A Septuagenarian With Progressive Hemiparesis

Oligoclonal Bands ◽

Hyperintense Lesion ◽

Fluid Analysis ◽

Hemiparetic Gait ◽

Disease Modifying Agents ◽

A 78-year-old man with no pertinent medical history sought care for an 18-month history of progressive right lower extremity weakness, gait impairment, and falls. On neurologic examination, he had a hemiparetic gait. He had normal higher cognitive function and cranial nerve function. Motor examination showed decreased bulk over the right hand with no fasciculations, mild spasticity over the right leg, and right hemiparesis with an upper motor neuron pattern. Deep tendon reflexes were brisk throughout his limbs, and he had an extensor plantar reflex on the right side. He had impaired vibratory sense at the toes, with otherwise normal sensory and coordination examinations. Magnetic resonance imaging (MRI) of the brain showed ovoid periventricular and punctate subcortical and deep white matter T2 hyperintense foci. Some of these had corresponding T1 hypointensity. MRI of the cervical spine showed 1 eccentrically located T2 hyperintense lesion over the right lateral aspect of C2. Cerebrospinal fluid analysis showed no pleocytosis, an increased protein concentration of 66 mg/dL, and 4 unique oligoclonal bands. A diagnosis of primary progressive multiple sclerosis, very late onset, was made. With any diagnosis of late-onset multiple sclerosis, a decision about whether multiple sclerosis disease-modifying agents are indicated should be carefully considered. Our older patient had a progressive disease course, and neuroimaging studies did not reveal evidence of active disease. Based on this, a decision was made to monitor him clinically and radiologically. Management of spasticity with regular daily stretching exercises was discussed with him. A first clinical manifestation of multiple sclerosis can occur at a later-than-typical age. Most studies consider an onset at age 50 years or older to be late-onset multiple sclerosis, whereas first symptoms occurring at age 60 years or older are commonly referred to as very late–onset MS.

Progressive Spasticity With a Single Magnetic Resonance Imaging Lesion

10.1093/med/9780197583425.003.0006 ◽

2021 ◽

pp. 20-21

Author(s):

Samantha A. Banks ◽

Eoin P. Flanagan

Keyword(s):

Magnetic Resonance Imaging ◽

Multiple Sclerosis ◽

Magnetic Resonance ◽

Oligoclonal Bands ◽

Resonance Imaging ◽

Fluid Analysis ◽

Skin Cancers ◽

Clinical Episode ◽

History Of ◽

A 59-year-old White man with a history of excised basal and squamous cell skin cancers was evaluated for gait difficulties. He had erectile dysfunction but no bowel or bladder dysfunction. He also reported fatigue. He began using a cane for ambulation 2 weeks before evaluation at our facility. His medications included vitamin D and sildenafil. He was a lifelong nonsmoker and had no family history of multiple sclerosis. Neurologic examination at the time of our evaluation 3 years after onset was notable for a positive Hoffman sign on the right and mild weakness of the right triceps but preserved strength elsewhere. He had a spastic gait with moderate spasticity in both lower extremities, hyperreflexic patellar and ankle jerks bilaterally, and bilateral positive Babinski sign. The remainder of the examination was essentially normal. Magnetic resonance imaging of the brain showed a single lesion at the cervicomedullary junction and medullary pyramids, more prominent on the right. There was also some accompanying atrophy that was also visible on cervical spine magnetic resonance imaging. Results of cerebrospinal fluid analysis showed a normal white blood cell count, increased protein concentration (108 mg/dL), and positive oligoclonal bands. The progressive nature of his symptoms, spinal fluid results, and lesion appearance were all consistent with a diagnosis of progressive solitary sclerosis. At the time this patient was seen, no immunomodulatory medications for progressive solitary sclerosis were approved, so no immunomodulatory medication was tried. Ongoing symptomatic management was recommended. Progressive solitary sclerosis is a rare variant of multiple sclerosis in which patients have a single central nervous system demyelinating lesion and development of motor progression attributable to that lesion. Patients can initially have a clinical episode followed by progression or can have a progressive course without an initial relapse.

