Diagnosis, Treatment, and Management of Dejerine-Roussy Syndrome: a Comprehensive Review.

PURPOSE OF REVIEW: Post-stroke pain represents a complex condition with few standardized diagnostic criteria. As such, the array of symptoms is often difficult to categorize and diagnose. Central post-stroke pain (CPSP), also known as Dejerine-Roussy syndrome, presents as painful paresthesia in any part of the body that is usually coupled with sensory abnormalities. RECENT FINDINGS: In patients who had experienced a cerebrovascular accident, CPSP typically affects the same areas of the body that are also impacted by the general motor and sensory deficits that result from stroke. Though it is generally debated, CPSP is thought to result from a lesion in any part of the central nervous system. Pain usually presents in the range of 3-6 months after the occurrence of stroke, manifesting contralaterally to the lesion, and most commonly involving the upper extremities. For the most accurate diagnosis of CPSP, a thorough history and clinical examination should be supplemented with imaging. Infarcted areas of the brain can be visualized using either CT or MRI. First-line treatment of CPSP is pharmacologic and consists of a three-drug regimen. Despite this, CPSP is often refractory to medical management producing only modest pain reduction in a limited subset of patients. Adverse effects associated with pharmacologic management of CPSP and frequent recalcitrance to treatment have driven alternative minimally invasive methods of pain control which include transcranial stimulation, deep brain stimulation, and neuromodulation. The aim of this review is to provide a comprehensive update to recent advances in the understanding of the treatment and management of CPSP.

Thalamic perforating artery stroke on computed tomography perfusion in a patient with coronavirus disease 2019.

Emerging evidence suggests that patients with coronavirus disease 2019 (COVID-19) are at risk of thromboembolic complications, including ischemic strokes. We present a case illustrating the value of CT perfusion to identify acute small subcortical infarcts in a patient with COVID-19 admitted to an intensive care unit for bilateral pneumonia and pulmonary embolism presenting with sudden right limb weakness.

Treatment of Déjerine-Roussy syndrome pain with scrambler therapy.

Aim: Déjerine-Roussy syndrome or central thalamic pain can be devastating, and treatment with drugs and even deep brain stimulation can be unsatisfactory. Scrambler therapy is a form of neuromodulation that uses external skin electrodes to send a 'non-pain' signal to the brain, with some success in difficult-to-treat syndromes such as neuromyelitis optica spectrum disorder. We used scrambler therapy to treat a patient with 6 years of disabling Déjerine-Roussy syndrome pain. Methods: A 56-year-old man received multiple daily then monthly treatments with electrode pairs placed just above the area of distal pain. Each treatment was for 40 min. Results: His allodynia and hyperalgesia resolved within 10 min, and his pain score fell to almost zero after 30 min. Months later, he resumed normal activity and is off all his pain medications. No side effects were noted. Conclusion: Scrambler therapy appeared to reverse 6 years of disabling pain safely and economically, and continues to be effective. Further multi-institutional trials are warranted for this rare syndrome.

Dorsal anterior cingulate cortex (ACC) deep brain stimulation (DBS): a promising surgical option for the treatment of refractory thalamic pain syndrome (TPS).

BACKGROUND: Neuroimaging evidences and previous successful case series of cingulotomy for cancer pain have disclosed the key-role of the dorsal anterior cingulate cortex (ACC) in the generation of the empathic and affective dimension of pain. The aim of this study is to assess the effectiveness and safety of ACC neuromodulation for the treatment of the thalamic pain syndrome (TPS), a chronic neuropathic disease often complicated by severe affective and emotional distress in the long term. METHOD: From January 2015 to April 2017, 5 patients with pure drug-refractory TPS underwent ACC deep brain stimulation (DBS) at our institution. Quantitative assessment of pain and health-related quality of life were performed 1 day before surgery and postoperatively at 6 and 18 months by using the numeric rating scale (NRS), the 36-item short-form health survey (SF-36), and the McGill pain and the EuroQol5-domain questionnaires. RESULTS: Mean age at surgery was 56.2 years (range, 47-66). NRS score improved by 37.9% at 6 months (range, - 22.2 to - 80%) and by 35% at 18 months (range, - 11.1 to - 80%). At the last follow-up, one patient reported a relevant pain reduction (NRS 2), only complaining of mild pain poorly interfering with activities of daily living. Concomitant improvements in the McGill and EuroQol5-domain pain questionnaires, SF-36 total and sub-item scores were also noticed at each follow-up. No surgical or stimulation-related complications occurred during the study period. CONCLUSIONS: ACC DBS may be a safe and promising surgical option to alleviate discomfort and improve the overall quality of life in a patient affected by drug-resistant TPS. Further prospective, larger, and randomized studies are needed to validate these findings.

