Movement Disorders In Adults Research Articles

Leksell Radiosurgery for Movement Disorders.

Tremor is the most prevalent movement disorder in adults. Patients who are refractory to medical management can explore surgical intervention. Deep-brain stimulation (DBS) and radiofrequency thalamotomy (RFT) are surgical procedures for intractable tremor that target the ventralis intermedius (VIM) nucleus to relieve contralateral tremor. For patients who are not candidates for surgical procedures, stereotactic radiosurgery (SRS) is a minimally invasive management option for tremor relief. SRS has been used for the elderly, those considered high surgical risk for other surgical procedures, those who are unresponsive to either DBS or RFT, and those who prefer a less invasive option. Radiosurgical thalamotomy is performed using a central dose of 130-140 Gy delivered to the VIM nucleus of the thalamus. The critical aspect of the dose planning procedure is the selection of the target, which requires a neurosurgeon experienced with movement disorder surgery. More than 90% of patients experience improvements in their tremor and quality of life after radiosurgical thalamotomy. Adverse radiation effects rates are low (4%). In selected cases, staged bilateral procedure can also be performed to relieve bilateral refractory tremor. In patients with tremor-predominant Parkinson's disease, VIM thalamotomy is also effective.

The relevance of gene panels in movement disorders diagnosis: A lab perspective.

Next-Generation Sequencing (NGS) is a group of new methods that allow sequencing a variable number of known genes (targeted resequencing) or even the whole human genome (whole genome sequencing-WGS) and have contributed to an exponential genetic knowledge growth, especially in rare diseases, in the past few years. Since 2015, in the Molecular Neurogenetics Unit of Neurological Institute "Carlo Besta", some gene panels have become available to screen all the known genes associated with Movement Disorders (MD) in children and adults as a diagnostic package. Over 221 patients analyzed (part of the Telethon Network of Genetic Biobanks - TNGB), pathogenic variants were found in 25 (11.31%), allowing a definitive genetic diagnosis. Among them, we found mutations in 10/114 patients with dystonia (8.8%); 10/59 patients with Parkinson's disease (16.9%); 1/25 patients with Neurodegeneration with Brain Iron Accumulation (NBIA) (4%) and 4/23 patients with neurotransmitter and biopterin metabolism synthesis defect (17.4%). Our results are in line with those published in literature; targeted resequencing does not replace Sanger sequencing totally, but its usage needs to be discussed with clinicians taking into account both the patient's clinical picture and radiological and neurophysiological data.

Pediatric movement disorders.

Pediatric movement disorders are commonly encountered clinical entities in the pediatric outpatient department. These disorders are a heterogenous group of disorders and may represent an underlying genetic disorder, a metabolic disorder or a hypoxic-ischemic insult during the perinatal period. Hyperkinetic movement disorders are more common as compared to hypokinetic disorders. This is unlike the situation in adult movement disorders where hypokinetic disorders are more often seen. A child's nervous system is more prone to hypoxic-ischemic insults due to its higher metabolic demands and the presence of an immature blood-brain barrier. The commonest movement disorders seen are tics, dystonia and chorea. Myoclonus is commonly associated with epilepsy syndromes. The aetiology of paediatric movement disorders depends on their course, their static or progressive nature, and whether an isolated symptom or an association with other neurological symptoms is present. The clue to the diagnosis is the proper recognition of the movement prevalent in the disorder.

Essential Tremor: β blockers a first-line therapy

Essential tremor is one of the most common movement disorders in adults and can affect both children and adults. Tremor may also be present in other locations, commonly the neck or the vocal cords. Patients with additional neurologic symptoms are now categorized as “essential tremor plus.” Additional clinical features associated with the condition include but are not limited to cognitive impairment, psychiatric disorders, and hearing loss. When treatment is needed, propranolol is considered a first-line treatment in the management of essential tremor.

