16,979 results match your criteria Chorea in Children


Chorea Hyperglycemia Basal Ganglia Syndrome in a 63-Year-Old Male.

Case Rep Med 2018 5;2018:9101207. Epub 2018 Nov 5.

U.S. Department of Veteran Affairs, Internal Medicine, 1970 Roanoke Blvd., Salem, VA 24153, USA.

Chorea hyperglycemia basal ganglia syndrome (CHBG) is a rare condition that manifests within the setting of uncontrolled nonketotic diabetes mellitus. The objective of this case report is to present a patient found to have CHBG and provide a timeline in terms of his workup and subsequent treatment. We also present a commentary on the current understanding of the pathophysiology and treatment and how this was applied to our patient. Read More

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November 2018

Complexity of the Genetics and Clinical Presentation of Spinocerebellar Ataxia 17.

Front Cell Neurosci 2018 23;12:429. Epub 2018 Nov 23.

Ataxia Centre, Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, London, United Kingdom.

Spinocerebellar ataxia type 17 (SCA17) is a rare autosomal dominant neurodegenerative disease caused by a CAG repeat expansion in the TATA-box binding protein gene (). The disease has a varied age at onset and clinical presentation. It is distinct from other SCAs for its association with dementia, psychiatric symptoms, and some patients presenting with chorea. Read More

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November 2018

Enzymatic and non-enzymatic pathways of kynurenines' dimerization: the molecular factors for oxidative stress development.

PLoS Comput Biol 2018 Dec 10;14(12):e1006672. Epub 2018 Dec 10.

Laboratory of Neurogenetics, Pavlov Institute of Physiology, Russian Academy of Sciences, Saint-Petersburg, Russia.

Kynurenines, the products of tryptophan oxidative degradation, are involved in multiple neuropathologies, such as Huntington's chorea, Parkinson's disease, senile dementia, etc. The major cause for hydroxykynurenines's neurotoxicity is the oxidative stress induced by the reactive oxygen species (ROS), the by-products of L-3-hydroxykynurenine (L-3HOK) and 3-hydroxyanthranilic acid (3HAA) oxidative self-dimerization. 2-aminophenol (2AP), a structural precursor of L-3HOK and 3HAA, undergoes the oxidative conjugation to form 2-aminophenoxazinone. Read More

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December 2018
1 Read

A discontinuous Galerkin model for fluorescence loss in photobleaching of intracellular polyglutamine protein aggregates.

BMC Biophys 2018 29;11. Epub 2018 Nov 29.

Department of Biochemistry and Molecular Biology, Campusvej 55, Odense M, 5230 Denmark.

Background: Intracellular phase separation and aggregation of proteins with extended poly-glutamine (polyQ) stretches are hallmarks of various age-associated neurodegenerative diseases. Progress in our understanding of such processes heavily relies on quantitative fluorescence imaging of suitably tagged proteins. Fluorescence loss in photobleaching (FLIP) is particularly well-suited to study the dynamics of protein aggregation in cellular models of Chorea Huntington and other polyQ diseases, as FLIP gives access to the full spatio-temporal profile of intensity changes in the cell geometry. Read More

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November 2018
5 Reads

Neurologic conditions and disorders of uremic syndrome of chronic kidney disease: Presentations, causes and treatment strategies.

Authors:
Sherifa A Hamed

Expert Rev Clin Pharmacol 2018 Dec 2. Epub 2018 Dec 2.

a Department of Neurology and Psychiatry , Assiut University Hospital , Assiut , Egypt.

Introduction: Uremic syndrome of chronic kidney disease (CKD) is a term used to describe clinical, metabolic and hormonal abnormalities associated with progressive kidney failure. It is a rapidly growing public health problem worldwide. Nervous system complications occur in every patient with uremic syndrome of CKD. Read More

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December 2018
1 Read

Treatment of psychiatric disturbances in common hyperkinetic movement disorders.

Expert Rev Neurother 2018 Dec 1. Epub 2018 Dec 1.

b Department of Human Neurosciences , Sapienza University of Rome , Rome.

