Weakness With Neck Flexion

2021 ◽  
pp. 10-13
Author(s):  
Brian G. Weinshenker
Keyword(s):  
Multiple Sclerosis ◽  
Spinal Cord ◽  
Central Nervous System ◽  
Demyelinating Disease ◽  
Progressive Multiple Sclerosis ◽  
Spinal Cord Lesion ◽  
Oligoclonal Bands ◽  
Primary Progressive Multiple Sclerosis ◽  
Neck Flexion ◽  
Cord Lesion

A 51-year-old woman sought care for difficulty “picking up” her right foot after walking for 30 minutes; with rest, the symptoms would subside. A few months later, she reported a “zinging” sensation in her right 4th and 5th fingers and down her right side with neck flexion. Brain magnetic resonance imaging showed tiny nonspecific lesions not suggestive of demyelinating disease and a single T2 hyperintensity at the cervicomedullary junction that did not enhance with gadolinium administration. Cerebrospinal fluid analysis showed 10 oligoclonal bands on isoelectric focusing electrophoresis and a mildly increased immunoglobulin G index of 0.73. The patient was diagnosed with solitary sclerosis, suspected to be a form of central nervous system demyelinating disease strongly related to multiple sclerosis. Treatment was based on a diagnosis of “primary progressive multiple sclerosis,” although the patient did not satisfy the criterion of dissemination in space. The patient was being treated with glatiramer acetate 40 mg 3 times weekly for a presumptive diagnosis of multiple sclerosis before evaluation at Mayo Clinic. After our evaluation, she was advised that no treatments at that time were efficacious to prevent deterioration in patients with primary progressive multiple sclerosis. The mainstay of treatment is physical medicine modalities, including principles of energy conservation and, when necessary, mobility aids, such as braces and canes. The presentation of disease in this patient suggested a chronic myelopathy. Her symptoms did not develop acutely and manifested only after she walked a distance. Symptoms involving the upper and lower extremities and precipitation of Lhermitte sign with neck flexion also suggested a spinal cord lesion, typically a demyelinating lesion. However, a single spinal cord lesion present at the cervicomedullary junction. Oligoclonal bands are detected in patients with central nervous system infections or paraneoplastic disorders that might be associated with myelopathy.

A proposed modification to the McDonald 2010 criteria for the diagnosis of primary progressive multiple sclerosis

2012 ◽  
Vol 19 (8) ◽  
pp. 1095-1100 ◽  
Author(s):  
SB Kelly ◽  
K Kinsella ◽  
M Duggan ◽  
N Tubridy ◽  
C McGuigan ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Spinal Cord ◽  
Diagnostic Criteria ◽  
Progressive Multiple Sclerosis ◽  
Diagnostic Sensitivity ◽  
Oligoclonal Bands ◽  
Primary Progressive Multiple Sclerosis ◽  
Primary Progressive ◽  
Mcdonald Criteria ◽  
The Brain

Background: The diagnostic criteria for primary–progressive multiple sclerosis (PPMS) have undergone revision over the last 20 years. Cerebrospinal fluid oligoclonal bands (CSFOBs) have received less emphasis in recent revisions of the McDonald criteria. The aim of this study was to examine the sensitivity of the diagnostic criteria for PPMS with particular reference to spinal cord criteria and examine the utility of CSFOBs in a cohort of PPMS patients. Methods: All new PPMS diagnoses between 1990 and 2011 were identified. Baseline clinical details and paraclinical evaluations including MRI of the brain, spinal cord, CSF and visually evoked responses (VERs) were assessed. The proportion of patients who met the requirements for diagnosis of PPMS on the basis of Thompson’s and the McDonald Criteria (2001, 2005, 2010) were determined. Results: There were 88/95 PPMS patients who had at least two diagnostic investigations. The sensitivity of Thompson’s and the McDonald 2001 criteria was 64%; the McDonald 2010 revisions gave the highest sensitivity (77%); the McDonald 2005 criteria had intermediate sensitivity (74%). The combination of CSFOBs and MRI of the brain yielded the greatest number of patients demonstrating dissemination in space (DIS) on only two investigations. VERs did not aid diagnosis. Reducing requirements for the number of spinal cord lesions (symptomatic or not) to one increased diagnostic sensitivity to 84%. Conclusion: An alternative criterion requiring two of: i) MRI of the brain with one or more lesions in two of three regions typical for demyelination; ii) the presence of one T2-weighted spinal cord plaque (typical for demyelination); iii) CSFOBs; would increase the diagnostic sensitivity for PPMS.