Evidence-based and consensus clinical practice guidelines for the iron treatment of restless legs syndrome/Willis-Ekbom disease in adults and children: an IRLSSG task force report

BackgroundBrain iron deficiency has been implicated in the pathophysiology of RLS, and current RLS treatment guidelines recommend iron treatment when peripheral iron levels are low. In order to assess the evidence on the oral and intravenous (IV) iron treatment of RLS and periodic limb movement disorder (PLMD) in adults and children, the International Restless Legs Syndrome Study Group (IRLSSG) formed a task force to review these studies and provide evidence-based and consensus guidelines for the iron treatment of RLS in adults, and RLS and PLMD in children. MethodsA literature search was performed to identify papers appearing in MEDLINE from its inception to July 2016. The following inclusion criteria were used: human research on the treatment of RLS or periodic limb movements (PLM) with iron, sample size of at least five, and published in English. Two task force members independently evaluated each paper and classified the quality of evidence provided. ResultsA total of 299 papers were identified, of these 31 papers met the inclusion criteria. Four studies in adults were given a Class I rating (one for IV iron sucrose, and three for IV ferric carboxymaltose); only Class IV studies have evaluated iron treatment in children. Ferric carboxymaltose (1000 mg) is effective for treating moderate to severe RLS in those with serum ferritin <300 μg/l and could be used as first-line treatment for RLS in adults. Oral iron (65 mg elemental iron) is possibly effective for treating RLS in those with serum ferritin ≤75 μg/l. There is insufficient evidence to make conclusions on the efficacy of oral iron or IV iron in children. ConclusionsConsensus recommendations based on clinical practice are presented, including when to use oral iron or IV iron, and recommendations on repeated iron treatments. New iron treatment algorithms, based on evidence and consensus opinion have been developed.

Parasomnias and Sleep-Related Movement Disorders in Older Adults.

Parasomnias and sleep-related movement disorders are important problems in older adults. Sleep paralysis is rare, but may occur in families. In a minority of patients with disorders of arousal, the episodes persist until the age of 70. Zolpidem and other medications may induce sleepwalking and sleep-related eating. Most patients with idiopathic rapid eye movement (REM) sleep behavior disorder eventually develop Parkinson's disease or dementia with Lewy bodies. Anti-IgLON5 disease includes abnormal behaviors in NREM and REM sleep. Restless legs syndrome prevalence increases with age. A severe form of periodic limb movements in sleep may mimic REM sleep behavior disorder.

Psychogenic Movement Disorders in Adults and Children: A Clinical and Video Profile of 58 Indian Patients.

Psychogenic movement disorders (PMDs) represent 2% to 20% of patients with movement disorders. There is a paucity of literature on PMD in children, with most existing literature relating to adults only. For this detailed report of 58 patients (33 adults and 25 children) with PMD, history was assessed in the form of disease onset, duration, precipitating factors, diagnosis, and outcome. Movement phenotype was classified in terms of its appearance as tremor, dystonia, myoclonus, chorea, and others. Clinical evaluations were done to document variability, distractibility, and entrainment. Surface electromyography studies were obtained in patients with tremor. Seven adults and 7 children were classified with "documented" PMD, and 26 adults and 18 children were classified with "clinically established" PMD. Abrupt onset was common in adults (75.75%) and children (68%). The most common precipitating factors were family and social issues (42.42%) in adults and fear of examination (40%) and school-related issues (24%) in children. Tremor was the most common PMD in both adults and children. The second most common PMD was hemifacial spasm in adults. The majority of patients had abrupt onset of symptoms, and tremor was the most common form of movement disorder. The second most common PMDs were hemifacial spasm in adults and dystonia and gait abnormality in children. The most common precipitating factors in adults were related to family and social issues; whereas, in children, examination and school-related issues were most common. Complete improvement was seen in only 405 of patients.

Movement Disorders in Adults With Intellectual Disability and Behavioral Problems Associated With Use of Antipsychotics?

