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.

[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.

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.

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.

[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.

[Essentials of the treatment based on syndrome differentiation belonging to the meridian theory by professor Wang Ju-yi].

This paper states the essentials of the treatment based on syndrome differentiation practiced by professor WANG Ju-yi's and his experience in clinic. The understanding of basic theory is a key for clinical practices using meridian theory. Based on the understanding of mutual transformations among six meridians, six qi, six zang, and six fu, the theory of meridians and collaterals can be applied to daily acupuncture clinical practices. Doctors should follow the processes of observing meridian, inspection of meridian, selection meridian, selection acupoints in the clinical practices. This paper lists the clinical examples of treating patients with thalamic lesion by using the method of diagnosis and examination practiced by professor WANG Ju-yi's.

Increased regional cerebral blood flow in the contralateral thalamus after successful motor cortex stimulation in a patient with poststroke pain.

The mechanisms underlying poststroke pain have not been clearly identified. Although motor cortex stimulation (MCS) sometimes reduces poststroke pain successfully, the exact mechanism is not yet known. For further investigation of the neural pathways involved in the processing of poststroke pain and in pain reduction by MCS, the authors used positron emission tomography (PET) scanning to determine significant changes in regional cerebral blood flow (rCBF). This 58-year-old right-handed man suffered from right-sided poststroke pain for which he underwent implantation of a stimulation electrode in the right motor cortex. After 30 minutes of stimulation, his pain was remarkably reduced (Visual Analog Scale scores decreased 8 to 1) and he felt warmth in his left arm. The rCBF was studied using PET scanning with 15O-labeled water when the patient was in the following states: before MCS (painful condition, no stimulation) and after successful MCS (painless condition, no stimulation). The images were analyzed using statistical parametric mapping software. State-dependent differences in global blood flow were covaried using analysis of covariance. Comparisons of the patient's rCBF in the painful condition with that in the painless condition revealed significant rCBF increases in the left rectus gyrus (BA11), left superior frontal lobe (BA9), left anterior cingulate gyms (BA32), and the left thalamus (p < 0.05, corrected). On the other hand, there were significant decreases in rCBF in the right superior temporal gyrus (BA22, p < 0.01, corrected) and the left middle occipital gyrus (BA19, p < 0.05, corrected). The efficacy of MCS was mainly related to increased synaptic activity in the thalamus, whereas the activations in the rectus gyrus, anterior cingulate gyrus, and superior frontal cortex as well as the inactivation of the superior temporal lobe may be related to emotional processes. This is the first report in which the contralateral thalamus was significantly activated and pain relief was achieved using MCS.

Neurogenic pain relief by repetitive transcranial magnetic cortical stimulation depends on the origin and the site of pain.

OBJECTIVE: Drug resistant neurogenic pain can be relieved by repetitive transcranial magnetic stimulation (rTMS) of the motor cortex. This study was designed to assess the influence of pain origin, pain site, and sensory loss on rTMS efficacy. PATIENTS AND METHODS: Sixty right handed patients were included, suffering from intractable pain secondary to one of the following types of lesion: thalamic stroke, brainstem stroke, spinal cord lesion, brachial plexus lesion, or trigeminal nerve lesion. The pain predominated unilaterally in the face, the upper limb, or the lower limb. The thermal sensory thresholds were measured within the painful zone and were found to be highly or moderately elevated. Finally, the pain level was scored on a visual analogue scale before and after a 20 minute session of "real" or "sham" 10 Hz rTMS over the side of the motor cortex corresponding to the hand on the painful side, even if the pain was not experienced in the hand itself. RESULTS: and discussion: The percentage pain reduction was significantly greater following real than sham rTMS (-22.9% v -7.8%, p = 0.0002), confirming that motor cortex rTMS was able to induce antalgic effects. These effects were significantly influenced by the origin and the site of pain. For pain origin, results were worse in patients with brainstem stroke, whatever the site of pain. This was consistent with a descending modulation within the brainstem, triggered by the motor corticothalamic output. For pain site, better results were obtained for facial pain, although stimulation was targeted on the hand cortical area. Thus, in contrast to implanted stimulation, the target for rTMS procedure in pain control may not be the area corresponding to the painful zone but an adjacent one. Across representation plasticity of cortical areas resulting from deafferentation could explain this discrepancy. Finally, the degree of sensory loss did not interfere with pain origin or pain site regarding rTMS effects. CONCLUSION: Motor cortex rTMS was found to result in a significant but transient relief of chronic pain, influenced by pain origin and pain site. These parameters should be taken into account in any further study of rTMS application in chronic pain control.

Reversal of thalamic hand syndrome by long-term motor cortex stimulation.