scholarly journals IMMUNOLOGY OF MULTIPLE SCLEROSIS

2018 ◽  
Vol 24 (2) ◽  
pp. 191 ◽  
Author(s):  
Uleng Bahrun ◽  
Chelvi Wijaya
Keyword(s):  
Multiple Sclerosis ◽  
Central Nervous System ◽  
Nervous System ◽  
Interferon Beta ◽  
The United States ◽  
Progressive Multiple Sclerosis ◽  
Plaque Formation ◽  
Neurological Deficits ◽  
Oligoclonal Bands ◽  
Primary Progressive Multiple Sclerosis

Multiple Sclerosis (MS) is an autoimmune disease that causes myelin destruction in the Central Nervous System (CNS). Characteristics of this disease are perivascular infiltration by inflammatory cells, demyelination and loss of axons, accompanied by multiple plaque formation in the brain and spinal cord. According to the National Multiple Sclerosis Society, 400,000 people suffer from MS in the United States and about 2.5 million people worldwide. The disease is usually diagnosed in patients aged 20-45 years and more often found in females than males with a ratio of 2: 1. Nevertheless, the etiology of MS is still unknown, but it is suspected that genetic and environmental factors may induce a response to central nervous system autoantigen, such as Epstein-Barr Virus (EBV) infection. It then causes edema, demyelination, axon destruction and loss of oligodendrosites, resulting in neurological deficits, plaque formation, scarring and reduced brain volume. Multiple sclerosis symptoms and signs actually vary greatly depending on the location of the lesions and the course of the various diseases. Multiple sclerosis, moreover, can be divided into four clinical categories, namely Clinically Isolated Syndrome (CIS), Relapsing Remitting Multiple Sclerosis (RRMS), Secondary Progressive Multiple Sclerosis (SPMS) and Primary Progressive Multiple Sclerosis (PPMS). Diagnosis of Multiple sclerosis is established based on clinical symptoms and supporting investigations, such as MRI, CSF and neurological examination. In CSF examination, oligoclonal bands are found in over 90% of patients, considered as one of the laboratory criteria supporting the diagnosis of MS. There are four kinds of drugs that have been approved by the FDA as disease modifying therapy for the initial treatment of MS patients, namely interferon beta-1a, subcutaneous (SC) interferon beta-1a, interferon beta-1b and glatiramer acetate  In conclusion, life expectancy in MS patients is only slightly reduced and survival is related to the disability occurred. 

scholarly journals Multiple sclerosis - etiology and diagnostic potential

2017 ◽  
Vol 71 (1) ◽  
pp. 0-0 ◽  
Author(s):  
Joanna Kamińska ◽  
Olga M. Koper ◽  
Kinga Piechal ◽  
Halina Kemona
Keyword(s):  
Multiple Sclerosis ◽  
Demyelinating Disease ◽  
Signs And Symptoms ◽  
Progressive Multiple Sclerosis ◽  
Oligoclonal Bands ◽  
Primary Progressive Multiple Sclerosis ◽  
Diagnostic Challenge ◽  
Csf Analysis ◽  
Diagnostic Potential ◽  
Diagnostic Sensitivity And Specificity

Multiple sclerosis (MS) is a chronic inflammatory and demyelinating disease of autoimmune originate. The main agents responsible for the MS development include exogenous, environmental, and genetic factors. MS is characterized by multifocal and temporally scattered central nervous system (CNS) damage which lead to the axonal damage. Among clinical courses of MS it can be distinguish relapsing-remitting multiple sclerosis (RRMS), secondary progressive multiple sclerosis (SPSM), primary progressive multiple sclerosis (PPMS), and progressive-relapsing multiple sclerosis (RPMS). Depending on the severity of signs and symptoms MS can be described as benign MS or malignant MS. MS diagnosis is based on McDonald’s diagnostic criteria, which link clinical manifestation with characteristic lesions demonstrated by magnetic resonance imaging (MRI), cerebrospinal fluid (CSF) analysis, and visual evoked potentials. Among CSF laboratory tests used to the MS diagnosis are applied: Tibbling & Link IgG index, reinbegrams, and CSF isoelectrofocusing for oligoclonal bands detection. It should be emphasized, that despite huge progress regarding MS as well as the availability of differentdiagnostics methods this disease is still a diagnostic challenge. It may result from fact that MS has diverse clinical course and there is a lack of single test, which would be of appropriate diagnostic sensitivity and specificity for quick and accurate diagnosis.