__To the Editors:__ We read with great interest the article by Scheifes and colleagues about movement disorders in adults with intellectual disability (ID) and behavioral problems associated with the use of antipsychotics. The authors examined movement disorders in this population. The study shows that 44% of the study populationwith ID and behavioral problems had at least 1 movement disorder (parkinsonism, dyskinesia, akathisia, and dystonia). These results are in line with research in other populations. High prevalence of movement disorders among psychiatric patients using antipsychotics is already known. The conclusion of this study is that the prevalence of movement disorders in people with ID and behavioral problems is high, especially in ID patients using antipsychotics. Current use of antipsychotic drug and a dose in target range seems significantly associated with the risk of having thesemovement disorders. However, in our opinion, there are limitations in the validity of the instruments and the design of the study that compromise the validity of the conclusions. The best available instruments are used despite the limited number of standardized assessments for this population. Unfortunately, the validity of these instruments is unknown for people with ID. In addition, there are avoidable omissions in other measurements that may have biased the results. Information about the medical history of the participants is missing; it is essential to know if the participants had the movement disorder at the moment of their admission in the centers. Other medication use is not included in the analysis, although it was reported. This information is important because it is also known that other medications, such as dopamine receptor blockers, antidepressants, and antiepileptics, can cause these movement disorders. Several cross-sectional studies about movement disorders in ID patients have been performed in the past few years (Table 1). None of these studies are generalizable. In the study population of Scheifes et al, the intelligence quotient (IQ) is much higher compared with the general ID population. It could be that the level of ID influences the prevalence of movement disorders. Because of a lack of valid measurements, several tools are used in different studies. Therefore, the comparison of the study results is difficult. This highlights the need for uniform instruments that are valid, reliable, practical, and feasible in the ID population. There are only 3 instruments developed and validated in people with ID, these are as follows: Akathisia Ratings of Movement Scale for akathisia (ARMS), Dyskinesia Identification System Condensed User Scale (DISCUS) for dyskinesia, and Matson Evaluation of Drug Side Effects (MEDS) for overall adverse effects of psychotropic drugs. In the case of other movement disorders, there are no instruments that are validated for the ID population. More research is needed to acquire valid instruments and subsequently reach consensus on recommended instruments. In the study population of Scheifes et al, most patients have a Diagnostic and Statistical Manual of Mental Disorders IV (DSM IV) classification, and antipsychotics are used on-label, comparable with the studies of Matson et al and Fodstad et al. In the conclusion of Scheifes et al, the results are compared with the study of de Kuijper et al, but these participants use the antipsychotics off-label as shown in Table 1. Therefore, these populations are not comparable. It is still unclear if antipsychotics cause movement disorders in the ID population. It could be a result of the ID itself, an adverse effect of the antipsychotics, or a result of the psychiatric disease. Studies done in this field are all cross-sectional (Table 1), and none of them examined the presence of movement disorders before the start of antipsychotics. Only Fodstad et al compared the use of antipsychotics in patients with or without a psychiatric disorder; the limitation of the study was a small number of participants. A long-term prospective follow-up study with different ID patient groups (eg, adults with ID with on-label use, off-label use, and without antipsychotics use) is needed. That study would be composed of an assessment of movement disorders before the start of antipsychotics, during follow-up, and after the discontinuation of antipsychotic use. In addition, there could be a control group with ID and behavioral problems but without antipsychotic use. In this way, a causal relationship could be demonstrated. In conclusion, further research is needed to develop instruments that diagnose movement disorders that are valid, reliable, practical, and feasible in the ID population.With these instruments, a prospective follow-up study can be done. This subject should no longer be on the sidelines of evidence-based medicine, because it has a great impact on the quality of life of patients with ID.

Movement Disorders in Adults With Intellectual Disability and Behavioral Problems Associated With Use of Antipsychotics.