Introduction: This paper reviews studies that have assessed the treatment of psychiatric disturbances in dystonia, tic disorders, Tourette syndrome, Huntington's disease and essential tremor. Areas covered: We searched for papers in English in Pubmed using the following keywords: blepharospasm, cervical dystonia, arm dystonia, laryngeal dystonia, spasmodic dysphonia, tic disorders, Tourette syndrome, Huntington's chorea, essential tremor, depression, anxiety, obsessive compulsive disorders, attention deficit hyperactivity disorders, psychosis, apathy. Expert commentary: Although psychiatric disturbances are frequent in hyperkinetic movement disorders, few controlled studies have assessed the treatment of psychiatric disturbances in such disorders. Read More

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December 2018
4 Reads

Successful pallidotomy for post-hyperglycemic hemichorea-ballism.

Parkinsonism Relat Disord 2018 Nov 28. Epub 2018 Nov 28.

Division of Neurosurgery, Toronto Western Hospital, University Health Network, 399 Bathurst Street, Toronto, M5T 2S8, Ontario, Canada. Electronic address:

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November 2018
1 Read
3.972 Impact Factor

Molecular Pathogenesis in Huntington's Disease.

Biochemistry (Mosc) 2018 Sep;83(9):1030-1039

Institute of Cytology, Russian Academy of Sciences, St. Petersburg, 194064, Russia.

Huntington's disease (HD) is a severe autosomal dominant neurodegenerative disorder characterized by a combination of motor, cognitive, and psychiatric symptoms, atrophy of the basal ganglia and the cerebral cortex, and inevitably progressive course resulting in death 5-20 years after manifestation of its symptoms. HD is caused by expansion of CAG repeats in the HTT gene, which leads to pathological elongation of the polyglutamine tract within the respective protein - huntingtin. In this review, we present a modern view on molecular biology of HD as a representative of the group of polyglutamine diseases, with an emphasis on conformational changes of mutant huntingtin, disturbances in its cellular processing, and proteolytic stress in degenerating neurons. Read More

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September 2018
2 Reads

Characteristics of Globus Pallidus Internus Local Field Potentials in Hyperkinetic Disease.

Front Neurol 2018 5;9:934. Epub 2018 Nov 5.

Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.

Dystonia and Huntington's disease (HD) are both hyperkinetic movement disorders but exhibit distinct clinical characteristics. Aberrant output from the globus pallidus internus (GPi) is involved in the pathophysiology of both HD and dystonia, and deep brain stimulation (DBS) of the GPi shows good clinical efficacy in both disorders. The electrode externalized period provides an opportunity to record local field potentials (LFPs) from the GPi to examine if activity patterns differ between hyperkinetic disorders and are associated with specific clinical characteristics. Read More

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November 2018
3 Reads

Regenerative Approaches in Huntington's Disease: From Mechanistic Insights to Therapeutic Protocols.

Front Neurosci 2018 2;12:800. Epub 2018 Nov 2.

Department of Neurobiology, Hellenic Pasteur Institute, Athens, Greece.

Huntington's Disease (HD) is a neurodegenerative disorder caused by a CAG expansion in the exon-1 of the IT15 gene encoding the protein Huntingtin. Expression of mutated Huntingtin in humans leads to dysfunction and ultimately degeneration of selected neuronal populations of the striatum and cerebral cortex. Current available HD therapy relies on drugs to treat chorea and control psychiatric symptoms, however, no therapy has been proven to slow down disease progression or prevent disease onset. Read More

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November 2018
4 Reads

Response to: A need to apply unified criteria to choreoballism associated with glycemic derangement.

Parkinsonism Relat Disord 2018 Jun 12. Epub 2018 Jun 12.

Mayo Clinic, Department of Neurology Rochester, USA. Electronic address:

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June 2018
1 Read

Chorea following extracorporeal circulation in adults: A case report and brief literature review.

Neurologia 2018 Nov 15. Epub 2018 Nov 15.

Servicio de Neurología y Unidad de Trastornos del Movimiento, Hospital Universitario La Paz, Madrid, España.

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November 2018
2 Reads

Alternative routes of administration of the neuroprotective pituitary adenylate cyclase activating polypeptide.

Curr Pharm Des 2018 Nov 11. Epub 2018 Nov 11.

Department of Anatomy, MTA-PTE PACAP Research Team, Centre for Neuroscience, University of Pecs Medical School. Hungary.

Pituitary adenylate cyclase activating polypeptide (PACAP) is a neuropeptide with diverse actions, including strong neurotrophic and neuroprotective effects. The aim of our present review is to provide a summary of the different approaches how in vivo neuroprotective effects can be achieved, emphasizing the potential translational values for future therapeutic applications. In the central nervous system, PACAP has been shown to have in vivo protective effects in models of cerebral ischemia, Parkinson's and Alzheimer's disease, Huntington chorea, traumatic brain and spinal cord injury and different retinal pathologies. Read More

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November 2018
13 Reads

Hemichorea associated with cavernous angioma and a small errhysis: A case report and literature review.

