Variety Of Movement Disorders Research Articles

Correlation between neuroimaging, neurological phenotype, and functional outcomes in Wilson's disease.

Wilson's disease (WD) is associated with a variety of movement disorders and progressive neurological dysfunction. The aim of this study was to correlate baseline brain magnetic resonance imaging (MRI) features with clinical phenotype and long-term outcomes in chronically treated WD patients. Patients were retrospectively selected from an institutional database. Two experienced neuroradiologists reviewed baseline brain MRI. Functional assessment was performed using the World Health Organization Disability Assessment Schedule 2.0 (WHODAS 2.0) scale, and disease severity was classified using the Global Assessment Scale for Wilson's Disease (GASWD). Of 27 patients selected, 14 were female (51.9%), with a mean (standard deviation [SD]) age at onset of 19.5 (7.1) years. Neurological symptoms developed in 22 patients (81.5%), with hyperkinetic symptoms being the most common (70.4%). Baseline brain MRI showed abnormal findings in 18 cases (66.7%), including T2 hyperintensities in 59.3% and atrophy in 29.6%. After a mean (SD) follow-up of 20.9 (11.0) years, WD patients had a mean score of 19.2 (10.2) on WHODAS 2.0 and 6.4 (5.7) on GASWD. The presence of hyperkinetic symptoms correlated with putaminal T2 hyperintensities (p = 0.003), putaminal T2 hypointensities (p = 0.009), and mesencephalic T2 hyperintensities (p = 0.009). Increased functional disability was associated with brain atrophy (p = 0.007), diffusion abnormalities (p = 0.013), and burden of T2 hyperintensities (p = 0.002). A stepwise regression model identified atrophy as a predictor of increased WHODAS 2.0 (p = 0.023) and GASWD (p = 0.007) scores. Atrophy and, to a lesser extent, deep T2 hyperintensity are associated with functional disability and disease severity in long-term follow-up of WD patients.

Dynamic Activity Model of Movement Disorders: The Fundamental Role of the Hyperdirect Pathway.

Schematic illustration of cortically induced dynamic activity changes of the output nuclei of the basal ganglia (the internal segment of the globus pallidus, GPi and the substantia nigra pars reticulata, SNr) in the healthy and diseased states. The height of the dam along the time course controls the expression of voluntary movements. Its alterations could cause a variety of movement disorders, such as Parkinson's disease and hyperkinetic disorders. © 2023 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.

The intralaminar thalamus: a review of its role as a target in functional neurosurgery.

The intralaminar thalamus, in particular the centromedian-parafascicular complex, forms a strategic node between ascending information from the spinal cord and brainstem and forebrain circuitry that involves the cerebral cortex and basal ganglia. A large body of evidence shows that this functionally heterogeneous region regulates information transmission in different cortical circuits, and is involved in a variety of functions, including cognition, arousal, consciousness and processing of pain signals. Not surprisingly, the intralaminar thalamus has been a target area for (radio)surgical ablation and deep brain stimulation (DBS) in different neurological and psychiatric disorders. Historically, ablation and stimulation of the intralaminar thalamus have been explored in patients with pain, epilepsy and Tourette syndrome. Moreover, DBS has been used as an experimental treatment for disorders of consciousness and a variety of movement disorders. In this review, we provide a comprehensive analysis of the underlying mechanisms of stimulation and ablation of the intralaminar nuclei, historical clinical evidence, and more recent (experimental) studies in animals and humans to define the present and future role of the intralaminar thalamus as a target in the treatment of neurological and psychiatric disorders.

Clinical spectrum and management of dystonia in patients with Japanese encephalitis: A systematic review.

