Professor Ulf Ziemann
Editor-in-Chief, Clinical Neurophysiology
The Editor’s Choice—
Cortical network dysfunction revealed by magnetoencephalography in carriers of spinocerebellar ataxia 1 or 2 mutation
Clinical Neurophysiology Volume 131, Issue 7
Visani E, Mariotti C, Nanetti L, Mongelli A, Castaldo A, Panzica F, Rossi Sebastiano D, Nigri A, Grisoli M, Franceschetti S, Canafoglia L (2020) Cortical network dysfunction revealed by magnetoencephalography in carriers of spinocerebellar ataxia 1 or 2 mutation. Clin Neurophysiol 131: 1548–1555
Hereditary spinocerebellar ataxias type 1 and 2 (SCA1, SCA2) are relatively rare CAG trinucleotide repeat disorders in the ATXN1 or ATXN2 genes. The carriers develop the disease across a wide span of ages, depending on CAG repeat length. The progressive ataxia is thought to be caused by degeneration of the cerebellum and its efferent pathways. The magnetoencephalography study by Visani and colleagues in this issue of Clinical Neurophysiology demonstrated, using a visually cued Go/No-go task that SCA1 and SCA2 patients also show cortical pathology by loss of side predominance for alpha and beta event-related desynchronization (ERD) and significantly weakened beta event-related synchronization. Moreover, preclinical gene mutation carriers exhibited significantly enhanced alpha ERD, in particular when close to the estimated time of symptom onset. The study is important for three reasons: (1) it demonstrates cortical pathology in SCA1 and SCA2; (2) it provides a potential biomarker for imminent disease onset in preclinical gene mutation carriers; (3) this may be of particular utility given the emergent development of causally effective gene therapies in SCA1 and SCA2 that very likely will be available in the near future.
Read editor’s choice collection of articles:
- Latency of subthalamic nucleus deep brain stimulation-evoked cortical activity as a potential biomarker for postoperative motor side effects
2020 June Vol. 131, Issue 6 - Characterizing vocal tremor in progressive neurological diseases via automated acoustic analyses
2020 May Vol. 131, Issue 5 - Visualization of electrophysiological activity at the carpal tunnel area using magnetoneurography
2020 April Vol. 131, Issue 4 - An Automatic Detection Method for 40-Hz Auditory Steady State Response and its Application in Prognosis of Comatose Patients
2020 March Vol. 131, Issue 3 -
Evidence-based guidelines on the therapeutic use of repetitive transcranial magnetic stimulation (rTMS): An update (2014–2018)
2020 February Vol. 131, Issue 2 - Measurement of axonal excitability: Consensus guidelines
2020 January Vol. 131, Issue 1 - Seventy years of our journal,
An electroencephalographer recalls the history of the Federation on the 70th anniversary of its journal, Clinical Neurophysiology,
An electromyographer recalls the history of the Federation on the 70th anniversary of its journal, Clinical Neurophysiology,
2019 December Vol. 130, Issue 12 - Analysis of electrooculography signals for the detection of Myasthenia Gravis
2019 November Vol. 130, Issue 11 - Stability of a chronic implanted brain-computer interface in late-stage amyotrophic lateral sclerosis
2019 October Vol. 130, Issue 10 - Standards for quantification of EMG and neurography
2019 September Vol. 130, Issue 9 - Seizures from transcranial magnetic stimulation 2012–2016: Results of a survey of active laboratories and clinics
2019 August Vol. 130, Issue 8 - Brain networks and their relevance for stroke rehabilitation
2019 July Vol. 130, Issue 7 - Botulinum neurotoxin serotype D – A potential treatment alternative for BoNT/A and B non-responding patients
2019 June Vol. 130, Issue 6 - Clinical utility and prospective of TMS-EEG
2019 May Vol. 130, Issue 5 - Assessing blink reflex circuits by three different afferent routes in Parkinson’s disease
2019 April Vol. 130, Issue 4 - Cortical networks are disturbed in people with cirrhosis even in the absence of neuropsychometric impairment
2019 March Vol. 130, Issue 3 - Interictal spike connectivity in human epileptic neocortex
2019 February Vol. 130, Issue 2 - Recommendations of the International Society of Intraoperative Neurophysiology for intraoperative somatosensory evoked potentials
2019 January Vol. 130, Issue 1 - Indications for neuromuscular ultrasound: Expert opinion and review of the literature
2018 December Vol. 129, Issue 12 - Decreasing subthalamic deep brain stimulation frequency reverses cognitive interference during gait initiation in Parkinson’s disease
2018 November Vol. 129, Issue 11 - Fasciculation intensity and disease progression in amyotrophic lateral sclerosis