Medicine (Baltimore) 2018 Oct;97(43):e12889

Departments of Neurosurgery.

Rationale: Chorea is a movement disorder characterized by randomly appearing involuntary movements of the face, neck, limbs, or trunk. Hemichorea is unilateral, involving one side of the body. Hemichorea is commonly caused by non-ketotic hyperglycemia and/or cerebrovascular injury to the contralateral basal ganglia. Read More

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October 2018
1 Read

Molecular Imaging in Huntington's Disease.

Int Rev Neurobiol 2018 29;142:289-333. Epub 2018 Aug 29.

Neurodegeneration Imaging Group (NIG), Institute of Psychiatry, Psychology and Neuroscience (IoPPN), King's College London, London, United Kingdom. Electronic address:

Huntington's disease (HD) is a rare monogenic neurodegenerative disorder caused by a trinucleotide CAG repeat expansion in the huntingtin gene resulting in the formation of intranuclear inclusions of mutated huntingtin. The accumulation of mutated huntingtin leads to loss of GABAergic medium spiny neurons (MSNs); subsequently resulting in the development of chorea, cognitive dysfunction and psychiatric symptoms. Premanifest HD gene expansion carriers, provide a unique cohort to examine very early molecular changes, occurring before the development of overt symptoms, to elucidate disease pathophysiology and identify reliable biomarkers of HD progression. Read More

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August 2018
8 Reads

Transient Generalized Chorea in Influenza A Encephalopathy.

Tremor Other Hyperkinet Mov (N Y) 2018 9;8:591. Epub 2018 Oct 9.

Department of Neurology, University of Lübeck, Lübeck, DE.

Background: Influenza A infections are a rare cause of movement disorders. Previously described patients have suffered from acute-onset myoclonus and/or dystonia or post-viral parkinsonism.

Case Report: We present the case of a 74-year-old female patient with transient generalized chorea due to influenza A-mediated encephalopathy. Read More

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December 2018
5 Reads

New tricks for an old dog: A repurposing approach of apomorphine.

Eur J Pharmacol 2018 Nov 2;843:66-79. Epub 2018 Nov 2.

Behavior and Basal Ganglia Research Unit (EA 4712), University of Rennes 1, Rennes, France; Institut des Neurosciences Cliniques de Rennes (INCR), Rennes, France; Movement Disorders Unit, Neurology Department, Pontchaillou University Hospital, Rennes, France.

Apomorphine is a 150-year old nonspecific dopaminergic agonist, currently indicated for treating motor fluctuations in Parkinson's disease. At the era of drug repurposing, its pleiotropic biological functions suggest other possible uses. To further explore new therapeutic and diagnostic applications, the available literature up to July 2018 was reviewed using the PubMed and Google Scholar databases. Read More

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November 2018
7 Reads

Phenytoin induced chorea: a rare adverse effect of the drug.

Curr Drug Saf 2018 Oct 31. Epub 2018 Oct 31.

B. J. Medical College, Ahmedabad. India.

Background: Dyskinetic neurological diseases are common presentations of adverse reaction to many therapeutic agents. Phenytoin, a widely used age old antiepileptic drug has been reported to cause dyskinesias, a rare adverse drug reaction (ADR) in adults with toxic therapeutic serum level. When the drug is used in combination with other drugs which augments free drug level of phenytoin or in patients of organic brain lesion, this side effect is very occasionally reported with even normal therapeutic drug level. Read More

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October 2018

Successful treatment of choreo-athetotic movements in a patient with an EEF1A2 gene variant.

SAGE Open Med Case Rep 2018 24;6:2050313X18807622. Epub 2018 Oct 24.

Department of Neurology, School of Medicine, Johns Hopkins University, Baltimore, MD, USA.

Pathogenic variants in , a gene encoding a eukaryotic translation elongation factor, have been previously reported in pediatric cases of epileptic encephalopathy and intellectual disability. We report a case of a 17-year-old male with a prior history of epilepsy, autism, intellectual disability, and the abrupt onset of choreo-athetotic movements. The patient was diagnosed with an variant by whole exome sequencing. Read More

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October 2018
7 Reads

Proceedings of the Ninth International Meeting on Neuroacanthocytosis Syndromes.