BackgroundJapanese encephalitis (JE) is a potentially fatal viral infection with a wide range of manifestations and can also present with a variety of movement disorders (MD) including dystonia. Dystonic features in JE are uncommon. Here, we have tried to summarize the clinical features and management of dystonia among JE patients with a comprehensive literature search.MethodsVarious databases, including PubMed, Embase, and Google Scholar, were searched against the predefined criteria using suitable keywords combination and boolean operations. Relevant information from observational and case studies was extracted according to the author, dystonic features, radiological changes in the brain scans, treatment options, and outcome wherever provided.ResultWe identified 19 studies with a total of 1547 JE patients, the diagnosis of which was confirmed by IgM detection in serum and/or cerebrospinal fluid in the majority of the patients (88.62%). 234 (15.13%) of JE patients had dystonia with several types of focal dystonia being present in 131 (55.98%) either alone or in combination. Neuroimaging showed predominant involvement of thalami, basal ganglia, and brainstem. Oral medications including anticholinergics, GABA agonists, and benzodiazepines followed by botulinum toxin were the most common treatment modalities.ConclusionDystonia can be a disabling consequence of JE, and various available medical therapies can significantly improve the quality of life. Owing to insufficient studies on the assessment of dystonia associated with JE, longitudinal studies with a larger number of patients are warranted to further clarify the clinical course, treatment, and outcome of dystonia.

Movement Disorders Associated with Hypogonadism.

A variety of movement disorders can be associated with hypogonadism. Identification of this association may aid in guiding workup and reaching an accurate diagnosis. We conducted a comprehensive and structured search to identify the most common movement disorders associated with hypogonadism. Only Case Reports and Case Series articles were included. Ataxia was the most common movement disorder associated with hypogonadism, including entities such as Gordon-Holmes syndrome, Boucher-Neuhäuser, Marinesco-Sjögren and Perrault syndrome. Tremor was also commonly described, particularly with aneuploidies such as Klinefelter syndrome and Jacob's syndrome. Other rare conditions including mitochondrial disorders and Woodhouse-Sakati syndrome are associated with dystonia and parkinsonism and either hypo or hypergonadotropic hypogonadism. We also highlight those entities where a combination of movement disorders is present. Hypogonadism may be more commonly associated with movement disorders than previously appreciated. It is important for the clinician to be aware of this association, as well as accompanying symptoms in order to reach a precise diagnosis.

The effect of voluntary movement with non‐dominant finger on the delay time of involuntary movement in a patient of mirror movement disorder

Mirror movement disorder is a condition in which intentional movements of one side of the body are mirrored by involuntary movements of the other side. This symptom is mainly observed in the distal upper limbs. Mirror movement disorder is present in patients with a variety of movement disorders, such as dystonia, Parkinson's disease, and essential tremor as well as in congenital nervous system disorders. Mirror movement disorder is also observed in healthy subjects even in several members of the same family with no other neurological signs (Congenital mirror movement disorder). Although it is known that mutations in the DCC or RAD51 gene is involved in the congenital mirror movement disorder, it is still far from the understanding of the mechanism in congenital mirror movement disorder since its prevalence is thought to be less than 1 in 1 million. In this study, we found the healthy child and her father who had mirror movement. We report the quantitative analysis of the mirror movement using videotape recording. We obtained informed consent for the videotape recording and the experimental procedures from all subjects. In the subject of the child, we recorded the movement of both index fingers during pressing the button of the picture book by right index finger. The movement of the left index finger was observed 33 ms after pressing, and the maximum speed of the movement was comparable. In the subject of her father, we recorded the movement of both fingers during we asked him to move right or left one finger. The delay time between right (dominant) and left (non‐dominant) fingers was 24 ± 8 ms when we asked him to move the dominant finger. However, this value was significantly increased when the non‐dominant fingers were asked to move (133 ± 47 ms). In conclusion, child and her father had same symptoms; the involuntary movement was observed after voluntary movement with delay time. Increased delay time between voluntary and involuntary movement during the non‐dominant fingers were asked to move might be due to use‐dependent plasticity. This should be studied in the future study with anatomical/physiological examination of the brain and comprehensive inspection of genes.This abstract is from the Experimental Biology 2019 Meeting. There is no full text article associated with this abstract published in The FASEB Journal.

Botulinum toxin in movement disorders.

