Sustainable Effects of 8-Year Intermittent Spinal Cord Stimulation in a Patient with Thalamic Post-Stroke Pain.

BACKGROUND: Central post-stroke pain (CPSP) is a central neuropathic pain syndrome secondary to a cerebrovascular accident. CPSP treatment usually begins with medication; however, this is associated with inadequate pain relief and adverse effects. Neurostimulation therapies, including spinal cord stimulation (SCS), have been developed for improved pain relief. We report a patient with thalamic pain who underwent 8-year cervical SCS in an intermittent mode. CASE DESCRIPTION: A 71-year-old man presented with left thalamic stroke that caused persistent allodynia and "pricking" sensations at right-side extremities. The pain did not respond well to several pain therapies, including medication, acupuncture, and nerve-blocking anesthesia. Subsequently, the severe and refractory pain caused dystonia in his right hand and seriously hindered recovery and rehabilitation of stroke sequelae. Further, the pain induced depression and severe anxiety mood status and had an effect on his functional activities of life. After SCS device implantation, the patient received intermittent stimulation with 90 minutes on/30 minutes off. A significant decrease in the patient's pain was observed with no serious side effects. After subtle programming of the implantable pulse generator, a significant improvement of his dystonia and affective mood was observed. Intermittent SCS allowed for persistent stimulation for 8 years. Taken together, this intervention allowed for an acceptable improvement of his functional quality of life. CONCLUSIONS: Our findings indicate that SCS is safe and efficacious for CPSP, including thalamic stroke pain. Long-term intermittent stimulation can preserve implantable pulse generator battery life and achieve sustained improvement of a patient's pain, movement, and affective mood status.

Deep Brain Stimulation and Thalamotomy for the Treatment of Dystonia Acquired by Moyamoya Disease with Stroke.

Background: Moyamoya disease (MMD) is a type of chronic cerebrovascular disease. Currently, revascularization surgery including direct/indirect procedure is recommended for symptomatic patients. However, some patients still respond poorly to the treatment or develop secondary symptoms. Case report: We report the first case of an MMD patient treated with deep brain stimulation (DBS) and thalamotomy. Symptoms of dystonia due to hemorrhage in the thalamus responded poorly to revascularization surgery, but were considerably alleviated by stereotactic neurosurgery. Discussion: Our case report provides a potential strategy for management of refractory symptomatic MMD patients with dystonia and also supports the combined efficacy of DBS with thalamotomies. Highlights: Approximately 30% of patients with Moyamoya disease (MMD) presenting movement symptoms do not respond well to revascularization surgery. We reported an MMD patient treated with deep brain stimulation (DBS) and thalamotomy with significant dystonia and dystonic tremor symptom amelioration. It indicates that DBS or stereotactic lesioning might be a potential treatment for the refractory movement symptoms of MMD.

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.

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.

Variant Type of Posterior Reversible Encephalopathy Syndrome Associated with Deep Brain Hemorrhage: Case Report and Review of the Literature.

BACKGROUND: Various radiologic patterns of posterior reversible encephalopathy syndrome (PRES) have been reported. Among them, PRES involving brainstem, thalamus, or deep white matter and lacking parieto-occipital edema is rare. Although PRES in general has a benign course, PRES-related intracranial hemorrhage has been associated with a poor prognosis. We report a case of variant type of PRES associated with deep brain hemorrhage and discuss the characteristics of PRES-related intracranial hemorrhage via a literature review. CASE DESCRIPTION: A woman aged 41 years with a history of untreated hypertension presented to our hospital complaining of severe headache and with an elevated blood pressure of 237/142 mmHg. Computed tomography revealed a hemorrhage in the left thalamus and basal ganglia. Magnetic resonance imaging revealed remarkable hyperintensity in the left cerebellum, pons, bilateral temporal lobes, bilateral basal ganglia, and bilateral cerebral white matter on fluid-attenuated inversion recovery imaging, which represented vasogenic edema. The parieto-occipital regions were unremarkable. Given this clinical presentation, PRES associated with deep brain hemorrhage was suspected. The patient received strict blood pressure control treatment, which resulted in gradual symptom improvement. Magnetic resonance images obtained 1 month after admission demonstrated an almost complete resolution of the edema. CONCLUSIONS: Although hemorrhage in the thalamus, basal ganglia, or brainstem is uncommon in patients with PRES, it may occur in patients with variant type of PRES involving these lesions. It is important to recognize the presence of variant patterns of clinical features and radiologic findings of PRES to allow for early identification and appropriate treatment.

Delayed-onset central poststroke pain due to degeneration of the spinothalamic tract following thalamic hemorrhage: A case report.