Authors:

Tremor Other Hyperkinet Mov (N Y) 2018 17;8:579. Epub 2018 Jul 17.

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November 2018
2 Reads

Treatment of Levodopainduced dyskinesia with Vitamin D: A Randomized, double-blind, placebo-controlled trial.

Neurol Int 2018 Sep 1;10(3):7737. Epub 2018 Oct 1.

Iran University of Medical Sciences, Tehran, Iran.

Dyskinesia refers to any involuntary movement, such as chorea, dystonia, ballism that affect any part of the body. Levodopa-induced dyskinesia is a neurological disorder that afflicts many patients with Parkinson disease usually 5 years after the onset of levodopa therapy and can cause severe disability. The pathophysiology of this dyskinesia is complex and not fully understood. Read More

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September 2018
4 Reads

Changes in recruitment of motor cortex excitation and inhibition in patients with drug-induced tardive syndromes.

Neurophysiol Clin 2018 Oct 23. Epub 2018 Oct 23.

Sobell Department of Motor Neuroscience and movement Disorders, National Hospital for Neurology and Neurosurgery, Queen Square, London, UK.

Objectives: It has recently been suggested that drug-induced tardive syndromes (TS) might be due to maladaptive plasticity, which increases motor excitability in cerebral cortex and basal ganglia. In order to test this hypothesis, we performed the first measurements of cortical excitability in TS.

Methods: Motor cortex excitability was examined using transcranial magnetic stimulation (TMS) in 22 TS patients and compared with that in 20 age and sex-matched healthy individuals. Read More

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October 2018
4 Reads

Paraneoplastic Puzzle: An Unusual Case of Hemichorea, Renal Cell Carcinoma, and LGI1 Antibody.

Mov Disord Clin Pract 2018 May-Jun;5(3):337-338. Epub 2018 May 7.

Department of Neurology Edward Hines Junior VA Hospital Hines IL USA.

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May 2018
1 Read

Vascular Pathology Causing Late Onset Generalized Chorea: A Clinico-Pathological Case Report.

Mov Disord Clin Pract 2017 Nov-Dec;4(6):819-823. Epub 2017 Sep 8.

Department of Neurology Centro Hospitalar do Porto Porto Portugal.

Background: Chorea may occur as a manifestation of an acute stroke. Patients with vascular-related chorea typically present with an acute or subacute onset of hemichorea, contralateral to the lesion.

Methods And Findings: In this clinico-pathological case, we report a 90-year-old female who presented, at age 81, with a transient episode of generalized chorea. Read More

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September 2017
8 Reads

Rating Scales for Motor Symptoms and Signs in Huntington's Disease: Critique and Recommendations.

Mov Disord Clin Pract 2018 Mar-Apr;5(2):111-117. Epub 2018 Jan 3.

Department of Neurological Sciences Rush University Medical Center Chicago Illinois USA.

Motor symptoms are a major feature of Huntington's disease (HD). The International Parkinson and Movement Disorder Society (MDS) commissioned the assessment of the clinimetric properties of motor rating scales in HD to make recommendations regarding their use, following previously established standardized criteria. After a systematic literature search, a total of 6 rating scales assessing motor symptoms and signs in HD were included for review. Read More

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January 2018
11 Reads

Movement disorders phenomenology in focal motor seizures.

Parkinsonism Relat Disord 2018 Oct 16. Epub 2018 Oct 16.

Department of Human Neurosciences, Sapienza University of Rome, Rome, Italy; IRCSS Neuromed, Pozzilli, IS, Italy.

Introduction: Although focal motor seizures may resemble one or more movement disorders their phenomenology and prevalence remain uncertain.

Methods: To examine the extent to which focal motor seizures can present with a phenomenology fulfilling diagnostic criteria for movement disorders, 100 consecutive patients with focal motor seizures were rated by movement disorders experts, epileptologists, and general neurologists.

Results: A focal motor seizure phenomenologically manifested as a defined movement disorder in 29% of the patients from a consecutive video-EEG documented cohort as per consensus among experts: myoclonus and dystonia (10 and 9 cases, respectively) were the most common movement disorders, followed by chorea (4), stereotypies (3) myoclonus-dystonia (2), and tremor (1). Read More

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October 2018
6 Reads

Restless Legs Syndrome in NKX2-1-related chorea: An expansion of the disease spectrum.

Brain Dev 2018 Oct 20. Epub 2018 Oct 20.