The authors describe a case of complete recovery from the so-called "thalamic hand" syndrome following chronic motor cortex stimulation in a 64-year-old man suffering from poststroke thalamic central pain. As of the 2-year follow-up examination, the patient's dystonia and pain are still controlled by electrical stimulation. It is speculated that a common mechanism in which the thalamocortical circuit loops are rendered out of balance may sustain hand dystonia and central pain in this case of thalamic syndrome. To the authors' knowledge this is the first reported case of its kind.

The clinical efficacy of neuroendoscope in surgical treatment for deafferentation pain.

Spinal cord stimulation (SCS) is one of the most minimally invasive and effective treatments for intractable pain. We report the efficacy of a very small diameter neuroendoscope on setting the electrode to the proper site in the epidural space. Our cases include thalamic hemorrhage, and each patient had unilateral intractable pain on L1 or less as the main complaint. They had been treated for over two years in other hospitals, but no significant relief was achieved. Because each patient had been given frequent epidural blocks, the adhesion in the epidural space was expected. In Group A (3 cases), we used very small diameter neuroendoscope to dissect adhesion in the epidural space and to make optimal space for lead placement under direct vision. Conventional lead placement under fluoroscopy was performed in Group B (3 cases). Medtronic's PISCES lead system was used for SCS. In Group A, stimulation and pain regions matched in all cases, and good pain relief was also achieved. In Group B, however, stimulation and pain regions matched incompletely and the increase in stimulation caused stimulation on the pain-free side.

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.

Treatment of deafferentation pain by chronic stimulation of the motor cortex: report of a series of 20 cases.

Twenty patients with deafferentation pain were treated by chronic stimulation of the motor cortex. The central fissure was localized using stereotactic MRI and the motor cortex was mapped using intra-operative somatosensory evoked potentials. Seven patients with trigeminal neuropathic pain experienced definite pain relief varying between 40 and 100%. Ten patients had central pain secondary to central nervous system lesions. A satisfactory long-lasting pain control (pain relief > 40%) was obtained in five of them (50% of cases). One patient with pain from peripheral nerve injury obtained more than 80% pain relief. Two patients had pain from spinal cord lesions. One did not respond but the other obtained an excellent long-term result. The location of the effective stimulation plots was in agreement with the somatotopic maps of the primary motor cortex. One patient developed a small extradural haematoma which resolved spontaneously. None of the patients developed seizure activity. This study confirms the potential value of motor cortex stimulation in the treatment of certain forms of intractable pain, especially in cases with trigeminal neuropathic pain.

Familial neurofibromatosis 1 with germinoma involving the basal ganglion and thalamus.

Intracranial germinoma associated with neurofibromatosis 1 (NF-1) has never been documented previously. We report a case of familial NF-1 with a germinoma involving the right basal ganglion and thalamus. A 12-year-old boy presented with multiple café-au-lait spots and a family history of neurofibromatosis in his mother, one of two siblings, and his maternal grandfather. His intracranial lesion was subtotally resected. Histologically, it was a pure germinoma. Serum alpha-feto protein and beta-human chorionic gonadotropin levels were within the normal range. Postoperative myelographic examination and cerebrospinal fluid cytology study showed no evidence of subarachnoid seeding. The patient received postoperative combination chemotherapy resulting in complete response and clearance of the residual tumor. Although this finding of an intracranial germinoma in a patient with familial NF-1 may be coincident, it is suggestive of a potential genetic predisposition. Longitudinal evaluation for the possibility of neoplasm, especially germ cell tumor, in basal ganglion lesions in NF-1 patients is necessary.

A case of symptomatic hemidystonia improved by ventroposterolateral thalamic electrostimulation.

A 16-year-old boy presented with a left anterothalamic lesion secondary to an extradural hematoma. A few months later, a right hemidystonia developed. The dystonic posture, which predominated in the right hand, was not associated with any motor deficit or sensory loss. Superficial sensory stimulation of the right palm and forearm and proprioceptive stimuli induced by passive movements of the right thumb decreased the dystonic posture. Electrical stimulation of the left ventroposterolateral nucleus of the thalamus induced a dramatic improvement in the dystonic postures and movement of the upper right limb. This finding suggests that the role of tactile and proprioceptive stimulation should be analyzed in patients presenting with a symptomatic hemidystonia. When such sensory stimulation is effective, thalamic stimulation may be tried in patients whose condition is incapacitating.

Effects of thalamic stimulation on tremor, balance, and step initiation: a single subject study.

