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Importance: Urate elevation, despite associations with crystallopathic, cardiovascular, and metabolic disorders, has been pursued as a potential disease-modifying strategy for Parkinson disease (PD) based on convergent biological, epidemiological, and clinical data. Objective: To determine whether sustained urate-elevating treatment with the urate precursor inosine slows early PD progression. Design, Participants, and Setting: Randomized, double-blind, placebo-controlled, phase 3 trial of oral inosine treatment in early PD. A total of 587 individuals consented, and 298 with PD not yet requiring dopaminergic medication, striatal dopamine transporter deficiency, and serum urate below the population median concentration (<5.8 mg/dL) were randomized between August 2016 and December 2017 at 58 US sites, and were followed up through June 2019. Interventions: Inosine, dosed by blinded titration to increase serum urate concentrations to 7.1-8.0 mg/dL (n = 149) or matching placebo (n = 149) for up to 2 years. Main Outcomes and Measures: The primary outcome was rate of change in the Movement Disorder Society Unified Parkinson Disease Rating Scale (MDS-UPDRS; parts I-III) total score (range, 0-236; higher scores indicate greater disability; minimum clinically important difference of 6.3 points) prior to dopaminergic drug therapy initiation. Secondary outcomes included serum urate to measure target engagement, adverse events to measure safety, and 29 efficacy measures of disability, quality of life, cognition, mood, autonomic function, and striatal dopamine transporter binding as a biomarker of neuronal integrity. Results: Based on a prespecified interim futility analysis, the study closed early, with 273 (92%) of the randomized participants (49% women; mean age, 63 years) completing the study. Clinical progression rates were not significantly different between participants randomized to inosine (MDS-UPDRS score, 11.1 [95% CI, 9.7-12.6] points per year) and placebo (MDS-UPDRS score, 9.9 [95% CI, 8.4-11.3] points per year; difference, 1.26 [95% CI, -0.59 to 3.11] points per year; P = .18). Sustained elevation of serum urate by 2.03 mg/dL (from a baseline level of 4.6 mg/dL; 44% increase) occurred in the inosine group vs a 0.01-mg/dL change in serum urate in the placebo group (difference, 2.02 mg/dL [95% CI, 1.85-2.19 mg/dL]; P<.001). There were no significant differences for secondary efficacy outcomes including dopamine transporter binding loss. Participants randomized to inosine, compared with placebo, experienced fewer serious adverse events (7.4 vs 13.1 per 100 patient-years) but more kidney stones (7.0 vs 1.4 stones per 100 patient-years). Conclusions and Relevance: Among patients recently diagnosed as having PD, treatment with inosine, compared with placebo, did not result in a significant difference in the rate of clinical disease progression. The findings do not support the use of inosine as a treatment for early PD. Trial Registration: ClinicalTrials.gov Identifier: NCT02642393.
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Progresión de la Enfermedad , Inosina/uso terapéutico , Enfermedad de Parkinson/tratamiento farmacológico , Ácido Úrico/sangre , Anciano , Biomarcadores/sangre , Proteínas de Transporte de Dopamina a través de la Membrana Plasmática/deficiencia , Método Doble Ciego , Femenino , Humanos , Inosina/efectos adversos , Cálculos Renales/inducido químicamente , Masculino , Persona de Mediana Edad , Enfermedad de Parkinson/sangre , Enfermedad de Parkinson/fisiopatología , Índice de Severidad de la Enfermedad , Insuficiencia del TratamientoRESUMEN
Parkinson's disease is a slowly progressive neurodegenerative disease that commonly affects people aged 60 years and above. So far, no treatment has been shown to halt or slow the progression of the disease and our options are limited to symptomatic management. Levodopa is the most preferred antiparkinsonian medication that provides excellent control of symptoms early in the disease. However, in most patients the response declines over time and complications of motor fluctuations and dyskinesia arise. Other medical therapies play an adjunctive role in the management, as they are not as effective as levodopa. Advanced therapies like deep brain stimulation (DBS) can provide effective control of symptoms in moderate to advanced disease. Deep brain stimulation surgery has recently been started in Pakistan. This review provides an overview of deep brain stimulation, its indications, patient selection process and details of surgery, expected benefits and limitations as well as its history and challenges in Pakistan.
