HTML Microdata document

This HTML5 document contains 416 embedded RDF statements represented using HTML+Microdata notation.

The embedded RDF content will be recognized by any processor of HTML5 Microdata.

Namespace Prefixes

Prefix	IRI
dbpedia-da	http://da.dbpedia.org/resource/
dbt	http://dbpedia.org/resource/Template:
wikipedia-en	http://en.wikipedia.org/wiki/
dbr	http://dbpedia.org/resource/
dbpedia-ar	http://ar.dbpedia.org/resource/
n19	http://commons.wikimedia.org/wiki/Special:FilePath/
dbpedia-fr	http://fr.dbpedia.org/resource/
n14	https://www.ncbi.nlm.nih.gov/bookshelf/
n37	http://dbpedia.org/resource/List_of_ICD-9_codes_320–389:
dcterms	http://purl.org/dc/terms/
rdfs	http://www.w3.org/2000/01/rdf-schema#
rdf	http://www.w3.org/1999/02/22-rdf-syntax-ns#
n6	http://d-nb.info/gnd/
n27	http://dbpedia.org/resource/File:
dbp	http://dbpedia.org/property/
n26	https://sp-foundation.org/
xsdh	http://www.w3.org/2001/XMLSchema#
dbo	http://dbpedia.org/ontology/
dbpedia-pt	http://pt.dbpedia.org/resource/
umbel-rc	http://umbel.org/umbel/rc/
n31	http://uz.dbpedia.org/resource/
dbc	http://dbpedia.org/resource/Category:
dbpedia-de	http://de.dbpedia.org/resource/
n35	http://www.acnr.co.uk/JF07/
dbpedia-pl	http://pl.dbpedia.org/resource/
yago	http://dbpedia.org/class/yago/
dbpedia-ru	http://ru.dbpedia.org/resource/
wikidata	http://www.wikidata.org/entity/
gold	http://purl.org/linguistics/gold/
dbpedia-nl	http://nl.dbpedia.org/resource/
yago-res	http://yago-knowledge.org/resource/
n17	https://global.dbpedia.org/id/
dbpedia-it	http://it.dbpedia.org/resource/
n40	http://www4.wiwiss.fu-berlin.de/diseasome/resource/diseases/
dbpedia-ca	http://ca.dbpedia.org/resource/
prov	http://www.w3.org/ns/prov#
foaf	http://xmlns.com/foaf/0.1/
dbpedia-zh	http://zh.dbpedia.org/resource/
n9	http://www.w3.org/2006/03/wn/wn20/instances/
freebase	http://rdf.freebase.com/ns/
dbpedia-es	http://es.dbpedia.org/resource/
owl	http://www.w3.org/2002/07/owl#

Statements

Subject Item: dbr:Robin_Surgeoner
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Roman_Polianskyi
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:FA2H
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:FARS2
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:List_of_abbreviations_for_diseases_and_disorders
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:List_of_diseases_(H)
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:MASA_syndrome
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Hereditary_Spastic_Paraplegia
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia
dbo:wikiPageRedirects: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:SPG23
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: n37:_diseases_of_the_nervous_system_and_sense_organs
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:List_of_genetic_disorders
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Rhizotomy
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Cytochrome_P450_omega_hydroxylase
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:ESCRT
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Intellectual_disability-spasticity-ectrodactyly_syndrome
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:L1_syndrome
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Vesicular_transport_adaptor_protein
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:List_of_neurological_conditions_and_disorders
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:List_of_neuromuscular_disorders
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:SPG5B_(gene)
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia
dbo:wikiPageRedirects: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Neuropathy_target_esterase
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:REEP2
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Mitochondrion
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Yop1p
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:VPS37A
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Andrea_Ballabio
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Kufor–Rakeb_syndrome
