Segmental Reflex, Long Latency Reflex, and Mixed Nerve Silent Period in Dystonia.

We hypothesized that "long latency reflexes" (LLRs), associated segmental reflex (SR), and mixed nerve silent periods (MnSPs) recorded on the distal upper extremity muscles would behave differently in patients with cervical dystonia and focal hand dystonia. We enrolled patients with cervical dystonia, generalized dystonia, focal hand dystonia, and healthy individuals. We recorded SR, LLRs, and MnSPs. The mean amplitude of SR on the affected side of focal hand dystonia was significantly lower (p = 0.010). The parameters related to LLRs and MnSPs were not different between groups. We suggest, using SR, LLRs, and MnSPs, we could not show an electrophysiological signature specific to dystonia.

Myoclonus and tremor in chronic inflammatory demyelinating polyneuropathy: a multichannel electromyography analysis.

BACKGROUND: Information regarding involuntary movements in chronic inflammatory polyneuropathy (CIDP) is gradually increasing. Our goal is to identify the types of involuntary movements in CIDP. METHODS: All patients who were followed with the diagnosis of CIDP were invited for clinical and electrophysiological evaluations. Demographic and clinical findings (age, gender, duration of illness, diagnosis, treatments) were noted. Clinical examination and multichannel surface electromyography were done. We also performed routine upper and lower extremity peripheral nerve conduction studies, F-waves, long latency reflexes, blink reflex, mixed nerve silent period and cutaneous silent period in all patients. RESULTS: Twenty-two patients accepted the invitation. Eleven patients with CIDP had involuntary movements. Ten (45.5%) patients with CIDP had tremor and seven (31.8%) had short-duration and high-amplitude myoclonus. Regarding demographic, clinical and electrophysiological features, there was no significant difference between patients with and without tremor. The latencies of R1, R2 and R2c components of BR were longer among CIDP patients without tremor compared to CIDP patients with tremor. Presence of myoclonus (p = 0.007) and delayed F-waves (p = 0.008) were associated with the presence of tremor. CONCLUSION: Tremor and myoclonus were frequent in CIDP. The fact that myoclonus was detected in the majority of patients only by multichannel surface EMG who were clinically evaluated as pure tremor suggests that a more detailed electrophysiological evaluation is required. There was no difference in the medications used or other clinical features between patients with and without tremor.

Postural tremor in L-2-hydroxyglutaric aciduria is associated with cerebellar atrophy.

OBJECTIVE: In this study, we performed analysis of brainstem reflexes and movement disorders using surface polymyogram in L-2-hydroxyglutaric aciduria (L2HGA). We also reviewed all cases in the literature with detailed clinical and radiological description to analyze the anatomical correlates of involuntary movements. PATIENTS AND METHOD: We performed surface electromyography of appropriate muscles, long-loop reflexes, and somatosensory evoked potentials and analyzed the neuroimaging findings in patients with L2HGA and recorded blink reflex (BR), auditory startle response (ASR), and startle response after somatosensory stimuli (SSS) in patients and healthy subjects. We also performed a systematic literature search to identify the association of neuroimaging findings and movements disorders in previous patients with L2HGA. RESULTS: Thirteen patients were enrolled in the study. Among them, ten had low-amplitude postural tremor with a frequency between 4 and 7 Hz. The tremor was predominant on distal parts of the upper extremities. Postural tremor was accompanied by negative myoclonus in one-third. The BR, ASR, and SSS, all, were hypoactive. There was a close association of postural tremor with cerebellar atrophy in patients who participated in this study and by the analysis of the previously reported patients. CONCLUSIONS: Low-amplitude postural tremor is common in L2HGA. It is related with cerebellar atrophy. Although the neuroimaging shows no overt lesions at the brainstem, there is a functional inhibition at this level.

Contribution of brainstem circuits in pathophysiology of NREM-sleep parasomnias.