Botulinum toxin has gained immense popularity since its introduction for therapeutic use. It is used in a variety of movement disorders like hemi-facial spasm, focal dystonias like blepharospasm, cervical dystonia, oromandibular dystonia, limb dystonias. It is also being used in patients with tremors, tics and for a variety of indications in Parkinson's disease as well. There are eight subtypes of toxins available, but type A and B are the ones used in movement disorder clinics. The toxin mainly acts by inhibiting the release of acetylcholine at the neuromuscular junction and causing weakness. Type B toxin has more effect over the autonomic nervous system and hence is preferred for hyper-secretory disorders. The use of electromyography and ultrasound further improve the accuracy of the procedure. It is a relatively safe therapeutic option with its effect lasting for around three months. It has very few side effects. The key is to start with the lowest possible dose and then gradually increase the dose depending upon the patient's response. Selecting the right muscles for injection is of utmost importance and is guided by the knowledge of anatomy of the muscles.

EP 31. Deep brain stimulation-induced neurogenesis is gender-independent

Background Deep brain stimulation (DBS) provides substantial clinical benefits for a variety of movement disorders and lately emerged as a potential treatment for cognitive and mood disorders. Regulation of adult hippocampal neurogenesis may play a chief role in mediating DBS effects. Nevertheless, there exist significant sex differences in the regulation of neurogenesis influenced mainly by gonadal hormone exposure. Objective To investigate the hippocampal neurogenic differences between male and female awake and unrestrained rats in response to unilateral anteromedial thalamic nucleus (AMN) stimulation. Methods Four groups of adult Sprague-Dawley male and female rats received unilateral stimulation ( n = 6 each) or sham surgery of electrode implantation with no current delivery ( n = 4 each) in the right AMN; A naive group of males and females ( n = 4 each) was also included. Rats received 4 injections (50 mg/Kg/injection) of 5′-bromo-2′-deoxyuridine (BrdU) 3 days post-surgery and were euthanized 24h later. The fractionator method was used together with confocal immunofluorescent analysis to probe for BrdU-, GFAP- and NeuN-positive cells in the dentate gyrus (DG). Results Focal neurogenesis was induced in the ipsilateral DG after AMN stimulation. Stimulation-induced effects were gender-independent and translated into a 76% increase in proliferation of neural stem/progenitor cells. Conclusions Despite the difference in basal neurogenic level between male and female rats, the current study demonstrates that DBS elicits gender-independent neurogenic effects that might have implications in the treatment of cognitive and behavioral disorders irrespective of sex of patients.

Probing the interaction of the ipsilateral posterior parietal cortex with the premotor cortex using a novel transcranial magnetic stimulation technique

ObjectiveFunctional imaging studies have shown that control of planned movement involves a distributed network that involves the premotor (PMv) and posterior parietal cortices (PPC). Similarly, anatomical studies show that these regions are densely interconnected via white matter tracts. We therefore hypothesized that the PPC influence over the motor cortex is partly via a connection with the PMv. MethodsUsing a novel three-pulse ipsilateral transcranial magnetic stimulation technique, we preconditioned the PPC (80% RMT) at ISIs from 4–15ms prior to stimulating the PMv and M1 at ISIs of 4 and 6ms. ResultsAs previously shown, PMv–M1 paired-pulse stimulation resulted in inhibition of the MEP (90% RMT, 4–6ms) and PPC–M1 paired-pulse stimulation resulted in facilitation of the MEP (90% RMT, 4–8ms). PPC–M1 paired-pulse stimulation at 80% RMT preconditioning had no effect on M1. PPC–PMv–M1 stimulation resulted in reversal of inhibition observed with PMv–M1 stimulation at ISIs ranging from 6 to 15ms. ConclusionsThe reversal of inhibition observed with PPC–PMv–M1 stimulation suggests that the parietal connection to the PMv plays a role in the modulation of M1. SignificanceThis is the first study to stimulate three intrahemispheric regions in order to test a disynaptic connection with M1. The described network may be important in a variety of movement disorders.

Efficacy of botulinum toxin type A in treatment of different forms of focal dystonias in the Serbian population: experience of the Botulinum Toxin Outpatients Department.