RATIONALE: Recent studies have used diffusion tensor tractography (DTT) to demonstrate that central poststroke pain (CPSP) was related to spinothalamic tract (STT) injury in patients with stroke. However, few studies have been reported about delayed-onset CPSP due to degeneration of the STT following a stroke. PATIENT'S CONCERNS: A 57-year-old female patient presented with right hemiparesis after stroke. Two weeks after onset, she did not report any pain. At approximately 6 months after onset, she reported pain in the right arm and leg, and the pain slowly intensified with the passage of time. At 14 months after onset, the characteristics and severity of her pain were assessed to be continuous pain without allodynia or hyperalgesia; tingling and cold-sensational pain in her right whole arm and leg (visual analog scale score: 5). DIAGNOSES: The patient was diagnosed as the right hemiparesis due to spontaneous thalamic hemorrhage. INTERVENTIONS: Clinical assessment and diffusion tensor imaging (DTI) were performed 2 weeks and 14 months after onset. OUTCOMES: She suffered continuous pain in her right whole arm and leg (visual analog scale score: 5). On DTT of the 2-week postonset DTI scans, the configuration of the STT was well-preserved in both hemispheres. However, in contrast to those 2-week postonset results, the 14-month postonset DTT results showed partial tearing and thinning in the left STT. Regardless, both the 2-week and 14-month postonset DTT showed that the left STT passed through the vicinity of the thalamic lesion. LESSONS: Diagnostic importance of performing a DTT-based evaluation of the STT in patients exhibiting delayed-onset CPSP following intracerebral hemorrhage.

Thalamic Lesions and Aphasia or Neglect.

PURPOSE OF REVIEW: When the thalamus is damaged, not only are there neurological symptoms such as sensory impairment, hemianopia, or motor control disorders, but there are also various neuropsychological symptoms. We discuss the basic anatomy and function of the thalamus followed by a discussion of thalamic aphasia and hemineglect. RECENT FINDINGS: Subcortical lesions in patients with hemineglect involved the anatomical network directly linked to the superior temporal gyrus. Furthermore, the pulvinar, part of the thalamus, may be associated with hemineglect. General linguistic tasks activated the thalami, depending on the difficulty, as well as the frontal and temporal lobes. The thalamus may play a role in language and be involved in the activation of cortical language areas, and in the linguistic integration function via verbal memory and semantic mechanisms. Thalamic lesions are associated with aphasia and hemineglect. It is possible the mechanisms and such neuropsychological may be different depending on the size and site of the thalamic lesion.

Persistent anterograde amnesia due to the artery of Percheron occlusion: a case report.

Bilateral thalamic infarction involving the artery of Percheron (AOP) can cause diagnostic difficulties due to the varying clinical presentations. AOP infarcts presented with isolated memory impairment are not common and the factors affecting the persistence of memory disorders are still unknown. A 41-year-old male patient was hospitalized with acute unconsciousness. MRI disclosed bilateral paramedian thalamic infarction The patient had isolated memory deficit and his anterograde amnesia continued without any change in the past decade. More cases might answer the questions concerning the intra- and extra-thalamic structures responsible for the amnesic syndrome and the factors affecting the persistence of the symptoms.

BILATERAL CENTRAL RETINAL VEIN OCCLUSIONS IN A YOUNG PATIENT WITH A HISTORY OF EOSINOPHILIC PNEUMONIA AND THALAMIC STROKE.

PURPOSE: To describe a case of a central retinal vein occlusion in a young patient with a history of eosinophilic pneumonia. METHODS: A retrospective case report of a 45-year-old woman with acute painless vision loss for 9 days after multiple episodes of eosinophilic pneumonia and thalamic stroke. Fluorescein angiography, spectral domain optical coherence tomography, and clinical examination were performed. She was then treated with intravitreal bevacizumab and pan-retinal photocoagulations. RESULTS: Retinal examination revealed tortuosity and dilatation of all branches of the central retinal vein and flame-shaped hemorrhages in all four quadrants of the right eye associated with cystoid macular edema, optic disc edema, and cotton wool spots. The left eye had mild venous dilatation and tortuosity with a few dot retinal hemorrhages in the far temporal periphery. The cystoid macular edema resolved after one intravitreal injection of bevacizumab and remained resolved at the most recent follow-up. Fluorescein angiography at the most recent follow-up revealed vasculitis in the far periphery of the nontreated eye. CONCLUSION: Central retinal vein occlusion in young patients is a rare condition often presenting as a manifestation of an underlying inflammatory or hematological disorder. Combined anti-vascular endothelial growth factor treatment and pan-retinal photocoagulation may have resolved the associated cystoid macular edema in this case, although continued observation is necessary.

Degeneration of paramedian nuclei in the thalamus induces Holmes tremor in a case of artery of Percheron infarction.

