Clinical Neurophysiological Evaluation for Simple Motor Tics.

Simple motor tics are sudden, nonrhythmic jerk like movements that are often preceded by premonitory urge and can be voluntarily suppressed. Some clinical characteristic of tics such as variability of movement, distractibility and fluctuating course can mimic functional movement disorders. Here we report a case of motor tics where the physiological approach in addition to careful clinical assessments is helpful to support the diagnosis of tic.

Introduction

Simple motor tics are sudden, rapid, recurrent, nonrhythmic jerk-like movements that are often preceded by premonitory urge (Kwak et al., 2003) and may be voluntarily suppressed for a brief period (Himle and Woods, 2005). Some clinical characteristics of motor tics such as sudden onset, variability of movement distributions, distractibility, suggestibility, temporary remissions and waxing and waning courses may lead to incorrect diagnosis of functional (psychogenic) movement disorders (Jankovic and Kurlan, 2011, Demartini et al., 2015). Careful history taking and electrophysiological study might help to differentiate between these disorders. Here we described a case of motor tics that illustrates the physiological approach. The patient gave informed consent for participating in an IRB approved protocol and for publishing the video.

Case description

A 36 year-old right-handed female was referred to our clinic for a second opinion concerning the presence of jerky movements all over the body, randomly in the hands, arms, legs and face, predominantly on the right for 2 months. The referring diagnosis was functional movement disorder. Before the movement, she sometimes had a feeling of something clawing in the certain part of the body and relieved whenever she moved it. She was able to suppress the jerky movement by clenching her fist as well as tensing up that particular muscle for a brief period, but it eventually built up again and she needed to move. The movements occurred more often when she was relaxed whereas they occurred less frequently when she was concentrating on something. She was taking diazepam to control these jerks without any benefit. She had history of similar movements when she was teenager for 5 years and was diagnosed as having a psychogenic movement disorder also at that time. These movements spontaneously disappeared without any treatment. She denied history of obsessive–compulsive symptoms and attention-deficit syndrome during childhood. Neurological examination demonstrated random jerky movements of the right fingers, arm, shoulder and the right leg. The movements were sometimes accompanied with urge to move. She was able to suppress them for a brief period (see video). These movements were sometimes distractible, but not entrainable with tapping maneuver. Magnetic resonance imaging was normal.

The diagnosis of our patient with jerky movements can either be motor tics or functional myoclonus due to variability of her movements. The clawing sensations prior to the movements and the feelings of relief after the movements are consistent with premonitory urge. Urge to move and ability to voluntarily suppress the movements favor tics rather than functional myoclonus. However, distractibility during the exam is suggestive of functional myoclonus. The history of similar movement during teenager further supports the diagnosis of tics that reoccurs in adulthood even if she was previously diagnosed as having a functional movement disorder. While we favored tic, the referring doctor was firm in the functional diagnosis; hence, electrophysiological study was done for further investigation.

Clinical neurophysiological evaluation

The first step in the evaluation of a hyperkinetic movement is multichannel surface electromyography (EMG) (Hallett, 2003a). The purpose of this investigation is to see the duration of the EMG bursts in the involved muscles and the relationship of the bursts in the different muscles, particularly in antagonist pairs. Here there were EMG bursts with variable duration of 300–1000 ms in the right deltoid, triceps, biceps, extensor carpi radialis (ECR) and flexor carpi radialis (FCR) muscle. The jerking movements demonstrated a triphasic pattern of the agonist and antagonist muscle activity resembling voluntary ballistic movements (Fig. 1).

Fig. 1

Multichannel surface EMG showed EMG bursts with variable durations in the triphasic pattern of the agonist and antagonist muscle activity resembling voluntary ballistic movement. FCR is flexor carpi radialis and ECR is extensor carpi radialis.

The second step is to evaluate the relationship of the EEG to the EMG bursting (Hallett, 2012). This is accomplished with EEG–EMG jerk-locked back-averaging. Multichannel EEG is recorded together with an active EMG channel, and the EEG before the EMG onset is averaged and evaluated. In this case, the back-averaging was performed with respect to the onset of EMG activity on the right triceps and averaging EEG in central and frontal leads (Fz, Cz, F3, F4, C3, C4). A steep rising negative potential was seen in the central leads (Cz and C3) beginning at 140 ms prior to the movement onset with amplitude of 8.5 and 5.9 μV consistent with the late Bereitschaftspotential (late BP) (Fig. 2). Then similar back-averaging was done with voluntary movements mimicking the involuntary movements. In this circumstance, a slow rising negative potential was seen in all leads beginning at 1100 ms prior to the movement onset with amplitude of 9.6–14.5 μV (Fig. 3).

Fig. 2

The EEG–EMG jerk-locked back-average showed the late Bereitschaftspotential (late BP) in the central leads (Cz and C3) at 140 ms prior to the movement onset with amplitude of 8.5 and 5.9 μV in the spontaneous jerks.

Fig. 3

The EEG–EMG jerk-locked back-average showed Bereitschaftspotential in all leads at 1100 ms prior to the movement onset with amplitude of 9.6–14.5 μV in the voluntary condition.

