Leber’s hereditary optic neuropathy associated with a multiple-sclerosis-like picture in a man

2011 ◽  
Vol 17 (6) ◽  
pp. 763-766 ◽  
Author(s):  
Antonella La Russa ◽  
Rita Cittadella ◽  
Virginia Andreoli ◽  
Paola Valentino ◽  
Francesca Trecroci ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Spinal Cord ◽  
Optic Neuropathy ◽  
Brain Mri ◽  
Resonance Imaging ◽  
Gadolinium Enhancement ◽  
Demyelinating Lesions ◽  
Male Patients ◽  
Magnetic Resonance Imaging Mri ◽  
Symptoms And Signs

A 35-year-old young man displayed Leber’s optic neuropathy (LHON) due to T14484C and multiple sclerosis (MS) phenotype that was dominated by symptoms and signs of spinal cord impairment. Magnetic resonance imaging (MRI) revealed demyelinating lesions extending from D6 to D11 in the spinal cord with gadolinium enhancement, while only three linear demyelinating lesions were seen on brain MRI. In the literature, a major involvement of the spinal cord was already reported in three of four male patients with the 14484 LHON mutation who developed MS, but the reasons of this peculiar association remain unknown, and further research in this area is needed.

Polyregional and hemispheric syndromes: a study of these uncommon first attacks in a CIS cohort

2007 ◽  
Vol 13 (6) ◽  
pp. 731-736 ◽  
Author(s):  
R. Pelayo ◽  
M. Tintoré ◽  
A. Rovira ◽  
J. Rio ◽  
C. Nos ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Multiple Sclerosis ◽  
Spinal Cord ◽  
Clinical Symptoms ◽  
Brain Mri ◽  
Resonance Imaging ◽  
Number Of Patients ◽  
Cns Demyelination ◽  
Magnetic Resonance Imaging Mri ◽  
Definition Of

Clinically isolated syndromes (CIS) classically refer to optic neuritis (ON), brainstem or spinal cord syndromes. Less common first episodes suggestive of central nervous system (CNS) demyelination, such as hemispheric or clinically polyregional syndromes, have been only slightly studied. The aim of this study was to describe these CIS topographies in our cohort of patient with a CIS. We evaluated 320 patients with a CIS, and classified the topographies of the attacks according to clinical symptoms only into CIS of the optic nerve (123), brainstem (78), spinal cord (89), hemispheric (6), polyregional (12) or undetermined (12) topographies. Patients underwent brain MRI within three months of their first attack, and again 12 months later. Conversion to multiple sclerosis (MS), determined either clinically or by magnetic resonance imaging (MRI), was evaluated according to topography. Hemispheric and polyregional syndromes were closer to brainstem or spinal cord syndromes than ON in clinical and MRI conversion terms, although a statistical analysis was not performed because of the small number of patients. There are differences between several studies in the definition, and, therefore, the prevalence of these so-called atypical CIS. Consensus on the denomination and definition of these syndromes must be reached. Multiple Sclerosis 2007; 13: 731-736. http://msj.sagepub.com

Onset of progressive motor impairment in patients with critical central nervous system demyelinating lesions

2020 ◽  
pp. 135245852094098
Author(s):  
Roman M Kassa ◽  
Elia Sechi ◽  
Eoin P Flanagan ◽  
Timothy J Kaufmann ◽  
Orhun H Kantarci ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Brain Mri ◽  
Age At Onset ◽  
Motor Impairment ◽  
Resonance Imaging ◽  
Total Brain ◽  
Demyelinating Lesions ◽  
Magnetic Resonance Imaging Mri ◽  
Brain And Spinal Cord

Objective: To compare progressive motor impairment onset attributable to a “critical” central nervous system (CNS) demyelinating lesion in patients with highly restricted versus unlimited magnetic resonance imaging (MRI) lesion burden. Methods: We identified 135 patients with progressive motor impairment for ⩾1 year attributable to a “critical” demyelinating lesion with: MRI burden of 1 lesion (“progressive solitary sclerosis”), 2–5 lesions (“progressive paucisclerosis”), or unrestricted (>5) lesions and “progressive unilateral hemiparesis.” Neuroradiology review of brain and spinal cord MRI documented unequivocally demyelinating lesions. Results: A total of 33 (24.4%) patients had progressive solitary sclerosis; 56 (41.5%) patients had progressive paucisclerosis; and 46 (34.1%) patients had progressive unilateral hemiparesis. Median age at onset of progressive motor impairment was younger in progressive solitary sclerosis (49 years; range 24–73) and progressive paucisclerosis (50 years; range 30–64) than in progressive unilateral hemiparesis (54 years; range 39–77; p = 0.02 and p = 0.003, respectively). Within progressive unilateral hemiparesis, motor-progression onset was similar between those with 4–10, 11–20, or >20 brain lesions (55, 54, 53 years of age, respectively; p = 0.44). Conclusion: Motor-progression age is similar, but paradoxically earlier, in cohorts with highly restricted CNS lesion burden than in those with unrestricted lesion burden with progressive unilateral hemiparetic MS. The “critical” demyelinating lesion rather than total brain MRI lesion burden is the major contributor to motor-progression onset in these cohorts.

