Clinical Neurophysiology Practice最新文献

Objective

Nerve conduction studies (NCS) require valid reference limits for meaningful interpretation. We aimed to further develop the extrapolated norms (e-norms) method for obtaining NCS reference limits from historical laboratory datasets for children and adults, and to validate it against traditionally derived reference limits.

Methods

We compared reference limits obtained by applying a further developed e-norms with reference limits from healthy controls for the age strata’s 9–18, 20–44 and 45–60 years old. The control data consisted of 65 healthy children and 578 healthy adults, matched with 1294 and 5628 patients respectively. Five commonly investigated nerves were chosen: The tibial and peroneal motor nerves (amplitudes, conduction velocities, F-waves), and the sural, superficial peroneal and medial plantar sensory nerves (amplitudes, conduction velocities). The datasets were matched by hospital to ensure identical equipment and protocols. The e-norms method was adapted, and reference limit calculation using both ±2 SD (original method) and ±2.5 SD (to compensate for predicted underestimation of population SD by the e-norms method) was compared to control data using ±2 SD. Percentage agreement between e-norms and the traditional method was calculated.

Results

On average, the e-norms method (mean ±2 SD) produced slightly stricter reference limits compared to the traditional method. Increasing the e-norms range to mean ±2.5 SD improved the results in children while slightly overcorrecting in the adult group. The average agreement between the two methods was 95 % (±2 SD) and 96 % (±2.5 SD).

Conclusions

The e-norms method yielded slightly stricter reference limits overall than ones obtained through traditional methods; However, much of the difference can be attributed to a few outlying plots where the raters found it difficult to apply e-norms correctly. The two methods disagreed on classification of 4–5% of cases. Our e-norms software is suited to analyze large amounts of raw NCS data; it should further reduce bias and facilitate more accurate ratings.

Significance

With small adaptations, the e-norms method adequately replicates traditionally derived reference limits, and is a viable method to produce reference limits from historical datasets.

Accurate and rapid diagnosis of amyotrophic lateral sclerosis (ALS) is essential in order to provide accurate information for patient and family, to avoid time-consuming investigations and to permit an appropriate management plan. ALS is variable regarding presentation, disease progression, genetic profile and patient reaction to the diagnosis. It is obviously important to exclude treatable conditions but, in most patients, for experienced neurologists the diagnosis is clear-cut, depending on the presence of progressive upper and lower motor neuron signs. Patients with signs of restricted lower motor neuron (LMN) or upper motor neuron (UMN) dysfunction may present diagnostic difficulty, but electromyography (EMG) is often a determinant diagnostic test since it may exclude other disorders. Transcranial magnetic stimulation may aid detection of UMN dysfunction, and brain and spinal cord MRI, ultrasound and blood neurofilament measurements, have begun to have clinical impact, although none are themselves diagnostic tests. Several sets of diagnostic criteria have been proposed in the past; all rely on clinical LMN and UMN signs in different anatomic territories, EMG changes, exclusion of other disorders, and disease progression, in particular evidence of spreading to other anatomic territories. Fasciculations are a characteristic clinical feature and increased importance is now attached to fasciculation potentials detected by EMG, when associated with classical signs of denervation and reinnervation. The Gold Coast diagnostic criteria rely on the presence of UMN and LMN signs in one (or more) anatomic territory, or LMN signs in two (or more) anatomic territories, recognizing the fundamental clinical requirements of disease progression and exclusion of other diseases. Recent studies confirm a high sensitivity without loss of specificity using these Gold Coast criteria. In considering the diagnosis of ALS a critical question for future understanding is whether ALS should be considered a syndrome or a specific clinico-pathologic entity; this can only be addressed in the light of more complete knowledge.

Objective

Methods

Results

Conclusions

Significance

Background

We present a case of a 46-year-old man with Lennox-Gastaut syndrome and drug-resistant epilepsy. An adjunctive neurostimulation therapy strategy was implemented involving bilateral deep brain stimulation (DBS) of the centromedian thalamic nucleus (CMT).

Methods

Robotically assisted implantation of bilateral DBS directional lead system with 8 contacts in the CMT was performed. The clinical course was assessed in repeated in-patient follow-ups.

Results

Initial DBS stimulation resulted in progressive ataxia and dysarthria significantly beyond the level seen before surgery. Deactivation of DBS coincided with improvement of dysarthria. A DBS stimulation paradigm with reduction of lateral and superior stimulation resulted in improvement and eventually complete resolution of the stimulation side effect.

Discussion

This case suggests that stimulation-induced dysarthria in DBS can be improved by targeted reduction of lateral and/or superior thalamic stimulation. When dysarthria and ataxia occur during DBS, directed stimulation to medial thalamic structures and more inferior electrode contacts offers a promising strategy to reduce side effects while maintaining positive effects.

Objective

Methods

Results

Conclusions

Significance

Objective

To assess impact of ultrasound guidance (USG) on patient’s perception of nerve conduction studies (NCS).

Methods

In this single-center, randomized, sham-controlled, parallel, single-blind trial, we evaluated ultrasound (US) in identifying NCS stimulation site. Consecutive adults (18–80 old) without neuropathy referred for NCS were electronically randomized 1:1 to USG or Sham US. The primary outcome was sensory supramaximal intensity (SSMI) for each site/nerve; motor supramaximal intensity (MSMI), amplitudes, number of non-routine muscle punctured, Visual Analogue Scale (VAS), satisfaction were secondary outcomes.

Results

290 participants were randomized, with 145 in the USG and 144 Sham US groups, respectively. No difference in SSMI, CMAP or SNAP, VAS, satisfaction was recorded. With USG, the median at the elbow and fibular MMSI were lower (p = 0.04; p = 0.02). With normal NCS or overweight and obese subgroups patients had lower median SSMI (p = 0.05/ p = 0.02), higher median and sural SNAP with normal NCS (p = 0.04; p = 0.007) and the sural SNAP for the expert US subgroup (p = 0.02).

Conclusions

USG is useful for nerves, that are anatomically variable or in obesity. The sural SNAP gain with US in the normal NCS subgroup could facilitate routine NCS.

Significance

In standard NCS the USG does not modify the patient’s tolerance.

Trial Registration: clinicaltrials.gov (NCT03868189).