Bilateral internuclear ophthalmoplegia in multiple sclerosis of very late onset

10.5327/1516-3180.716 ◽

2021 ◽

Author(s):

Guilherme Drumond Jardini Anastacio ◽

Stella de Angelis Trivellato ◽

Hendrick Henrique Fernandes Gramasco ◽

Ana Claudia Pires Carvalho ◽

Omar Gurrola Arambula ◽

...

Keyword(s):

Multiple Sclerosis ◽

Visual Acuity ◽

Demyelinating Disease ◽

Hip Prosthesis ◽

Age Of Onset ◽

Late Onset ◽

Oligoclonal Bands ◽

Internuclear Ophthalmoplegia ◽

Lower Limb Pain ◽

Context: Multiple sclerosis (MS) is the most common demyelinating disease of the central nervous system. The average age of onset is in the range of 28 to 31 years, but it can occur from the first year of life or after the seventh decade. If diagnosed over 60 years of age, it is called very late onset, with low prevalence. Bilateral internuclear ophthalmoplegia is a rare syndrome characterized by bilateral restriction of the adduction of the eyes in the conjugated horizontal gaze, which is generally secondary to demyelinating and vascular diseases. Case report: A 64-year-old man, brown, with a history of alcoholism, gout, hypothyroidism, arterial hypertension and generalized osteoarthritis with a right hip prosthesis with difficulty walking, presented a five-day subacute bilateral visual turbidity and binocular horizontal diplopia. On physical examination, he presented bilateral internuclear ophthalmoplegia, with a reduction in bilateral visual acuity worse on the right, in addition to lower limb pain pseudoparesis, with bilateral Babinski sign. Investigation with visual evoked potential demonstrated bilateral demyelinating optic neuropathy on the right, cerebrospinal fluid with more than two oligoclonal bands present, neuroaxis magnetic resonance imaging showing a change in the signal of the right optic nerve and multiple demyelinating lesions, fulfilling the criteria for dissemination in space and time. Therapy with Methylprednisolone 1g / day for 5 days was performed, with improvement of the condition, maintaining only visual sequelae with decreased visual acuity, and disease-modifying therapy was also started with stabilization of the condition. Conclusions: Despite being more rare, very late-onset MS should be considered as a diagnosis, even with singular presentations as in the case reported.

Progression in disability and regional grey matter atrophy in relapsing–remitting multiple sclerosis

10.1177/1352458513493034 ◽

2013 ◽

Vol 20 (2) ◽

pp. 202-213 ◽

Cited By ~ 22

Author(s):

Louis Hofstetter ◽

Yvonne Naegelin ◽

Lukas Filli ◽

Pascal Kuster ◽

Stefan Traud ◽

...

Keyword(s):

Multiple Sclerosis ◽

Grey Matter ◽

Matched Pair ◽

Disability Progression ◽

Point Change ◽

3 Dimensional ◽

Relapsing Remitting ◽

Background: In multiple sclerosis (MS) regional grey matter (GM) atrophy has been associated with disability progression. Objective: The aim of this study was to compare regional GM volume changes in relapsing–remitting MS (RRMS) patients with progressive and stable disability, using voxel-based morphometry (VBM). Methods: We acquired baseline and 1-year follow-up 3-dimensional (3D) T1-weighted magnetic resonance imaging (MRI) data of RRMS patients, using two 1.5-Tesla scanners. Patients were matched pair-wise with respect to age, gender, disease duration, medication, scanner and baseline Expanded Disability Status Scale (EDSS) into 13 pairs, with either progressive EDSS (≥ 1 point change y-1) or stable EDSS, as well as into 29 pairs with either progressive Multiple Sclerosis Functional Composite (MSFC) at ≥ 0.25% decrease in y-1 in any component, or stable MSFC. We analysed longitudinal regional differences in GM volumes in the progressive and stable EDSS and MSFC groups, respectively, using VBM. Results: Significant GM volume reductions occurred in the right precuneus, in the progressive EDSS group. Differential between-group effects occurred in the right precuneus and in the postcentral gyrus. Further longitudinal GM volume reductions occurred in the right orbicular gyrus, in the progressive MSFC group, but no between-group differences were observed (non-stationary cluster-wise inference, all Pcorrected < 0.05). Conclusion: These results suggested a direct association of disability progression and regional GM atrophy in RRMS.