Preferential spinal cord volume loss in primary progressive multiple sclerosis

2018 ◽  
Vol 25 (7) ◽  
pp. 947-957 ◽  
Author(s):  
Charidimos Tsagkas ◽  
Stefano Magon ◽  
Laura Gaetano ◽  
Simon Pezold ◽  
Yvonne Naegelin ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Spinal Cord ◽  
Clinical Outcomes ◽  
Brain Mri ◽  
Progressive Multiple Sclerosis ◽  
Lesion Volume ◽  
Primary Progressive Multiple Sclerosis ◽  
Primary Progressive ◽  
Over Time ◽  
Spinal Cord Volume

Background: Little is known on longer term changes of spinal cord volume (SCV) in primary progressive multiple sclerosis (PPMS). Objective: Longitudinal evaluation of SCV loss in PPMS and its correlation to clinical outcomes, compared to relapse-onset multiple sclerosis (MS) subtypes. Methods: A total of 60 MS age-, sex- and disease duration-matched patients (12 PPMS, each 24 relapsing-remitting (RRMS) and secondary progressive MS (SPMS)) were analysed annually over 6 years of follow-up. The upper cervical SCV was measured on 3D T1-weighted magnetization-prepared rapid gradient-echo (MPRAGE) images using a semi-automatic software (CORDIAL), along with the total brain volume (TBV), brain T2 lesion volume (T2LV) and Expanded Disability Status Scale (EDSS). Results: PPMS showed faster SCV loss over time than RRMS ( p < 0.01) and by trend ( p = 0.066) compared with SPMS. In contrast to relapse-onset MS, in PPMS SCV loss progressed independent of TBV and T2LV changes. Moreover, in PPMS, SCV was the only magnetic resonance imaging (MRI) measurement associated with EDSS increase over time ( p < 0.01), as opposed to RRMS and SPMS. Conclusion: SCV loss is a strong predictor of clinical outcomes in PPMS and has shown to be faster and independent of brain MRI metrics compared to relapse-onset MS.

A DTI study of the spinal cord lesion in patients with multiple sclerosis during the follow-up after relapse

2016 ◽  
Vol 116 (2. Vyp. 2) ◽  
pp. 21 ◽  
Author(s):  
S. N. Morozova ◽  
V. V. Bryukhov ◽  
O. V. Trifonova ◽  
E. I. Kremneva ◽  
M. V. Krotenkova
Keyword(s):  
Multiple Sclerosis ◽  
Spinal Cord ◽  
Spinal Cord Lesion ◽  
Cord Lesion

The Importance of CSF Oligoclonal Bands in the Diagnosis of Primary Progressive Multiple Sclerosis (P01.139)

Neurology ◽  
2012 ◽  
Vol 78 (Meeting Abstracts 1) ◽  
pp. P01.139-P01.139
Author(s):  
S. Kelly ◽  
K. Kinsella ◽  
M. Duggan ◽  
C. McGuigan ◽  
N. Tubridy ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Progressive Multiple Sclerosis ◽  
Oligoclonal Bands ◽  
Primary Progressive Multiple Sclerosis ◽  
Primary Progressive ◽  
Csf Oligoclonal Bands

scholarly journals Ongoing microstructural changes in the cervical cord underpin disability progression in early primary progressive multiple sclerosis

2020 ◽  
Vol 27 (1) ◽  
pp. 28-38 ◽  
Author(s):  
Rosa Cortese ◽  
Carmen Tur ◽  
Ferran Prados ◽  
Torben Schneider ◽  
Baris Kanber ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Spinal Cord ◽  
Brain Mri ◽  
Progressive Multiple Sclerosis ◽  
Cervical Cord ◽  
Primary Progressive Multiple Sclerosis ◽  
Disability Progression ◽  
Mixed Effect ◽  
Primary Progressive ◽  
Over Time