[Resuscitation acupuncture for thalamic pain:a randomized controlled trial].

OBJECTIVE: To compare the effects between resuscitation acupuncture and pregabalin for thalamic pain and their impacts on plasma P substance (SP) and ß-endorphin (ß-EP). METHODS: Sixty-four patients were randomly assigned into an acupuncture group and a western medication group, 32 cases in each one. Based on conventional western methods, pregabalin capsule was used orally in the western medication group, 75 mg a time,twice a day; resuscitation acupuncture was applied in the acupuncture group. The main acupoints were Shuigou (GV 26), Neiguan (PC 6), Sanyinjiao (SP 6). Patients with upper limb pain were attached affected Jiquan (HT 1), Chize (LU 5), and Hegu (LI 4); lower limb pain, affected Weizhong (BL 40), Zusanli (ST 36); hea-dache, bilateral Fengchi (GB 20), Wangu (GB 12), and Yifeng (TE 17), twice a day. Treatment was given 6 d a week for 8 weeks in the two groups. The changes of simplified McGill pain questionnaire (SF-MPQ), plasma SP and ß-EP were observed before and after 4-week, 8-week treatment, as well as at follow-up, namely, 3 months after treatment. Also, clinical effects were evaluated. RESULTS: The total effective rate of the acupuncture group was 50.0% (16/32) after 4-week treatment, which was similar to 46.9% (15/32) in the western medication group (P>0.05). While after 8-week treatment and at follow-up, the total effective rates of the acupuncture group were 90.6% (29/32) and 84.4% (27/32), which were better than 65.6% (21/32) and 40.6% (13/32) of the western medication group correspondingly (both P<0.05). After 4-week, 8-week treatment and at follow-up, the pain scores of the acupuncture group were lower than that before treatment (all P<0.05). After 4-week and 8-week treatment, the pain scores of the western medication group were lower than that before treatment (both P<0.05). After 8-week treatment and at follow-up, the pain scores of the acupuncture group were superior to thoseof the western medication group (both P<0.05). After 4-week and 8-week treatment,the contents of plasma SP reduced compared with those before treatment in the two groups (all P<0.05), and plasma ß-EP increased (all P<0.05). After 8-week treatment, SP content of the acupuncture group was apparently lower than that of the western medication group (P<0.05), and ß-EP increased more obviously (P<0.05). CONCLUSIONS: Resuscitation acupuncture can effectively relieve the symptoms of thalamic pain with stable and long-term effect, and it is better than pregabalin. Meanwhile, the acupuncture can increase ß-EP and reduce SP.

Deep Brain Stimulation for the Treatment of Dejerine-Roussy Syndrome.