Botulinum toxin (BTX) irreversibly inhibits presynaptic acetylcholine release with subsequent relaxation of abnormally contracting muscles. It is an effective and well tolerated treatment with long-term benefit in a variety of movement disorders and other neurological and non-neurological disturbances. The aim of our study was to present our experience with BTX type A in treatment of different forms of focal dystonias. A hundred of patients with different focal dystonias (spastic torticollis, blepharospasm and graphospasm) from the Botulinum Toxin Outpatients Department, Clinic for Neurology, Clinical Center of Serbia, were included in the study. All the patients were examined and rated at baseline visit prior to BTX application and on the following visit, after 3-4 months, using self-assessment improvement questionnaire and standardized rating scales. . The improvement of 50% was presented in 68.2% of all (199) the analyzed applications. Independent predictors of good response to the therapy (improvement ≥ 50%) were male sex (p = 0.011), the presence of sensory trick (p = 0.013) and the total number of BTX applications (p = 0.002). The patients with spastic torticollis and blepharospasm showed a statistically significantly better BTX effect (improvement 57.3 ± 27.5% and 54.1 ± 28.3%), respectively than the graphospasm group (26.7 ± 25.6%). Most of the patients did not have therapy complications (81.4% and 72% in two applications). Side effects in the remaining patients (muscle weakness, dysphagia, ptosis, double vision, neck weakness and lacrimal dysfunction) lasted for 28.3 ± 18.6 days after the first treatment and 32.5 ± 36.2 days after the second one. BTX is safe and highly effective in long-term treatment of patients with different forms of focal dystonia, with only mild and well-tolerated side-effects.

Long-Term Efficacy and Safety of Botulinum Toxin Injections in Dystonia

Local chemodenervation with botulinum toxin (BoNT) injections to relax abnormally contracting muscles has been shown to be an effective and well-tolerated treatment in a variety of movement disorders and other neurological and non-neurological disorders. Despite almost 30 years of therapeutic use, there are only few studies of patients treated with BoNT injections over long period of time. These published data clearly support the conclusion that BoNT not only provides safe and effective symptomatic relief of dystonia but also long-term benefit and possibly even favorably modifying the natural history of this disease. The adverse events associated with chronic, periodic exposure to BoNT injections are generally minor and self-limiting. With the chronic use of BoNT and an expanding list of therapeutic indications, there is a need to carefully examine the existing data on the long-term efficacy and safety of BoNT. In this review we will highlight some of the aspects of long-term effects of BoNT, including efficacy, safety, and immunogenicity.

Successful Treatment of Severe Bruxism with OnabotulinumtoxinA in Patients with Post Anoxic Brain Injury (P01.237)

Objective: Intramuscularly administered OnabotulinumtoxinA is an effective treatment for a variety of movement disorders. Severe bruxism, which can generate discomfort, pain, tongue laceration and tooth fragmentation, is a frequent complication of anoxic brain injury. Severe bruxism is usually amenable to medical treatment. We report successful symptomatic treatment of severe bruxism with OnabotulinumtoxinA in four patients with anoxic brain injury. Background Four patients with severe bruxism secondary to post anoxic brain injury were seen on the inpatient and/or outpatient service at Henry Ford Health System between November 2003 and August 2011 and treated with OnabotulinumtoxinA. Design/Methods: All four patients were females, three were African American and one Caucasian. Ages ranged from twenty three to sixty three years old. All four patients received OnabotulinumtoxinA injection to the bilateral masseter and/or temoporalis muscles (total of 50-100units were injected during each visit). Two of the four patients received multiple injections separated by two weeks to 3 months due to severity of the symptoms. Results: All four patients responded positively to the injections. No side effects were reported in any of the studied patients. Conclusions: OnabotulinumtoxinA injections are an efficacious and valuable treatment for patients with severe bruxism post anoxic brain injury. Disclosure: Dr. Murshed has nothing to disclose. Dr. Dakka has nothing to disclose. Dr. Angus has nothing to disclose.

Exploring Layers of Meaning with Deep Brain Stimulation Patients

Tens of thousands of patients have received deep brain stimulation (DBS) implants that aim to treat a variety of movement disorders, including Parkinson's disease (PD). We share Muller and Christen...

Poster 176: Neuropsychology and Functional Performance in Daily Activities Associated With Pontine and Subcortical Demyelination: A Case Study

Objective: Central pontine and extra pontine myelinolysis (CPEM) is a rare neurological disease characterized by demyelination of the pons and other subcortical regions, secondary to rapid normalization of hyponatremia. A variety of movement disorders, psychiatric and behavioral manifestations can occur. However, very few studies on neuropsychological functioning and none on functional performance in daily activities have been reported.

