Is Video-Oculography a Predictive Test for Myasthenia Gravis with Ocular Symptoms?

Background and Objective: The aim of this study was to evaluate the contribution of extraocular muscle function testing with video-oculography (VOG), which is a noninvasive and easily applicable method of recording eye movement with digital cameras, to the diagnosis of myasthenia gravis (MG) in patients without any clinical eye movement abnormalities. Methods and Materials: The study included 18 patients prediagnosed with ocular MG: MG Group (N = 7) with abnormal, and non-MG Group (N = 11) with normal single-fiber electromyography. Control group included 50 healthy volunteers. Ocular movements were recorded with the EyeSeeCam VOG device. Results: The inward latency of the 10° horizontal saccade and the downward latency of the 10° vertical saccade were significantly delayed; and the downward amplitude of the 10° vertical saccade was significantly lower in the MG group. Receiver operating characteristic curve analyses showed high specificity values for the discrimination of MG patients. Conclusions: This study supports the usefulness of the VOG device in revealing subclinical extraocular muscle involvement in MG.

Default mode and dorsal attention network involvement in visually guided motor sequence learning.

Motor sequence learning (MSL) paradigms are often used to investigate the neural processes underlying the acquisition of complex motor skills. Behavioral and neuroimaging studies have indicated an early stage in which spatial learning is prominent and a late stage of automatized performance after multiple training periods. Functional magnetic resonance imaging (fMRI) studies yielded both decreased and increased activations of the sensorimotor and association areas. However, task-negative and task-positive intrinsic connectivity networks (ICNs), the default mode (DMN) and dorsal attention (DAN) networks involved in governing attention demands during various task conditions were not specifically addressed in most studies. In the present fMRI study, a visually guided MSL (VMSL) task was used for bringing roles of visuospatial and motor attention into foreground in order to investigate the role of attention-related ICNs in MSL. Seventeen healthy, right-handed participants completed training and test sessions of VMSL during fMRI on the 1st day. Then, after daily training for three consecutive days outside the scanner, they were re-tested during the 5th day's scanning session. When test session after early learning period was compared with training session, activation decrease was observed in the occipito-temporal fusiform cortex, while task-related suppression of DMN was reduced. Reduced deactivation after early learning was correlated with decreased error rates. After late learning stage we observed activation decreases in bilateral superior parietal lobules of task-positive DAN, dorsal precunei, and cerebellum. Reduced activity in left posterior parietal and right cerebellar regions were correlated with gains in speed, error rate, respectively. This dissociation in activity changes of DMN and DAN related areas suggests that DAN shows high contribution during both early and late MSL stages, possibly due to attention requirement for automatization of spatial and temporal aspects of motor sequence. In contrast, spatial learning occurring during early MSL stage was sufficient for releasing DMN resources.

Slow repetitive transcranial magnetic stimulation in refractory juvenile myoclonic epilepsies.

OBJECTIVE: The objective of the study was to investigate the effects of slow repetitive transcranial magnetic stimulation (rTMS) on patients with refractory juvenile myoclonic epilepsy (JME). METHODS: One thousand pulses with the intensity of 120% active motor threshold (AMT) at 0.2 Hz frequency were applied on 5 consecutive days in 10 patients with refractory JME. Sham rTMS was performed after 3 months. Electroencephalography (EEG) examinations were performed before rTMS, on the 5th day, and 1, 2, 4, and 8 weeks after rTMS. Resting motor threshold (RMT), AMT, and cortical silent periods (CSPs) were recorded before the application and at the end of day 5. The changes in the quality of life were evaluated using the Quality of Life in Epilepsy Inventory (QOLIE-31). RESULTS: No adverse effects were observed. The number of seizures decreased by 29-50%, and interictal discharge durations decreased 2 weeks after the real rTMS. No significant difference was observed between the AMT and RMT values recorded before and after the stimulations. Statistically significant increases in CSP duration and quality of life scores were found following real rTMS. Repetitive transcranial magnetic stimulation may be considered as a safe treatment option in refractory JME. CONCLUSION: This study provides some positive evidence that rTMS may be effective in resistant JME.

Neuronavigated rTMS inhibition of right pars triangularis anterior in stuttering: Differential effects on reading and speaking.