2018 October Vol. 129, Issue 10 - Multi-component intrinsic brain activities as a safe alternative to cortical stimulation for sensori-motor mapping in neurosurgery
2018 September Vol. 129, Issue 9 - Implementing Motor Unit Number Index (MUNIX) in a large clinical trial: Real world experience from 27 centres
2018 August Vol. 129, Issue 8 - Decrease of motor cortex excitability following exposure to a 20 Hz magnetic field as generated by a rotating permanent magnet
2018 July Vol. 129, Issue 7 - Phantom somatosensory evoked potentials following selective intraneural electrical stimulation in two amputees
2018 June Vol. 129, Issue 6 - Clinical utility of EEG in diagnosing and monitoring epilepsy in adults
2018 May Vol. 129, Issue 5 - Vestibulo-cochlear function in inflammatory neuropathies
2018 April Vol. 129, Issue 4 - Sleep patterns associated with the severity of impairment in a large cohort of patients with chronic disorders of consciousness
2018 March Vol. 129, Issue 3 - Somatosensory Evoked Potentials and Central Motor Conduction Times in children with dystonia and their correlation with outcomes from Deep Brain Stimulation of the Globus pallidus internus
2018 February Vol. 129, Issue 2 - Abnormal functional connectivity of high-frequency rhythms in drug-naïve schizophrenia
2018 January Vol. 129, Issue 1 - Ear-EEG detects ictal and interictal abnormalities in focal and generalized epilepsy – A comparison with scalp EEG monitoring
2017 December Vol. 128 Issue 12 - Long term correlation of subthalamic beta band activity with motor impairment in patients with Parkinson’s disease
2017 November Vol. 128 Issue 11 - The standardized EEG electrode array of the IFCN
2017 October Vol. 128 Issue 10 - Low intensity transcranial electric stimulation: Safety, ethical, legal regulatory and application guidelines
2017 September Vol. 128 Issue 9 - Reduced electrode arrays for the automated detection of rhythmic and periodic patterns in the intensive care unit: Frequently tried, frequently failed?
2017 August Vol. 128 Issue 8 - A novel methodology for assessing laryngeal and vagus nerve integrity in patients under general anesthesia
2017 July Vol. 128 Issue 7 - The evolution of motor cortical dysfunction in amyotrophic lateral sclerosis
2017 June Vol. 128 Issue 6 - Parietomotor connectivity in the contralesional hemisphere after stroke: A paired-pulse TMS study
2017 May Vol. 128 Issue 5 - Early disturbances in multimodal evoked potentials as a prognostic factor for long-term disability in relapsing-remitting multiple sclerosis patients
2017 April Vol. 128 Issue 4 - Pain perception in patients with chronic disorders of consciousness: What can limbic system tell us?
2017 March Vol. 128 Issue 3 - TMS combined with EEG in genetic generalized epilepsy: A phase II diagnostic accuracy study
2017 February Vol. 128 Issue 2 - Haemodialysis alters peripheral nerve morphology in end-stage kidney disease
2017 January Vol. 128 Issue 1 - The significance of ultrasonographic carpal tunnel outlet measurements in the diagnosis of carpal tunnel syndrome
2016 December Vol. 127 Issue 12 - Motor cortical dysfunction develops in spinocerebellar ataxia type 3
2016 November Vol. 127 Issue 11 - Intraoperative spinal cord monitoring using low intensity transcranial stimulation to remove post-activation depression of the H-reflex
2016 October Vol. 127 Issue 10 - The relationship between seizures, interictal spikes and antiepileptic drugs
2016 September Vol. 127 Issue 9 - Disease-specific sparing of the anterior semicircular canals in bilateral vestibulopathy
2016 August Vol. 127 Issue 8 - Brain areas with epileptic high frequency oscillations are functionally isolated in MEG virtual electrode networks
2016 July Vol. 127 Issue 7 - Cortical correlates of susceptibility to upper limb freezing in Parkinson’s disease
2016 June Vol. 127 Issue 6 - Normal pressure hydrocephalus: Increase of utricular input in responders to spinal tap test
2016 May Vol. 127 Issue 5 - Ascending vestibular drive is asymmetrically distributed to the inferior oblique motoneuron pools in a subset of hemispheric stroke survivors
2016 April Vol. 127 Issue 4 - Language mapping using high gamma electrocorticography, fMRI, and TMS versus electrocortical stimulation
2016 March Vol. 127 Issue 3 - Short pauses in thalamic deep brain stimulation promote tremor and neuronal bursting
2016 February Vol. 127 Issue 2 - Yield of MRI, high-density electric source imaging (HD-ESI), SPECT and PET in epilepsy surgery candidates
2016 January Vol. 127 Issue 1