuclear Neutrophils ◽  
Inflammatory Cell Infiltrate ◽  
Unexpected Finding ◽  
Stereotactic Brain Biopsy ◽  
True Variant

Marburg’s variant of multiple sclerosis is a rapidly progressive and malignant form of multiple sclerosis (MS) that usually leads to severe disability or death within weeks to months without remission. Few cases have been described in the literature since the original description by Marburg. The classic pathological findings usually include highly destructive zones of extensive demyelination, necrosis with dense cellular infiltrate, and giant reactive astrocytes. We report a case of a 31-year-old woman with Marburg’s variant of MS who, over a period of eight months, became totally disabled, blind, and quadriplegic, with vocal cord paralysis, requiring a tracheostomy. The patient underwent diagnostic stereotactic brain biopsy. Clinical findings, magnetic resonance imaging (MRI), serologic and cerebrospinal fluid (CSF) findings, and neuropathology are discussed. MRI showed extensive white matter involvement in the brain and spinal cord that continuously progressed over time. A diagnostic stereotactic brain biopsy revealed extensive active demyelination with unexpected finding of active vasculitis and fibrinoid necrosis with a vascular inflammatory cell infiltrate, including polymorphonuclear neutrophils and rare eosinophils. Serologic work-up for vasculitis and neuromyelitis optica was unremarkable and the CSF showed only one oligoclonal band (OCB) not present in serum. This is the second case of Marburg’s variant of MS that demonstrated both demyelination and vasculitis. In our case these features were demonstrated simultaneously, even though the demyelination was the predominant pathological finding. Since vasculitis is not a feature of classic MS, these findings pose the question as to whether Marburg’s variant of MS is a true variant or different entity altogether.

CRYAB modulates the activation of CD4+ T cells from relapsing–remitting multiple sclerosis patients

2013 ◽  
Vol 19 (14) ◽  
pp. 1867-1877 ◽  
Author(s):  
Que Lan Quach ◽  
Luanne M Metz ◽  
Jenna C Thomas ◽  
Jonathan B Rothbard ◽  
Lawrence Steinman ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
T Cells ◽  
Cytokine Production ◽  
Clinical Signs ◽  
Healthy Controls ◽  
Relapsing Remitting ◽  
Remitting Multiple Sclerosis ◽  
Multiple Sclerosis Patients ◽  
Relapsing Remitting Multiple Sclerosis

Background: Suppression of activation of pathogenic CD4+ T cells is a potential therapeutic intervention in multiple sclerosis (MS). We previously showed that a small heat shock protein, CRYAB, reduced T cell proliferation, pro-inflammatory cytokine production and clinical signs of experimental allergic encephalomyelitis, a model of MS. Objective: We assessed whether the ability of CRYAB to reduce the activation of T cells translated to the human disease. Methods: CD4+ T cells from healthy controls and volunteers with MS were activated in vitro in the presence or absence of a CRYAB peptide (residues 73–92). Parameters of activation (proliferation rate, cytokine secretion) and tolerance (anergy, activation-induced cell death, microRNAs) were evaluated. Results: The secretion of pro-inflammatory cytokines by CD4+ T cells was decreased in the presence of CRYAB in a subset of relapsing–remitting multiple sclerosis (RRMS) participants with mild disease severity while no changes were observed in healthy controls. Further, there was a correlation for higher levels of miR181a microRNA, a marker upregulated in tolerant CD8+ T cells, in CD4+ T cells of MS patients that displayed suppressed cytokine production (responders). Conclusion: CRYAB may be capable of suppressing the activation of CD4+ T cells from a subset of RRMS patients who appear to have less disability but similar age and disease duration.