Functional neuroimaging studies show an overactivation of speech and language related homologous areas of the right hemisphere in persons who stutter. In this study, we inhibited Broca's homologues using 1 Hz repetitive transcranial magnetic stimulation (rTMS) and assessed its effects on stuttering severity. The investigated cortical areas included pars opercularis (BA44), anterior and posterior pars triangularis (BA45), mouth area on the primary motor cortex (BA4). We collected reading and speaking samples before and after rTMS sessions and calculated the percentage of syllables stuttered. Only right anterior pars triangularis stimulation induced significant changes in speech fluency. Notably, the effects were differential for reading and speaking conditions. Overall, our results provide supportive evidence that right anterior BA45 may be a critical region for stuttering. The observed differential effects following the inhibition of right anterior BA45 merits further study of contributions of this region on different language domains in persons who stutter.

Parameters derived from compound muscle action potential scan for discriminating amyotrophic lateral sclerosis-related denervation.

INTRODUCTION: The objective of this study was to determine compound muscle action potential (CMAP) scan parameters and MScanFit motor unit number estimation (MUNE) in patients with amyotrophic lateral sclerosis (ALS) and to compare the results in the abductor pollicis brevis (APB) to those in the abductor digiti minimi (ADM). METHODS: CMAP scans were recorded from the APB and ADM in 35 patients with ALS and 21 controls. MScanFit MUNE, neurophysiological index (NI), step%, returner%, and D50 were calculated. RESULTS: CMAP scan parameters including the returner%, MScanFit MUNE, and NI can distinguish ALS with high sensitivity and specificity. The electrophysiological parameters, with the exception of D50 (the number of largest consecutive differences of recorded responses generating 50% of maximum CMAP), showed more pronounced changes in the APB than in the ADM, even though most of the patients had normal APB/ADM amplitude ratios. DISCUSSION: CMAP scan parameters and MScanFit MUNE can be used in the evaluation of denervation and reinnervation and may herald the "split hand" in ALS.

A SOD2 Polymorphism is Associated with Abnormal Quantitative Sensory Testing in Type 2 Diabetic Patients.

INTRODUCTION: Diabetes mellitus (DM) affects peripheral nerves inducing diabetic polyneuropathy (DPN). Mitochondrial dysfunction and oxidative stress are potential causes of DPN. METHODS: Nerve conduction studies were performed in 20 type 2 DM patients (11 with DPN) and 15 healthy controls. Perception threshold values of cold, warm and vibration were measured by quantitative sensory testing. Variants of a superoxide dismutase 2 (SOD2) gene single nucleotide polymorphism (SNP) (rs5746136) were determined by polymerase chain reaction (PCR) and following NexteraXT DNA Library. RESULTS: DPN patients showed significantly increased threshold values. DM patients without DPN and healthy controls showed comparable values. TT variant of the SOD2 SNP was more prevalent in DM and DPN patients. DM patients with the TT variant displayed increased perception threshold values. CONCLUSIONS: Quantitative sensory testing is not superior to standard nerve conduction studies in DPN. Carriers of SOD2 SNP manifested increased sensory threshold levels which is important in further corroborating the link between oxidative stress and DPN.

Electromyography in Pediatric Population.

INTRODUCTION: Electrodiagnostic evaluation provides an important extension to the neurological examination for the evaluation of pediatric neuromuscular disease. Many pediatric neuromuscular diseases are analogous to those seen in the adult. However, the relative frequency of these illnesses varies greatly when different age populations are compared. The purpose of the present study is to provide a retrospective analysis of children referred to our electromyography (EMG) laboratory for electrophysiological examinations. METHODS: We retrospectively reviewed electrodiagnostic records of patients aged between 0-15 years, from January 2004 to June 2013. Patients were classified as having plexopathy, nerve root lesions, polyneuropathy, myopathy, mononeuropathy, anterior horn cell disease, neuromuscular transmission disorder, facial nerve palsy, and other rare disorders. RESULTS: We reviewed totally 5563 pediatric records, which was on the average 578 studies per year. It was about 14% of the all EMG examinations performed in our laboratory. When we looked at all the procedures, 3271 of the records included needle EMGs, 170 of them were single fiber EMGs, 100 of them were repetitive nerve stimulations, and 52 of them were evoked potentials. The results were normal in 55% of the cases. As a result of electrophysiological examinations, the common diagnoses were: plexopathy (28.6%), polyneuropathy (7.4%), and myopathy (6.6%) in patients aged 0-5 years (41.2% of all records); myopathy (9.4%), PNP (8.5%), mononeuropathy (6.4%), and plexopathy (5.9%) in 6-10 years (28.2% of all records); PNP (11.3%), myopathy (6.6%), and mononeuropathy (5.6%) in 11-15 years (30.6% of all records). CONCLUSION: Infants and toddlers mostly suffered from brachial plexopathy which can be prevented by proper obstetrical management. Nerve conduction studies and EMG yielded diagnostic importance for demyelinating neuropathy and myopathy in patients older than 6 years of age.

