Thalamic Deep Brain Stimulation May Improve Restless Legs Syndrome in Patients With Essential Tremor.

OBJECTIVES: To determine change in restless legs syndrome (RLS) symptoms in essential tremor (ET) patients undergoing bilateral thalamic ventral intermedius (VIM) deep brain stimulation (DBS) surgery. MATERIALS AND METHODS: We retrospectively reviewed our database of ET patients with RLS who had undergone VIM DBS for tremor from 2012 to 2020. We reviewed the patients with available International Restless Leg Syndrome Study Group RLS scale scores before and after DBS. Percentage of responders, defined as proportion of patients experiencing three or more point improvement of RLS scores post-DBS, was calculated. We performed two-tailed t-test of pre-DBS and post-DBS RLS scores. RESULTS: We identified 13 patients with ET and RLS who had undergone bilateral VIM DBS, of whom nine (69%) were responders post-DBS. Five of 13 patients (38%) had complete resolution of RLS post-DBS. For all patients, mean pre-DBS RLS score was 15.8 ± 7.9 which improved by 46% post-DBS to a mean of 8.5 ± 8.8 (p = 0.007). Four patients rated their RLS scale one night with the stimulator OFF and another night with the stimulator ON. The mean RLS score with stimulator ON was 15.5 ± 7.6 which improved by 53% to a mean of 6.25 ± 7.8 (p = 0.008), with two having complete resolution of RLS with stimulator ON. Of the nine responders, six preferred to keep their stimulator ON at night due to relief of RLS and better subjective quality of sleep. CONCLUSIONS: We report for the first time improvement of RLS in patients with ET after bilateral thalamic DBS. Although many ET patients with nonrechargeable DBS systems switch off their stimulator at night to conserve battery life, those with RLS may potentially benefit from keeping their stimulator ON at night to relieve their RLS.

Tardive Blepharospasm May Respond to Bilateral Pallidal Deep Brain Stimulation.

Background: To date, there have been no reports of tardive blepharospasm being treated with deep brain stimulation (DBS), though there have been two reports of focal blepharospasm responding favorably to bilateral pallidal DBS. Case: A 34 year old man with tardive blepharospasm that was refractory to oral medications as well as botulinum toxin types A and B underwent bilateral pallidal DBS under general anesthesia. He had significant improvement of his severe blepharospasm by one and half months post-DBS which was sustained at last follow-up 30 months post-DBS. The best programming parameters included pulse widths of 90-100 µsec, frequencies of 140-150 Hz, and stimulating the ventral contacts in each side. Conclusion: Our case represents the first report of medically refractory tardive blepharospasm responding favorably to bilateral pallidal DBS.

Short-Lasting Unilateral Neuralgiform Headache With Conjunctival Injection and Tearing (SUNCT) Improves With Bilateral Ventral Tegmental Area Deep Brain Stimulation.

BACKGROUND: Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) is a severe headache disorder characterized by clustered episodes of extreme pain. Refractory to most interventions, ipsilateral unilateral ventral tegmental area (VTA) deep brain stimulation (DBS) has been previously reported to be efficacious in 14 cases. METHODS AND RESULTS: Herein, we report the first case of an individual with medically refractory SUNCT who underwent bilateral VTA DBS. The patient experienced better improvement of his headaches with bilateral stimulation compared to unilateral stimulation. He also had a return of severe headaches within a few hours after his stimulator was switched off during sleep, with rebound worsening of his headaches over several days. The main side effects were double vision and difficulty focusing while reading, which were observed primarily with unipolar stimulation that required high voltages, high frequencies, and multiple negative contacts to control the headaches. The side effects were minimized with bipolar and interleaving stimulation with comparable control of his headaches. CONCLUSION: Bilateral VTA DBS may be effective in alleviating medical refractory SUNCT.

Adductor Spasmodic Dysphonia Improves with Bilateral Thalamic Deep Brain Stimulation: Report of 3 Cases Done Asleep and Review of Literature.

Background: To date, there are only six published reports of adductor spasmodic dysphonia (SD) responding to awake thalamic deep brain stimulation (DBS). Methods: We retrospectively reviewed cases of Essential Tremor (ET) with SD that were seen in our center from 2012 to 2020. We further identified those that have undergone thalamic DBS, and had a blinded laryngologist rate first the audio voice recordings before and after DBS using the Unified Spasmodic Dysphonia Rating Scale (USDRS), and the video recordings last to rate the related movements and facial grimacing. Results: We identified three cases of adductor SD with ET that had undergone bilateral ventralis intermedius (VIM) DBS under general anesthesia. All patients noted improvement of their limb and voice tremor, as well as their SD post-DBS. Although improvement of tremor was observed even with initial programming in all three, improvement of SD was noted only upon reaching higher amplitudes or wider pulse widths. Blinded voice assessments showed improvement of USDRS scores post-DBS compared to pre-DBS, and with stimulator on compared to stimulator off. Discussion: We report the first three cases of SD responding favorably to bilateral VIM asleep DBS and summarize the nine cases so far of SD who have undergone thalamic DBS.

Emotional Reactivity as a Vulnerability for Psychogenic Nonepileptic Seizures? Responses While Reliving Specific Emotions.

OBJECTIVE: Dysfunction in emotional processes is a hypothesized contributor to functional neurological disorders (FNDs), yet few studies have evoked real-time emotion during multimethod assessment incorporating subjective, behavioral, and psychophysiological indicators. This approach may reveal clinical and neurobiological vulnerability to FND and clarify how dysfunctional emotional processes serve as perpetuating factors. METHODS: Eleven participants with video-EEG-confirmed diagnoses of psychogenic nonepileptic seizures (PNES) were compared with 49 seizure-free trauma control subjects (TCs) with or without clinically elevated posttraumatic stress symptoms (25 clinically elevated [TC-clin], 24 not clinically elevated [TC-nonclin]). Participants recalled and described memories evoking anger, shame, happiness, and neutral feelings. RESULTS: Even though PNES patients and TCs reported similar amounts of emotional experience, PNES patients reported more difficulty reliving emotions and were less likely to complete the relived shame task. During and after reliving happiness, PNES and TC-clin groups showed respiratory sinus arrhythmia (RSA) decreases, indicating parasympathetic withdrawal, whereas the TC-nonclin group showed RSA increases. CONCLUSIONS: Findings from this pilot study are consistent with previous research and clinical observations that emotional engagement may be more effortful for PNES patients. Patterns of RSA change, which may also point to greater effortful engagement, were similar in PNES and TC-clin groups, suggesting that traumatic stress reactions may play a part. At the same time, experience of greater difficulty or avoidance may be even greater among PNES patients. Especially when regulatory resources are already limited, accumulated effort, coupled with self-threatening contexts such as shame, may be particularly problematic for those with PNES and perhaps other FNDs.

Orthostatic Tremor is Responsive to Bilateral Thalamic Deep Brain Stimulation: Report of Two Cases Performed Asleep.

Background: Orthostatic tremor (OT) is a hyperkinetic movement disorder characterized by rapid tremor in the lower extremities or trunk upon standing. Case Report: We report two patients presenting with OT, whose symptoms improved markedly following asleep bilateral thalamic deep brain stimulation (DBS) surgery. Discussion: Medically refractory OT can respond favorably to asleep bilateral DBS surgery similar to awake surgery, and may have the advantages of less psychological trauma to the patient, shorter procedure times, and less exposure to anesthesia.