Child Neurology and Psychiatry Unit, Santa Maria Nuova Hospital IRCCS, Reggio Emilia, Italy.

Background: Molecular technologies are expanding our knowledge about genetic variability underlying early-onset non-progressive choreic syndromes. Focusing on NKX2-1-related chorea, the clinical phenotype and sleep related disorders have been only partially characterized.

Methods: We propose a retrospective and longitudinal observational study in 7 patients with non-progressive chorea due to NKX2-1 mutations. Read More

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October 2018
8 Reads

PDE10A and ADCY5 mutations linked to molecular and microstructural basal ganglia pathology.

Mov Disord 2018 Oct 21. Epub 2018 Oct 21.

Neurodegeneration Imaging Group, Institute of Psychiatry, Psychology and Neuroscience, King's College London, London, UK.

Background: Striatal cyclic adenosine monophosphate activity modulates movement and is determined from the balance between its synthesis by adenylate cyclase 5 (ADCY5) and its degradation by phosphodiesterase 10A (PDE10A).

Objective: We assessed the integrity of striatocortical pathways, in vivo, in 2 genetic hyperkinetic disorders caused by ADCY5 and PDE10A mutations.

Methods: We studied 6 subjects with PDE10A and ADCY5 mutations using [ C]IMA107 PET, [ I]FP-CIT Single-photon emission computed tomography (SPECT) and multimodal MRI to investigate PDE10A and dopamine transporter availability, neuromelanin-containing neurons, and microstructural white and gray matter changes, respectively. Read More

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October 2018
2 Reads
5.680 Impact Factor

Neuropsychiatric Disorder Associated with Group G Infection.

Case Rep Pediatr 2018 23;2018:6047318. Epub 2018 Sep 23.

Department of Pediatrics, Niigata University, Niigata, Japan.

Immune-mediated central nervous system manifestations of group A -hemolytic (GABHS) infection include Sydenham's chorea, pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection (PANDAS)-which includes tic and obsessive compulsive disorders-and a variety of neurobehavioral disorders. We report a case of subspecies (group G ) (GGS) infection associated with involuntary movements, complex tics, and emotional lability in an 11-year-old Japanese girl. Serum IgM and IgG antibodies to lysoganglioside were positive, and she responded rapidly to intravenous immunoglobulin treatment. Read More

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September 2018
6 Reads

F-fluorodeoxyglucose positron emission tomography and magnetic resonance imaging evaluation of chorea.

Neurol Int 2018 Sep 5;10(3):7780. Epub 2018 Sep 5.

Division of Neurology, Respirology, Endocrinology and Metabolism, Department of Internal Medicine, University of Miyazaki, Japan.

Chorea is thought to be caused by deactivation of the indirect pathway in the basal ganglia circuit. However, few imaging studies have evaluated the basal ganglia circuit in actual patients with chorea. We investigated the lesions and mechanisms underlying chorea using brain magnetic resonance imaging (MRI) and F-fluorodeoxyglucose positron emission tomography (FDG-PET). Read More

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September 2018
1 Read

Movement disorders associated with neuronal antibodies.

Acta Neurol Scand 2018 Oct 18. Epub 2018 Oct 18.

Department of Neurology, Govind Ballabh Pant Postgraduate Institute of Medical Education and Research, New Delhi, India.

Movement disorders are one of the common clinical features of neurological disease associated with neuronal antibodies which is a group of potentially reversible disorder. They can present with hypokinetic or hyperkinetic types of involuntary movements and may have other associated neurological symptoms. The spectrum of abnormal movements associated with neuronal antibodies is widening. Read More

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October 2018
1 Read

P/Q and N-type Voltage-gated Calcium Channel Binding Antibodies Associated with Paraneoplastic Chorea and Mixed Invasive Ductal and Lobular Carcinoma of the Breasts in an Elderly Patient.

Cureus 2018 Aug 4;10(8):e3097. Epub 2018 Aug 4.

Department of Neurology, University of Ilinois at Chicago, Chicago, USA.

Paraneoplastic neurologic syndromes are a group of immune-mediated, cancer-associated disorders affecting the nervous system. While these syndromes are not understood fully, they are reportedly caused by an immune response against common antigens expressed by the cancer and nervous system. We describe the course of a patient who suffered paraneoplastic chorea before being diagnosed with breast cancer. Read More

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August 2018
1 Read

Movement disorders in emergency settings: a prospective study.