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:KIAA0196
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:20-Hydroxyeicosatetraenoic_acid
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Distal_hereditary_motor_neuronopathies
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Distal_hereditary_motor_neuropathy_type_V
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Familial_spastic_paralysis
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia
dbo:wikiPageRedirects: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:RIPK5
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Adolf_Strümpell
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia
dbp:knownFor: dbr:Hereditary_spastic_paraplegia
dbo:knownFor: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Ciliopathy
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Foot_drop
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:KCNA2
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:KIF1A
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Konzo
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Familial_spastic_paraparesis
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia
dbo:wikiPageRedirects: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:HSP
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia
dbo:wikiPageDisambiguates: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:AP5B1
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:AP5M1
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:AP5S1
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:AP5Z1
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Griffin_LaMarre
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia
dbp:disability: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Hereditary_sensory_and_autonomic_neuropathy_type_I
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Hereditary_spastic_paraplegia
rdf:type: yago:WikicatSyndromes yago:Complex105870365 yago:NervousDisorder114084880 yago:WikicatMusculoskeletalDisorders yago:Disease114070360 yago:WikicatGeneticDisordersWithNoOMIM yago:WikicatGeneticDisorders umbel-rc:AilmentCondition yago:GeneticDisease114151139 yago:Imperfection114462666 yago:PhysicalCondition114034177 dbo:Disease dbo:Band yago:Attribute100024264 yago:Condition113920835 yago:Illness114061805 yago:IllHealth114052046 yago:Content105809192 yago:Cognition100023271 yago:Whole105869584 yago:PathologicalState114051917 yago:Concept105835747 yago:PsychologicalFeature100023100 yago:WikicatCytoskeletalDefects yago:Abstraction100002137 yago:Defect114464005 yago:Idea105833840 owl:Thing yago:State100024720 yago:Disorder114052403 wikidata:Q12136 yago:Syndrome105870790 yago:WikicatNeurodegenerativeDisorders yago:WikicatNeurologicalDisorders
rdfs:label: Paraparesia espástica familiar Спастическая спинальная параплегия Síndrome de Strumpell Lorrain Paraplegia espàstica hereditària Choroba Strümplla Paraparesi spastica ereditaria Hereditaire spastische paraparese Spastische Paraplegie 遺傳性痙攣性下半身麻痺 Hereditary spastic paraplegia شلل نصفي تشنجي موروث Paraplégie spastique familiale
rdfs:comment: Hereditaire spastische paraparese (HSP), ook wel de ziekte van Strümpell of ziekte Strümpell-Lorrain genoemd, is een dominant-erfelijke spastische paraparese. De symptomen, de intensiteit en de leeftijd waarop de eerste symptomen beginnen, kunnen sterk uiteenlopen, zelfs binnen één familie. De ziekte is niet te genezen. Hereditary spastic paraplegia (HSP) is a group of inherited diseases whose main feature is a progressive gait disorder. The disease presents with progressive stiffness (spasticity) and contraction in the lower limbs. HSP is also known as hereditary spastic paraparesis, familial spastic paraplegia, French settlement disease, Strumpell disease, or Strumpell-Lorrain disease. The symptoms are a result of dysfunction of long axons in the spinal cord. The affected cells are the primary motor neurons; therefore, the disease is an upper motor neuron disease. HSP is not a form of cerebral palsy even though it physically may appear and behave much the same as spastic diplegia. The origin of HSP is different from