Primary Bilateral Thalamic Astrocytoma Presenting With Head Tremor, Ataxia, and Dementia

Back to table of contents Previous article Next article LETTERFull AccessPrimary Bilateral Thalamic Astrocytoma Presenting With Head Tremor, Ataxia, and DementiaÁlvaro Machado M.D.,Manuel Ribeiro M.D.,Margarida Rodrigues M.D.Carla Ferreira M.D.,Rui Almeida M.D.,Isabel Santana M.D., Ph.D.,Lígia Castro M.D.Stirling Carpenter M.D.,Álvaro Machado M.D.Search for more papers by this author,Manuel Ribeiro M.D.Search for more papers by this author,Margarida Rodrigues M.D.Search for more papers by this authorCarla Ferreira M.D.Search for more papers by this author,Rui Almeida M.D.Search for more papers by this author,Isabel Santana M.D., Ph.D.Search for more papers by this author,Lígia Castro M.D.Search for more papers by this authorStirling Carpenter M.D.Search for more papers by this author,Published Online:1 Jul 2010https://doi.org/10.1176/jnp.2010.22.3.352.e7AboutSectionsPDF/EPUB ToolsAdd to favoritesDownload CitationsTrack Citations ShareShare onFacebookTwitterLinked InEmail To the Editor: Primary bilateral thalamic gliomas are exceedingly rare and usually present with behavioral and cognitive abnormalities, sparing motor and sensory functions. 1 Brain imaging reveals bilateral homogeneous enlargement of thalamic nuclei, which appear hyperintense on T2-weighted imaging. 1Case ReportA 42-year-old woman was seen for 6-month progressive behavioral disturbances. At that time she started being unusually aggressive and impatient, complaining of headaches, aching pain in the right leg, and head tremor. The behavioral changes worsened slowly, and she grew incapable of doing things she previously did, such as cooking, cleaning the house, administering medicine to her mother, and caring for her 13-year-old daughter. Relatives reported that she walked with poor balance. When seen, she thought she was in 1970 and in another hospital and that the reason for the consultation was a bloody nipple discharge. Ocular movements were uncoordinated, and she had a rhythmic nodding head tremor, choreoathetoid movement of the right hand, and bilateral cerebellar signs. Muscle strength and sensory functions, as well as visual fields, were normal. Neuropsychological assessment showed moderate to severe impairment of executive function, language, semantic and episodic memory, and visuospatial abilities. Noticeable was a severe involvement of temporoparietal cortical functions, with Gerstmann and Balint syndromes. Patient brain MRI ( Figure 1 , panels A and B) disclosed a bithalamic diffuse homogeneous lesion, hyperintense on T2 and iso-intense on T1-weighted images, without restricted diffusivity or gadolinium uptake. EEG showed an 8-Hz background activity. Electromyography characterized the head tremor as rhythmic at 3 Hz. A stereotaxic biopsy was performed, with removal of tissue from the right thalamus. FIGURE 1. Patient Brain MRIA. PD-weighted axial image shows a hyperintense infiltrative lesion involving both thalami, including the pulvinar; B. DWI reveals slight high signal in the enlarged bilateral thalami (without restricted diffusion)Histological examination showed astrocytosis with minor nuclear anaplasia and inconspicuous cytoplasm. A few normal large thalamic neurons were found. Spongiform changes were absent, as were mitotic figures. Numerous GFAP-positive processes were present. The diagnosis was of astrocytoma, grade 2 (WHO). On an oncology consultation, only palliative care was proposed. The patient died 3 months later.Discussion As a complex integrating system, the thalami play a central role in nervous system function. Pain and dysesthesia can reflect ventral posterior nuclei involvement, since they receive temperature and pain afferents from the contralateral hemibody. 2 A variety of movement disorders have been related to lesions affecting the ventral inferior or ventral intermediate nuclei, as well as the centromedianum and pulvinar, presumably because of their interconnections with the basal ganglia. 3 Cerebellar symptoms can result from dysfunction of the ventral anterior, ventral lateral, intralaminar, or reticular nuclei, which are functionally linked to the cerebellum. 4 The thalami also participate in cortical functioning, by three major networks: temporoparietoccipital, connected with the pulvinar; prefrontal, mainly related with the dorsomedial nuclei; and limbic, which receives efferents from the anterior thalamic nuclei. 4 Additionally, intralaminar nuclei have a central role in arousal and, with the dorsomedial nuclei, in attention. 5 We believe these known functions can explain all of our patient’s symptoms, even the most atypical and, to our knowledge, not previously described, as the head tremor, the ataxia, and the combined Gerstmann and Balint syndromes. Neurology Department, Hospital de São Marcos, Braga, PortugalNeuroradiology Department, Hospital de São MarcosNeurology Department, Hospital de São MarcosNeurosurgery Department, Hospital de São MarcosNeurology Department, Hospitais da Universidade de Coimbra, Coimbra, PortugalPathology Department, Hospital de São João, Porto, Portugal