RATIONALE: Holmes' tremor is an uncommon neurologic disorder following brain insults, and its pathogenesis is undefined. The interruption of the dento-rubro-thalamic tract and secondary deterioration of the nigrostriatal pathway are both required to initiate Holmes' tremor. We used nuclear medicine imaging tools to analyze a patient with concurrent infarction in different zones of each side of the thalamus. Finding whether the paramedian nuclear groups of the thalamus were injured was a decisive element for developing Holmes' tremor. PATIENT CONCERNS: A 36-year-old woman was admitted to our department due to a bilateral paramedian thalamic infarction. Seven months after the stroke, a unilaterally involuntary trembling with irregularly wavering motions occurring in both her left hand and forearm. DIAGNOSIS: Based on the distinct features of the unilateral coarse tremor and the locations of the lesions on the magnetic resonance imaging (MRI), the patient was diagnosed with bilateral paramedian thalamic infarction complicated with a unilateral Holmes' tremor. INTERVENTIONS: The patient refused our recommendation of pharmacological treatment with levodopa and other dopamine agonists based on personal reasons and was only willing to accept physical and occupational training programs at our outpatient clinic. OUTCOMES: We utilized serial anatomic and functional neuroimaging of the brain to survey the neurologic deficit. A brain magnetic resonance imaging showed unequal recovery on each side of the thalamus. The residual lesion appeared larger in the right-side thalamus and had gathered in the paramedian area. A brain perfusion single-photon emission computed tomography (SPECT) revealed that the post-stroke hypometabolic changes were not only in the right-side thalamus but also in the right basal ganglion, which was anatomically intact. Furthermore, the brain Technetium-99m-labeled tropanes as a dopamine transporter imaging agents scan ( Tc-TRODAT-1) displayed a secondary reduction of dopamine transporters in the right nigrostriatal pathway which had resulted from the damage on the paramedian nuclear groups of the right-side thalamus. LESSONS: Based on the functional images, we illustrated that a retrograde degeneration originating from the thalamic paramedian nuclear groups, and extending forward along the direct innervating fibers of the mesothalamic pathway, played an essential role towards initiating Holmes' tremor.

Mirror therapy for involuntary movement due to chronic thalamic hemorrhage: a case report.

BACKGROUND: Some stroke survivors suffer from involuntary movement, which often disturbs upper-limb function, but there are few effective modality options. CASE REPORT: A 70-year-old man presented with persisting right-upper-limb hemiballism due to left thalamic hemorrhage ten years before. We provided inpatient rehabilitation, including repetitive-facilitative exercise and task-related training, and prescribed clonazepam from day 5 after admission. However, the affected upper limb showed no significant change and remained nonfunctional. When we introduced mirror therapy (MT) to the rehabilitation program on day 14, hemiballism immediately decreased during the MT session, so we added daily MT sessions of 20 min/day. At discharge on day 42, he was able to use his right hand for eating meals and folding laundry. CLINICAL REHABILITATION IMPACT: This shows that MT may have the potential to be a novel therapeutic method for treating involuntary movement. Further research is needed to elucidate mechanisms by which MT may reduce hemiballism.

Third Ventricular Cerebrospinal Fluid Cysts of Thalamic Origin: Review of Embryologic Origin, Presentation, and Management Strategies with a Case Series.

OBJECTIVE: Third ventricular cerebrospinal fluid (CSF) cysts of thalamic origin are rare. The objective of this study is to review their possible pathogenesis, clinical presentation, and management strategies with a case series describing management via an endoscopic approach with fenestration using a single burr-hole technique. METHODS: A systematic literature review of reported cases of thalamic cysts was conducted with further meta-analysis of CSF cysts that involve the third ventricle. The mode of presentation, pathologic analysis, surgical management, and outcomes were analyzed. RESULTS: Twenty-two studies reported between 1990 and 2013 described 42 cases of thalamic cyst. Of those cases, 13 were consistent with CSF cyst that originated in the thalamus and involved the third ventricle. Eight cases (61.5%) were treated via endoscopic fenestration, 2 cases (15.4%) were surgically drained, 2 cases (15.4%) were stereotactically aspirated, and 1 case (7.69%) was observed. The most common presenting symptoms were gait disturbance (26.3%) and headaches (26.3%) followed by tremors (15.8%) and weakness (15.8%). In our series, a single burr-hole technique was a successful definitive treatment, with an average period of 23 months. CONCLUSIONS: Third ventricular CSF cysts of thalamic origin most commonly present with hydrocephalus. They can be safely definitively treated via endoscopic fenestration to the CSF circulation using a single burr-hole technique. Long-term follow-up shows lasting improvement in symptoms without reaccumulation of the cyst.