OBJECTIVE: We aimed to investigate the brainstem circuits to reveal if there was any abnormality in these circuits in clinically diagnosed patients with NREM parasomnias during wakefulness. METHODS: Twelve patients with NREM-sleep parasomnia diagnosed according to ICSD-3 criteria and a control group of 16 healthy subjects were enrolled into our study. We analyzed the auditory startle reflex (ASR), blink reflex (BR), prepulse inhibition (PPI) of BR and recovery excitability of BR. RESULTS: There was a trend for longer responses from orbicularis oculi and sternocleidomastoid muscles after auditory stimulation in the patients compared to those in the healthy subjects. The recovery percentages at 200 ms and 300 ms showed a borderline significance in the patients. No significant difference was found in the R2-PPI between the patients and healthy subjects. CONCLUSIONS: Our results suggest a mildly enhanced ASR and relatively early facilitation of BR excitability in patients with NREM-sleep parasomnia during daytime. Although our findings suggest involvement of brainstem networks in NREM-sleep parasomnia during wakefulness, it would be better to study these networks at night and during daytime to see if there is any contribution.

Prepulse inhibition and recovery of trigemino-cervical reflex in patients with cervical dystonia.

OBJECTIVE: We aimed to analyze the prepulse inhibition (PPI) and recovery rate (R) of the trigeminocervical reflex (TCR) in patients with cervical dystonia (CD). METHODS: We enrolled 15 patients with CD and 16 healthy subjects. TCR was recorded over splenius capitis after infraorbital nerve stimulation. For TCR-PPI, we applied a prepulse stimulus to the left second finger 100 ms prior to the test stimulus and the percentage of change of response to test stimulus was calculated. For TCR-R, we applied paired infraorbital stimuli at interstimulus interval (ISIs) of 300 ms and the percentage of change of the second compared to the first response was calculated. RESULTS: TCR-PPI and TCR-R values were higher (less inhibition and greater recovery) on both sides in the patient group compared to healthy subjects. There was high correlation between TCR-PPI and TCR-R on both sides in patients with dystonia (p < 0.005). We did not find any significant relationship between TCR-R or TCR-PPI and side of dystonic posture. CONCLUSIONS: We showed disturbed modulation of TCR in CD patients. In CD, a general inhibition of the inhibitory pathways and facilitation of the excitatory pathways occur. Although TCR was recorded directly on the affected muscles in CD, symmetric abnormal TCR findings in CD suggest that these findings are probably secondary to altered function of higher order centers rather than being directly related to the pathophysiological process.

Cutaneous silent period in patients with spinal muscular atrophy type 2 and type 3.

OBJECTIVE: The aim of this study was to examine cutaneous silent periods (CSPs) in spinal muscular atrophy (SMA) type 2 and type 3 patients. METHODS: Fourteen patients with SMA and 14 healthy subjects were included. CSPs were recorded from thenar muscles after painful stimulation of the index finger during slight thumb abduction. CSP parameters (including onset latency, duration, index of suppression, long-loop reflex, and post-inhibitory rebound activity) were measured. All parameters were compared between SMA patients and healthy subjects using the Mann-Whitney U test. We then correlated CSP parameters to Hammersmith Functional Motor Scale Expanded (HFMSE) scores in SMA patients by Spearman-rho testing. RESULTS: No CSP parameter except E3% (i.e., the post-inhibitory rebound activity relative to pre-stimulus baseline) differed significantly between SMA patients and healthy subjects. E3% was significantly smaller in patients than healthy individuals. HFMSE scores of SMA patients correlated negatively with CSP duration and positively with E3%. DISCUSSION: Smaller E3%, correlating with longer CSP duration, in SMA patients with more severe clinical findings and therefore more marked motoneuron loss suggests that the E3 component following the CSP is directly affected by motoneuron loss.

Late component of trigemino-cervical reflex: changes according to age and gender.

BACKGROUND: Trigemino-cervical reflex (TCR) is a protective reflex which is elicited by the stimulation of any branch of the trigeminal nerve. After infraorbital stimulation, an early and late components have been described. The aim of this study was to find out whether there are age- or gender-related changes in the long-latency (RII) component of TCR. METHOD: We included consecutive 53 healthy subjects (20 men, 37.7%) who had normal neurological examination. The mean age was 45.1 ± 14.3 years (age range 18-75 years). TCR was recorded simultaneously from bilateral sternocleidomastoid (SCM) and splenius capitis (SC) muscles with surface electrodes after stimulating right or left infraorbital branch of the trigeminal nerve, separately. We compared latency, amplitude, and duration according to gender and age. RESULTS: The amplitudes of SC responses were significantly higher in women compared to men. The duration of SCM response was significantly longer in subjects above the age of 50 years compared to younger patients. The latency of the SC response was significantly delayed above the age of 40 years. CONCLUSION: There are age- and gender-related changes in TCRs probably due to changes in the motoneurons of the SC and SCM muscles.