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Estimulación Encefálica Profunda , Enfermedades Neurodegenerativas , Enfermedad de Parkinson , Núcleo Subtalámico , Humanos , Persona de Mediana Edad , Pakistán , Enfermedad de Parkinson/terapia , Resultado del TratamientoRESUMEN
BACKGROUND/AIMS: In 1996, Nebraska became the first state in the United States to establish a Parkinson's disease (PD) Registry. The objectives of this study were to determine the most common comorbid conditions among PD patients receiving inpatient and outpatient services in Nebraska between 2004 and 2012, and to examine whether PD patients had increased risks of these conditions. METHODS: Statewide linkage was performed between Nebraska PD Registry data and hospital discharge database. The cohort comprised of 3,852 PD inpatients and 19,260 non-PD inpatients, and 5,217 PD outpatients and 26,085 non-PD outpatients. Referent subjects were matched to PD patients by age at initial hospital admissions or visits, gender, and county of residence using systematic random-sampling method. RESULTS: Compared to non-PD inpatients, PD inpatients were at higher risks for dementia (relative risk [RR] 2.29; 95% CI 2.14-2.45), mood disorders (RR 1.57; 95% CI 1.44-1.70), gastrointestinal disorders (RR 1.15; 95% CI 1.06-1.25), and urinary tract infections (RR 1.33; 95% CI 1.22-1.45), while PD outpatients had higher risks for spondylosis (RR 1.23; 95% CI 1.09-1.38), genitourinary disorders (RR 1.48; 95% CI 1.29-1.69), gastrointestinal disorders (RR 1.59; 95% CI 1.38-1.84), and dementia (RR 2.83; 95% CI 2.38-3.37) than non-PD outpatients. CONCLUSIONS: The findings highlight PD as a multisystem neurodegenerative disorder, and this information is crucial for creating strategies to better prevent and manage PD complications.
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Demencia/epidemiología , Enfermedades Gastrointestinales/epidemiología , Trastornos del Humor/epidemiología , Enfermedad de Parkinson/epidemiología , Infecciones Urinarias/epidemiología , Anciano , Anciano de 80 o más Años , Comorbilidad , Femenino , Hospitalización , Humanos , Masculino , Persona de Mediana Edad , Nebraska/epidemiología , Sistema de Registros , Estudios RetrospectivosRESUMEN
OBJECTIVE: Parkinson's disease (PD) impairs motor and non-motor functions. Driver strategies to compensate for impairments, like avoiding driving in risky environments, may reduce on-road risk at the cost of decreasing driver mobility, independence, and quality of life (QoL). It is unclear how PD symptoms link to driving risk exposure, strategies, and QoL. We assessed associations between PD symptoms and driving exposure (1) overall, (2) in risky driving environments, and (3) in relationship to QoL. METHODS: Twenty-eight drivers with idiopathic PD were assessed using the Movement Disorders Society-Unified Parkinson's Disease Rating Scale (MDS-UPDRS) and RAND 36-Item Short Form Health Survey (SF-36). Real-world driving was monitored for 1 month. Overall driving exposure (miles driven) and risky driving exposure (miles driven in higher risk driving environments) were assessed across PD symptom severity. High traffic, night, and interstate roads were considered risky environments. RESULTS: 18,642 miles (30,001 km) driven were collected. Drivers with PD with worse motor symptoms (MDS-UPDRS Part III) drove more overall (b = 0.17, P < .001) but less in risky environments (night: b = -0.35, P < .001; interstate roads: b = -0.23, P < .001; high traffic: b = -0.14, P < .001). Worse non-motor daily activities symptoms (MDS-UPDRS Part I) did not affect overall driving exposure (b = -0.05, P = .43) but did affect risky driving exposure. Worse non-motor daily activities increased risk exposure to interstate (b = 0.36, P < .001) and high traffic (b = 0.09, P = .03) roads while reducing nighttime risk exposure (b = -0.15, P = .01). Daily activity impacts from motor symptoms (MDS-UPDRS Part II) did not affect distance driven. Reduced driving exposure (number of drives per day) was associated with worse physical health-related QoL (b = 2.87, P = .04). CONCLUSIONS: Results provide pilot data revealing specific PD symptom impacts on driving risk exposure and QoL. Drivers with worse non-motor impairments may have greater risk exposure. In contrast, drivers with worse motor impairments may have reduced driver risk exposure. Reduced driving exposure may worsen physical health-related QoL. Results show promise for using driving to inform clinical care.