Neurol Sci 2018 Oct 15. Epub 2018 Oct 15.

Movement Disorders Division, Department of Neurosciences, Biomedicine and Movement Sciences, Neurology Unit, University of Verona, Verona, Italy.

Introduction: Acute movement disorders (MD) are etiologically heterogeneous entities. Since studies on the relative frequency of different MD and their underlying diseases are limited, we performed a prospective study to investigate the spectrum of various MD and their causes in patients presenting with acute MD in an emergency room (ER) setting.

Objective: To describe the spectrum and outcomes of acute MD in a prospective cohort and to guide its management. Read More

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October 2018
4 Reads

The "sick dancers": The construction of medical knowledge about the "epidemic of dance" in Itapagipe, Salvador, Bahia (1882-1901).

Stud Hist Philos Biol Biomed Sci 2018 Oct 9;71:32-40. Epub 2018 Oct 9.

Postgraduate Program in Social History, Federal University of Pará, Rua Augusto Corrêa, 01 Campus Universitário Do Guamá, Belém, Pará, Brazil. Electronic address:

The goal of this paper is to analyze a little-known set of documents referring to a "Dancing Epidemic" that took place in Itapagipe, a suburb of Salvador, capital of the province of Bahia, Brazil, in 1882. Through the studies of a group of physicians, especially Raimundo Nina Rodrigues (1862-1906), a psychiatrist and anthropologist from the Bahia School of Medicine, the medical knowledge built on this unique phenomenon in Brazilian history is examined. The case in particular involved a crowd that spread through the streets of Itapagipe, attracting the interest of the medical classes, who were intrigued by the symptoms of motor incoordination the patients manifested. Read More

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October 2018
1 Read

VMAT2 Inhibitors in Neuropsychiatric Disorders.

CNS Drugs 2018 Dec;32(12):1131-1144

Parkinson's Disease Center and Movement Disorders Clinic, Baylor College of Medicine, 7200 Cambridge St., 9th floor, Houston, TX, 77030, USA.

The basal ganglia and dopaminergic pathways play a central role in hyperkinetic movement disorders. Vesicular monoamine transporter 2 (VMAT2) inhibitors, which deplete dopamine at presynaptic striatal nerve terminals, are a class of drugs that have long been used to treat hyperkinetic movement disorders, but have recently gained more attention following their development for specific indications in the United States. At present, there are three commercially available VMAT2 inhibitors: tetrabenazine, deutetrabenazine, and valbenazine. Read More

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December 2018
16 Reads

TSFM mutations cause a complex hyperkinetic movement disorder with strong relief by cannabinoids.

Parkinsonism Relat Disord 2018 Sep 29. Epub 2018 Sep 29.

Department of Neurodegenerative Diseases, Hertie-Institute for Clinical Brain Research & Center of Neurology, University of Tuebingen, Tuebingen, Germany; German Research Center for Neurodegenerative Diseases (DZNE), University of Tuebingen, Tuebingen, Germany. Electronic address:

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September 2018
7 Reads

An unusual presentation of dystonia and chorea from intraventricular pneumocephalus.

Surg Neurol Int 2018 21;9:193. Epub 2018 Sep 21.

Department of Neurosurgery, Northshore University Health System, Evanston, Illinois, USA.

Background: Pneumocephalus is a common finding following intracranial procedures, typically asymptomatic and resolves within several days. However, in some cases, pneumocephalus presents with headache, encephalopathy, or symptoms of elevated intracranial pressure. Here, we present a case of iatrogenic tension pneumocephalus following endoscopic sinus surgery, presenting as abnormal involuntary movements resembling a movement disorder with choreiform movements. Read More

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September 2018
7 Reads

Novel Imaging Biomarkers for Huntington's Disease and Other Hereditary Choreas.

Curr Neurol Neurosci Rep 2018 Oct 5;18(12):85. Epub 2018 Oct 5.

Department of Clinical Neuroscience, Centre for Psychiatry Research, Karolinska Institutet and Stockholm County Council, R5:02 Karolinska University Hospital, SE-171 76, Stockholm, Sweden.

Purpose Of The Review: Imaging biomarkers for neurodegenerative disorders are primarily developed with the goal to aid diagnosis, to monitor disease progression, and to assess the efficacy of disease-modifying therapies in support to clinical outcomes that may either show limited sensitivity or need extended time for their evaluation. This article will review the most recent concepts and findings in the field of neuroimaging applied to Huntington's disease and Huntington-like syndromes. Emphasis will be given to the discussion of potential pharmacodynamic biomarkers for clinical trials in Huntington's disease (HD) and of neuroimaging tools that can be used as diagnostic biomarkers in HD-like syndromes. Read More

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October 2018
1 Read
3.060 Impact Factor

History and future challenges of the subthalamic nucleus as surgical target: Review article.