Antipsychotic drugs are prescribed to approximately 30% to 40% of adults with intellectual disability (ID) and behavioral problems despite lack of evidence of effectiveness and potential adverse effects, including movement disorders. The aim of this study was to examine the prevalence of movement disorders (dyskinesia, akathisia, dystonia, and parkinsonism) in in-patient adults with mild to borderline ID and behavioral problems associated with use of antipsychotics. Prevalence of movement disorders was measured with a standardized protocol. The strength of the association between antipsychotic drug use and movement disorders was assessed using logistic regression analysis. Almost half (44.0%) of 134 in-patient adults with ID and behavioral problems had any movement disorder. Parkinsonism, dyskinesia, akathisia, and dystonia were present in, respectively, 36.6%, 11.2%, 9.0%, and 0.7% of patients with ID. It appeared that current use of any antipsychotic drug (odds ratio, 3.0; 95% confidence interval, 1.0-8.4) and a dose in target range (odds ratio, 5.5; 95% confidence interval, 1.5-20.4) were significantly associated with the risk of having movement disorders. The prevalence of movement disorders in people with ID and behavioral problems is high, especially in ID patients using antipsychotics. More attention is needed for these movement disorders and their potential impact.

Clinical, Genetic, and Radiological Features of Extrapyramidal Movement Disorders in Mitochondrial Disease.

Extrapyramidal movement disorders associated with mitochondrial disease are difficult to treat and can lead to considerable disability. Moreover, potential new treatment trials on the horizon highlight the importance of genotype-phenotype associations and deep phenotyping of the movement disorders related to mitochondrial disease. To describe the phenotype, genetic etiology, and investigation of extrapyramidal movement disorders in a large and well-defined mitochondrial disease cohort. An observational cohort study at a single national referral center. Among 678 patients (87% adults) followed up at the Newcastle mitochondrial disease specialized referral center between January 1, 2000, and January 31, 2015, 42 patients (12 pediatric, 30 adult) with genetic or biochemical evidence of mitochondrial disease and with 1 or more predefined extrapyramidal movement disorders (parkinsonism, dystonia, tremor, chorea, and restless legs syndrome) were included. We investigated the prevalence and genetic causes of dystonia and parkinsonism as well as radiological findings in the context of movement disorders in mitochondrial disease. All patients were interviewed and examined. All available medical notes and clinical, radiological, and genetic investigations were reviewed. Forty-two patients (mean [SD] age, 37 [25] years; 38% female) with mitochondrial disease (12 pediatric [age range, 4-14 years], 30 adult [age range, 20-81 years]) with extrapyramidal movement disorders were identified. Dystonia manifested in 11 pediatric patients (92%), often in the context of Leigh syndrome; parkinsonism predominated in 13 adult patients (43%), among whom 5 (38%) harbored either dominant (n = 1) or recessive (n = 4) mutations in POLG. Eleven adult patients (37%) manifested with either generalized or multifocal dystonia related to mutations in mitochondrial DNA, among which the most common were the m.11778G>A mutation and mutations in MT-ATP6 (3 of 11 patients [27%] each). Bilateral basal ganglia lesions were the most common finding in brain magnetic resonance imaging, usually associated with generalized dystonia or Leigh syndrome. Dystonia, often associated with Leigh syndrome, was the most common extrapyramidal movement disorder among pediatric patients with mitochondrial disease. Parkinsonism was the most prevalent extrapyramidal movement disorder in adults and was commonly associated with POLG mutations; dystonia was predominantly associated with mitochondrial DNA mutations. These findings may help direct genetic screening in a busy neurology outpatient setting.