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Enfermedad de Parkinson , Humanos , Enfermedad de Parkinson/diagnóstico , Calidad de Vida , Accidentes de Tránsito , Índice de Severidad de la EnfermedadRESUMEN
Background: Driving is a complex, everyday task that impacts patient agency, safety, mobility, social connections, and quality of life. Digital tools can provide comprehensive real-world (RW) data on driver behavior in patients with Parkinson's disease (PD), providing critical data on disease status and treatment efficacy in the patient's own environment. Objective: This pilot study examined the use of driving data as a RW digital biomarker of PD symptom severity and dopaminergic therapy effectiveness. Methods: Naturalistic driving data (3974 drives) were collected for 1 month from 30 idiopathic PD drivers treated with dopaminergic medications. Prescriptions data were used to calculate levodopa equivalent daily dose (LEDD). The association between LEDD and driver mobility (number of drives) was assessed across PD severity, measured by the Movement Disorder Society Unified Parkinson's Disease Rating Scale (MDS-UPDRS). Results: PD drivers with worse motor symptoms based on self-report (Part II: P = 0.02) and clinical examination (Part III: P < 0.001) showed greater decrements in driver mobility. LEDD levels >400 mg/day were associated with higher driver mobility than those with worse PD symptoms (Part I: P = 0.02, Part II: P < 0.001, Part III: P < 0.001). Conclusions: Results suggest that comprehensive RW driving data on PD patients may index disease status and treatment effectiveness to improve patient symptoms, safety, mobility, and independence. Higher dopaminergic treatment may enhance safe driver mobility in PD patients with worse symptom severity.
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Introduction: Orthostatic tremor (OT) is a rare neurological disorder characterized by a sensation of instability while standing. Very few clinical signs have been described for OT to date. Finding other symptoms and signs could prove valuable for this hard-to-recognized disease. Methods: This protocol is part of the University of Nebraska Medical Center Orthostatic Tremor longitudinal study. It was noted that OT patients flex their toes and sometimes the foot arch while standing (Plantar Grasp). They reported doing this to "grab" the floor and improve stability. This paper analyses the diagnostic test characteristics of the patient-self-reported Plantar Grasp, a new sign in OT. Results: There were 34 OT patients (88% females), and 20 controls (65% females). Eighty-eight percent of patients with OT reported the plantar grasp sign and none of the controls. The Plantar Grasp Sign was found to be very sensitive (88%), and extremely specific (100%) in our cohort. Non-weighted Negative Likelihood Ratio (NLR) was 0.12. And the 3% prevalence-weighted NLR was so low that the negative post-test probability was close to zero. Conclusion: Due to its high sensitivity, specificity, and ideal likelihood ratio, we propose that the Plantar Grasp sign could be considered to screen patients with possible OT. Further studies are needed to determine the specificity of this sign in OT versus other balance disorders.
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Background: Deep brain stimulation (DBS) is used as a treatment option for patients diagnosed with a form of obsessive-compulsive disorder (OCD) that is highly resistant to conventional treatment methods. In 2009, DBS was granted a humanitarian device exemption-approval by the Food and Drug Administration after promising preliminary data. Monitoring of long-term safety data through post market surveillance of adverse events has not yet been conducted for DBS in OCD patients. This study aims to address this critical knowledge gap. Methods: All patient- and device-related (PR; DR) reports from January 1, 2012, to December 31, 2021, were downloaded and compiled from the manufacturer and user facility device experience (MAUDE) database pertaining to DBS for OCD using the product class name "Deep Brain Stimulator For OCD." Data in this study were examined using descriptive statistics to evaluate for frequency of reporting. Results: The most frequently reported PR adverse event categories included psychiatric (40%), neurological (19%), other (14%), decreased therapeutic response (10%), and infections (10%). The most frequent DR reports were high impedance (14%), energy output problem (7%), battery problem (7%), malposition of device (7%), and improper/incorrect procedure or method (7%). Conclusion: The PR and DR adverse events in our study align with the previous findings of adverse events. They also further solidify that DBS for refractory OCD may be a viable option for the right patient population. However, further studies are essential given the limitations of the MAUDE database.