Primary Bilateral Thalamic Astrocytoma Presenting With Head Tremor, Ataxia, and Dementia

Back to table of contents Previous article Next article LETTERFull AccessPrimary Bilateral Thalamic Astrocytoma Presenting With Head Tremor, Ataxia, and DementiaÁlvaro Machado M.D.,Manuel Ribeiro M.D.,Margarida Rodrigues M.D.Carla Ferreira M.D.,Rui Almeida M.D.,Isabel Santana M.D., Ph.D.,Lígia Castro M.D.Stirling Carpenter M.D.,Álvaro Machado M.D.Search for more papers by this author,Manuel Ribeiro M.D.Search for more papers by this author,Margarida Rodrigues M.D.Search for more papers by this authorCarla Ferreira M.D.Search for more papers by this author,Rui Almeida M.D.Search for more papers by this author,Isabel Santana M.D., Ph.D.Search for more papers by this author,Lígia Castro M.D.Search for more papers by this authorStirling Carpenter M.D.Search for more papers by this author,Published Online:1 Jul 2010https://doi.org/10.1176/jnp.2010.22.3.352.e7AboutSectionsPDF/EPUB ToolsAdd to favoritesDownload CitationsTrack Citations ShareShare onFacebookTwitterLinked InEmail To the Editor: Primary bilateral thalamic gliomas are exceedingly rare and usually present with behavioral and cognitive abnormalities, sparing motor and sensory functions. 1 Brain imaging reveals bilateral homogeneous enlargement of thalamic nuclei, which appear hyperintense on T2-weighted imaging. 1Case ReportA 42-year-old woman was seen for 6-month progressive behavioral disturbances. At that time she started being unusually aggressive and impatient, complaining of headaches, aching pain in the right leg, and head tremor. The behavioral changes worsened slowly, and she grew incapable of doing things she previously did, such as cooking, cleaning the house, administering medicine to her mother, and caring for her 13-year-old daughter. Relatives reported that she walked with poor balance. When seen, she thought she was in 1970 and in another hospital and that the reason for the consultation was a bloody nipple discharge. Ocular movements were uncoordinated, and she had a rhythmic nodding head tremor, choreoathetoid movement of the right hand, and bilateral cerebellar signs. Muscle strength and sensory functions, as well as visual fields, were normal. Neuropsychological assessment showed moderate to severe impairment of executive function, language, semantic and episodic memory, and visuospatial abilities. Noticeable was a severe involvement of temporoparietal cortical functions, with Gerstmann and Balint syndromes. Patient brain MRI ( Figure 1 , panels A and B) disclosed a bithalamic diffuse homogeneous lesion, hyperintense on T2 and iso-intense on T1-weighted images, without restricted diffusivity or gadolinium uptake. EEG showed an 8-Hz background activity. Electromyography characterized the head tremor as rhythmic at 3 Hz. A stereotaxic biopsy was performed, with removal of tissue from the right thalamus. FIGURE 1. Patient Brain MRIA. PD-weighted axial image shows a hyperintense infiltrative lesion involving both thalami, including the pulvinar; B. DWI reveals slight high signal in the enlarged bilateral thalami (without restricted diffusion)Histological examination showed astrocytosis with minor nuclear anaplasia and inconspicuous cytoplasm. A few normal large thalamic neurons were found. Spongiform changes were absent, as were mitotic figures. Numerous GFAP-positive processes were present. The diagnosis was of astrocytoma, grade 2 (WHO). On an oncology consultation, only palliative care was proposed. The patient died 3 months later.Discussion As a complex integrating system, the thalami play a central role in nervous system function. Pain and dysesthesia can reflect ventral posterior nuclei involvement, since they receive temperature and pain afferents from the contralateral hemibody. 2 A variety of movement disorders have been related to lesions affecting the ventral inferior or ventral intermediate nuclei, as well as the centromedianum and pulvinar, presumably because of their interconnections with the basal ganglia. 3 Cerebellar symptoms can result from dysfunction of the ventral anterior, ventral lateral, intralaminar, or reticular nuclei, which are functionally linked to the cerebellum. 4 The thalami also participate in cortical functioning, by three major networks: temporoparietoccipital, connected with the pulvinar; prefrontal, mainly related with the dorsomedial nuclei; and limbic, which receives efferents from the anterior thalamic nuclei. 4 Additionally, intralaminar nuclei have a central role in arousal and, with the dorsomedial nuclei, in attention. 5 We believe these known functions can explain all of our patient’s symptoms, even the most atypical and, to our knowledge, not previously described, as the head tremor, the ataxia, and the combined Gerstmann and Balint syndromes. Neurology Department, Hospital de São Marcos, Braga, PortugalNeuroradiology Department, Hospital de São MarcosNeurology Department, Hospital de São MarcosNeurosurgery Department, Hospital de São MarcosNeurology Department, Hospitais da Universidade de Coimbra, Coimbra, PortugalPathology Department, Hospital de São João, Porto, Portugal