Amyotrophic Lateral Sclerosis and Multiple Sclerosis Overlap: A Case Report

Case Reports in Medicine ◽

10.1155/2012/324685 ◽

2012 ◽

Vol 2012 ◽

pp. 1-4 ◽

Cited By ~ 5

Author(s):

Francesca Trojsi ◽

Anna Sagnelli ◽

Giovanni Cirillo ◽

Giovanni Piccirillo ◽

Cinzia Femiano ◽

...

Keyword(s):

Multiple Sclerosis ◽

Amyotrophic Lateral Sclerosis ◽

Muscular Atrophy ◽

Past History ◽

Pathological Process ◽

Oligoclonal Bands ◽

Csf Analysis ◽

The Right ◽

Lateral Sclerosis

The concurrence of amyotrophic lateral sclerosis (ALS) and multiple sclerosis (MS) is extremely rare. We reported the case of a 33-year-old woman with a past history of paresthesias at the right hand, who developed progressive quadriparesis with muscular atrophy of limbs and, finally, bulbar signs and dyspnea. Clinical and neurophysiologic investigations revealed upper and lower motor neuron signs in the bulbar region and extremities, suggesting the diagnosis of ALS. Moreover, magnetic resonance imaging (MRI) and cerebrospinal fluid (CSF) analysis demonstrated 3 periventricular and juxtacortical lesions, hyperintense in T2 and FLAIR sequences, and 3 liquoral immunoglobulin G (IgG) oligoclonal bands, consistent with diagnosis of primary progressive MS (PPMS). This unusual overlap of ALS and MS leads to the discussion of a hypothetical common pathological process of immunological dysfunction in these two disorders, although the role of immune response in ALS remains ambivalent and unclear.

Cervical cord myelin abnormality is associated with clinical disability in multiple sclerosis

10.1177/13524585211001780 ◽

2021 ◽

pp. 135245852110017

Author(s):

Lisa Eunyoung Lee ◽

Irene M Vavasour ◽

Adam Dvorak ◽

Hanwen Liu ◽

Shawna Abel ◽

...

Keyword(s):

Multiple Sclerosis ◽

Dorsal Column ◽

Cervical Cord ◽

In Vivo Measurement ◽

Subclinical Disease ◽

Healthy Control ◽

Relapsing Remitting Multiple Sclerosis

Background: Myelin water imaging (MWI) was recently optimized to provide quantitative in vivo measurement of spinal cord myelin, which is critically involved in multiple sclerosis (MS) disability. Objective: To assess cervical cord myelin measurements in relapsing-remitting multiple sclerosis (RRMS) and progressive multiple sclerosis (ProgMS) participants and evaluate the correlation between myelin measures and clinical disability. Methods: We used MWI data from 35 RRMS, 30 ProgMS, and 28 healthy control (HC) participants collected at cord level C2/C3 on a 3 T magnetic resonance imaging (MRI) scanner. Myelin heterogeneity index (MHI), a measurement of myelin variability, was calculated for whole cervical cord, global white matter, dorsal column, lateral and ventral funiculi. Correlations were assessed between MHI and Expanded Disability Status Scale (EDSS), 9-Hole Peg Test (9HPT), timed 25-foot walk, and disease duration. Results: In various regions of the cervical cord, ProgMS MHI was higher compared to HC (between 9.5% and 31%, p ⩽ 0.04) and RRMS (between 13% and 26%, p ⩽ 0.02), and ProgMS MHI was associated with EDSS ( r = 0.42–0.52) and 9HPT ( r = 0.45–0.52). Conclusion: Myelin abnormalities within clinically eloquent areas are related to clinical disability. MWI metrics have a potential role for monitoring subclinical disease progression and adjudicating treatment efficacy for new therapies targeting ProgMS.

Heterogeneous pathological processes account for thalamic degeneration in multiple sclerosis: Insights from 7 T imaging

10.1177/1352458517726382 ◽

2017 ◽

Vol 24 (11) ◽

pp. 1433-1444 ◽

Cited By ~ 17

Author(s):

Céline Louapre ◽

Sindhuja T Govindarajan ◽

Costanza Giannì ◽

Nancy Madigan ◽

Jacob A Sloane ◽

...