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Medical education faces a difficult challenge today; an exponential increase in knowledge and the rise and rise of disruptive technologies are making traditional education obsolete. As the world nears the era of Industry and Healthcare 4.0, the medical community needs to keep up and prepare physicians for a hyper-connected digital world. Virtual neurological care is poised to be at the forefront of care delivery claims, yet the virtual communication of neurological knowledge is still in its infancy. This increasing digitalization of care and education is both an opportunity and a challenge. With this paper, the authors aim to bridge the gap between technology and neurological education. After a thorough review of recent literature and assessing current trends, the authors propose that contemporary medical education must adhere to the following tenets: Hybrid, Mobile, Mixed-reality, Open Access, Collaborative, Peer-reviewed, Intelligent, Game-based, and Global. We identify and align education objectives with the needs of future digital neurologists. The authors also discuss real-world advances that are aligned to serve the next generation of patients and providers.
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Alzheimer's disease (AD) and Parkinson's disease (PD) are neurodegenerative disorders that affect millions of individuals worldwide. As incidence of these conditions increases with age, there will undoubtedly be an increased prevalence of cases in the near future. Neuroinflammation is a hallmark in the development and progression of neurodegenerative diseases and prevention or resolution of chronic neuroinflammation may represent a novel approach to treatment. The present review highlights the potential of the anti-inflammatory and pro-resolving effects of polyunsaturated fatty acid (PUFA)-derived mediators (Specialized Pro-resolving Mediators-SPM) in neurodegenerative disorders. PUFA-derived SPM are biosynthesized in response to chemicals produced from acute inflammatory responses. Preclinical studies from both AD and PD models suggest a dysregulation of SPM and their receptors in neurological disorders. Decreased SPM may be due to inadequate substrate, an imbalance between SPM and pro-inflammatory mediators or a disruption in SPM synthesis. SPMs hold great promise for neuroprotection in AD by altering expression of pro-inflammatory genes, modulating macrophage function, serving as a biomarker for AD status, and promoting resolution of neuroinflammation. In PD, data suggest SPM are able to cross the blood-brain barrier, inhibit microglial activation and decrease induced markers of inflammation, possibly as a result of their ability to downregulate NFκB signaling pathways. Several in vivo and in vitro studies suggest a benefit from administration of SPMs in both neurodegenerative disorders. However, extrapolation of these outcomes to humans is difficult as no models are able to replicate all features of AD or PD. Minimal data evaluating these PUFA-derived metabolites in humans with neurodegenerative disorders are available and a gap in knowledge exists regarding behavior of SPM and their receptors in patients with these conditions. There is also large gap in our knowledge regarding which lipid mediator would be most effective in which model of AD or PD and how dietary intake or supplementation can impact SPM levels. Future direction should include focused, translational efforts to investigate SPM as an add-on (in addition to standard treatment) or as standalone agents in patients with neurodegenerative disorders.
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Oromandibular dystonia (OMD) is a chronic focal dystonia that involves the mouth, jaw, and tongue. It may cause repetitive or sustained dystonic movements, which can be very disabling for patients. It is usually a life-long disorder with numerous treatment options that are, most often, partially curative. In our experience, the best modality to treat OMD is botulinum toxin (BoNT) injections, which not only provide long-term relief but also have fewer adverse effects compared to other medications. Although multiple small- and large-scale studies support this fact, there is still a need for evidence from large randomized clinical trials. Jaw-closing dystonia responds very well to BoNT injections compared to other subtypes of OMD. This review discusses in detail the evidence, injection technique, and typical starting doses for botulinum injection.
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Background: Orthostatic tremor (OT) is characterized by a sensation of instability while standing, associated with high frequency (1318 Hz) tremor in the legs. Small retrospective series have reported electroencephalography (EEG) findings in OT with discordant results. Methods: We prospectively enrolled 30 OT subjects. Mean age = 68.3 (range 5487) with mean disease duration 16.3 years (range 444). A modified 1020 system EEG recording with additional midline electrodes was obtained. EMG electrodes were placed on quadricep muscles. EEG recording was performed at rest, during sleep and while standing unassisted. Results: In all subjects, EEG showed normal background, normal drowsiness and/or stage 2 sleep, and normal responses to hyperventilation and photic stimulation. These normal results persisted during stance. EEG abnormalities were found in 3 subjects (anterior-mid temporal slow activity), but were not position-dependent and were judged unlikely to be related to OT. Tremor artifact while standing was noted in all subjects, however it was measurable in 26 with frequency in the OT range in 25. When compared with EMG, the average difference in frequency was small at 1.2 Hz (range 0.52.5, p 0.46). Visual EEG analysis in OT patients did not reveal electrographic abnormalities even upon standing unassisted. Discussion: EEG was normal on this prospective, relatively large OT series. Clinicians interpreting video-EEGs should be aware of the OT artifact that can be seen in EEG and EKG leads mostly while standing.