Clinical, Electrophysiological, and Serological Evaluation of Patients with Cramp-Fasciculation Syndrome.

INTRODUCTION: Cramp-fasciculation syndrome (CFS) is a rare peripheral nerve hyperexcitability syndrome. There are only a few reports on clinical and serological profile of a CFS cohort that was followed up by a single outpatient clinic. METHODS: Clinical, electrophysiological, and serological features of 6 CFS patients (5 men, 1 woman; 27-65 years old) were investigated. RESULTS: All patients presented with cramps, fasciculations, muscle pain, and autonomic symptoms, and 2 also reported numbness and burning sensation in limbs, suggestive of neuropathic pain. Antibodies to uncharacterized voltage-gated potassium channel (VGKC)-complex proteins were found in 2 patients and to contactin-associated protein-like 2 (CASPR2) in 1 patient. None of the patients had a tumor. Most of the patients revealed prolonged after-discharges following tibial nerve stimulation. Nerve conduction studies and R-R interval variability tests were normal, whereas sympathetic skin responses were increased in amplitude in 3 seronegative patients. Five patients showed favorable response to carbamazepine or pregabalin treatment, whereas 1 VGKC-antibody-positive patient was resistant to carbamazepine and immunosuppressant treatment. CONCLUSION: Neuropathic pain and VGKC-complex antibodies may be encountered in CFS patients. Although autonomic symptoms are commonly found in CFS, routine autonomic system tests which are done in electrophysiology laboratories might yield normal results.

A Novel Electrophysiological Method in the Diagnosis of Pudendal Neuropathy: Position-related Changes in Pudendal Sensory Evoked Potentials.

OBJECTIVE: To examine the diagnostic value of pudendal somatosensory evoked potentials (SEPs) in pudendal nerve entrapment (PNE) neuropathy by stimulating the 2 sides separately after provocation by a standard sitting position. Routine pudendal SEPs performed in the supine position with bilateral simultaneous stimulation may fail to show the abnormality because the complaints of PNE appear or worsen in the sitting position. METHODS: Forty-nine patients with PNE and 16 controls were included. SEP recordings were performed by stimulating the dorsal nerve of penis or clitoris on either side. The recordings were performed at the initial supine position, at the beginning and end of the provocation by sitting position, and at the second supine position. RESULTS: Amplitude loss in the SEP responses after prolonged sitting was significantly more pronounced on the symptomatic sides of the patients. Approximately 45% decrease in the SEP amplitude or an amplitude value less than 1.5 µV at the end of sitting are the parameters to be used with high selectivity. CONCLUSION: The dynamic pudendal SEP study described herein seems to be more helpful in PNE diagnosis than in conventional SEPs.

Sensorimotor Integration During Motor Learning: Transcranial Magnetic Stimulation Studies.

The effect of sensory signals coming from skin and muscle afferents on the sensorimotor cortical networks is entitled as sensory-motor integration (SMI). SMI can be studied electrophysiologically by the motor cortex excitability changes in response to peripheral sensory stimulation. These changes include the periods of short afferent inhibition (SAI), afferent facilitation (AF), and late afferent inhibition (LAI). During the early period of motor skill acquisition, motor cortex excitability increases and changes occur in the area covered by the relevant zone of the motor cortex. In the late period, these give place to the morphological changes, such as synaptogenesis. SAI decreases during learning the motor skills, while LAI increases during motor activity. In this review, the role of SMI in the process of motor learning and transcranial magnetic stimulation techniques performed for studying SMI is summarized.

Quantitative electromyography of the frontalis muscle.