Changes in brainstem excitatory and inhibitory pathways in dry eye syndrome.

BACKGROUND: We hypothesized that there may be changes in sensory integration pathways in patients with dry eye. To confront this issue, we analyzed blink reflex (BR), prepulse modulation (PPM) of BR, and excitability recovery of BR to paired stimuli in 17 experimental subjects with dry eye syndrome. METHOD: We included 17 experimental subjects, 8 with primary and 9 with secondary, dry eye syndrome. We also examined a control group of 14 age and gender matched control subjects. After clinical evaluation, we recorded BR, PPM of BR (at 50 and 100 ms intervals) and BR percentage recovery to paired stimulation (at 300 and 500 ms intervals). RESULTS: None of the patients had any spasm activity. Experimental subjects had significantly larger R2 and R2c AUCs, significantly greater excitability recovery at 300 ms interval and significantly reduced R2 and R2c prepulse inhibition, in comparison to control subjects. Experimental subjects with primary dry eye syndrome had higher number of spontaneous blinks than experimental subjects with secondary dry eye syndrome (54.0 ±â€¯10.3 for primary dry eye and 43.5 ±â€¯13.3 secondary dry eye). CONCLUSION: Our results are compatible with increased excitability and abnormalities in sensorimotor integration in blink reflex circuits of patients with dry eye. This suggests the development of adaptive changes in brainstem synaptic activity, aimed at facilitation of blinking in the context of increased sensory input from corneal irritation.

Ulnar Neuropathy at Elbow in Patients With Type 2 Diabetes Mellitus.

PURPOSE: In diabetes mellitus (DM), upper extremity entrapment neuropathies are suggested to be a component of polyneuropathy (PNP). Our aim is to examine the presence of ulnar neuropathy at the elbow (UNE) and its relation to other findings including PNP in symptomatic and asymptomatic type-2 DM patients who were admitted for routine examinations. METHODS: The study included all cases referred for electromyography because of type-2 DM between November 2017 and May 2018. Demographic and clinical characteristics were recorded. Routine electromyography examinations in all cases included the following: bilateral motor conduction of the median, ulnar, peroneal, and tibial nerves and sensory conduction of the median, ulnar, and sural nerves. For ulnar nerve examination, stimuli were given at the wrist, below the elbow, and above the elbow. Electrophysiological findings were evaluated according to the American Association of Neuromuscular and Electrodiagnostic Medicine criteria. RESULTS: Eighty-two patients with type-2 DM and 144 upper extremities were included in the study. Of the 82 patients who participated in the study, 3 had findings suggesting ulnar neuropathy, and electrophysiology confirmed UNE in only one. Electrophysiological studies showed UNE in 36 patients. Other diagnoses identified by electrophysiology were carpal tunnel syndrome and PNP. Ulnar neuropathy at the elbow was more commonly associated with PNP compared with carpal tunnel syndrome. Gender and PNP were independent risk factors for the development of UNE. CONCLUSIONS: Although the majority of diabetic patients were asymptomatic for the UNE, approximately one third of all patients with DM were found to have UNE. Ulnar neuropathy at the elbow is closely related with PNP.

Prepulse modulation and recovery of trigemino-cervical reflex in normal subjects.

OBJECTIVE: In this study, we analyzed the inhibitory control on the trigemino-cervical reflex (TCR), and whether or not prepulse modulation (PPM) has an effect on TCR. Thus, we studied the PPM of TCR. We hypothesized that TCR would presumably be under the modulatory effect after the prepulse stimulus similar to blink reflex (BR). We also studied the recovery of TCR which was previously shown. METHODS: We included 13 healthy individuals. All subjects underwent recordings of TCR, TCR-PPM, and recovery of TCR. For TCR-PPM, a subthreshold stimulus to second finger 50 or 100 ms before the test stimulus was applied. For recovery of TCR, two stimuli at the infraorbital nerve were applied at 300, 500, and 800 ms interstimulus intervals (ISIs). RESULTS: There was an inhibition of bilateral late responses of TCR at the ISIs of both 50 ms and 100 ms. There was no change of latencies. Full recovery of TCR did not develop even at the ISI 800 ms. DISCUSSION: We have provided an evidence for the TCR-PPM in healthy subjects for the first time in this study. The prepulse inhibition is attributed to the functions of the pedunculopontine tegmental nucleus. Our study provides a strong indication that there are connections between pedunculopontine tegmental nucleus and trigemino-cervical circuit, which produces TCR.