Keyword(s):

Multiple Sclerosis ◽

Lesion Volume ◽

Thalamic Lesion ◽

Cortical Lesions ◽

Focal Lesions ◽

Thalamic Degeneration ◽

Relapsing Remitting ◽

Thalamic Lesions ◽

Magnetic Resonance Imaging Mri

Background: Thalamic degeneration impacts multiple sclerosis (MS) prognosis. Objective: To investigate heterogeneous thalamic pathology, its correlation with white matter (WM), cortical lesions and thickness, and as function of distance from cerebrospinal fluid (CSF). Methods: In 41 MS subjects and 17 controls, using 3 and 7 T imaging, we tested for (1) differences in thalamic volume and quantitative T2* (q-T2*) (2) globally and (3) within concentric bands originating from the CSF/thalamus interface; (4) the relation between thalamic, cortical, and WM metrics; and (5) the contribution of magnetic resonance imaging (MRI) metrics to clinical scores. We also assessed MS thalamic lesion distribution as a function of distance from CSF. Results: Thalamic lesions were mainly located next to the ventricles. Thalamic volume was decreased in MS versus controls ( p < 10−2); global q-T2* was longer in secondary progressive multiple sclerosis (SPMS) only ( p < 10−2), indicating myelin and/or iron loss. Thalamic atrophy and longer q-T2* correlated with WM lesion volume ( p < 0.01). In relapsing-remitting MS, q-T2* thalamic abnormalities were located next to the WM ( p < 0.01 (uncorrected), p = 0.09 (corrected)), while they were homogeneously distributed in SPMS. Cortical MRI metrics were the strongest predictors of clinical outcome. Conclusion: Heterogeneous pathological processes affect the thalamus in MS. While focal lesions are likely mainly driven by CSF-mediated factors, overall thalamic degeneration develops in association with WM lesions.

At the heart of primary progressive multiple sclerosis: three cases with diffuse MRI abnormalities only

10.1177/1352458507084591 ◽

2008 ◽

Vol 14 (3) ◽

pp. 428-430 ◽

Cited By ~ 4

Author(s):

JNP Zwemmer ◽

JCJ Bot ◽

B. Jelles ◽

F. Barkhof ◽

CH Polman

Keyword(s):

Multiple Sclerosis ◽

Clinical Course ◽

Primary Progressive Multiple Sclerosis ◽

Resonance Imaging ◽

Focal Lesions ◽

Primary Progressive ◽

Brain And Spinal Cord ◽

Made In

We present three patients with a clinical course and cerebrospinal fluid findings consistent with a diagnosis of primary progressive multiple sclerosis (PPMS). Extensive and repeated magnetic resonance imaging (MRI) examinations showed only diffuse abnormality in brain and spinal cord, but no focal lesions. We propose that these cases represent the most pure form of PPMS, even though according to currently applied criteria this diagnosis can not be made in the absence of focal lesions on MRI. Multiple Sclerosis 2008; 14: 428—430. http://msj.sagepub.com

Early predictors of multiple sclerosis after a typical clinically isolated syndrome

10.1177/1352458514533397 ◽

2014 ◽

Vol 20 (13) ◽

pp. 1721-1726 ◽

Cited By ~ 24

Author(s):

Aurélie Ruet ◽

Georgina Arrambide ◽

Bruno Brochet ◽

Cristina Auger ◽

Eva Simon ◽

...

Keyword(s):

Multiple Sclerosis ◽

At Risk ◽

High Specificity ◽

Clinically Isolated Syndrome ◽

Oligoclonal Bands ◽

Mcdonald Criteria ◽

Early Predictors ◽

Patients At Risk ◽

Periventricular Lesions

Background: The 2010 McDonald criteria allow diagnosing multiple sclerosis (MS) with one magnetic resonance imaging (MRI) scan. Nevertheless, not all patients at risk fulfil criteria at baseline. Other predictive factors (PFs) are: age ≤40 years, positive oligoclonal bands (OBs), and ≥3 periventricular lesions. Objective: The purpose of this study was to evaluate the 2010 McDonald criteria performance and to assess other PFs in patients without dissemination in space (DIS). Methods: Patients with clinically isolated syndrome (CIS) underwent baseline MRI and OB determination with clinical and radiological follow-up. Adjusted hazard ratios (aHRs) for clinically definite MS were estimated for DIS, dissemination in time (DIT), and DIS+DIT. Diagnostic properties at two years were calculated. In cases without DIS, combinations of ≥2 PFs were assessed. Results: A total of 652 patients were recruited; aHRs were 3.8 (2.5–5.8) for DIS, 4.2 (1.9–9.2) for DIT, and 8.6 (5.4–13.8) for DIS+DIT. Sensitivities were 69.6%, 42.3%, and 36.4%, and specificities were 67.3%, 87.9%, and 90.2%, respectively. In patients without DIS, aHRs varied between 2.7–5.5 and specificities ranged from 73.5–89.7% for PF combinations. Conclusion: The high specificity of the 2010 McDonald criteria is confirmed. In patients without DIS, PF combinations could be helpful in identifying those at risk for MS.