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Mareo , Temblor , Electroencefalografía , Electromiografía , Humanos , Estudios Prospectivos , Estudios Retrospectivos , Temblor/diagnósticoRESUMEN
A broad set of conditions may present with an exaggerated startle reflex in clinics. This, combined with the overall rarity of these disorders, may pose diagnostic uncertainty in the mind of the treating physician. Herein, we report a case of a patient who presented to us with the complaint of exaggerated startle reflex and outline a simple approach towards characterisation of these disorders.
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Enfermedades del Sistema Nervioso Central/patología , Hiperekplexia/diagnóstico , Hiperekplexia/etiología , Reflejo de Sobresalto , Adulto , Anticonvulsivantes/uso terapéutico , Clonazepam/uso terapéutico , Humanos , Hiperekplexia/tratamiento farmacológico , Masculino , Evaluación de SíntomasRESUMEN
Teleneurology has provided access to neurological expertise and state-of-the-art stroke care where previously they have been inaccessible. The use of Artificial Intelligence with machine learning to assist telestroke care can be revolutionary. This includes more rapid and more reliable diagnosis through imaging analysis as well as prediction of hospital course and 3-month prognosis. Intelligent Electronic Medical Records can search free text and provide decision assistance by analyzing patient charts. Speech recognition has advanced enough to be reliable and highly convenient. Smart contextually aware communication and alert programs can enhance efficiency of patient flow and improve outcomes. Automated data collection and analysis can make quality improvement and research projects quicker and much less burdensome. Despite current challenges, these synergistic technologies hold immense promise in enhancing the clinician experience, helping to reduce physician burnout while improving patient health outcomes at a lower cost. This brief overview discusses the multifaceted potential of AI use in telestroke.
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In-person resident didactics are traditionally limited to the faculty within a single institution. Tele-education efforts have been implemented in neurology to various degrees historically, but the coronavirus disease 2019 (COVID-19) pandemic has necessitated a broad and immediate overhaul in neurology didactic training. To respond to the immediate need for resident didactics, we created a rapid onset, volunteer tele-education didactic series publicized on online forums to the American Academy of Neurology A.B. Baker Section via Synapse and the Women Neurologists Group via Facebook. We describe how, with just 1 week of lead time, we created an ongoing neurology lecture series featuring faculty from across the country lecturing on a diverse range of neurology topics. The series is ongoing and draws upwards of 120 residents per lecture. Tele-education offers unique benefits to enhance the education of all neurology trainees everywhere.
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Educación a Distancia/métodos , Educación de Postgrado en Medicina , Neurología/educación , Betacoronavirus , COVID-19 , Infecciones por Coronavirus , Humanos , Pandemias , Neumonía Viral , SARS-CoV-2RESUMEN
Background: The exact pathophysiology of primary Orthostatic Tremor (OT) is unknown. A central oscillator is assumed, and previous imaging studies show involvement of cerebellar pathways. However, the presence of ataxia on clinical exam is disputed. We set out to study ataxia in OT prospectively. Methods: EMG-confirmed primary OT subjects and spousal controls received a neurological exam with additional semiquantitative evaluations of ataxia as part of a multinational, prospective study. These included detailed limb coordination (DLC), detailed stance and gait evaluation (DS), and the Brief Ataxia Rating Scale (BARS). Intra- and inter-rater reliability were assessed and satisfactory. Results: 34 OT subjects (mean age = 67 years, 88% female) and 21 controls (mean age = 66 years, 65% male) were enrolled. Average disease duration was 18 years (range 4-44). BARS items were abnormal in 88% of OT patients. The OT subjects were more likely to have appendicular and truncal ataxia with significant differences in DLC, DS and BARS. Ocular ataxia and dysarthria were not statistically different between the groups. Discussion: Mild-to-moderate ataxia could be more common in OT than previously thought. This is supportive of cerebellar involvement in the pathophysiology of OT. We discuss possible implications for clinical care and future research. Highlights: Previous studies of Primary Orthostatic Tremor (OT) have proposed pathophysiologic involvement of the cerebellar pathways.However, presence of ataxia has not been systematically studied in OT.This is a prospective comprehensive ataxia assessment in OT compared to controls. Mild-to-moderate appendiculo-truncal ataxia was found to be common in OT.