Cortical modulation of brainstem circuits is abnormal in cervical dystonia.

OBJECTIVE: In healthy subjects, magnitude of hand-evoked blink reflex (BR) is increased if the stimulated hand is positioned in the peripersonal space. Here, we aimed to investigate the modulation of hand-evoked BR in peripersonal space to understand alterations of cortical modulations of subcortical structures in cervical dystonia. PATIENTS AND METHOD: We recruited 23 patients with idiopathic cervical dystonia and 21 healthy subjects, all of whom had hand-evoked BR. The reflex was recorded while stimulated hand was close to face ('peripersonal space) and far away from face (extrapersonal space). Comparisons were done between two conditions in each group. RESULTS: In extrapersonal space, magnitude of the reflex was bigger in patients compared to healthy subjects. In peripersonal space, magnitude of the reflex was increased in healthy subjects whereas it was reduced in patients. Comparisons of patients with and without sensory trick disclosed reflex magnitude was reduced in peripersonal space in patients with sensory trick whereas it did not change between two conditions in patients without sensory trick. DISCUSSION: Enhancement of hand-evoked BR is a normal phenomenon that provides evidence for top-down modulation of its neural circuitry. In cervical dystonia, the normal modulation is lost which suggests abnormal modulatory effect of higher-order centers upon brainstem circuits. The pattern of alteration also differs if there is no sensory trick.

Differential modulation of prepulse inhibition of the blink reflex in peripersonal versus extrapersonal space.

OBJECTIVE: Threatening stimuli encountered in peripersonal space (PPS) are processed differently from those encountered in extrapersonal space (EPS). This phenomenon is attributed to tonic top-down modulation. We hypothesized that prepulse inhibition (PPI) of a reflex, which has a protective function, may change according to whether the conditioning stimulus appears in PPS or EPS. We aimed to compare the strength of the PPI according to whether stimulation was delivered in PPS or EPS. METHODS: The study included 23 healthy subjects with a mean age of 36.8±9.1years. Recordings of blink reflex (BR) after supraorbital stimulation (so-BR) were performed. Recordings of BR after prepulse stimulation to the median nerve 100ms prior to the supraorbital stimulation were acquired with the ipsilateral hand 50-60cm from the face (EPS) and approximately 3-4cm from the face (PPS). Changes of response magnitudes were compared between PPS and EPS conditions. RESULTS: R2 area-under-the curve of so-BR was reduced after prepulse stimulation of median nerve in all subjects while the hand was in EPS. Although the R2 magnitude was also decreased after prepulse stimulation while the hand was in PPS, the percentage of reduction with the hand in PPS was significantly smaller compared to that with the hand in EPS. CONCLUSION: Reduction in R2 magnitude after prepulse stimulation 100ms prior to test pulse is recognized (PPI). Although PPI was observed under both conditions, PPI of so-BR was attenuated when the stimulus was presented in the PPS. Therefore, our study provides evidence for modulation of PPI of so-BR in PPS and may suggest top-down modulation of the neural circuitry underlying PPI.

Startle responses after different stimulus modalities differ in stroke.

OBJECTIVES: The auditory startle reaction (ASR) and startle reflex to somatosensory inputs (SSS) are stereotypical responses to sudden and unexpected stimuli, which are generated in the caudal brainstem reticular formation. Changes of ASR are relatively well known in stroke. Here, we aimed to investigate central pathways of SSS and plasticity changes of SSS circuits in different stages and localizations of stroke, by comparing with ASR. METHODS: We prospectively included 39 patients with stroke between June 2009 and June 2013, and 23 age and gender-matched healthy subjects. ASR and SSS were recorded over orbicularis oculi, sternocleidomastoid, biceps brachii (BB), and abductor policis brevis muscles (APB) using surface electrodes. RESULTS: There were supratentorial and infratentorial lesions in both acute and chronic stages. Overall, ASR probability was similar between groups (P=0.981). However, ASR probability was increased for BB and APB recordings on symptomatic sides of stroke patients with high amplitudes and long durations, most prominently on symptomatic sides of pontine strokes. Latencies and presence rates of SSS did not differ between any subgroups of stroke and healthy subjects. CONCLUSION: ASR is facilitated in arm and hand muscles on symptomatic sides of stroke patients, whereas SSS did not show any significant changes according to stroke.