PURPOSE: Identification of abnormal motor unit potentials (MUP), in particular those caused by myopathic processes, is generally difficult in facial muscles because the MUPs are already low in amplitude and short in duration. Therefore, quantitative measures for the limits of abnormality are needed more frequently for these muscles, especially for the pathological processes predominantly affecting the craniofacial area. In this study, we aimed to determine some quantitative values that may help us to differentiate "myogenic" and "neurogenic" processes in periocular muscles. METHODS: The frontalis muscle was examined unilaterally with multi-MUP and interference pattern analyses in 32 normal subjects, 31 patients with different types of myopathy, and 13 patients with facial palsy. Abnormality limits were calculated by using five previously suggested methods, two of which were based on group means and the other three on determining the outlier values. The sensitivity and specificity of multi-MUP and interference pattern parameters in discriminating abnormal findings by using these different methods were analyzed. RESULTS: Statistically significant differences for all MUP parameters, except for the frequency, was found between the groups (P < 0.001). Motor unit potential duration, size index, and amplitude were the most valuable parameters for distinguishing the individual abnormalities. However, their discriminating power was higher for neuropathies than for myopathies. Interference pattern analysis was found not to have any superior sensitivity to MUP analysis. In this study, the outlier analysis that depends on the 5th to 95th percentile limits of the pooled data provided the best power for the discrimination of both neuropathies and myopathies from normal subjects. CONCLUSION: Multi-MUP analysis seems to be helpful in differentiating the patients with neurogenic and-to a lesser extent-myogenic facial muscle involvement.

Simple electrodiagnostic method for Morton neuroma.

INTRODUCTION: We describe a simple and quickly applied electrodiagnostic method for confirming the diagnosis of interdigital neuropathy caused by Morton neuroma (MN). METHODS: Interdigital nerves II-III and III-IV were stimulated with surface electrodes simultaneously touching the lateral side of 1 toe and the medial side of the other. Recording was also made with surface electrodes. The results of 20 normal controls and 14 patients with MN were evaluated. RESULTS: The amplitude and peak latency values elicited in the patients as well as the interside differences revealed an acceptable abnormality rate between 57.1% and 71.4%. CONCLUSIONS: Although the most popular and effective method of MN diagnosis is clinical evaluation supported by imaging, electrophysiological studies can, in selected patients, provide valuable information.

Diagnostic use of dermatomal somatosensory-evoked potentials in spinal disorders: Case series.

UNLABELLED: OBJECTIVE/CONTEXT: Dermatomal somatosensory-evoked potentials (dSEPs) may be valuable for diagnostic purposes in selected cases with spinal disorders. DESIGN: Reports on cases with successful use of dSEPs. FINDINGS: Cases 1 and 2 had lesions causing multiple root involvement (upper to middle lumbar region in Case 1 and lower sacral region in Case 2). Cystic lesions in both cases seemed to compress more than one nerve root, and stimulation at the center of the involved dermatomes in dSEPs helped to reveal the functional abnormality. Cases 3 and 4 had lesions involving the spinal cord with or without nerve root impairment. In Case 3, an magnetic resonance imaging (MRI)-verified lesion seemed to occupy a considerable volume of the lower spinal cord, causing only very restricted clinical sensory and motor signs. In Case 4, a cervical MRI showed a small well-circumscribed intramedullary lesion at right C2 level. All neurophysiological investigations were normal in the latter two patients (motor, tibial, and median somatosensory-evoked potentials in Case 3, and electromyography in both) except for the dSEPs. CONCLUSIONS: Objectifying the presence and degree of sensory involvement in spinal disorders may be helpful for establishing diagnoses and in therapeutic decision-making. Valuable information could be provided by dSEPs in selected patients with multiple root or spinal cord involvement.

Excitability changes at brainstem and cortical levels in blind subjects.

OBJECTIVES: This study was designed to search potential changes in trigemino-facial system in blind subjects by the use of relatively well-established electrophysiological methods. Excitability changes in the motor cortex were also investigated by transcranial magnetic stimulation studies (TMS) with the expectation of finding some abnormal interactions between the cortex and brainstem. METHODS: Twenty blind (BS) and 13 control subjects (CoS) were included in the study. Blink reflex and its recovery with paired electrical stimulation were studied at 150, 200, 300, 400 and 500 ms interstimulus intervals (ISI). Facial F waves elicited by buccal branch stimulation were recorded from nasalis muscles. Motor cortex excitability with recordings from left first dorsal interosseus muscle was studied by using magnetically elicited silent periods and paired magnetic stimuli, subthreshold conditioning and suprathreshold test, given at ISIs of 2, 3, 4, 10, 12, 15 and 20 ms. RESULTS: Blink reflex recovery was significantly reduced in BS group comparing to CoS at 400 and 500 ms ISIs. This difference between the groups was more prominent for the responses evoked by the initial stimulation side and faded away with stimulations on the contralateral side. Facial F wave amplitudes and F/M amplitude ratios were higher in BS group. In TMS studies, the early inhibitions at 2 and 4 ms were found to be significantly less in BS as compared to that of CoS. CONCLUSIONS: The reduced blink reflex recovery and its fast restoration with continuing stimulation might be explained by conditioning and extinction processes which have been shown to be mainly carried out by cerebellar-brainstem pathways. Our TMS studies showed reduced intracortical inhibition in the motor cortices of BS cases and facial F wave studies revealed the possible effect of this altered excitability on the facial motor nuclei. SIGNIFICANCE: Firing probabilities of facial motor neurons in BS are probably determined by the equilibrium between the low-set excitability of blink reflex interneurons and the enhanced excitability brought on by the descending motor pathways.