Mov Disord 2018 Oct 4;33(10):1540-1550. Epub 2018 Oct 4.

Department of Neurosurgery, Neurology and Neurosciences, Clínica Universidad de Navarra, University of Navarra, Pamplona, Spain.

For many years the subthalamic nucleus had a poor reputation among neurosurgeons as a result of the acute movement disorders that develop after its lesion or manipulation through different surgical procedures. However, this nucleus is now considered a key structure in relation to parkinsonism, and it is currently one of the preferred therapeutic targets for Parkinson's disease. The implication of the subthalamic nucleus in the pathophysiology of chorea and in the parkinsonian state is thought to be related to its role in modulating the basal ganglia, a fundamental circuit in movement control. Read More

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October 2018
6 Reads

Genetic landscape of pediatric movement disorders and management implications.

Neurol Genet 2018 Oct 26;4(5):e265. Epub 2018 Sep 26.

Division of Clinical and Metabolic Genetics (D.C., G.B., R.D.C., S.M.-A.), Department of Pediatrics, Toronto, Ontario, Canada; Department of Medical Genetics (K.S.), University of Alberta, Edmonton, Canada; Department of Pediatrics (A.E., J.K., R.D.C., S.M.-A.), University of Toronto; the Emergency Medicine Division (A.E.), Department of Paediatrics, The Hospital for Sick Children; Division of Neurology (J.K.), Department of Paediatrics, The Hospital for Sick Children,; Genetics and Genome Biology Program (R.D.C., S.M.-A.), Research Institute, The Hospital for Sick Children; and Institute of Medical Sciences (S.M.-A.), University of Toronto, Toronto, Ontario, Canada.

Objective: To identify underlying genetic causes in patients with pediatric movement disorders by genetic investigations.

Methods: All patients with a movement disorder seen in a single Pediatric Genetic Movement Disorder Clinic were included in this retrospective cohort study. We reviewed electronic patient charts for clinical, neuroimaging, biochemical, and molecular genetic features. Read More

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October 2018
9 Reads

Management of antiphospholipid syndrome.

Ann Rheum Dis 2018 Oct 3. Epub 2018 Oct 3.

Department of Rheumatology, Dubai Hospital, Dubai, United Arab Emirates

Antiphospholipid syndrome, also known as 'Hughes Syndrome', is an autoimmune disease characterised by a set of clinical manifestations, almost all of which are direct or indirect sequelae of a hypercoagulable state involving the venous, and to a lesser extent the arterial vasculature. The incidence and prevalence of antiphospholipid syndrome are estimated at approximately 5 de novo cases per 100 000 per year and 40-50 cases per 100 000 individuals, respectively. The clinical spectrum of antiphospholipid syndrome involves haematological (thrombocytopaenia, venous thrombosis), obstetrical (recurrent pregnancy loss), neurological (stroke, transient ischaemic attack, migraine, seizures, cognitive dysfunction, chorea, transverse myelitis, multiple sclerosis), cardiovascular (cardiac valve disease), dermatological (livedo reticularis and racemosa, skin ulceration and necrosis), renal (glomerulonephritis, renal thrombotic microangiopathy) and orthopaedic (avascular necrosis of bones, non-traumatic fractures) manifestations, among others. Read More

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October 2018
2 Reads

Tetrabenazine Treatment Patterns and Outcomes for Chorea Associated with Huntington Disease: A Retrospective Chart Review.

J Huntingtons Dis 2018 ;7(4):345-353

University of Alabama School of Medicine, Birmingham, AL, USA.

Background: Huntington disease (HD) is a neurodegenerative disorder characterized by motor impairments (including chorea), along with behavioral, psychiatric, and cognitive symptoms. Tetrabenazine was the first US Food and Drug Administration (FDA)-approved treatment for chorea related to HD.

Objective: To examine pharmacologic treatment patterns among patients using tetrabenazine, including reasons for treatment initiation, non-initiation, dose adjustments, and discontinuation, and to quantify the burden of chorea based on healthcare resource utilization. Read More

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January 2018
12 Reads

Epileptic chorea: Another window into neural networks?

