keyword
MENU ▼
Read by QxMD icon Read
search

Chorea

keyword
https://www.readbyqxmd.com/read/29137086/chorea-associated-with-nonketotic-hyperglycemia-a-case-report-with-atypical-imaging-changes
#1
Xianchao Chang, Wenke Hong, Hu Yu, Yindan Yao
INTRODUCTION: This is a unique case of nonketotic hyperglycemic (NKH) chorea in 84-year-old Asian woman. The patient had a history of type 2 diabetes mellitus more than 30 years, but had a poor control of blood sugar. She complained of acute onset of bilateral limb involuntary activities, and being easy to fall within a week. Laboratory testing disclosed hyperglycemia (669 mg/dL), glycated hemoglobin (14%), and normal ketones. The brain computed tomography scan and magnetic resonance imaging did not disclose any abnormality in the basal ganglion unlike most cases...
November 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29130591/familial-choreoathetosis-due-to-novel-heterozygous-mutation-in-pde10a
#2
Dhanya L Narayanan, Dipti Deshpande, Aneek Das Bhowmik, Dandu R Varma, Ashwin Dalal
PDE10A encodes a dual cAMP-cGMP phosphodiesterase that is enriched in the medium spiny neurons of the corpus striatum in the brain and plays an important role in basal ganglia circuitry. Three unrelated patients with childhood onset chorea and striatal abnormalities on MRI brain with heterozygous de novo variants in PDE10A have been described previously. Two families with eight affected individuals with biallelic mutations in PDE10A have also been described previously. We report a family with multiple affected individuals with childhood onset chorea, striatal abnormalities, and a novel heterozygous mutation, c...
November 12, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/29128164/grey-matter-volume-loss-is-associated-with-specific-clinical-motor-signs-in-huntington-s-disease
#3
Emma M Coppen, Milou Jacobs, Annette A van den Berg-Huysmans, Jeroen van der Grond, Raymund A C Roos
BACKGROUND: Motor disturbances are clinical hallmarks of Huntington's disease (HD) and involve chorea, dystonia, hypokinesia and visuomotor dysfunction. Investigating the association between specific motor signs and different regional volumes is important to understand the heterogeneity of HD. OBJECTIVE: To investigate the motor phenotype of HD and associations with subcortical and cortical grey matter volume loss. METHODS: Structural T1-weighted MRI scans of 79 HD patients and 30 healthy controls were used to calculate volumes of seven subcortical structures including the nucleus accumbens, hippocampus, thalamus, caudate nucleus, putamen, pallidum and amygdala...
November 2, 2017: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/29120264/treatment-options-for-chorea
#4
H Bashir, J Jankovic
Chorea is defined as jerk-like movements that move randomly from one body part to another. It is due to a variety of disorders and although current symptomatic therapy is quite effective there are few etiology- or pathogenesis-targeted therapies. The aim of this review is to summarize our own experience and published evidence in the treatment of chorea. Areas covered: After evaluating current guidelines and clinical practices for chorea of all etiologies, PubMed was searched for the most recent clinical trials and reviews using the term 'chorea' cross referenced with specific drug names...
November 9, 2017: Expert Review of Neurotherapeutics
https://www.readbyqxmd.com/read/29119300/longitudinal-outcomes-of-children-with-pediatric-autoimmune-neuropsychiatric-disorder-associated-with-streptococcal-infections-pandas
#5
Jill Leon, Rebecca Hommer, Paul Grant, Cristan Farmer, Precilla D'Souza, Riley Kessler, Kyle Williams, James F Leckman, Susan Swedo
Little is known about the natural history of children with pediatric autoimmune neuropsychiatric disorder associated with streptococcal infections (PANDAS). This study prospectively followed 33 children with PANDAS for up to 4.8 years (mean 3.3 ± 0.7 years) after enrollment in a 24-week randomized, double-blind, placebo-controlled trial of intravenous immunoglobulin (IVIG) (N = 35). Fourteen of eighteen children randomized to placebo received open label IVIG 6 weeks after the blinded infusion, so follow-up results reported below largely reflect outcomes in a population of children who received at least one dose of IVIG...
November 8, 2017: European Child & Adolescent Psychiatry
https://www.readbyqxmd.com/read/29103424/autoantibody-associated-movement-disorders-in-children-proven-and-proposed
#6
Harvey S Singer
Movement disorders secondary to autoantibodies in children represent a rapidly expanding group of conditions. Once considered to be limited to poststreptococcal Sydenham's chorea or rare cases of childhood systemic lupus erythematosus, a variety of antibody-related movement abnormalities are now seen as part of noninfectious autoimmune encephalitis or within an expanding list of postinfectious disorders. In this article, several proposed autoantibody-mediated movement disorders in children are reviewed. In each one, there is a hypothesized antibody biomarker that is believed to be pathogenic and cause the clinical symptoms...