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Ataxia/fisiopatología , Mareo/fisiopatología , Temblor/fisiopatología , Anciano , Anciano de 80 o más Años , Ataxia/epidemiología , Estudios de Casos y Controles , Mareo/epidemiología , Electromiografía , Femenino , Análisis de la Marcha , Humanos , Masculino , Persona de Mediana Edad , Prevalencia , Estudios Prospectivos , Temblor/epidemiologíaRESUMEN
In 2015, the US Food and Drug Administration approved levodopa-carbidopa intestinal gel (LCIG; also known as carbidopa-levodopa enteral suspension in the US) for the treatment of motor fluctuations in patients with advanced Parkinson's disease. LCIG provides a continuous infusion of levodopa and carbidopa by means of a portable pump and percutaneous endoscopic gastrojejunostomy tube. The delivery system has a two-fold pharmacokinetic advantage over orally administered carbidopa/levodopa. First, levodopa is delivered in a continuous rather than intermittent, pulsatile fashion. Second, delivery to levodopa's site of absorption in the jejunum bypasses the stomach, thereby avoiding issues with erratic gastric emptying. In blinded prospective clinical trials and observational studies, LCIG has been shown to significantly decrease "off" time, increase "on" time without troublesome dyskinesia, and reduce dyskinesia. Consistent with procedures in previous studies, LCIG initiation and titration in the pivotal US clinical trial were performed in the inpatient setting and followed a standardized protocol. In clinical practice, however, initiation and titration of LCIG have a great degree of flexibility and, in the US, almost always take place in the outpatient setting. Nonetheless, there remains a significant amount of clinician uncertainty regarding titration in outpatient clinical practice. This review aims to shed light on and provide guidance as to the current methods of titration in the outpatient setting, as informed by the medical literature and the authors' experiences. FUNDING: AbbVie, Inc. Plain language summary available for this article.
Results from recent studies have shown that continuous infusion of levodopa-carbidopa intestinal gel (LCIG) into the jejunum (a part of the small intestine) effectively manages the motor and nonmotor complications (e.g., tremor, extreme stiffness in arms and legs, difficulty walking, and impaired balance) experienced by patients with advanced Parkinson's disease (PD). LCIG is administered by a portable pump directly into the patient's jejunum by a permanent tube that is inserted surgically. LCIG therapy is beneficial to advanced PD patients over orally administered carbidopa/levodopa for two reasons. First, oral carbidopa/levodopa moves from the stomach to the small intestine where it is intermittently absorbed into the blood stream. LCIG is administered continuously and offers better symptom control for longer. Results from clinical trials and observational studies have shown that LCIG significantly decreases "off" time (poor motor control) and increases "on" time (good motor control) in advanced PD patients without troublesome dyskinesia, which results from the higher doses of oral levodopa required to treat the symptoms. Second, LCIG is absorbed in the jejunum, thereby bypassing the stomach where problems can occur because of inconsistent stomach emptying. In the US, titration of LCIG is performed mostly in an outpatient setting. Some clinicians may view titration of LCIG to be too complex and variable, so they avoid using LCIG therapy for their PD patients. Fortunately, emerging data and clinicians' expanding experience with LCIG have shown that titration can be easily managed in an outpatient setting, allowing for more customized therapeutic regimens for patients.
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Antiparkinsonianos/uso terapéutico , Carbidopa/uso terapéutico , Levodopa/uso terapéutico , Enfermedad de Parkinson/tratamiento farmacológico , Anciano , Combinación de Medicamentos , Femenino , Geles , Humanos , Infusiones Parenterales , Masculino , Persona de Mediana Edad , Estudios Observacionales como Asunto , Medicina de Precisión , Estudios Prospectivos , Estados UnidosRESUMEN
Neurology still remains one of the most underserved specialties of medicine in Pakistan with roughly one neurologist per million people. Movement disorders (MD) are neurological problems that interfere with patient's motor abilities and diagnosis is typically clinical. In this review, we describe a practical approach to common MD emergencies that may be encountered by a non-neurologist physician, emphasizing on formulating a working diagnosis and their immediate management. Movement disorder emergencies can be classified based on MD phenomenology and we will provide a brief overview of dystonia including acute dystonic reaction, PAID syndrome and dystonic storm; chorea, myoclonus including serotonin syndrome and startle disease; and rigidity including neuroleptic malignant syndrome and malignant hyperthermia.
