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.

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.

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.

[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.

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.

Improved dexterity after chronic electrical stimulation of the motor cortex for central pain: a special relevance for thalamic syndrome.

OBJECTIVES: To demonstrate that motor cortex stimulation (MCS) could improve motor function in patients with neuropathic pain. METHODS: In this prospective clinical study of 38 patients referred for MCS as treatment for their neuropathic pain, we collected any declaration of improvement in motor performance that could be attributed to MCS. RESULTS: Ten patients (26%) declared a benefit in their motor function. Eight presented objective evidence of recovered dexterity for rapid alternating movements. A minor proportion had improvement in dystonic posture (n = 2), but none had detectable increased motor strength or tonus changes. Overall, 73% of the patients with limb ataxia declared a benefit after MCS. In 6 out of 10 patients (60%), the anatomic lesion responsible for pain was restricted to the lateral aspect of the thalamus. All of them had either clinical or electrophysiological evidence of lemniscal dysfunction (proprioceptive ataxia). No correlation was found between the scores of pain relief and the modification of motor status. The correlation between thalamic lesions and benefits in motor performance was significant (Fisher's exact test, two-tailed, p = 0.0017). CONCLUSIONS: Up to 26% of patients estimated that MCS improved their motor outcome through recovered dexterity and in cases of lateral thalamic lesions.

Moyamoya disease presenting as thalamic hemorrhage in a patient with neuromyelitis optica and Sjögren's syndrome.

A 52-year-old woman was admitted to the hospital with right thalamic hemorrhage. A carotid angiogram revealed occlusion of the terminal portions of the bilateral internal carotid arteries with basal moyamoya vessels, which was diagnosed as moyamoya disease (MMD). At 31 years of age, she was diagnosed with multiple sclerosis because of optic neuritis and myelitis. Paraplegia appeared 14 days after admission. T2-weighted thoracic magnetic resonance imaging revealed a high intensity lesion extending from T4 to T6. Her left upper limb was partially paralytic and her lower limbs exhibited paraplegia and dysesthesia. Anti-aquaporin 4 and anti-Sjögren's syndrome-A and -B antibodies were positive. The pathogenesis of neuromyelitis optica may be associated with such immunologic factors, but there are no reports of simultaneous presentations of neuromyelitis optica and MMD. Autoimmunity may be associated with the etiology of MMD.

Deep brain stimulation for Dejerine-Roussy syndrome: case report.

BACKGROUND: The term "central post-stroke pain" is more appropriate to describe neuropathic pain following a cerebrovascular accident. Most patients complain of burning and other symptoms like lacerating and shooting pain. Treatment options for central pain are limited in number and efficacy. CASE REPORT: This paper reports on a 47-year-old man with central post-stroke pain refractory to treatment. The patient underwent insertion of a deep brain stimulator utilizing the Leksell frame. The target was the left centromedian thalamic nuclei. He had a qualitative symptomatic improvement. CONCLUSION: Deep brain stimulation can be a useful tool when all other modalities have failed. It is a minimally invasive neurosurgical procedure that may improve the quality of life in carefully selected (often desperate) patients with central post-stroke pain.

[Dynamic observation on therapeutic features of thalamic pain by mind calming, blood activating and pain relief acupuncture and carbamazepine].

OBJECTIVE: To observe therapeutic features of thalamic pain by mind Calming, blood activating and pain relief acupuncture and Carbamazepine. METHODS: Crossover trial design was adopted. Eleven patients with confirmed diagnosis of thalamic pain were randomly assigned to two groups according to the minimal unbalance index method, i.e., Group I (Six patients received acupuncture first and then Western medicine.) and Group II (Five patients received Western medicine first and then acupuncture). The therapeutic course for each group was ten days. There was a ten-day elution phase between the two therapeutic methods. The total therapeutic course was thirty days. Eleven patients were enrolled in the two groups for statistical analysis. The therapeutic efficacy was assessed with visual analogue scale (VAS) and the pain assessment scale of Anderson Cancer Center in the USA (MD Pain Evaluation value) respectively. The VAS and MD values of the two groups were recorded every day to get the dynamic curve. RESULTS: The VAS and MD values obviously decreased in the two groups after treatment (P<0.05). The pain curves of the two groups showed a declining trend during the treatment. A gradual and stable descending process was shown in the acupuncture group. But a greater decrease first appeared in the Western medicine group, then a comparatively greater decrease occurred after one platform stage, showing ladder-shaped curve. CONCLUSIONS: Cumulative potency may be the main analgesic effects of acupuncture. Western medicine may possibly play a role by rapid initiate effect.

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.

Glasgow Coma Scale and hematoma volume as criteria for treatment of putaminal and thalamic intracerebral hemorrhage.

