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https://www.readbyqxmd.com/read/26190011/atypical-presentation-of-costeff-syndrome-severe-psychomotor-involvement-and-electrical-status-epilepticus-during-slow-wave-sleep
#1
Nirit Carmi, Dorit Lev, Esther Leshinsky-Silver, Yair Anikster, Lubov Blumkin, Sara Kivity, Tally Lerman-Sagie, Ayelet Zerem
BACKGROUND: Costeff syndrome or OPA3-related 3-methylglutaconic aciduria is an autosomal recessive neurodegenerative disorder characterized by early onset optic atrophy and choreoathetosis with later onset of ataxia and spasticity. Costeff syndrome is prevalent among Iraqi Jews. METHODS: We describe a 5 year old girl from Syrian Jewish origin with an atypical presentation of Costeff syndrome. RESULTS: The patient presented with asymmetric optic atrophy, severe dystonia and choreoathetosis and global developmental regression at the age of 7 months; no achievement of independent walking and only minimal speech; and appearance of electrical status epilepticus during slow wave sleep in the second year of life with further deterioration...
November 2015: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/25546440/where-does-chorea-come-from-cortical-excitability-findings-challenge-classic-pathophysiological-concepts
#2
EDITORIAL
Mark Hallett, Jose Obeso
No abstract text is available yet for this article.
February 2015: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/25139915/psychosis-with-huntington-s-disease-role-of-antipsychotic-medications
#3
Jonathan Ding, Amin Muhammad Gadit
This is a case of a 60-year-old man who presented with a 6-month history of increasing agitation and emotional volatility. His family brought him to the emergency room as they were concerned about his threatening and aggressive behaviour. The patient was initially incoherent and uncooperative. During the interview, the patient's family revealed that he had a previous diagnosis of Huntington's disease; there was also a family history of personality changes preceding Huntington's chorea. The patient was admitted to the psychiatric inpatient unit and started on low-dose risperidone...
2014: BMJ Case Reports
https://www.readbyqxmd.com/read/25024581/undulating-tongue-in-wilson-s-disease
#4
M Nagappa, S Sinha, Js Saini, Ps Bindu, Ab Taly
We report an unusual occurrence of involuntary movement involving the tongue in a patient with confirmed Wilson's disease (WD). She manifested with slow, hypophonic speech and dysphagia of 4 months duration, associated with pseudobulbar affect, apathy, drooling and dystonia of upper extremities of 1 month duration. Our patient had an uncommon tongue movement which was arrhythmic. There was no feature to suggest tremor, chorea or dystonia. It might be described as athetoid as there was a writhing quality, but of lesser amplitude...
April 2014: Annals of Indian Academy of Neurology
https://www.readbyqxmd.com/read/24919927/increased-intracortical-inhibition-in-hyperglycemic-hemichorea-hemiballism
#5
Jie-Yuan Li, Robert Chen
Hemichorea-hemiballism (HC-HB) in uncontrolled diabetes mellitus is an uncommon manifestation of hyperglycemia. The pathophysiology of hyperglycemic HC-HB is not well understood. A previous report showed increased intracortical inhibition in the motor cortex in a patient with diabetes with HC-HB. The objective of this study is to investigate motor cortex excitability in patients with hyperglycemic HC-HB. We hypothesized that intracortical inhibition measured with transcranial magnetic stimulation, which likely reflects the excitability of cortical γ-aminobutyric acid (GABA)ergic circuits, would be impaired in patients with hyperglycemic HC-HB...
February 2015: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/24909435/changes-in-motor-cortical-excitability-in-patients-with-sydenham-s-chorea
#6
Eman M Khedr, Mohamed A Ahmed, Anwar M Ali, Reda Badry, John C Rothwell
BACKGROUND AND PURPOSE: The neurophysiological characteristics of motor cortex have been well characterized in patients with Huntington's disease. We present the first data on cortical excitability in patients with Sydenham's chorea. METHODS: Motor cortex excitability was examined using transcranial magnetic stimulation in 16 patients in the early clinical stages of Sydenham's chorea and in 17 age- and sex-matched control subjects. Investigations included resting and active motor threshold, motor evoked potential, input-output curves, contralateral silent period, and transcallosal inhibition...
February 2015: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/24356039/sympathomimetic-syndrome-choreoathetosis-and-acute-kidney-injury-following-bath-salts-injection
#7
Grerk Sutamtewagul, Vineeta Sood, Kenneth Nugent
"Bath salts" is a well known street drug which can cause several cardiovascular and neuropsychiatric symptoms. However, only one case of acute kidney injury has been reported in the literature. We present a case with sympathomimetic syndrome, choreoathetosis, gustatory and olfactory hallucinations, and acute kidney injury following the use of bath salts. A 37-year-old man with past medical history of hypertension and depression was brought to the emergency center with body shaking. Three days before admission he injected 3 doses of bath salts intravenously and felt eye pain with blurry vision followed by a metallic taste, strange smells, profuse sweating, and body shaking...