This study was conducted to evaluate the clinically apparent balance improvements in a patient with Parkinson's disease who had stimulating electrodes surgically implanted to the VIM nucleus of the right thalamus for control of left-upper-extremity tremor. Experiments were conducted to determine if balance improved simply because the large-amplitude upper-extremity tremor was reduced or if the neural control of balance improved. Using EMGs and forceplate recordings, we quantified the effects of the thalamic stimulation on the contralateral upper-extremity tremor and on the lower-extremity postural muscle activations for quiet stance, step initiation, and equilibrium responses to surface displacements. The results demonstrated that, beside reducing the amplitude and destabilizing effects of the upper-extremity tremor, the thalamic stimulation was also effective in reducing tremor activity of the trunk and contralateral lower-extremity muscles. In addition, the contralateral lower-extremity muscle activation patterns, strengths, and durations for the balance tasks were enhanced during stimulation. These results suggest that thalamic stimulation improved this patient's balance by reducing tremor in the contralateral extremities and by increasing burst duration and magnitude of the tibialis anterior, which functions as the postural prime mover for the step initiation and balance tasks.

Los síndromes de lesión talámica

En la unidad de Neurología del Centro Hospitalario San Juan de Dios de Bogotá, durante cuatro años (1986 a 1989), se estudiaron en forma consecutiva 25 pacientes con lesiones talámaticas no fatales. Se registraron los hallazgos neurológicos, neurosicológicos y neurooftalmológicos y los diagnósticos se confirmaron por tomografía computarizada (TC). Fueron 14 mujeres y 11 varones con una edad promedio de 52.5 y un rango de 25 a 84 años. La lesión talámica fue de origen vascular en 24 casos, ocho por infarto isquémico, cuatro por infarto hemorrágico y 12 con hematomas parenquimatosos. Diecisiete pacientes tenían hipertensión arterial sistémica y el único factor de riesgo en otros dos era el consumo de cocaína base (basuco). Ocho infartos se presentaron en el tálamo derecho, 12 en el izquierdo y cinco pacientes tuvieron lesiones bilaterales, uno de ellos con un glioma complobado por biopsia. En 5 pacientes con lesiónes bilateral se observó el síndrome del "Tope" de la arteria basilar, por compromiso del pedículo retromamilar; en todos ellos encontramos alteraciones sensitivomotoras, cerebelosas, oculomotoras bilaterales y demencia. Solamente un paciente presentó el clasicó síndrome de hiperpatía (Dejerine-Roussy). En los restantes se observaron asociaciones de síndromes sensitivomotores, cerebelosos, neurooftalmológicos, neuropsicológicos, y del comportamiento motor que remedan con frecuencia los hallazgos clínicos de la alteración cortical frontal, temporal o parietal.

Treatment of thalamic pain by chronic motor cortex stimulation.

All forms of therapy, including chronic stimulation of the thalamic relay nucleus, can provide satisfactory pain control in only 20%-30% of cases of thalamic pain syndrome. In order to develop a more effective treatment for thalamic pain syndrome, we investigated the effects of stimulation of various brain regions on the burst hyperactivity of thalamic neurons recorded in cats after deafferentiation of the spinothalamic pathway. Complete, long-term inhibition of the burst hyperactivity was induced by stimulation of the motor cortex. Based on this experimental finding, we treated seven cases of thalamic pain syndrome by chronic motor cortex stimulation employing epidural plate electrodes. Excellent or good pain control was obtained in all cases without any complications or side effects. During the stimulation, an increase in regional blood flow of the cerebral cortex and thalamus, a marked rise in temperature of the painful skin regions, and improved movements of the painful limbs were observed. These results suggest that thalamic pain syndrome can be most effectively treated by chronic motor cortex stimulation.

Chronic motor cortex stimulation for the treatment of central pain.

Twelve patients with deafferentation pain secondary to central nervous system lesions were subjected to chronic motor cortex stimulation. The motor cortex was mapped as carefully as possible and the electrode was placed in the region where muscle twitch of painful area can be observed with the lowest threshold. 5 of the 12 patients reported complete absence of previous pain with intermittent stimulation at 1 year following the initiation of this therapy. Improvements in hemiparesis was also observed in most of these patients. The pain of these patients was typically barbiturate-sensitive and morphine-resistant. Another 3 patients had some degree of residual pain but considerable reduction of pain was still obtained by stimulation. Thus, 8 of the 12 patients (67%) had continued effect of this therapy after 1 year. In 3 patients, revisions of the electrode placement were needed because stimulation became incapable of inducing muscle twitch even with higher stimulation intensity. The effect of stimulation on pain and capability of producing muscle twitch disappeared simultaneously in these cases and the effect reappeared after the revisions, indicating that appropriate stimulation of the motor cortex is definitely necessary for obtaining satisfactory pain control in these patients. None of the patients subjected to this therapy developed neither observable nor electroencephalographic seizure activity.