August 2017: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/29097081/paraneoplastic-autoimmune-movement-disorders
#7
Thien Thien Lim
PURPOSE OF REVIEW: To provide an overview of paraneoplastic autoimmune disorders presenting with various movement disorders. RECENT FINDINGS: The spectrum of paraneoplastic autoimmune disorders has been expanding with the discovery of new antibodies against cell surface and intracellular antigens. Many of these paraneoplastic autoimmune disorders manifest as a form of movement disorder. With the discovery of new neuronal antibodies, an increasing number of idiopathic or neurodegenerative movement disorders are now being reclassified as immune-mediated movement disorders...
October 13, 2017: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/29089255/huntington-disease-care-from-the-past-to-the-present-to-the-future
#8
T A Mestre, K Shannon
INTRODUCTION: Huntington disease is a progressive neurodegenerative disorder without a cure. Its clinical presentation makes complex the care of patients with HD, further impacted by the progressive loss of dependence and disability. Intuitively, HD management calls for multispecialty care. METHODS: Literature review and expert-based statement. RESULTS: Chorea is the only indication for symptomatic treatments in HD. Surgical therapies are experimental, and exercise-based physical interventions have been assessed but in small feasibility studies...
August 10, 2017: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/29080203/deutetrabenazine-a-review-in-chorea-associated-with-huntington-s-disease
#9
Young-A Heo, Lesley J Scott
Oral deutetrabenazine (Austedo™), a reversible inhibitor of vesicular monoamine transporter type 2 (VMAT2) that is structurally related to tetrabenazine is approved for the treatment of chorea symptoms associated with Huntington's disease (HD). In the pivotal 12-week phase III FIRST-HD trial (n = 90), deutetrabenazine, at doses titrated for optimal chorea control and tolerability (maintenance dosage range 12-48 mg/day), was significantly more effective for controlling chorea in HD patients than placebo...
November 2017: Drugs
https://www.readbyqxmd.com/read/29066237/huntington-s-disease-like-2-with-an-expansion-mutation-of-the-junctophilin-3-gene-first-reported-case-from-botswana
#10
C Ocampo, R Daimari, A A Oyekunle
Huntington's disease-like 2 (HDL2) is a rare autosomal dominant progressive neurodegenerative disorder commonly seen in adults. It was first described in a large African-American family in the United States. HDL2 clinically resembles Huntington's disease (HD) and causes adult-onset relentlessly progressive movement, emotional and cognitive dysfunction. Onset is usually in the fourth decade with slow progression to death. We present a 47-year-old male Botswana native, with a four-year-history of chorea, slurred speech, mood instability, cognitive impairment and weight loss...
October 21, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/29061241/central-nervous-system-manifestations-of-antiphospholipid-syndrome
#11
REVIEW
Jonathan Graf
Neurologic manifestations are common in patients with antiphospholipid antibodies and include stroke, seizures, dementia, cognitive dysfunction, chorea, migraine, psychosis, and demyelinating disease. Many of these disorders mimic their idiopathic counterparts, yet treatment for antiphospholipid antibody-associated disease can be quite different compared with treatment of CNS disease not associated with these antibodies. For patients with antiphospholipid antibody-associated neurologic disease, anticoagulation or immunosuppressive therapy or both may significantly improve their symptoms...
November 2017: Rheumatic Diseases Clinics of North America
https://www.readbyqxmd.com/read/29053777/movement-disorders-with-neuronal-antibodies-syndromic-approach-genetic-parallels-and-pathophysiology
#12
Bettina Balint, Angela Vincent, Hans-Michael Meinck, Sarosh R Irani, Kailash P Bhatia
Movement disorders are a prominent and common feature in many autoantibody-associated neurological diseases, a group of potentially treatable conditions that can mimic infectious, metabolic or neurodegenerative disease. Certain movement disorders are likely to associate with certain autoantibodies; for example, the characteristic dyskinesias, chorea and dystonia associated with NMDAR antibodies, stiff person spectrum disorders with GAD, glycine receptor, amphiphysin or DPPX antibodies, specific paroxysmal dystonias with LGI1 antibodies, and cerebellar ataxia with various anti-neuronal antibodies...
September 25, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/29050651/-movement-disorders-an-update
#13
M Béreau, C Tranchant
Movement disorders (tremor, chorea, dystonia, tics, and myoclonus) are related to basal ganglia and/or interconnected brain areas dysfunction. Clinical examination is a key point in order to characterize the abnormal movement and identify associated signs that can guide etiological approach. Iatrogenic diseases will be systematically ruled out before conducting additional investigations (brain MRI, electrophysiological studies). Wilson disease, but also other treatable metabolic and/or genetic diseases, and auto-immune diseases will be systematically considered...
October 16, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/29046206/-clinical-and-genetic-analyses-of-a-family-with-atypical-nonketotic-hyperglycinemia-caused-by-compound-heterozygous-mutations-in-the-gldc-gene
#14
Tie-Jia Jiang, Jing-Jing Jiang, Jia-Lu Xu, Jing Zhen, Pei-Fang Jiang, Feng Gao
Nonketotic hyperglycinemia (NKH) is an autosomal recessive hereditary disease caused by a defect in the glycine cleavage system and is classified into typical and atypical NKH. Atypical NKH has complex manifestations and is difficult to diagnose in clinical practice. This article reports a family of NKH. The parents had normal phenotypes, and the older brother and the younger sister developed this disease in the neonatal period. The older brother manifested as intractable epilepsy, severe spastic diplegia, intellectual disability, an increased level of glycine in blood and cerebrospinal fluid, an increased glycine/creatinine ratio in urine, and an increased ratio of glycine concentration in cerebrospinal fluid and blood...