BACKGROUND/AIMS: Patients who suffer from Dejerine-Roussy syndrome commonly experience severe poststroke hemibody pain which has historically been attributed to thalamic lesions. Despite pharmacological treatment, a significant proportion of the population is resistant to traditional therapy. Deep brain stimulation is often appropriate for the treatment of resistant populations. In this review we aim to summarize the targets that are used to treat Dejerine-Roussy syndrome and provide insight into their clinical efficacy. METHODS: In reviewing the literature, we defined stimulation success as achievement of a minimum of 50% pain relief. RESULTS: Contemporary targets for deep brain stimulation are the ventral posterior medial/ventral posterior lateral thalamic nuclei, periaqueductal/periventricular gray matter, the ventral striatum/anterior limb of the internal capsule, left centromedian thalamic nuclei, the nucleus ventrocaudalis parvocellularis internis, and the posterior limb of the internal capsule. CONCLUSIONS: Due to technological advancements in deep brain stimulation, its therapeutic effects must be reevaluated. Despite a lack of controlled evidence, deep brain stimulation has been effectively used as a therapeutic in clinical pain management. Further clinical investigation is needed to definitively evaluate the therapeutic efficacy of deep brain stimulation in treating the drug-resistant patient population.

Thalamic pain alleviated by stellate ganglion block: A case report.

RATIONALE: Thalamic pain is a distressing and treatment-resistant type of central post-stroke pain. Although stellate ganglion block is an established intervention used in pain management, its use in the treatment of thalamic pain has never been reported. PATIENT CONCERNS: A 66-year-old woman presented with a 3-year history of severe intermittent lancinating pain on the right side of the face and the right hand. The pain started from the ulnar side of the right forearm after a mild ischemic stroke in bilateral basal ganglia and left thalamus. Weeks later, the pain extended to the dorsum of the finger tips and the whole palmar surface, becoming more severe. Meanwhile, there was also pain with similar characteristics emerging on her right face, resembling atypical trigeminal neuralgia. DIAGNOSES: Thalamic pain was diagnosed. INTERVENTIONS: After refusing the further invasive treatment, she was suggested to try stellate ganglion block. OUTCOMES: After a 3-day period of pain free (numerical rating scale: 0) postoperatively, she reported moderate to good pain relief with a numerical rating scale of about 3 to 4 lasting 1 month after the first injection. Pain as well as the quality of life was markedly improved with less dose of analgesic agents. LESSONS: Stellate ganglion block may be an optional treatment for thalamic pain.

[Spinal cord stimulation for thalamic pain: Case report and review of the current literature]. / Hochzervikale Rückenmarkstimulation bei Thalamusschmerz : Fallbericht und Übersicht zur aktuellen Literatur.

BACKGROUND: Spinal cord stimulation (SCS) is an established procedure for treatment of chronic neuropathic pain of peripheral origin. The efficacy of SCS in case of central poststroke pain (CPSP), especially thalamic pain, has not been adequately proven. OBJECTIVES: The efficacy of SCS as an extracranial neurostimulation method for the management of central pain syndrome was investigated. MATERIALS AND METHODS: In this study, relevant pharmacological and nonpharmacological measures for central pain management were reviewed. A case of successful SCS for thalamic pain after ischemic insult is presented. Explanatory approaches of pathophysiological processes and a review of the current literature underline our results. RESULTS: In the case presented, SCS was found effective in the treatment of thalamic pain. CONCLUSION: The efficacy of SCS might be caused by segmental and supraspinal processes and collaboration of activating and inhibiting pathways. The integrity of the spinothalamic tract is mandatory. SCS is a treatment option for central pain syndrome, especially thalamic pain. Comparable studies confirm the potency of this technique. In contrast to other neuromodulation procedures spinal cord stimulation is less invasive, has a lower perioperative risk and is often less expensive. Further studies are needed to define its potential and role in the treatment of thalamic pain.

Deep brain stimulation in the treatment of Holmes tremor - a long-term case observation.

We present the patient with Holmes tremor secondary to the infarction of thalamus, successfully treated with the deep brain stimulation (DBS) of the area between ventralis oralis anterior and zona incerta for a long time, in whom the severe tremor reappeared after removal of the DBS lead. This is the first presentation of the effective DBS on this location. Our case does not support the hypothesis that the DBS treatment could lead to sustained relief of symptoms after cessation of stimulation.

Communication skills and thalamic lesion: Strategies of rehabilitation.