J Neurol Sci 2018 Nov 11;394:138-140. Epub 2018 Sep 11.

James J. and Joan A. Gardner Center for Parkinson disease and Movement Disorders, Department of Neurology and Rehabilitation Medicine, University of Cincinnati, Cincinnati, OH, USA. Electronic address:

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November 2018
4 Reads
2.474 Impact Factor

Streptococcal Serology in Acute Rheumatic Fever Patients: Findings From 2 High-income, High-burden Settings.

Pediatr Infect Dis J 2019 Jan;38(1):e1-e6

Royal Darwin Hospital.

Background: Globally, there is wide variation in streptococcal titer upper limits of normal (ULN) for antistreptolysin O (ASO) and anti-deoxyribonuclease B (ADB) used as an evidence of recent group A streptococcal infection to diagnose acute rheumatic fever (ARF).

Methods: We audited ASO and ADB titers among individuals with ARF in New Zealand (NZ) and in Australia's Northern Territory. We summarized streptococcal titers by different ARF clinical manifestations, assessed application of locally recommended serology guidelines where NZ uses high ULN cut-offs and calculated the proportion of cases fulfilling alternative serologic diagnostic criteria. Read More

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January 2019
1 Read

An Update on the Treatment of Chorea.

Curr Treat Options Neurol 2018 Sep 25;20(10):44. Epub 2018 Sep 25.

Veterans Affairs Medical Center, Department of Neurology, Bronx Mount Sinai School of Medicine, New York, NY, 10029, USA.

Purpose Of Review: There are many causes for chorea, including genetic, autoimmune, pharmacological, and structural lesions. Where appropriate, treatment is based on reversing the underlying cause of chorea; many cases are self-limited, resolving when the primary disorder is treated. This review focuses on the management of chorea due to untreatable causes. Read More

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September 2018
1 Read

Worsening Choreoathetosis in Huntington's Disease with Fluoxetine, Lisdexamfetamine, and Melatonin: A Case Report.

Innov Clin Neurosci 2018 Jul-Aug;15(7-8):27-31. Epub 2018 Aug 1.

Dr. Hamilton is with the Department of Pharmacy, Veteran's Affairs Montana Medical Center-Fort Harrison, in Helena, Montana.

Cognitive, affective, and sleep disturbances can be found in patients with Huntington's disease (HD), and medications used to treat these HD-related sequela can also impact HD-related movement disorders. We present the case of a 52-year-old Caucasian man with previously undiagnosed HD who exhibited significant choreoathetoid movements that improved with discontinuation of fluoxetine and lisdexamfetamine upon hospital admission. Following diagnosis of HD through genetic testing, he was administered 5mg of oral melatonin on two consecutive evenings, which resulted in worsening choreoathetosis. Read More

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August 2018
1 Read

Life expectancy and mortality in chorea-acanthocytosis and McLeod syndrome.

Parkinsonism Relat Disord 2018 Sep 14. Epub 2018 Sep 14.

Department of Neurology, Ludwig-Maximilians-Universität München, Munich, Germany.

Objective: To document life expectancy and causes of death in chorea-acanthocytosis (ChAc) and McLeod syndrome (MLS).

Methods: We reviewed our personal databases and the published literature to identify cases of ChAc and MLS for whom adequate information was available regarding ages of disease onset and death, cause of death, and other clinical information.

Results: Adequate information was obtained on 52 patients with ChAc and 34 with MLS. Read More

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September 2018
3.972 Impact Factor

Unravelling of the paroxysmal dyskinesias.

J Neurol Neurosurg Psychiatry 2018 Sep 21. Epub 2018 Sep 21.

Sobell Department For Motor Neuroscience and Movement Disorders, Institute of Neurology, University College London, London, UK.

Paroxysmal dyskinesias (PxD) refer to a rare group of clinically and genetically heterogeneous disorders presenting with recurrent attacks of abnormal movements, typically dystonia, chorea or a combination thereof, without loss of consciousness. Classically, PxD have been categorised according to their triggers and duration of the attacks, but increasing evidence suggests that there is a certain degree of clinical and genetic overlap and challenges the concept that one phenotype is attributable to one single aetiology. Here we review the increasing spectrum of genetic conditions, as well as of other non-genetic disorders, that might present with PxD, provide criteria for case definition and propose a diagnostic workup to reach a definitive diagnosis, on which treatment is heavily dependent. Read More

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September 2018
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