Hyperkalemic Periodic Paralysis and Permanent Weakness: 3-T MR Imaging Depicts Intracellular<sup>23</sup>Na Overload—Initial Results

Amarteifio, Erick; Nagel, Armin M.; Weber, Marc‐André; Jurkat‐Rott, Karin; Lehmann‐Horn, Frank

doi:10.1148/radiol.12110980

Cited by 41 publications

(30 citation statements)

References 29 publications

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“…The majority of respondents noted progressive permanent muscle weakness during childhood (69.2 %), while only a minority of individuals reported such changes during teenage years (33.3 %) and from age 20–39 (30.0 %). This is particularly significant as prior literature reports that permanent muscle weakness is a problem of older adults [8, 13]. In line with prior literature, 84.6 % of respondents age 40 and up reported worsening permanent muscle weakness.…”

Section: Resultssupporting

confidence: 75%

“…Serum creatine kinase (CK) values may be elevated, and some individuals exhibit calf hypertrophy. The muscles are typically well-developed [6, 11]; however, a large proportion of individuals with hyperPP develop a chronic progressive proximal myopathy as they age [10, 13]. Parenthetically, individuals without interictal myotonia are much more susceptible to developing this progressive myopathy than are individuals with myotonia [1, 8].…”

Section: Introductionmentioning

confidence: 99%

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Characterization of hyperkalemic periodic paralysis: a survey of genetically diagnosed individuals

et al. 2013

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This exploratory study aims to create an evidence-based comprehensive characterization of hyperkalemic periodic paralysis (hyperPP). HyperPP is a rare genetic disorder that causes episodes of flaccid paralysis. Disease descriptions in the literature are based upon isolated clinical encounters and case reports. We describe the experience of a large cohort of genetically diagnosed individuals with hyperPP. We surveyed genetically characterized individuals age 18 and over to assess disease comorbidities, diagnostic testing, management, and quality of life issues relevant to hyperPP. Myotonia was reported by 55.8 % of subjects and paramyotonia by 45.3 %. There is a relative risk of 3.6 (p < 0.0001) for thyroid dysfunction compared to the general population. Twenty-five percent of subjects experienced their sentinel attack in the second decade of life. It took an average of 19.4 years and visits to four physicians to arrive at the diagnosis of hyperPP. In addition to limbs and hands being affected during attacks, 26.1 % of subjects reported their breathing musculature was affected and 62.0 % reported their facial muscles were affected. There was a lifelong trend of increasing attack frequency, which was particularly common during childhood and adolescence. Approximately one-third of individuals experienced progressive myopathy. Permanent muscle weakness was evident and worsened during childhood and after age 40. Those with no chronic treatment regimen have a RR of 2.3 for inadequate disease control compared to those taking long-term medications. This study revealed a multitude of heretofore unidentified characteristics of hyperPP, in addition to providing a different perspective on some previously held notions regarding the condition.Electronic supplementary materialThe online version of this article (doi:10.1007/s00415-013-7025-9) contains supplementary material, which is available to authorized users.

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Section: Resultssupporting

confidence: 75%

Section: Introductionmentioning

confidence: 99%

Characterization of hyperkalemic periodic paralysis: a survey of genetically diagnosed individuals

et al. 2013

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“…The same kind of study was also performed on patients with hyperkalemic periodic paralysis (hyperPP) by Amarteifio et al [181]. They showed that sodium MRI can detect increased myoplasmic sodium content in HyperPP patients with permanent weakness, as they are affected by an incomplete inactivation of muscular sodium channels [182].…”

Section: Biomedical Applicationsmentioning

confidence: 99%

Sodium MRI: Methods and applications

Madelin

Lee

Regatte

et al. 2014

Progress in Nuclear Magnetic Resonance Spectroscopy

190

209

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Sodium NMR spectroscopy and MRI have become popular in recent years through the increased availability of high-field MRI scanners, advanced scanner hardware and improved methodology. Sodium MRI is being evaluated for stroke and tumor detection, for breast cancer studies, and for the assessment of osteoarthritis and muscle and kidney functions, to name just a few. In this article, we aim to present an up-to-date review of the theoretical background, the methodology, the challenges and limitations, and current and potential new applications of sodium MRI.

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“…Quantitative measurement of the sodium concentration in cartilage via MRI can thus serve as a direct proxy for PG content, potentially allowing the detection of very early degenerative changes in cartilage that portend the onset of osteoarthritis (as discussed for both T1-rho and gagCEST imaging above). Other musculoskeletal applications of quantitative sodium MRI, which could all benefit from the higher SNR achievable at 7 Tesla, include imaging of sodium concentrations in muscle [for assessment of muscular dystrophies and channelopathies (53)(54)(55)(56)(57)(58), and muscle response to exercise (59)(60)(61)(62)], imaging of sodium concentrations in tendon (14), and even assessment of intervertebral disc health (63,64). …”

Section: Sodium Mrimentioning

confidence: 99%

Quantitative techniques for musculoskeletal MRI at 7 Tesla

Bangerter

Taylor

Tarbox

et al. 2016

Quant. Imaging Med. Surg.

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Whole-body 7 Tesla MRI scanners have been approved solely for research since they appeared on the market over 10 years ago, but may soon be approved for selected clinical neurological and musculoskeletal applications in both the EU and the United States. There has been considerable research work on musculoskeletal applications at 7 Tesla over the past decade, including techniques for ultra-high resolution morphological imaging, 3D T2 and T2* mapping, ultra-short TE applications, diffusion tensor imaging of cartilage, and several techniques for assessing proteoglycan content in cartilage. Most of this work has been done in the knee or other extremities, due to technical difficulties associated with scanning areas such as the hip and torso at 7 Tesla. In this manuscript, we first provide some technical context for 7 Tesla imaging, including challenges and potential advantages. We then review the major quantitative MRI techniques being applied to musculoskeletal applications on 7 Tesla whole-body systems.

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Hyperkalemic Periodic Paralysis and Permanent Weakness: 3-T MR Imaging Depicts Intracellular²³Na Overload—Initial Results

Abstract: 23Na MR imaging depicts increased myoplasmic Na+ in HyperPP with permanent weakness. Na+ overload may cause muscle degeneration developing with age. 23Na MR imaging may have potential to aid monitoring of medical treatment that reduces this overload.

Cited by 41 publications

References 29 publications

Characterization of hyperkalemic periodic paralysis: a survey of genetically diagnosed individuals

Characterization of hyperkalemic periodic paralysis: a survey of genetically diagnosed individuals

Sodium MRI: Methods and applications

Quantitative techniques for musculoskeletal MRI at 7 Tesla

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Hyperkalemic Periodic Paralysis and Permanent Weakness: 3-T MR Imaging Depicts Intracellular23Na Overload—Initial Results

Abstract: 23Na MR imaging depicts increased myoplasmic Na+ in HyperPP with permanent weakness. Na+ overload may cause muscle degeneration developing with age. 23Na MR imaging may have potential to aid monitoring of medical treatment that reduces this overload.

Cited by 41 publications

References 29 publications

Characterization of hyperkalemic periodic paralysis: a survey of genetically diagnosed individuals

Characterization of hyperkalemic periodic paralysis: a survey of genetically diagnosed individuals

Sodium MRI: Methods and applications

Quantitative techniques for musculoskeletal MRI at 7 Tesla

Contact Info

Product

Resources

About

Hyperkalemic Periodic Paralysis and Permanent Weakness: 3-T MR Imaging Depicts Intracellular²³Na Overload—Initial Results