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Distonía/terapia , Trastornos del Movimiento/complicaciones , Mioclonía/terapia , Corea/etiología , Corea/terapia , Delirio/etiología , Delirio/terapia , Distonía/etiología , Urgencias Médicas , Humanos , Hipertermia Maligna/etiología , Hipertermia Maligna/terapia , Mioclonía/etiología , Síndrome Neuroléptico Maligno/etiología , Síndrome Neuroléptico Maligno/terapia , PakistánRESUMEN
PURPOSE OF REVIEW: Orthostatic tremor (OT) is a rare disorder characterized by tremor and a feeling of unsteadiness while standing that resolve upon walking or sitting. A pathognomonic 13-18 Hz tremor is seen on surface EMG while standing. Though its clinical features have been better defined over time, much of its pathophysiology remains unknown and treatment options are limited. We review here recent developments in both of these areas. RECENT FINDINGS: Several recent studies have furthered our understanding of the central oscillatory network involved in OT. fMRI and 18F-FDG-PET studies have identified a ponto-cerebello-thalamo-cortical network underlying OT, though the nature of its dysfunction remains unknown. Randomized trials of treatments for OT are few, so most data are from case reports or small case series. Clonazepam and gabapentin are likely the most effective medical therapies, while bilateral ventral intermediate nucleus deep brain stimulation shows promise for refractory cases. Though much about OT remains unknown, our understanding of its pathophysiology has improved through recent studies. Treatment benefit is overall modest and inconsistent, though better understanding of the disease could lead to new avenues for treatment.
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BACKGROUND: Deep brain stimulation (DBS) is a well-established neuromodulation therapy for advanced Parkinson disease, essential tremor and dystonia. In as much as this therapy is being developed in the Middle East, a better understanding of the incidence and prevalence of movement disorders, health care, and social framework is required for the region. METHODS: We reviewed current literature on the incidence and prevalence of various movement disorders in the Middle East amenable to DBS surgery. We also assessed recent efforts to develop DBS in the region. RESULTS: Available data on incidence and prevalence of movement disorders in the Middle East are old, inconclusive, and conflicting. We identify key areas such as cultural background, availability of accessible information, training, infrastructure, and public support groups in the region that may pose challenges. CONCLUSIONS: The Middle East is projected to be a growing market for neuromodulation. The available data on incidence and prevalence of movement disorders is from studies that were small, partial, and old, with inconsistent results, highlighting the need for newer, better-designed, and larger studies. DBS in the Middle East will need assessment of incidence and prevalence of movement disorders, existing challenges to its application, and focused efforts on key opportunities to foster development of DBS for this underserved region. This article is an attempt to identify and explore these challenges and propose solutions in anticipation.
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Estimulación Encefálica Profunda , Congresos como Asunto , Trastornos Distónicos/epidemiología , Trastornos Distónicos/terapia , Educación Médica Continua/organización & administración , Humanos , Incidencia , Cooperación Internacional , Medio Oriente/epidemiología , Trastornos del Movimiento/epidemiología , Trastornos del Movimiento/terapia , Neurología/educación , Neurología/organización & administración , Enfermedad de Parkinson/epidemiología , Enfermedad de Parkinson/terapia , Educación del Paciente como Asunto , PrevalenciaRESUMEN
INTRODUCTION: Parkinson's disease (PD) is increasing in prevalence due to a growing elderly population. Although there is no cure, there are exercise therapies and medications for mild to moderate disease. For more advanced disease, infusion or surgical interventions including deep brain stimulation surgery, levodopa carbidopa intestinal gel, and subcutaneous apomorphine infusion are considered. As these interventions become increasingly available, it is imperative for a neurologist involved in the care of advanced PD to be aware of the indications and timing for these interventions. Areas covered: This article attempts to identify different patient profiles and matches them with suggested advanced therapies for PD. There is limited literature providing guidance to a busy neurologist to match the most appropriate advanced therapy to the right patient profile. This article attempts to fill that void. Expert commentary: When matching patient profiles to therapy, several features must be considered: age, frailty, cognitive status, phenotype (predominant tremor vs. akinetic rigid), side effect or complication profile (dyskinesia, hallucinations, dysautonomia), and patient's comfort with invasive therapy options.