Paroxysmal kinesigenic dyskinesia–like phenotype in multiple sclerosis

10.1177/1352458517702535 ◽

2017 ◽

Vol 23 (13) ◽

pp. 1795-1797 ◽

Cited By ~ 4

Author(s):

Roxana Pop ◽

Stefan Kipfer

Keyword(s):

Multiple Sclerosis ◽

Gene Mutations ◽

Brain Magnetic Resonance Imaging ◽

Acute Onset ◽

Intravenous Steroid ◽

Paroxysmal Kinesigenic Dyskinesia ◽

Hyperkinetic Movement Disorder ◽

The Right ◽

Rapid Regression

In April 2015, a 20-year-old woman with multiple sclerosis (MS) presented with acute onset of repetitive abnormal postures and choreatic movements of the right arm, precipitated by voluntary movements (online video 1 and 2). Brain magnetic resonance imaging (MRI) showed a new active MS lesion involving the basal ganglia on the left side (Figure 1(a)). Intravenous steroid treatment resulted in rapid regression of this paroxysmal kinesigenic dyskinesia (PKD)-like hyperkinetic movement disorder. The patient became asymptomatic within 3 months. PKD is characterized by recurrent uni- or bilateral choreoathetosis and usually represents an autosomal dominant inherited disorder caused by PRRT2 gene mutations. As in the present case, a PKD-like phenotype may be associated with MS relapses in presumably genetic negative cases.

Pilot trial of intravenous autologous culture-expanded mesenchymal stem cell transplantation in multiple sclerosis

10.1177/1352458517703802 ◽

2017 ◽

Vol 24 (4) ◽

pp. 501-511 ◽

Cited By ~ 32

Author(s):

Jeffrey A Cohen ◽

Peter B Imrey ◽

Sarah M Planchon ◽

Robert A Bermel ◽

Elizabeth Fisher ◽

...

Keyword(s):

Multiple Sclerosis ◽

Scale Score ◽

Pilot Trial ◽

Expanded Disability Status Scale ◽

Serious Adverse Events ◽

Mesenchymal Stem Cell Transplantation ◽

Disability Status ◽

Magnetic Resonance Imaging Mri

Background: Mesenchymal stem cells (MSCs) exhibit immunomodulatory, tissue-protective, and repair-promoting properties in vitro and in animals. Clinical trials in several human conditions support the safety and efficacy of MSC transplantation. Published experience in multiple sclerosis (MS) is modest. Objective: To assess feasibility, safety, and tolerability and explore efficacy of autologous MSC transplantation in MS. Methods: Participants with relapsing-remitting multiple sclerosis (RRMS) or secondary progressive multiple sclerosis (SPMS), Expanded Disability Status Scale score 3.0–6.5, disease activity or progression in the prior 2 years, and optic nerve involvement were enrolled. Bone-marrow-derived MSCs were culture-expanded and then cryopreserved. After confirming fulfillment of release criteria, 1–2 × 106 MSCs/kg were thawed and administered IV. Results: In all, 24 of 26 screened patients were infused: 16 women and 8 men, 10 RRMS and 14 SPMS, mean age 46.5, mean Expanded Disability Status Scale score 5.2, 25% with gadolinium-enhancing magnetic resonance imaging (MRI) lesions. Mean cell dosage (requiring 1–3 passages) was 1.9 × 106 MSCs/kg (range, 1.5–2.0) with post-thaw viability uniformly ⩾95%. Cell infusion was tolerated well without treatment-related severe or serious adverse events, or evidence of disease activation. Conclusion: Autologous MSC transplantation in MS appears feasible, safe, and well tolerated. Future trials to assess efficacy more definitively are warranted.