scholarly journals Concurrence of Juvenile Idiopathic Arthritis and Multiple Sclerosis

2011 ◽  
Vol 2011 ◽  
pp. 1-2
Author(s):  
Ben Abdelghani Kaouther ◽  
Souabni Leila ◽  
Belhadj Salwa ◽  
Zakraoui Leith
Keyword(s):  
Multiple Sclerosis ◽  
Juvenile Idiopathic Arthritis ◽  
Visual Evoked Potential ◽  
Evoked Potential ◽  
Brain Mri ◽  
Resonance Imaging ◽  
Cerebral Magnetic Resonance Imaging ◽  
Need To Evaluate ◽  
Magnetic Resonance Imaging Mri ◽  
The Brain

We report a 21-year-old female patient known to have Juvenile idiopathic arthritis (JIA) who later developed multiple sclerosis (MS). The disease was documented on the brain and cerebral magnetic resonance imaging (MRI) and the visual evoked potential. Our case emphasizes the need to evaluate the symptoms and brain MRI carefully. The concurrence of MS and JIA is uncommon. The possible relationship between the 2 diseases was discussed.

scholarly journals Ocular flutter as presenting manifestation of pediatric MOG antibody–associated demyelination: A case report

2018 ◽  
Vol 25 (1) ◽  
pp. 122-125 ◽  
Author(s):  
Marianthi Breza ◽  
Nikoletta Smyrni ◽  
Georgios Koutsis ◽  
Evangelos Anagnostou ◽  
John Tzartos ◽  
...  
Keyword(s):  
Brain Mri ◽  
Brain Magnetic Resonance Imaging ◽  
Oligoclonal Bands ◽  
Resonance Imaging ◽  
Ocular Flutter ◽  
Demyelinating Lesions ◽  
History Of ◽  
Mog Antibodies ◽  
Magnetic Resonance Imaging Mri ◽  
Csf Oligoclonal Bands

A 13-year-old girl presented with a 5-day history of oscillopsia. On examination, ocular flutter and mild cerebellar signs were found. Brain magnetic resonance imaging (MRI) revealed four periventricular and subcortical non-enhancing lesions. Cerebrospinal fluid (CSF) oligoclonal bands were negative. Neuroblastoma or other malignancies were not found. She responded well to a corticosteroid–intravenous immunoglobulin (IVIG) combination and remained symptom-free for 3 years until presenting again with isolated ocular flutter. Brain MRI at this time remained atypical for classic multiple sclerosis (MS) with a predominance of juxtacortical demyelinating lesions. CSF was positive for oligoclonal bands. Serum myelin oligodendrocyte glycoprotein (MOG) antibodies were present. Ocular flutter can be the presenting feature of MOG antibody–associated pediatric demyelination.

scholarly journals Slowly expanding/evolving lesions as a magnetic resonance imaging marker of chronic active multiple sclerosis lesions

2018 ◽  
Vol 25 (14) ◽  
pp. 1915-1925 ◽  
Author(s):  
Colm Elliott ◽  
Jerry S Wolinsky ◽  
Stephen L Hauser ◽  
Ludwig Kappos ◽  
Frederik Barkhof ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Multiple Sclerosis ◽  
Magnetic Resonance ◽  
Brain Mri ◽  
Brain Magnetic Resonance Imaging ◽  
Tissue Loss ◽  
Jacobian Determinant ◽  
Resonance Imaging ◽  
T2 Lesions ◽  
Magnetic Resonance Imaging Mri

Background: Chronic lesion activity driven by smoldering inflammation is a pathological hallmark of progressive forms of multiple sclerosis (MS). Objective: To develop a method for automatic detection of slowly expanding/evolving lesions (SELs) on conventional brain magnetic resonance imaging (MRI) and characterize such SELs in primary progressive MS (PPMS) and relapsing MS (RMS) populations. Methods: We defined SELs as contiguous regions of existing T2 lesions showing local expansion assessed by the Jacobian determinant of the deformation between reference and follow-up scans. SEL candidates were assigned a heuristic score based on concentricity and constancy of change in T2- and T1-weighted MRIs. SELs were examined in 1334 RMS patients and 555 PPMS patients. Results: Compared with RMS patients, PPMS patients had higher numbers of SELs ( p = 0.002) and higher T2 volumes of SELs ( p < 0.001). SELs were devoid of gadolinium enhancement. Compared with areas of T2 lesions not classified as SEL, SELs had significantly lower T1 intensity at baseline and larger decrease in T1 intensity over time. Conclusion: We suggest that SELs reflect chronic tissue loss in the absence of ongoing acute inflammation. SELs may represent a conventional brain MRI correlate of chronic active MS lesions and a candidate biomarker for smoldering inflammation in MS.