La esencia del temblor esencial: bases neuroquímicas

Essential tremor is the most frequent movement disorder in adults. It has been considered a benign disease, but can result in significant physical and psychosocial disability. Pharmacological treatment is still not very satisfactory. Its causation, pathophysiology and anatomy remain only partially understood. An understanding of its neurochemical basis is essential to be able to develop more efficient therapies. We review what is currently known in this field in order to motivate further research and ideas that allow an enhanced understanding of the disease and which foster the development of new pharmacological therapies. We review the studies conducted to date in humans and in animal models of neurotransmitters (gamma-aminobutyric acid, glutamate, noradrenalin, serotonin, adenosine), proteins and other neurochemical phenomena, such as T-type calcium channels, in essential tremor. Four neurochemical dysfunctions have been described that basically occur in the cerebellum and the inferior olivary nucleus: alteration of the GABAergic system, increased post-inhibitory rebound via T-type calcium currents, decreased neuronal inhibition mechanisms and an increase in excitatory neurotransmitter activity. These neurochemical dysfunctions would involve an increase in the activity of the deep neurons of the cerebellum with an oscillatory activity that would shift to the thalamic nucleus and the motor cortex, which in turn would lead to the appearance of tremor. Further research is needed to be able to confirm these hypotheses and to continue to advance towards achieving more efficient pharmacological treatments for patients with essential tremor.

Magnetic resonance guided focused ultrasound pallidotomy for Parkinson’s disease

Parkinson’s disease (PD) is one of the most common movement disorders in adults, often cannot be adequately controlled with medical treatment, and thereby treated with the neurosurgical procedures. Unilateral pallidotomy, making a lesion in the globus pallidus pars interna (GPi), was the predominant surgical technique until early 1900s. Thereafter, deep brain stimulation (DBS) of the subthalamic nucleus (STN) has become a mainstream surgical technique for managing not only the cardinal dopaminergic features of PD, but also levodopa-induced motor fluctuations and dyskinesia. Recent review studies, however, reported that unilateral pallidotomy also has long-term effect on both cardinal symptoms (contralateral tremor and rigidity) and motor complications (contralateral dyskinesia and motor fluctuations). Currently, transcranial magnetic resonance guided focused ultrasound (MRgFUS), without necessity of opening the cranium, has been developed as a minimal invasive surgical technique, generating precise and focal thermal lesion in the brain compared to previous radiofrequency thermal lesion.

TREM2 R47H variant and risk of essential tremor: a cross-sectional international multicenter study.

Essential tremor (ET) is the most frequent movement disorder in adults. Its pathophysiology is not clearly understood, however there is growing evidence showing common etiologic factors with other neurodegenerative disorders such as Alzheimer's and Parkinson's diseases (AD, PD). Recently, a rare p.R47H substitution (rs75932628) in the TREM2 protein (triggering receptor expressed on myeloid cells 2; OMIM: *605086) has been proposed as a risk factor for AD, PD and amyotrophic lateral sclerosis (ALS). The objective of the study was to determine whether TREM2 p.R47H allele is also a risk factor for developing ET. This was a cross-sectional multicenter international study. An initial case-control cohort from Spain (n = 456 ET, n = 2715 controls) was genotyped. In a replication phase, a case-control series (n = 897 ET, n = 1449 controls) from different populations (Italy, Germany, North-America and Taiwan) was studied. Owed to the rarity of the variant, published results on p.R47H allele frequency from 14777 healthy controls from European, North American or Chinese descent were additionally considered. The main outcome measure was p.R47H (rs75932628) allelic frequency. There was a significant association between TREM2 p.R47H variant and ET in the Spanish cohort (odds ratio [OR], 5.97; 95% CI, 1.203-29.626; p = 0.042), but it was not replicated in other populations. These results argue in favor of population-specific differences in the allelic distribution and suggest that p.R47H (rs75932628) variant may contribute to the susceptibility of ET in Spanish population. However, taking into account the very low frequency of p.R47H, further confirmatory analyses of larger ET series are needed.

The pleiotropic movement disorders phenotype of adult ataxia-telangiectasia.

