Cortical contributions to the flail leg syndrome: Pathophysiological insights

Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration
Parvathi MenonS Vucic

Abstract

Cortical hyperexcitability has been identified as an intrinsic feature of amyotrophic lateral sclerosis (ALS). Consequently, the aim of the present study was to determine whether cortical hyperexcitability formed the pathophysiological basis for the flail leg syndrome (FL), an atypical ALS variant. Cortical excitability studies were undertaken on 18 FL patients, using the threshold tracking transcranial magnetic stimulation (TMS) technique, and results were compared to healthy controls, upper and lower limb-onset ALS as well as bulbar-onset and the flail arm variant ALS. Results showed that cortical hyperexcitability was a feature of FL and was heralded by a significant reduction of short-interval intracortical inhibition (FL 7.2 ± 1.8%; controls 13.2 ± 0.8%, p <0.01) and cortical silent period (CSP) duration (FL 181.7 ± 10.8ms; controls 209.8 ± 3.4ms; p <0.05) along with an increase in motor evoked potential amplitude (FL 29.2 ± 5.1%; controls 18.9 ± 1.2%, p <0.05). The degree of cortical hyperexcitability was comparable between FL and other ALS phenotypes, defined by site of disease onset. In addition, the CSP duration correlated with biomarkers of peripheral neurodegeneration in FL. In conclusion, cortical hyperexcitability ...Continue Reading

References

May 12, 1998·The Journal of Physiology·R HanajimaI Kanazawa
Jan 22, 2002·Clinical Neurophysiology : Official Journal of the International Federation of Clinical Neurophysiology·Maria Teresa DesiatoMaria D Caramia
Nov 30, 2005·Muscle & Nerve·Steve VucicMatthew C Kiernan
Jan 11, 2007·Journal of Neurology, Neurosurgery, and Psychiatry·Steve Vucic, Matthew C Kiernan
Jan 1, 2008·Clinical Neurophysiology : Official Journal of the International Federation of Clinical Neurophysiology·Mamede de CarvalhoMichael Swash
May 13, 2008·Brain : a Journal of Neurology·Steve VucicMatthew C Kiernan
Jul 9, 2008·Amyotrophic Lateral Sclerosis : Official Publication of the World Federation of Neurology Research Group on Motor Neuron Diseases·Paul TalmanSusan Mathers
Jun 10, 2009·Archives of Neurology·Renske M Van den Berg-VosLeonard H Van den Berg
Sep 4, 2009·Journal of Neurology, Neurosurgery, and Psychiatry·Steve Vucic, Matthew C Kiernan
Jan 7, 2010·Neuropathology : Official Journal of the Japanese Society of Neuropathology·Zen KobayashiHidehiro Mizusawa
Feb 8, 2011·Lancet·Matthew C KiernanMargaret C Zoing
Mar 16, 2011·Journal of Neurology, Neurosurgery, and Psychiatry·Adriano ChiòUNKNOWN PARALS study group
Sep 29, 2012·Clinical Neurophysiology : Official Journal of the International Federation of Clinical Neurophysiology·Parvathi MenonSteve Vucic
Dec 25, 2012·Journal of Neurology, Neurosurgery, and Psychiatry·Steve VucicMatthew C Kiernan
Apr 26, 2013·Brain : a Journal of Neurology·Steve VucicMatthew C Kiernan
Aug 24, 2013·Journal of Clinical Neuromuscular Disease·Mazen M DimachkieRichard J Barohn
Jun 15, 2014·Trends in Neurosciences·Steve VucicMatthew C Kiernan
Sep 18, 2014·Clinical Neurophysiology : Official Journal of the International Federation of Clinical Neurophysiology·Parvathi MenonSteve Vucic
Feb 17, 2015·European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies·N GeevasingaS Vucic

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Citations

Oct 28, 2016·Nature Reviews. Neurology·Nimeshan GeevasingaSteve Vucic
Nov 11, 2016·Neurotherapeutics : the Journal of the American Society for Experimental NeuroTherapeutics·Steve Vucic, Matthew C Kiernan
Dec 23, 2016·Journal of Neurology, Neurosurgery, and Psychiatry·Nidhi GargMatthew C Kiernan
Aug 7, 2018·Current Opinion in Neurology·Steve Vucic, Seward B Rutkove
Apr 13, 2017·Neural Regeneration Research·Stéphane MathisGwendal Le Masson
Jun 13, 2019·International Journal of Molecular Sciences·Mehdi A J van den BosSteve Vucic
Jun 3, 2021·Brain Sciences·Thanuja Dharmadasa
Jun 13, 2021·Neuroscience Letters·Steve VucicMatthew C Kiernan

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