Incidence of enhancement of the trigeminal nerve on MRI in patients with multiple sclerosis

2002 ◽  
Vol 8 (1) ◽  
pp. 64-67 ◽  
Author(s):  
A HC van der Meijs ◽  
I L Tan ◽  
F Barkhof
Keyword(s):  
Multiple Sclerosis ◽  
Trigeminal Nerve ◽  
Distal Part ◽  
Phase Iii ◽  
Resonance Imaging ◽  
Gadolinium Enhancement ◽  
Phase Iii Clinical Trial ◽  
Mri Scans ◽  
Peripheral Type ◽  
Magnetic Resonance Imaging Mri

The purpose of this study is to review the incidence of contrast enhancement of the trigeminal nerve on magnetic resonance imaging (MRI) in a large cohort of patients with multiple sclerosis (MS). MRI scans of 851 MS patients participating in a phase III clinical trial were reviewed for the presence of gadolinium enhancement on postcontrast T1-weighted images. If enhancement was present, it was documented whether this involved only the proximal or also the distal part of the fifth nerve, and whether the abnormality was unilateral or bilateral. In 24 (2.8%) patients, enhancement of the trigeminal nerve was observed, and was bilateral in 16 (66.7%) of those. In 19 (79.2%) patients with abnormal nerves, enhancement extended to the distal part of the trigeminal nerve (into Meckel’s cave). The results of this study indicate a high, probably clinically silent, incidence of trigeminal nerve demyelination in MS and frequent involvement of the peripheral type of myelin in MS.

Incidence of enhancement of the trigeminal nerve on MRI in patients with multiple sclerosis

2002 ◽  
Vol 8 (1_suppl) ◽  
pp. 64-67 ◽  
Author(s):  
Ahc van der Meijs ◽  
IL Tan ◽  
F. Barkhof
Keyword(s):  
Multiple Sclerosis ◽  
Trigeminal Nerve ◽  
Distal Part ◽  
Phase Iii ◽  
Resonance Imaging ◽  
Gadolinium Enhancement ◽  
Phase Iii Clinical Trial ◽  
Mri Scans ◽  
Peripheral Type ◽  
Magnetic Resonance Imaging Mri

The purpose of this study is to review the incidence of contrast enhancement of the trigeminal nerve on magnetic resonance imaging (MRI) in a large cohort of patients with multiple sclerosis (MS). MRI scans of 851 MS patients participating in a phase III clinical trial were reviewed for the presence of gadolinium enhancement on postcontrast TI-weighted images. If enhancement was present, it was documented whether this involved only the proximal or also the distal part of the fifth nerve, and whether the abnormality was unilateral or bilateral. In 24 (2.8%) patients, enhancement of the trigeminal nerve was observed, and was bilateral in 16 (66.7%) of those. In 19 (79.2%) patients with abnormal nerves, enhancement extended to the distal part of the trigeminal nerve (into Meckel's cave). The results of this study indicate a high, probably clinically silent, incidence of trigeminal nerve demyelination in MS and frequent involvement of the peripheral type of myelin in MS. Multiple Sclerosis (2002) 8, 64-67

Air pollution is associated to the multiple sclerosis inflammatory activity as measured by brain MRI

2017 ◽  
Vol 24 (12) ◽  
pp. 1578-1584 ◽  
Author(s):  
Roberto Bergamaschi ◽  
Andrea Cortese ◽  
Anna Pichiecchio ◽  
Francesca Gigli Berzolari ◽  
Paola Borrelli ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Air Pollution ◽  
Brain Mri ◽  
Brain Magnetic Resonance Imaging ◽  
Inflammatory Activity ◽  
Resonance Imaging ◽  
Increased Risk ◽  
Immune Therapies ◽  
Magnetic Resonance Imaging Mri ◽  
The Relationship

Background: Some environmental factors have been already associated to increased risk of multiple sclerosis (MS), but it is plausible that additional factors might play a role. Objective: To investigate in MS patients the relationship between inflammatory activity, detected by brain magnetic resonance imaging (MRI) with gadolinium (Gd), and air pollution, namely, particulate matters with diameter less than 10 μm (PM10). Methods: We analyzed from 52 remitting MS patients 226 brain MRIs, 34% with (Gd+MRI) and 66% without (Gd-MRI) T1-Gd-enhancing lesions. Daily recording of PM10 in the 30 days before MRI examination was obtained by monitors depending on the residence of subjects. Results: PM10 levels in the 5, 10, 15, 20, and 25 days before brain MRIs were higher (plus 16%, 21%, 24%, 25%, and 21%, respectively) with reference to Gd+MRI versus Gd-MRI. There was a significant association between Gd+MRI and PM10 levels ( p = 0.013), independent of immune therapies, smoker status, and season. In patients who had two repeated MRIs with opposite outcomes (Gd-MRI and Gd+MRI), PM10 levels were strongly higher in concurrence with Gd+MRI ( p < 0.0001). Conclusion: Our findings suggest that air pollution may be a risk factor for MS favoring inflammatory exacerbations.