AIM: To describe the speech rehabilitation history of patients with thalamic lesions. BACKGROUND: Thalamic lesions can affect speech and language according to diverse thalamic nuclei involved. Because of the strategic functional position of the thalamus within the cognitive networks, its lesion can also interfere with other cognitive processes, such as attention, memory and executive functions. Alterations of these cognitive domains contribute significantly to language deficits, leading to communicative inefficacy. This fact must be considered in the rehabilitation efforts. MATERIALS AND METHODS: Whereas evaluation of cognitive functions and communicative efficiency is different from that of aphasic disorder, treatment should also be different. The treatment must be focused on specific cognitive deficits with belief in the regaining of communicative ability, as well as it occurs in therapy of pragmatic disorder in traumatic brain injury: attention process training, mnemotechnics and prospective memory training. RESULTS: According to our experience: (a) there is a close correlation between cognitive processes and communication skills; (b) alterations of attention, memory and executive functions cause a loss of efficiency in the language use; and (c) appropriate cognitive treatment improves pragmatic competence and therefore the linguistic disorder. CONCLUSION: For planning a speech-therapy it is important to consider the relationship between cognitive functions and communication. The cognitive/behavioral treatment confirms its therapeutic efficiency for thalamic lesions.

Barriers to investigator-initiated deep brain stimulation and device research.

The success of device-based research in the clinical neurosciences has overshadowed a critical and emerging problem in the biomedical research environment in the United States. Neuroprosthetic devices, such as deep brain stimulation (DBS), have been shown in humans to be promising technologies for scientific exploration of neural pathways and as powerful treatments. Large device companies have, over the past several decades, funded and developed major research programs. However, both the structure of clinical trial funding and the current regulation of device research threaten investigator-initiated efforts in neurologic disorders. The current atmosphere dissuades clinical investigators from pursuing formal and prospective research with novel devices or novel indications. We review our experience in conducting a federally funded, investigator-initiated, device-based clinical trial that utilized DBS for thalamic pain syndrome. We also explore barriers that clinical investigators face in conducting device-based clinical trials, particularly in early-stage studies or small disease populations. We discuss 5 specific areas for potential reform and integration: (1) alternative pathways for device approval; (2) eliminating right of reference requirements; (3) combining federal grant awards with regulatory approval; (4) consolidation of oversight for human subjects research; and (5) private insurance coverage for clinical trials. Careful reformulation of regulatory policy and funding mechanisms is critical for expanding investigator-initiated device research, which has great potential to benefit science, industry, and, most importantly, patients.

Deep brain stimulation of the ventral striatum/anterior limb of the internal capsule in thalamic pain syndrome: study protocol for a pilot randomized controlled trial.

BACKGROUND: Chronic neuropathic pain in thalamic pain syndrome remains intractable. Its poor response is ascribed to destruction of the integrated neuromatrix in experience of pain. Deep brain stimulation is a promising technique to modulate activity of implicated structures. However, traditional approaches targeting sensori-motor substrates have failed to affect disability. The offending lesion in thalamic pain syndrome that almost invariably destroys sensory pain pathways may render these classical approaches ineffective. Instead, we hypothesize that targeting structures representing emotion and affective behavior-ventral striatum/anterior limb of the internal capsule, may alleviate disability. METHODS/DESIGN: We present the design of our phase I randomized, double-blinded, sham-controlled, crossover trial that examines safety, feasibility and efficacy of our proposed approach. In our ongoing trial, we intend to enroll ten patients with thalamic pain syndrome. Following implantation, patients are randomized to receive active deep brain stimulation to the ventral striatum/anterior limb of the internal capsule or sham for 3 months, after which they are crossed over. The primary endpoint is Pain Disability Index. Other outcomes include visual analog scale, depression and anxiety inventories, quality of life, and functional neuroimaging. DISCUSSION: Designing trials of deep brain stimulation for pain is challenging owing to the ethical-scientific dilemma of introducing a control arm, complicated blinding, heterogeneous etiologies, patient expectations, and inadequate assessment of disability. The quality of evidence in the field is classified as level III (poor) because it mainly includes a multitude of uncontrolled case series reporting variable outcomes, with little regard for the placebo effect related to implantation. Without valid data on efficacy, use of deep brain stimulation for pain remains "off label". We present our trial design to discuss feasibility of conducting sham-controlled phase I studies that may represent significant refinement for the field. Double-blinding would reduce influence of patient expectations and therapeutic confusion amongst investigators. With a cross-over approach, the dilemma regarding including a control group can be mitigated. Use of homogeneous etiology, measurement of disability, depression and quality of life, besides pain perception, all represent strategies to evaluate efficacy rigorously. Functional imaging would serve to define mechanisms underlying observed effects and may help optimize future targeting. TRIAL REGISTRATION: Clinicaltrials.gov NCT01072656.