The study was interpreted as supporting the suspected diagnosis of chronic motor tics. The reasoning for this interpretation is given below. The patient was sent back to the referring neurologist who now accepted the diagnosis. Subsequently the patient started taking tetrabenazine 25 mg for tic control with significant improvement.

Discussion

Resolving the differential diagnosis between tics and functional myoclonus can be difficult (van der Salm et al., 2013). Simple motor tics are sudden, brief, repetitive jerk-like movements that vary in frequency, intensity and distribution (Jankovic and Kurlan, 2011, Hallett, 2015). Some clinical features of motor tics including variability of movements, sudden onset, distractibility, suggestibility, temporary remission and fluctuating courses are also frequently observed in functional myoclonus (Gupta and Lang, 2009, Jankovic and Kurlan, 2011, Baizabal-Carvallo et al., 2014, Demartini et al., 2015). Motor tics often have a premonitory urge and may be temporarily suppressed (Kwak et al., 2003, Himle and Woods, 2005). Lack of premonitory sensation, inability to suppress the movements, lack of typical rostrocaudal tic distribution over time, adult onset, absence of childhood and family history of tics and coexistence of other functional movement disorders seem to point to functional myoclonus resembling tics (Baizabal-Carvallo et al., 2014, Demartini et al., 2015). However, not all patients with tics have premonitory sensations and some patients with functional jerks reported some sensations before the movements (van der Salm et al., 2010). The differential diagnosis of the jerky movements in our patient is mainly either motor tics or functional myoclonus. She reported some clawing sensations prior to the movements and relief after moving consistent with premonitory urge. Urge to move and ability to voluntarily suppress are typical for tics rather than functional myoclonus. Distractibility can be observed in both tics and functional myoclonus (Jankovic and Kurlan, 2011, Demartini et al., 2015). The fact that she has a history of similar symptoms during teenage years further supports the diagnosis of tics. However, fluctuating course and history of spontaneous remission may happen in both conditions. With uncertainty of clinical assessment, further electrophysiological evaluation should be considered.

The electrophysiological study with multichannel surface EMG might not be helpful in distinguishing between functional myoclonus and motor tics because both types of movement are variable in frequency, duration and spreading pattern. In addition, EMG bursts in tic movements have triphasic pattern resembling voluntary ballistic movement (Hallett, 2003b, Apartis, 2014) as demonstrated in our case. The value of doing this here is to rule out other disorders such as cortical myoclonus. Also, the study will help identify good muscles to use for the second step of the evaluation, the EEG–EMG correlation. The right triceps was selected as the best muscle for EEG–EMG jerk-locked back-average because it had the earliest onset. It is important to use the earliest muscle for back-averaging. If a later muscle is used, then some of the EEG activity will be after the movement onset, and it is critical to see what is happening before movement.

EEG–EMG jerk-locked back-average may be useful for differential diagnosis. The Bereitschaftspotential (BP) is the cortical potential originally described as preceding voluntary movements. The early BP (BP1) is the initial slow rising segment of BP and indicates the involvement of the supplementary motor areas and premotor cortices bilaterally during preparation of movement. The late BP (BP2) is the steep rising segment of BP starting about 400 ms prior to the movement onset and indicates the involvement of contralateral motor cortex and premotor cortex for selection of appropriate movement and the initiation of movement execution (Shibasaki and Hallett, 2006). In prior studies, patients with motor tics had either no BP prior to the movement or BP with short duration within the range of late BP (Obeso et al., 1981, Karp et al., 1996, van der Salm et al., 2012). A recent study found that 86% of patients with functional myoclonus had BP prior to the movement onset with earlier onset while only 43% of patients with motor tics had a BP and most of them (29%) had only the early component. Moreover, the absence of BP prior to the voluntary movements is highly specific (0.98) for functional myoclonus (van der Salm et al., 2012). In our case, we demonstrated BP in the central lead contralateral to the side of movement at 150 ms prior to the movement onset consistent with late BP. Although the presence of BP showed low specificity for the diagnosis of functional myoclonus and tics, 0.68 and 0.26 respectively, the specificity of the presence of only late BP has not been studied. The presence of both components of BP in the voluntary movements mimicking the involuntary movements in addition to the presence of late BP are helpful for diagnosis of tics in our case, since it confirms that this patient’s brain can generate the full BP. The presence of a late BP in tic patients suggests that tics are movements originating from a subcortical generator such as the insula (Bohlhalter et al., 2006), bypassing the premotor cortex (Hallett, 2015), and tic expression might be a volitional response to internal urge (Hallett, 2003b, Yael et al., 2015). This is different from functional myoclonus where the movements share some physiology of voluntary movements without perception of voluntariness (Hallett, 2010). The diagnosis of motor tics rather than psychogenic movement disorders in this case was aided by the presence of only a late BP.

Conclusion

Simple motor tics and functional jerks share some clinical features. Combination of careful clinical assessment and electrophysiological study demonstrating an isolated late Bereitschaftspotential is helpful for differentiation between motor tics and functional movement disorders.

Conflict of interest

Dr. Pattamon Panyakaew: Anandamahidol Foundation Scholarship under the Royal Patronage of His Majesty the King of Thailand. Dr. Hyun Joo Cho: Fellowship from the Dystonia Medical Research Foundation. Dr. Mark Hallett: None.

Funding sources for study

NINDS Intramural Program.