T1 hypointense lesion load in secondary progressive multiple sclerosis: a comparison of pre versus post contrast loads and of manual versus semi automated threshold techniques for lesion segmentation

1998 ◽  
Vol 4 (5) ◽  
pp. 408-412 ◽  
Author(s):  
J I O'Riordan ◽  
M Gawne Cain ◽  
A Coles ◽  
L Wang ◽  
D AS Compston ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Strong Correlation ◽  
Measurement Techniques ◽  
Progressive Multiple Sclerosis ◽  
Secondary Progressive Multiple Sclerosis ◽  
Lesion Volume ◽  
Mri Findings ◽  
Lesion Load ◽  
Post Contrast ◽  
Secondary Progressive

Magnetic resonance imaging (MRI) is increasingly being used as a monitoring tool for disease activity in therapeutic trials in multiple sclerosis. There is, however, only a limited relationship between MRI findings and clinical outcome measurements. It has been suggested that hypointense lesion load on T1 weighted imaging has a better correlation with disability than the more conventional T2 hyper intense lesion load. This study was undertaken to (i) evaluate different measurement techniques used to quantify T1 hypointense lesion load, and (ii) to compare lesion load as measured using different parameters and disability. Twenty-five patients with secondary progressive multiple sclerosis, mean age of 40 years (23-57), mean EDSS 5.7 (4-7) were analysed. T2 lesion load on FSE correlated well with both the hypointense lesion load on T1 pre-gadolinium (r=0.8, P50.0001) and T1 post-gadolinium (r=0.8, P50.0001) but less so with the enhancing lesion load (r=0.4, P50.05). There was a very strong correlation with T1 hypo-intense lesion volume pre and post gadolinium (r=0.96, P50.001). However, the EDSS was not correlated with the T2 lesion load (r=70.27, P=0.2), T1 pre-gadolinium load (r=70.3, P=0.1), T1 post gadolinium load (r=70.4, P=0.7) and enhancing lesion load (r=70.28, P=0.2), or with the degree of hypointensity of T1 weighted images determined using the threshold technique. There is a strong correlation between T1 hypointense lesion volume both pre and post gadolinium and also between T1 and T2 lesion volumes.

519 Restless Legs and Periodic Limb Movements in 86 patients with Multiple Sclerosis

SLEEP ◽  
2021 ◽  
Vol 44 (Supplement_2) ◽  
pp. A205-A205
Author(s):  
Davide Sparasci ◽  
Raffaele Ferri ◽  
Anna Castelnovo ◽  
Claudio Gobbi ◽  
Chiara Zecca ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Diagnostic Criteria ◽  
Large Population ◽  
Female Gender ◽  
Clinical Findings ◽  
Periodic Limb Movements ◽  
Limb Movements ◽  
Cross Sectional ◽  
Restless Legs ◽  
Maintenance Of Wakefulness Test

Abstract Introduction The aim of this study was to assess the prevalence of restless legs syndrome (RLS), periodic limb movements during sleep (PLMS) and their overlap in a large population of patients with multiple sclerosis (MS), and to compare clinical and paraclinical findings between patients with and without RLS/PLMS. Methods In this cross–sectional, observational, instrumental study, eighty-six patients (M/F: 27/59; mean age 48.0 ± 10.8 years) with a diagnosis of MS underwent a structured telephone interview assessing the five standard diagnostic criteria for RLS. Seventy-six participants underwent Video-polysomnography and Maintenance of Wakefulness Test (MWT). Instrumental and clinical findings were subsequently statistically compared to investigate their association with RLS and PLMS index (PLMSI). Results RLS and PLMS (PLMSI ≥15/h) prevalence in patients with MS was of 31.4% and 31.6% respectively. Among patients with RLS, 37.5% had a PLMSI ≥15/h. In the group with PLMS, 37.5% met all diagnostic criteria for RLS. No differences were found between patients with and without RLS (F = 0.99, p = 0.45), and between patients with and without a PLMSI ≥15/hour (F = 0.32 p = 0.94) on the pool of clinical and instrumental variables. Conclusion RLS is highly prevalent and severe in patients with MS. The prevalence of PLMS is comparable to the general population. The low percentage of patients with RLS having a high PLMSI, together with the absence of correlation between RLS and female gender and older age, support the existence of a distinct symptomatic form of RLS in MS. Support (if any):

Sign in / Sign up

Export Citation Format

Share Document