Antipsychotic Induced Bruxism Treated with Clozapine

Antipsychotic Induced Bruxism Treated with Clozapine

Motor cortex stimulation in patients with Parkinson disease: 12-month follow-up in 4 patients

Since the initial 1991 report by Tsubokawa et al., stimulation of the M1 region of cortex has been used to treat chronic pain conditions and a variety of movement disorders. A Medline search of the literature published between 1991 and the beginning of 2007 revealed 459 cases in which motor cortex stimulation (MCS) was used. Of these, 72 were related to a movement disorder. More recently, up to 16 patients specifically with Parkinson disease were treated with MCS, and a variety of results were reported. In this report the authors describe 4 patients who were treated with extradural MCS. Although there were benefits seen within the first 6 months in Unified Parkinson's Disease Rating Scale Part III scores (decreased by 60%), tremor was only modestly managed with MCS in this group, and most benefits seen initially were lost by the end of 12 months. Although there have been some positive findings using MCS for Parkinson disease, a larger study may be needed to better determine if it should be pursued as an alternative surgical treatment to DBS.

Hemidystonia Associated With Focal Status Epilepticus as Incipient Manifestations in Probable Creutzfeldt-Jakob Disease

We report a 70-year-old man who manifested with hemidystonia associated with focal status epilepticus. The subsequent clinical symptoms and signs including rapid progressive dementia, and generalized myoclonus and the presence of 14-3-3 protein in cerebrospinal fluid, typical magnetic resonance imaging and eletroencephalography findings provide the evidences for diagnosis of probable Creutzfeldt-Jako b disease (CJD). Movement disorders or epilepsy rarely occur in the early stage of CJD. Furthermore, co-occurrence of both disorders in the early stage has been hardly reported. To the best of the authors’ knowledge, this is the first report in Korea of CJD presenting as dystonia. Our case suggests that CJD can present with a variety of movement disorders.

Motor Cortex Stimulation for Pain and Movement Disorders

Since initial reports in the early 1990s, stimulation of the M1 region of the cortex (MCS) has been used to treat chronic refractory pain conditions and a variety of movement disorders. A Medline search of literature between 1991 and 2007 revealed 512 cases using MCS. Although most of these relate to the treatment of pain (422), 84 of them involve movement disorders. More recently, several studies have specifically looked at treating Parkinson's disease (PD) with MCS. We report here several of our own cases using MCS to treat poststroke and non-poststroke pain syndromes and movement disorders (n = 8), PD (n = 4), ET (n = 2), and cortico-basal degeneration (n = 1). We also cover the essential history of this procedure and our current research using computational modeling to understand further the underlying mechanisms of MCS.