{"orpha":97355,"name":"Caribbean parkinsonism","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Atypical parkinsonism in the Caribbean"],"definition":"Parkinsonism with dementia of Guadeloupe is characterised by symmetrical bradykinesia, predominantly axial rigidity, postural instability with early falls and cognitive decline with prominent features of frontal lobe dysfunction.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-97355/","orphanet_url":"https://www.orpha.net/en/disease/detail/97355","names":{"fr":{"name":"Parkinsonisme des Caraïbes","synonyms":["Parkinsonisme atypique des Caraïbes"],"definition":"Le parkinsonisme atypique de Guadeloupe est caractérisé par une bradykinésie symétrique, une rigidité musculaire à prédominance axiale, une instabilité posturale avec des chutes précoces et un déclin cognitif avec des signes de dysfonction des lobes frontaux."},"de":{"name":"Karibisches Parkinson-Syndrom","synonyms":["Atypisches Parkinson-Syndrom der Karibik"],"definition":"Der Parkinsonismus mit Demenz vom Typ Guadeloupe ist gekennzeichnet durch symmetrische Bradykinesie, vorwiegend axiale Rigidität, instabile Haltung mit früher Sturzneigung und durch kognitiven Abbau mit prominenten Zeichen einer Frontalhirnstörung."},"es":{"name":"Parkinsonismo caribeño","synonyms":["Parkinsonismo atípico en el Caribe"],"definition":"El parkinsonismo con demencia de Guadalupe está caracterizado por bradicinesia simétrica, rigidez principalmente axial, inestabilidad postural con caídas precoces y deterioro cognitivo con características destacadas de disfunción del lóbulo frontal."},"it":{"name":"Parkinsonismo con demenza della Guadalupa"},"nl":{"name":"Caribisch parkinsonisme","synonyms":["Atypisch parkinsonisme in de Caraïben"],"definition":"Parkinsonisme met dementie, Guadeloupe-type wordt gekenmerkt door symmetrische bradykinesie, overwegend axiale rigiditeit, posturale instabiliteit met vroege vallen, en cognitieve aftakeling met prominente kenmerken van disfunctie van frontale kwab."},"pt":{"name":"Parkinsonismo com demência de Guadeloupe"},"pl":{"name":"Odmiana karaibska choroby Parkinsona","synonyms":["Atypical parkinsonism in the Caribbean"]},"cs":{"name":"Guadeloupský parkinsonský komplex","synonyms":["Atypical parkinsonism in the Caribbean"]},"uk":{"name":"Карибський паркінсонізм","synonyms":["Атиповий паркінсонізм у Карибському басейні"],"definition":"Parkinsonism with dementia of Guadeloupe is characterised by symmetrical bradykinesia, predominantly axial rigidity, postural instability with early falls and cognitive decline with prominent features of frontal lobe dysfunction."},"zh":{"name":"加勒比海帕金森病","synonyms":["加勒比海非典型帕金森病"],"definition":"瓜德罗普氏痴呆帕金森病的特点是对称性运动迟缓，主要表现为轴性僵硬，姿势不稳伴早期跌倒，认知功能减退，额叶功能障碍是其显著特征。"},"tr":{"name":"Karayip parkinsonizmi","synonyms":["Karayipler'de atipik parkinsonizm"]}},"xrefs":[{"source":"ICD-10","code":"F02.3*","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"G20+","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"8A00.1Y","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0019977","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4275027","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98006,"name":"Rare neurologic disease"},"parents":[{"orpha":89043,"name":"Rare dementia","classifications":[181]},{"orpha":306666,"name":"Rare parkinsonian syndrome due to neurodegenerative disease","classifications":[181]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Adult","Elderly"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2018-06-11","source":"29525296[PMID]_18941145[PMID]_18816693[PMID]_17303592[PMID]_1210440304[PMID]_217621[PMID]_11912113[PMID]","terms":[{"hpo":"HP:0002067","label":"Bradykinesia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001300","label":"Parkinsonism","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002063","label":"Rigidity","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0012332","label":"Abnormal autonomic nervous system physiology","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002345","label":"Action tremor","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002186","label":"Apraxia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0005341","label":"Autonomic bladder dysfunction","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002120","label":"Cerebral cortical atrophy","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000726","label":"Dementia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001332","label":"Dystonia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003458","label":"EMG: myopathic abnormalities","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000727","label":"Frontal lobe dementia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000738","label":"Hallucinations","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000571","label":"Hypometric saccades","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0007045","label":"Midline brain calcifications","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001336","label":"Myoclonus","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001278","label":"Orthostatic hypotension","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0030902","label":"Palmomental reflex","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002172","label":"Postural instability","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0007240","label":"Progressive gait ataxia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002193","label":"Pseudobulbar behavioral symptoms","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002360","label":"Sleep abnormality","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012753","label":"T2 hypointense basal ganglia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002119","label":"Ventriculomegaly","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000511","label":"Vertical supranuclear gaze palsy","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0010549","label":"Weakness due to upper motor neuron dysfunction","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100315","label":"Lewy bodies","frequency":"Excluded (0%)","criteria":null}]}}