January 2014: Clinical Nephrology
https://www.readbyqxmd.com/read/23518044/akathisia-in-association-with-herpes-simplex-encephalitis-relapse-and-opercular-syndrome-in-children
#8
Ozan Kocak, Coskun Yarar, Ayten Yakut, Arzu Ekici, Sevgi Yimenicioglu, Suzan Saylisoy
We report a 2-year-old boy with herpes simplex virus type 1 encephalitis (HSE) and opercular syndrome who presented with clinical relapse characterized by chorea-like involuntary movements that suggest akathisia. The patient initially presented with multiple focal seizures that cause epilepsia partialis continua, polymerase chain reaction (PCR) for herpes simplex virus type 1 was positive. He developed hypersalivation, speech and swallowing difficulties within 30days. Based on these findings the patient was diagnosed as having opercular syndrome due to HSE...
February 2014: Brain & Development
https://www.readbyqxmd.com/read/23280482/analysis-of-cyp2d6-genotype-and-response-to-tetrabenazine
#9
Raja Mehanna, Christine Hunter, Anthony Davidson, Joohi Jimenez-Shahed, Joseph Jankovic
Tetrabenazine is effective in the treatment of the chorea associated with Huntington disease and other hyperkinetic movement disorders. Following oral administration, tetrabenazine is hepatically transformed into 2 active metabolites that are CYP2D6 substrates. There are 4 CYP2D6 genotypes: poor metabolizers, intermediate metabolizers, extensive metabolizers, and ultrarapid metabolizers. CYP2D6 genotyping was performed on sequential subjects treated with tetrabenazine, but results were not known at the time of titration...
February 2013: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/22314929/therapeutics-in-huntington-s-disease
#10
Annie Killoran, Kevin M Biglan
OPINION STATEMENT: There is no specific treatment for Huntington's disease (HD). Its many symptoms of motor, psychiatric, and cognitive deterioration are managed with symptomatic relief, rehabilitation, and support. The only drug approved by the US Food and Drug Administration (FDA) for the treatment of HD is an antichoreic agent, tetrabenazine, but this drug is used sparingly because of uneasiness regarding its propensity to cause depression and suicidality in this population, which is already at risk for these complications...
February 8, 2012: Current Treatment Options in Neurology
https://www.readbyqxmd.com/read/21397177/azithromycin-induced-agitation-and-choreoathetosis
#11
Osman Farooq, Zebunnissa Memon, Sasko D Stojanovski, Howard S Faden
We report a child who developed agitation and choreoathetoid movements with azithromycin therapy on 2 separate occasions. In both instances, the symptoms resolved when the antibiotic was discontinued. By means of the Naranjo adverse drug reaction probability scale, we classified this event as a probable adverse drug reaction (score of 6 points). To our knowledge, this is the first published case of azithromycin-induced agitation with choreoathetosis. Because this is a widely used medication for many common infectious conditions, including otitis media and pneumonia, this potential serious adverse reaction should be considered...
April 2011: Pediatric Neurology
https://www.readbyqxmd.com/read/19577909/-acute-poststreptoccocal-chorea-an-atypical-postoperative-reaction-following-cardiac-surgery-for-mitral-valvulopathy
#12
V Brousse, N Bahi-Buisson, V Lucet, A Deloche, V Abadie
We report on a 12-year-old patient from Congo who presented acute chorea following cardiac surgery for poststreptococcal mitral valvulopathy. She showed severe and asymmetrical chorea, associated with motor impersistence and agitation. Biological investigations disclosed inflammatory signs and brain MRI was normal. Due to the negative results of the biological and morphological investigations, the diagnosis of Sydenham chorea was suspected. High doses of oral steroids resulted in a dramatic improvement of the chorea as well as the behavior disturbance within 1 month...
August 2009: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/19344915/complex-movement-disorders-in-primary-antiphospholipid-syndrome-a-case-report
#13
Miryam Carecchio, Cristoforo Comi, Claudia Varrasi, Alessandro Stecco, Pier Paolo Sainaghi, Kailash Bhatia, Alessandro Carriero, Roberto Cantello, Francesco Monaco
Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by recurrent thrombotic events and/or pregnancy morbidity that may be isolated (Primary APS) or associated with other diseases, mainly of autoimmune origin (Secondary APS). A variety of neurological symptoms may occur in association with the disease, including movement disorders. We report on a 79 year old woman with an unremarkable past medical history who progressively developed psychomotor agitation and insomnia through a period of four months, followed by an acute onset complex hyperkinetic syndrome with chorea, focal left foot dystonia, oral dyskinesias and severe speech impairment...