October 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/29044699/diabetic-striatopathy-manifesting-as-severe-consciousness-disturbance-with-no-involuntary-movements
#15
Hiroki Sato, Makoto Hamano, Etsuko Fushimi, Toshiaki Takahashi, Yohei Horikawa, Satoru Horiguchi
BACKGROUND: Diabetic striatopathy, one of the complications of diabetes mellitus, is characterized by involuntary movements, including hemichorea and hemiballismus, and the presence of hyperintense lesions on T1-weighted magnetic resonance imaging of the striatum. CASE REPORT: We present a case of diabetic striatopathy manifesting as severe consciousness disturbance without chorea or ballismus. A 58-year-old man was admitted to our hospital in a state of unconsciousness...
October 17, 2017: Diabetic Medicine: a Journal of the British Diabetic Association
https://www.readbyqxmd.com/read/29028509/delayed-onset-mri-findings-in-acute-chorea-related-to-anoxic-brain-injury
#16
Mehdi Ghasemi, Firas Kaddouh, Anindita Deb, Margaret A Owegi
Anoxic brain injury can manifest with various abnormal movements. We describe acute chorea in a young patient with anoxic brain injury due to chlordiazepoxide toxicity who had delayed radiographic lesions in bilateral globus pallidus. Although brain MRI 8days after the anoxic event was unremarkable, repeat brain MRI 15days after the event showed T2 hyperintensities and enhancement within the bilateral globus pallidi. It is possible that MRI brain findings of bilateral basal ganglia lesions may appear later than onset of chorea in anoxic brain injury...
October 5, 2017: Clinical Imaging
https://www.readbyqxmd.com/read/28983422/speech-language-pathology-evaluation-and-management-of-hyperkinetic-disorders-affecting-speech-and-swallowing-function
#17
REVIEW
Julie M Barkmeier-Kraemer, Heather M Clark
BACKGROUND: Hyperkinetic dysarthria is characterized by abnormal involuntary movements affecting respiratory, phonatory, and articulatory structures impacting speech and deglutition. Speech-language pathologists (SLPs) play an important role in the evaluation and management of dysarthria and dysphagia. This review describes the standard clinical evaluation and treatment approaches by SLPs for addressing impaired speech and deglutition in specific hyperkinetic dysarthria populations. METHODS: A literature review was conducted using the data sources of PubMed, Cochrane Library, and Google Scholar...
2017: Tremor and Other Hyperkinetic Movements
https://www.readbyqxmd.com/read/28979614/-exceptionally-good-response-to-sodium-valproate-in-patients-with-recurrent-sydenham-s-chorea
#18
Siham Bouchal, Ouarda Ouali, Mouhamed Faouzi Belahsen
Sydenham's chorea is the most common acquired cause of chorea in the third world. We report a case of recurrent chorea successfully treated with sodium valproate. Miss A.C, aged 16, with a history of recurrent angina reported an episode of choreic movements 2 years before requiring treatment with haloperidol and prevention of rheumatic fever. The patient interrupted her treatment with occurrence of a relapse a few months later. Brain MRI and transthoracic ultrasound were normal. Preventive treatment with extencilline and haloperidol was restarted without any improvement, hence the treatment with sodium valproate...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28971447/huntington-disease-genetics-prevention-and-therapy-approaches
#19
Christos Yapijakis
Huntington's chorea or Huntington disease (HD) is a late-onset autosomal dominant neurodegenerative disorder caused by a trinucleotide repeat expansion. The multidisciplinary study of HD has been the focus of an international collaborating effort of basic and applied research for several decades. HD was the first human genetic disease mapped using linkage analysis of DNA polymorphisms and became a paradigm for scores of genes mapped in the same manner. Presymptomatic and prenatal testing have been available for HD families in the last 30 years, following genetic counseling and careful bioethical guidelines...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28969740/unilateral-thalamic-infarct-presenting-as-a-convulsive-seizure
#20
Rajesh Kumar, Hazim Brohi, Afshan Mughul
Lesions of the thalamus and those extending into midbrain can cause various types of movement disorders such as dystonia, asterixis and ballism-chorea. Seizures are rare manifestation of thalamic disorder. Occurrence of seizures in bilateral thalamic infarct has been reported; but seizures in unilateral thalamic infarct have been reported very rarely. Literature review showed only single case of perinatal unilateral thalamic infarct presenting with seizures. We are reporting a unique case of convulsive seizure at the onset of unilateral thalamic infarct in an adult male, which has never been reported to the best of our knowledge...
September 2017: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
keyword
keyword
20374
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"