To assess the clinical spectrum of ataxia-telangiectasia (A-T) in adults, with a focus on movement disorders. A total of 14 consecutive adults with A-T were included at 2 tertiary adult movement disorders centers and compared to 53 typical patients with A-T. Clinical evaluation, neurophysiologic and video-oculographic recording, imaging, laboratory investigations, and ATM analysis were performed. In comparison with typical A-T cases, our patients demonstrated later mean age at onset (6.1 vs 2.5 years, p < 0.0001), later loss of walking ability (p = 0.003), and longer survival (p = 0.0039). The presenting feature was ataxia in 71% and dysarthria and dystonia in 14% each. All patients displayed movement disorders, among which dystonia and subcortical myoclonus were the most common (86%), followed by tremor (43%). Video-oculographic recordings revealed mostly dysmetric saccades and 46% of patients had normal latencies (i.e., no oculomotor apraxia) and velocities. The α-fetoprotein (AFP) level was normal in 7%, chromosomal instability was found in 29% (vs 100% of typical patients, p = 0.0006), and immunoglobulin deficiency was found in 29% (vs 69%, p = 0.057). All patients exhibited 2 ATM mutations, including at least 1 missense mutation in 79% of them (vs 36%, p = 0.0067). There is great variability of phenotype and severity in A-T, including a wide spectrum of movement disorders. Karyotype and repeated AFP level assessments should be performed in adults with unexplained movement disorders as valuable clues towards the diagnosis. In case of a compatible phenotype, A-T should be considered even if age at onset is late and progression is slow.

Transcranial Direct Current Stimulation of the Cerebellum in Essential Tremor: A Controlled Study

Essential tremor (ET) is a common movement disorder in adults. Success of drug treatment for ET remains poor and is often unsatisfactory [ [1] Louis E.D. Essential tremor. Lancet Neurol. 2005; 4: 100-110 Abstract Full Text Full Text PDF PubMed Scopus (312) Google Scholar ].

The Treatment of Restless Legs Syndrome and Periodic Limb Movement Disorder in Adults—An Update for 2012: Practice Parameters with an Evidence-Based Systematic Review and Meta-Analyses

A systematic literature review and meta-analyses (where appropriate) were performed to update the previous AASM practice parameters on the treatments, both dopaminergic and other, of RLS and PLMD. A considerable amount of literature has been published since these previous reviews were performed, necessitating an update of the corresponding practice parameters. Therapies with a STANDARD level of recommendation include pramipexole and ropinirole. Therapies with a GUIDELINE level of recommendation include levodopa with dopa decarboxylase inhibitor, opioids, gabapentin enacarbil, and cabergoline (which has additional caveats for use). Therapies with an OPTION level of recommendation include carbamazepine, gabapentin, pregabalin, clonidine, and for patients with low ferritin levels, iron supplementation. The committee recommends a STANDARD AGAINST the use of pergolide because of the risks of heart valve damage. Therapies for RLS secondary to ESRD, neuropathy, and superficial venous insufficiency are discussed. Lastly, therapies for PLMD are reviewed. However, it should be mentioned that because PLMD therapy typically mimics RLS therapy, the primary focus of this review is therapy for idiopathic RLS. (Reprinted with permission from SLEEP 2012; 35(8):1039–1062)

BNSU SURVEILLANCE PROJECT: MYOCLONUS DYSTONIA SYNDROME

BackgroundMyoclonus Dystonia Syndrome (MDS) is a childhood onset, alcohol responsive movement disorder characterised by trunk and upper limb myoclonus and dystonia of the neck and hands. Inheritance is autosomal dominant...