Inflammatory activity following motor progression due to critical CNS demyelinating lesions

2020 ◽  
pp. 135245852094874
Author(s):  
Shreya Nayak ◽  
Elia Sechi ◽  
Eoin P Flanagan ◽  
Steven Messina ◽  
Roman Kassa ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Multiple Sclerosis ◽  
Progressive Multiple Sclerosis ◽  
Inflammatory Activity ◽  
Resonance Imaging ◽  
Disease Modifying Therapies ◽  
Demyelinating Lesions ◽  
Disease Modifying ◽  
Magnetic Resonance Imaging Mri

Background: New inflammatory activity is of unclear frequency and clinical significance in progressive multiple sclerosis (MS); it is uncertain in patient cohorts with motor progression due to critical demyelinating lesions. Objectives: The aim of this study is to determine the likelihood of central nervous system (CNS) inflammatory activity, assessed by new clinical relapses or active magnetic resonance imaging (MRI) lesions, following onset of motor progression due to critical demyelinating lesions. Methods: Patients with progressive upper motor neuron impairment for ⩾1 year attributable to critical demyelinating lesions with single CNS lesion (progressive solitary sclerosis (PSS)), 2 to 5 total CNS demyelinating lesions (progressive “pauci-sclerosis” (PPS)), or >5 CNS demyelinating lesions and progressive exclusively unilateral monoparesis or hemiparesis (PUHMS) were identified. Clinical data were reviewed for acute MS relapses, and subsequent MRI was reviewed for active T1-gadolinium-enhancing or T2-demyelinating lesions. Results: None of the 91 patients (22 PSS, 40 PPS, 29 PUHMS) identified experienced clinical relapses over a median clinical follow-up of 93 months (range: 12–518 months). Nine patients (10%) developed active lesions over median 84 months radiologic follow-up (range: 12–518 months). Active lesions occurred in 24% PUHMS, 5% PSS, and 3% PPS cohorts. Conclusion: New inflammatory activity, defined by active lesions and clinical relapses following motor progression in patients with critical demyelinating lesions, is low. Disease-modifying therapies that reduce demyelinating relapses and active MRI lesions are of uncertain benefit in these cohorts.

Mean upper cervical cord area (MUCCA) measurement in long-standing multiple sclerosis: Relation to brain findings and clinical disability

2014 ◽  
Vol 20 (14) ◽  
pp. 1860-1865 ◽  
Author(s):  
Marita Daams ◽  
Florian Weiler ◽  
Martijn D Steenwijk ◽  
Horst K Hahn ◽  
Jeroen JG Geurts ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Spinal Cord ◽  
Brain Magnetic Resonance Imaging ◽  
Cervical Cord ◽  
Healthy Controls ◽  
Spinal Cord Atrophy ◽  
Disability Status ◽  
Upper Cervical ◽  
Male Patients ◽  
Magnetic Resonance Imaging Mri

Background: The majority of patients with multiple sclerosis (MS) present with spinal cord pathology. Spinal cord atrophy is thought to be a marker of disease severity, but in long-disease duration its relation to brain pathology and clinical disability is largely unknown. Objective: Our aim was to investigate mean upper cervical cord area (MUCCA) in patients with long-standing MS and assess its relation to brain magnetic resonance imaging (MRI) measures and clinical disability. Methods: MUCCA was measured in 196 MS patients and 55 healthy controls using 3DT1-weighted cervical images obtained at 3T MRI. Clinical disability was measured using the Expanded Disability Status Scale (EDSS), Nine-Hole-Peg test (9-HPT), and 25 feet Timed Walk Test (TWT). Stepwise linear regression was performed to assess the association between MUCCA and MRI measures, and between MUCCA and clinical disability. Results: MUCCA was smaller (mean 11.7%) in MS patients compared with healthy controls (72.56±9.82 and 82.24±7.80 mm2 respectively; p<0.001), most prominently in male patients. MUCCA was associated with normalized brain volume, and number of cervical cord lesions. MUCCA was independently associated with EDSS, TWT, and 9-HPT. Conclusion: MUCCA was reduced in MS patients compared with healthy controls. It provides a relevant marker for clinical disability in long-standing disease, independent of other MRI measures.

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