Background: Pathology in the spinal cord of patients with primary progressive multiple sclerosis (PPMS) contributes to disability progression. We previously reported abnormal Q-space imaging (QSI)-derived indices in the spinal cord at baseline in patients with early PPMS, suggesting early neurodegeneration. Objective: The aim was to investigate whether changes in spinal cord QSI over 3 years in the same cohort are associated with disability progression and if baseline QSI metrics predict clinical outcome. Methods: Twenty-three PPMS patients and 23 healthy controls recruited at baseline were invited for follow-up cervical cord 3T magnetic resonance imaging (MRI) and clinical assessment after 1 year and 3 years. Cord cross-sectional area (CSA) and QSI measures were obtained, together with standard brain MRI measures. Mixed-effect models assessed MRI changes over time and their association with clinical changes. Linear regression identified baseline MRI indices associated with disability at 3 years. Results: Over time, patients deteriorated clinically and showed an increase in cord QSI indices of perpendicular diffusivity that was associated with disability worsening, independently of the decrease in CSA. Higher perpendicular diffusivity and lower CSA at baseline predicted worse disability at 3 years. Conclusion: Increasing spinal cord perpendicular diffusivity may indicate ongoing neurodegeneration, which underpins disability progression in PPMS, independently of the development of spinal cord atrophy.

scholarly journals A Long Interval from a Spinal Cord Lesion to a Subsequent Brain Lesion in Primary Central Nervous System Vasculitis

2019 ◽  
Vol 58 (10) ◽  
pp. 1485-1489
Author(s):  
Tomoya Kon ◽  
Yukihisa Funamizu ◽  
Chieko Suzuki ◽  
Tsugumi Sato ◽  
Hidekachi Kurotaki ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Central Nervous System ◽  
Nervous System ◽  
Brain Lesion ◽  
Spinal Cord Lesion ◽  
Central Nervous System Vasculitis ◽  
Cord Lesion

scholarly journals B-27 Primary Progressive Multiple Sclerosis in an Older Man

2019 ◽  
Vol 34 (6) ◽  
pp. 973-973
Author(s):  
C Schieszler-Ockrassa ◽  
F Bylsma
Keyword(s):  
Multiple Sclerosis ◽  
Cognitive Dysfunction ◽  
Processing Speed ◽  
Demyelinating Disease ◽  
Progressive Multiple Sclerosis ◽  
Fine Motor ◽  
Primary Progressive Multiple Sclerosis ◽  
Primary Progressive ◽  
Motor Dexterity ◽  
One Year

Abstract Objective Multiple sclerosis (MS) is an autoimmune demyelinating disease more common in women than men, usually diagnosed between 20-30 years of age. Approximately 50% of individuals with MS develop cognitive dysfunction, with men and progressive MS subtype cases at higher risk (Beatty & Aupperle, 2002). Mr. Doe’s case is unique because he was diagnosed with primary progressive MS at age 56 and demonstrated only mild cognitive dysfunction. Method Mr. Doe presented to his neurologist with complaints of extreme fatigue, slowed processing, and sensory and motor disturbances. He was seen for neuropsychological evaluation one year after diagnosis and was reassessed one year later. He reported worsening mood including passive suicidal ideation since diagnosis. He reported difficulties with work duties (attorney) and household demands due to gradual motor and sensory disturbances, slowed processing speed, fatigue, and mood disturbance. Results Mr. Doe’s initial neuropsychological assessment revealed variability in auditory working memory, weakness in sustained visual attention, and mild deficits in upper extremity fine motor dexterity. Memory, executive functioning, language, and processing speed were all intact unless a motor component was involved (mild decline after one year). His cognitive performances remained generally stable after one year, but depression, anxiety, and hopelessness levels were all significantly worse. Conclusions Although Mr. Doe’s impairments are extremely mild and somewhat unexpected given the primary progressive MS diagnosis, his gender, and age, the affected domains are consistent with the diagnosis. This case demonstrates the importance of understanding base rates for conditions we assess, but also not ruling out lower base rate conditions.

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