Tractography-guided stimulation of somatosensory fibers for thalamic pain relief.

BACKGROUND: The spinothalamocortical tract (STC) is seen as a neural tract responsible for or involved in the generation or transmission of thalamic pain. Either the thalamus itself or the posterior limb of the internal capsule (PLIC) are targets for deep brain stimulation (DBS) in patients with thalamic pain, but due to its low contrast, conventional MRI cannot visualize the STC directly. OBJECTIVES: To show the feasibility of integrating diffusion tensor imaging-based tractography into the stereotactic treatment planning for identification of an object-oriented lead trajectory that allows STC-DBS with multiple electrode contacts. METHODS: Diffusion tensor imaging was performed in 4 patients with thalamic pain. The STC was modeled and integrated into the stereotactic treatment planning for DBS. DBS-lead implantation was done according to trajectory planning along the modeled STC at the level of the PLIC. RESULTS: After implantation, electrode stimulation was possible over a length of more than 20 mm with a tractography-based trajectory along the PLIC part of the STC. After a follow-up of 12 months, pain relief of more than 40% was achieved in 3 of 4 patients with rating on a visual analogue scale. In 1 patient, stimulation failed to reach any long-lasting positive effects. CONCLUSIONS: Integrating tractography data into stereotactic planning of DBS in thalamic pain is technically feasible. It can be used to identify a lead trajectory that allows for multiple contact stimulation along the STC at the level of the PLIC. Due to long-lasting positive stimulation effect, tractography-guided stimulation of sensory fibers seems to be beneficial for thalamic pain relief.

Stroke syndromes and clinical management.

The knowledge of brain syndromes is essential for stroke physicians and neurologists, particularly those that can be extremely difficult and challenging to diagnose due to the great variability of symptom presentation and yet of clinical significance in terms of potential devastating effect with poor outcome. The diagnosis and understanding of stroke syndromes has improved dramatically over the years with the advent of modern imaging, while the management is similar to general care as recommended by various guidelines in addition to care of such patients on specialized units with facilities for continuous monitoring of vital signs and dedicated stroke therapy. Such critical care can be provided either in the acute stroke unit, the medical intensive care unit or the neurological intensive care unit. There may be no definitive treatment at reversing stroke syndromes, but it is important to identify the signs and symptoms for an early diagnosis to prompt quick treatment, which can prevent further devastating complications following stroke. The aim of this article is to discuss some of the important clinical stroke syndromes encountered in clinical practice and discuss their management.

Current differential diagnoses and treatment options of vascular occlusions presenting as bilateral thalamic infarcts: a review of the literature.

bilateral thalamic infarctions are rare and usually caused by vascular occlusions. When symptomatic, it is important to make a distinction between different vascular etiologies in order to provide an effective and timely therapeutic response. Clinical presentations may not adequately differentiate between the vascular etiologies alone. It is therefore important to use imaging technologies to distinguish appropriately the origin of the infarct so that proper treatment can be administered. Advanced imaging techniques, such as CT angiography and MR angiography, have proved useful for distinguishing between arterial and venous causes of bithalamic infarctions. Bilateral thalamic venous infarctions can be treated with anticoagulation medication and with thrombolysis in more severe cases. Bilateral thalamic arterial infarctions may be treated with thrombolysis.