cerebral palsy. Despite this, some of the same anti-spasticity medications used in spast Die spastischen Paraplegien (SPG), auch spastische Spinalparalysen genannt, stellen eine Gruppe neurodegenerativer Erkrankungen dar, die sporadisch oder hereditär (erblich) auftreten. Die hereditären spastischen Paraplegien (kurz HSP, auch Strümpell-Lorrain-Syndrom) sind genetisch heterogen, weil Mutationen in unterschiedlichen Genen die Erkrankung auslösen können. Der Erbgang ist autosomal-dominant, autosomal-rezessiv oder X-chromosomal-rezessiv. Es handelt sich um eine Degeneration der Pyramidenseiten- und Vorderstrangbahnen. A síndrome Strumpell-Lorrain (SSL; também grafada como Strumpell Lorraine), ou Paraparesia espástica familiar, é um grupo de doenças hereditárias, cuja principal característica é a rigidez progressiva e contração (espasticidade) nos membros inferiores, como resultado de uma lesão ou disfunção dos nervos. A paraparesia espástica familiar foi descrita pela primeira vez em 1883 por , neurologista alemão, e mais tarde foi descrita de forma mais ampla em 1888, por Maurice Lorrain, um médico francês. La paraplegia espàstica hereditària (PEH) és un grup de malalties genètiques, la característica principal de les quals és un trastorn progressiu de la marxa. La malaltia presenta una rigidesa progressiva (espasticitat) i una contracció en les extremitats inferiors. La PEH també es coneix com a paraparèsia espàstica hereditària, paraplegia espàstica familiar o síndrome de Strumpell-Lorrain. La malaltia de Strumpell és la forma més habitual de la PEH. La paraparesi spastica ereditaria o familiare, anche chiamata sindrome o malattia di Strumpell-Lorrain, è un gruppo di malattie ereditarie caratterizzate principalmente da rigidità e spasticità della muscolatura degli arti inferiori conseguente a una lesione o a una disfunzione dei nervi. Fu identificata per la prima volta dal neurologo tedesco Adolph Strümpell nel 1883 e successivamente descritta più approfonditamente dal medico francese Maurice Lorrain, nel 1888. El Síndrome de Strumpell-Lorrain", también conocido como Paraplejía Espástica Hereditaria', Paraparesia Espástica Hereditaria, Paraplejía Espástica Familiar o Síndrome del Asentamiento Francés, es un grupo de padecimientos hereditarios cuya característica principal es la rigidez progresiva y contracción (espasticidad) en los miembros inferiores, como resultado del deterioro o disfunción de los nervios. 遺傳性痙攣性下半身麻痺是一種遺傳病，其會導致下肢無力與，複雜病症甚至會有痙攣、失智、等。此遺傳病的盛行率佔人口的10萬分之1.27。遺傳方面，其遺傳方式為並未確定，可能為體染色體顯性遺傳、體染色體隱性遺傳或是X染色體性聯隱性遺傳。 Une paraplégie spastique familiale ou maladie de Strümpell-Lorrain est un ensemble de maladies rares, de transmission génétique, qui affecte le système nerveux central. Elle se caractérise par une faiblesse musculaire très progressive atteignant les membres inférieurs s’accompagnant d’une hypertonie musculaire entraînant une raideur du membre inférieur. Elle peut conduire à l'emploi de cannes, et, dans certains cas, d'un fauteuil roulant.Cette maladie n'est pas aujourd'hui curable. Le traitement est symptomatique. Normalement, elle n'affecte pas l'espérance de vie. الشلل النصفي التشنجي الموروث (بالإنجليزية: Hereditary spastic paraplegia)‏ أو اختصارا HSP، هو مجموعة من الأمراض الوراثية النادرة، والتي تتمثل إحدى سماتها الرئيسية في اضطراب تدريجي في المشي. يظهر المرض على شكل تصلب تدريجي (التشنج) وانكماش في الأطراف السفلية. يعرف هذا المرض أيضا باسم الخزل السفلي التشنجي الوراثي، أو مرض المستوطنات الفرنسية، أو أيضا مرض سترومبل لورايين. الأعراض هي ناجة عن خلل وظيفي في محاور عصبية على مستوى النخاع الشوكي. بحيث تكون الخلايا المصابة هي الخلايا العصبية الحركية الرئيسية؛ وبالتالي، فهو مرض يصيب العصبونات الحركية العليا. Choroba Strümplla (dziedziczna paraplegia spastyczna, ang. HSP hereditary spastic paraplegia) – choroba neurodegeneracyjna, charakteryzująca się nasiloną paraparezą i dodatkowymi objawami ze strony nerwu wzrokowego, móżdżku i kory mózgowej. Chorobę jako pierwszy opisał niemiecki neurolog Adolf von Strümpell (1853-1925). Спастическая спинальная параплегия — это неврологическое заболевание верхнего двигательного нейрона (motor neuron disease).