P 98. The aberrant motor and premotor cortex in essential tremor

Essential tremor (ET) probably represents the most common movement disorders in adults. Dysfunction of cerebellar system involving cerebello-rubral-thalamic loop was mostly suggested in the pathogenesis of ET. However, tremor eliminated after cerebral vascular insults over the corresponding motor cortex were reported in some patients. Electrophysiological studies demonstrated regional cortical activity in accordance with the tremor frequency and the tremor easily being reset by single magnetic shock over primary motor cortex, implying the cerebral cortex may participate in the tremor generation. To investing the mechanisms response for alternating the amplitude of electromyogram (EMG) response to transcranial magnetic stimulation (TMS) after prior conditioning of continuous theta-burst stimulation (cTBS), a well-known efficient intervention tool producing transient plastic change over regional cortex, to primary motor cortex (M1) or premotor cortex (PM) in patients with ET. We compared the physiological parameters including motor evoked potential (MEP), cortical silent period (CSP) and short-interval intracortical inhibition (SICI) before and after applying cTBS of 600 pulses at an intensity of 80% action motor threshold to M1 or PM in normal control (NC) and patients of ET. (1) Although cTBS given to either M1 or PM was capable of producing suppression effect on the motor cortical excitability in ET patients, the effects lasted much shorter as compared to those on NCs. (M1 cTBS, Fig. 1 ; PM cTBS, Fig. 2 ) (2) The amount of intra-cortical inhibition was decreased in NCs after applying cTBS on either M1 or PM. In contrast, no signify change was found after applying the same method to ET patients. (3) CSP was insignificantly prolonged by either M1 or PM cTBS in control group but not in ET patients. In this study, we failed to clarify the role of PM in the pathogenesis of ET. However, reduced plasticity and less modifiable motor cortical inhibitory circuits in response to M1 and PM TBS supported the theory of GABA defect in ET.

Abnormal Regional Homogeneity in Patients with Essential Tremor Revealed by Resting-State Functional MRI

Essential tremor (ET) is one of the most common movement disorders in human adults. It can be characterized as a progressive neurological disorder of which the most recognizable feature is a tremor of the arms or hands that is apparent during voluntary movements such as eating and writing. The pathology of ET remains unclear. Resting-state fMRI (RS-fMRI), as a non-invasive imaging technique, was employed to investigate abnormalities of functional connectivity in ET in the brain. Regional homogeneity (ReHo) was used as a metric of RS-fMRI to assess the local functional connectivity abnormality in ET with 20 ET patients and 20 age- and gender-matched healthy controls (HC). The ET group showed decreased ReHo in the anterior and posterior bilateral cerebellar lobes, the bilateral thalamus and the insular lobe, and increased ReHo in the bilateral prefrontal and parietal cortices, the left primary motor cortex and left supplementary motor area. The abnormal ReHo value of ET patients in the bilateral anterior cerebellar lobes and the right posterior cerebellar lobe were negatively correlated with the tremor severity score, while positively correlated with that in the left primary motor cortex. These findings suggest that the abnormality in cerebello-thalamo-cortical motor pathway is involved in tremor generation and propagation, which may be related to motor-related symptoms in ET patients. Meanwhile, the abnormality in the prefrontal and parietal regions may be associated with non-motor symptoms in ET. These findings suggest that the ReHo could be utilized for investigations of functional-pathological mechanism of ET.

Komplikationsanalyse von spinalen Eingriffen bei adulten zentralen Bewegungsstörungen und Skoliosen

Adult central movement disorders, malpostures, and scolioses can have their cause in various neurological underlying diseases such as Morbus Parkinson, Pisa syndrome, or segmental dystonia. Important clinical characteristics are marked postural distortions such as camptocormia (bent spine) or laterocollis. In cases of these adult scolioses, surgical spine treatment puts high demands on the surgeon. Surgery in Parkinson's disease, for example, is associated with serious surgery-specific as well as general complications. The more rarely occurring Pisa syndrome is an entity primarily requiring medical therapy. A series of ten case reports of patients with Morbus Parkinson and Pisa syndrome who underwent spinal surgery is presented and discussed. From these reports, treatment recommendations have been derived and complemented by references from the literature. An extensive MEDLINE search was performed for this purpose. In patients suffering from Parkinson's disease, even minor surgical interventions can lead to instability of whole spine segments or even the entire spine. Implant loosening, adjacent segment instability, general perioperative complications, and progressive malposture due to disease progress can bring forth disastrous treatment courses. Spinal fixation should be performed long-segmented in combination with ventral stabilisation. Due to osteoporosis, pedicle screw cement augmentation is recommended in this collective. If the diagnosis of Pisa syndrome is established, an optimised preoperative preparation should be initiated in close cooperation with neurologists. In many cases medical therapy is sufficient and surgical interventions can be avoided.