June 15, 2009: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/18439931/-esophageal-achalasia-sleep-disorders-and-chorea-in-a-tauopathy-without-ophthalmoplegia-parkinsonian-syndrome-nor-dementia-progressive-supranuclear-palsy-clinicopathological-study
#14
E Kaphan, J-F Pellissier, M Rey, D Robert, M Auphan, A Ali Chérif
CONTEXT: Progressive supranuclear palsy (PSP) is classically characterized by supranuclear ophthalmoplegia, paroxysmal imbalance with backward falling, axial dystonia, rigidity, pseudobulbar palsy and cognitive dysfunction. However, incomplete or atypical clinical presentation has been previously reported, but in all these cases, the patients had at least one of the main clinical features of the disease (ophthalmoplegia, parkinsonian syndrome or cognitive dysfunction). CASE REPORT: A 60-year-old woman presented with nocturnal agitation and choreiform movements...
April 2008: Revue Neurologique
https://www.readbyqxmd.com/read/18175786/the-neurotoxicity-and-safety-of-treatment-with-cefepime-in-patients-with-renal-failure
#15
J Sonck, G Laureys, D Verbeelen
BACKGROUND: Cases of cefepime neurotoxicity have been sporadically reported in patients with renal failure. The neurotoxicity of cefepime might be underestimated and the frequency of its neurotoxic effects may be insufficiently recognized. METHODS: We retrospectively reviewed the files of patients with renal failure who were treated with cefepime and who developed neurological complications. RESULTS: All 8 patients developed decreased conscience, confusion, agitation, global aphasia, myoclonus, chorea-athetosis, convulsions and coma...
March 2008: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/17533963/-diphenhydramine-poisoning-presented-with-psychotic-like-symptoms-and-choreic-involuntary-movement-report-of-two-cases
#16
Kikuko Shobugawa, Yasuo Hirose, Yasushi Hori, Mika Ito, Manami Fujisawa, Ken Kumagai
We encountered two cases of diphenhydramine-containing hypnotics overdose, exhibiting delirium and involuntary choreic movement. In case 1, a 32-year-old man ingested 24 tablets of Drewell, each containing 25 mg diphenhydramine, in a suicidal attempt. About four hours after ingestion, he showed generalized convulsion, delirium, and involuntary choreic movements. Intravenous diazepam was ineffective and he was sedated with continuous infusion of propofol. About 15 hours after ingestion and on discontinuing the sedation, he became alert and did not show any neurological abnormality...
April 2007: Chūdoku Kenkyū: Chūdoku Kenkyūkai Jun Kikanshi, the Japanese Journal of Toxicology
https://www.readbyqxmd.com/read/17251884/cisatracurium-in-weakening-doses-assists-in-weaning-from-sedation-and-withdrawal-following-extended-use-of-inhaled-isoflurane
#17
Mehrengise K Cooper, Scot T Bateman
OBJECTIVE: Isoflurane was used to treat a patient with status asthmaticus refractive to standard therapeutic measures. The patient developed a significant withdrawal syndrome when the isoflurane was weaned. A case is reported here where this withdrawal syndrome was treated successfully by using a weakening dose neuromuscular blockade with cisatracurium. DESIGN: Case report. SETTING: Pediatric critical care unit. PATIENT: A 4-yr-old girl with severe reactive airways disease...
January 2007: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/16476934/tetrabenazine-as-antichorea-therapy-in-huntington-disease-a-randomized-controlled-trial
#18
RANDOMIZED CONTROLLED TRIAL
(no author information available yet)
BACKGROUND: Tetrabenazine (TBZ) selectively depletes central monoamines by reversibly binding to the type 2 vesicular monoamine transporter. Open-label reports indicate TBZ is effective in treating chorea. OBJECTIVE: To examine the safety, efficacy, and dose tolerability of TBZ for treating chorea in Huntington disease (HD). METHODS: The authors randomized 84 ambulatory patients with HD to receive TBZ (n = 54) or placebo (n = 30) for 12 weeks...
February 14, 2006: Neurology
https://www.readbyqxmd.com/read/15261885/distinguishing-pandas-from-sydenham-s-chorea-case-report-and-review-of-the-literature
#19
REVIEW
Ronald van Toorn, Henriete H Weyers, Johan F Schoeman
Children with Sydenham's chorea and PANDAS (Pediatric autoimmune neuropsychiatric disorders associated with streptococcal throat infections) share an array of neuropsychiatric symptoms and distinguishing one from the other, especially at onset can prove challenging. It is, however, important to distinguish between these two post-streptococcal disorders since their response to therapy differs. Children with Sydenham's chorea require long-term benzathine penicillin prophylaxis to reduce the risk of rheumatic heart disease...
2004: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/11511702/neuropsychiatric-aspects-of-huntington-s-disease
#20
J S Paulsen, R E Ready, J M Hamilton, M S Mega, J L Cummings
OBJECTIVE: Neuropsychiatric symptoms are common in Huntington's disease and have been considered its presenting manifestation. Research characterising these symptoms in Huntington's disease is variable, however, encumbered by limitations within and across studies. Gaining a better understanding of neuropsychiatric symptoms is essential, as these symptoms have implications for disease management, prognosis, and quality of life for patients and caregivers. METHOD: Fifty two patients with Huntington's disease were administered standardised measures of cognition, psychiatric symptoms, and motor abnormalities...
September 2001: Journal of Neurology, Neurosurgery, and Psychiatry
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