Short segment incremental study in ulnar neuropathy at the wrist: report of three cases and review of the literature.

Ulnar nerve lesions may occur at different sublocations at wrist and may involve various branches of the nerve. Standard neurophysiological studies are generally insufficient in revealing these lesions. Demonstration of conduction block and/or focal slowing of nerve conduction is the most definitive electrodiagnostic evidence for the localization of segmental demyelination. Short-segment incremental study (SSIS) is a sensitive technique for detecting the ulnar neuropathy at the wrist (UNW). We report 3 cases of UNW caused by ganglion cysts in Guyon's canal which were studied by using SSIS across the wrist. Even though SSIS is a time-consuming and technically demanding method, it increases the electrodiagnostic potential of detecting segmental demyelination in this location.

Motor unit number estimation in facial paralysis.

The value of motor unit number estimation (MUNE) in determining the prognosis of acute peripheral facial paralysis (PFP) was evaluated in 89 patients with PFP on days 6, 8, 11, 14, 20, and 30 of PFP and repeated once per month until complete recovery or the end of the first year. The symptomatic/asymptomatic side ratios of the compound muscle action potential (CMAP) amplitudes recorded from nasalis muscles and MUNEs studied using the incremental method by recording from the same muscle were assessed with regard to three outcome groups (Group I, complete recovery; Group II, mild dysfunction; Group III, moderate-moderately severe dysfunction). CMAP and MUNE ratios were parallel to each other in all patient groups throughout the observation period with lower values in the more severe groups. However, CMAP amplitude loss was significantly greater than the MUNE loss in the first 3 weeks of PFP. The MUNE method is not superior to CMAP size in determining prognosis in PFP. However, the significant disparity between the CMAP and MUNE ratios in the early period may have some physiological relevance with regard to the pathophysiology of the Wallerian degeneration process and deserves further research into its potential sources.

Excitability of facial nucleus and related brain-stem reflexes in hemifacial spasm, post-facial palsy synkinesis and facial myokymia.

OBJECTIVE: To compare the electrophysiological excitability characteristics of the facial nucleus and related structures in hemifacial spasm (HFS), post-facial palsy synkinesis (PFPS) and facial myokymia (FM). METHODS: Facial F-waves, blink reflex recoveries and magnetically elicited silent periods (SP) were prospectively studied in 17 HFS, 17 PFPS, 8 FM cases and in 13 controls. Earlier unpublished observations on abnormal impulse transmission in 36 HFS and 29 PFPS cases were also included. RESULTS: Enhanced F-waves were recorded on the symptomatic side in PFPS and HFS cases with a tendency to be more pronounced in PFPS. HFS and PFPS groups both showed an earlier blink reflex recovery, more prominent in PFPS patients, when stimulated and/or recorded on the symptomatic side. Unelicitable SPs were encountered after 24/39 stimulations in 5 patients with PFPS and rarely in HFS cases. Duration of elicitable SPs did not change remarkably. FM group had similar characteristics as normal controls in the 3 electrophysiological tests. Latencies of the lateral and synkinetic spread responses were significantly prolonged in the earlier PFPS group as compared to HFS. In two-point stimulation, both groups showed a greater latency shift in late responses, again more pronounced in PFPS. CONCLUSIONS: PFPS and HFS cases had similar enhanced excitability patterns at the facial nucleus and related brain-stem structures, more marked on the symptomatic side and more obvious in the PFPS group. Findings elicited in the FM group were thought to be caused by asynchronous hyperactivity of facial motoneurons. SIGNIFICANCE: In this comparative electrophysiological study, similar excitability patterns were found in HFS and PFPS groups, albeit with different intensities.