BACKGROUND: The decision to administer conservative or surgical treatment for putaminal and thalamic ICH is still a controversial issue. This study was undertaken to examine the decision-making criteria for these 2 treatments. METHODS: In a retrospective study, case records of 400 patients with spontaneous putaminal and thalamic hemorrhage who underwent conservative treatment (n = 201) and surgical treatment (n = 199) over the past 5 years were examined. Conservative treatment included hypertonic solution treatment and hypertension control. Surgical treatments included endoscopic surgery, craniotomy, and stereotactic aspiration. Preoperative GCS score and ICH volume were the major evaluating factors, and comparison of the 30-day mortality rate and 6-month BI score was used for outcome evaluation. RESULTS: In patients with a GCS score of 13 to 15, there was no difference in mortality between conservative and surgical treatments. At a GCS score of 9 to 12 and ICH volume of less than 30 mL, the mortality rate with surgical treatment (10.5%) was lower than that with conservative treatment (20.0%, P < .05). At a GCS score of 3 to 8 and ICH volume of at least 30 mL, surgical treatment was for life saving. Mortality rates were lower for conservative treatment than for surgical treatment when the GCS score was 3 to 12 and ICH volume less than 30 mL. Endoscopic surgery had a better functional outcome compared with craniotomy and stereotactic aspiration when the GCS score was at least 9 (P < .001 and P < .02, respectively). Those in conservative treatment received a better BI score than those in surgical treatment did when the ICH volume was less than 40 mL (P < .001). CONCLUSIONS: Intracerebral hemorrhage volume is probably more important than GCS score in determining treatment. Our nonrandomized data could be interpreted to show that conservative treatment is suggested at GCS score of at least 13 or when ICH volume is less than 30 mL, regardless of GCS score. Surgical treatment could be recommended at GCS score of less than 12 with ICH volume of at least 30 mL for life saving. Endoscopic surgery may improve the functional outcomes because it is less invasive and effectively removes the ICH at GCS score of at least 9.

Can vestibular caloric stimulation be used to treat Dejerine-Roussy Syndrome?

Dejerine-Roussy Syndrome (thalamic pain syndrome) is characterised by the development of chronic, severe pain in the contralateral half of the body after a thalamic stroke. It is often largely refractory to treatment. In this paper we draw together a number of disparate pieces of knowledge to propose a novel therapy for this condition. There is already substantial evidence from neurological disease that the brain's left hemisphere serves to "smooth over" discrepancies in sensory input in order to impose order and maintain the existing view of the world around us. Conversely the right hemisphere acts on discrepant sensory input to cause a re-evaluation of one's world view. Based on this, it was proposed by Harris that pain is an organism's response to discrepancy. It is already known that cold water vestibular caloric irrigation of the ear leads to activation of a number of areas in the contralateral hemisphere - including the insular cortex. Indeed it is known that - presumably because it also activates the right parietal lobe - this technique can be used to treat anosognosia, somatoparaphrenia and neglect. In addition to being activated by vestibular stimulation, it has been shown that the posterior insula has a somatotopic map of the body for painful stimuli. We speculate that phylogenetically, close anatomical proximity between the pain and vestibular areas of the brain makes sense; as it would allow modulation of otherwise disabling chronic pain, when the organism makes a sudden movement to avoid a predator. Given Harris's theory we propose that post stroke thalamic pain may represent a pathological amplification of the thalamic posterior insular response to pain due to discrepant sensory input. Based on all the above we go on to hypothesise that cold vestibular caloric stimulation will be effective in treating Dejerine-Roussy Syndrome and we present provisional evidence from two patients which supports this conclusion. If our hypothesis is correct this will be the first time in clinical neurology that a chronic disorder, long considered refractory to treatment, is relieved by a simple non-invasive procedure.

[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.

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.

[Case of straight sinus venous thrombosis presenting as depression and disorientation due to bilateral thalamic lesions].

A 45-year-old man was admitted to our hospital because of progressive inactivity and mild disturbance of consciousness which appeared two weeks ago. Brain CT revealed symmetric hypointensity of bilateral thalamus, and the lesion appeared hyperintensity on T2 weighted MRI image. He was first considered as immune-mediated cerebritis, and steroid pulse therapy was applied, but the clinical features were not improved. The diagnosis of cerebral venous thrombosis was established, when MR venography (MRV) showed severe stenosis in straight sinus. Consciousness was improved after the start of anticoagulation therapy, but mild dementia was remained as a sequela. MRV was useful to distinguish straight sinus thrombosis from cerebritis in this case.

Unilateral thalamic deep brain stimulation for refractory essential tremor and Parkinson's disease tremor.

OBJECTIVE: To determine the efficacy and tolerability of unilateral thalamic deep brain stimulation (DBS) for patients with medically refractory essential tremor (ET) and the tremor associated with Parkinson's disease (PD). BACKGROUND: The tremor of ET and PD may produce functional disability despite optimal medical therapy. Several reports have demonstrated efficacy of thalamic DBS in this scenario. METHODS: Preoperative and 3-month postoperative tremor ratings were compared in 33 patients (14 ET and 19 PD) with severe tremor. Evaluations included Unified Parkinson's Disease Rating Scale (UPDRS) scores for PD patients and a modified Unified Tremor Rating Scale in ET patients. Open-label and blinded data (unknown activation status) were obtained. RESULTS: ET patients demonstrated an 83% reduction (p < 0.0001) in observed contralateral arm tremor. All measures of tremor including writing samples, pouring tests, subjective functional surveys, and disability scores improved significantly. PD patients demonstrated an 82% reduction (p < 0.0001) in contralateral tremor and significant improvement in disability and global impressions. There was, however, no meaningful improvement in other motor aspects of the disease, and the total UPDRS part II (activities of daily living) score did not change. Adverse events, more common in ET patients, were generally mild and were usually eliminated by adjustment of the device parameters. CONCLUSIONS: Thalamic DBS is a safe and effective treatment of ET and the tremor of PD. In PD, its use should be limited to patients in whom high-amplitude tremor results directly in significant functional disability.

Fetal cortical transplants ameliorate thalamic atrophy ipsilateral to neonatal frontal cortex lesions.

Thalamic atrophy develops ipsilateral to neonatal frontal cortex lesions. Fetal cortex transplants placed in these lesions at birth ameliorate the thalamic atrophy. This could be due to trophic effects of the transplants on the neonatal host thalamus, host cortex, or both.