foaf:name: Hereditary spastic paraplegia
dbp:name: Hereditary spastic paraplegia
foaf:depiction: n19:Overview_of_HSP_pathogenesis_on_cellular_level._Identified_affected_genes_in_each_pathway_are_depicted.jpg
dcterms:subject: dbc:Systemic_atrophies_primarily_affecting_the_central_nervous_system dbc:Rare_diseases dbc:Genetic_disorders_with_no_OMIM dbc:Cytoskeletal_defects
dbo:wikiPageID: 233735
dbo:wikiPageRevisionID: 1111635927
dbo:wikiPageWikiLink: dbr:Tibialis_anterior dbr:NIPA1 dbr:USP8 dbr:AMPD2 dbr:L1CAM dbr:National_Institutes_of_Health dbr:Kufor–Rakeb_syndrome dbr:KLC2 dbr:FARS2 dbr:Martsolf_syndrome dbr:MRI dbr:MEGDEL_syndrome dbr:KIF5A dbr:VPS37A dbr:REEP2 dbr:UCHL1 dbr:TFG_(gene) dbr:Pelizaeus–Merzbacher_disease dbr:Axon dbr:SPG11 dbr:Deafness dbr:Kyphoscoliosis_peptidase dbr:SPG15_(disease) dbr:Orthopedic_surgery dbr:SERAC1 dbr:CAPN1 dbr:Troyer_syndrome dbr:PLP1 dbr:Genetic_test dbr:Motor_neuron dbr:KIF1C dbr:Spastin dbr:Spastic_cerebral_palsy dbr:Paresthesia dbc:Systemic_atrophies_primarily_affecting_the_central_nervous_system dbr:ENTPD1 dbr:ALDH18A1 dbr:RNA_polymerase_III dbr:Oxybutynin_chloride dbr:SPAST dbr:PLA2G6 dbr:CCT5_(gene) dbr:SEMA3A dbr:Hamstring dbr:Cro_System dbr:CPT1C dbr:Tizanidine dbr:Upper_motor_neuron dbr:AP4E1 dbr:AP4S1 dbr:Clonazepam dbr:Spinal_cord dbr:Spasm dbr:Baclofen n27:Overview_of_HSP_pathogenesis_on_cellular_level._Identified_affected_genes_in_each_pathway_are_depicted.jpg dbr:Corticospinal_tract dbr:Spinocerebellar_tract dbr:Jaw_jerk dbr:Corpus_callosum dbr:Ataxia dbr:B4GALNT-1 dbr:Demyelination dbr:FA2H dbr:Anita_Harding dbr:Warburg_Micro_syndrome dbr:Spasticity dbr:Life_expectancy dbr:Fasciculus_gracilis dbr:KIAA0196 dbr:Mutations dbr:ARL6IP1 dbr:Cerebral_palsy dbr:Neurogenic_bladder_dysfunction dbr:Amyotrophy dbr:RAB3GAP2 dbr:Iliopsoas dbr:ZFYVE26 dbr:HSP60 dbr:MASA_syndrome dbr:Intellectual_disability dbr:Selective_dorsal_rhizotomy dbr:ATAD3A dbr:Lison_syndrome dbr:OMIM dbr:B4GALNT1 dbr:ATP13A2 dbr:Ichthyosis dbr:Dementia dbr:REEP1 dbr:Harel-Yoon_syndrome dbr:AP4B1 dbr:AP4M1 dbr:NRP1 dbr:Diazepam dbr:AP5Z1 dbr:ATL1 dbr:Multiple_sclerosis dbr:Leukodystrophy dbr:PGAP1 dbr:Granulin dbr:RIPK5 dbr:SLC16A2 dbr:SPG7 dbr:WDR48 dbr:ERLIN1 dbr:Tolterodine dbc:Rare_diseases dbr:ERLIN2 dbr:X-linked_recessive dbr:Pyramidal_tract dbr:Spastic_diplegia dbr:ACP33 dbr:Epilepsy dbr:Paraplegin dbc:Genetic_disorders_with_no_OMIM dbr:SLC33A1 dbr:Allan–Herndon–Dudley_syndrome dbr:PNPLA6 dbr:RTN2 dbr:MAG_(gene) dbr:KCNA2 dbr:GJC2 dbr:Differential_diagnosis dbr:KIF1A dbr:Atlastin dbr:Physical_therapy dbr:Neuropathy dbr:Spastic_ataxia_2 dbr:Adolph_Strümpell dbr:Antidepressants dbr:United_States dbr:GBA2 dbr:MARS_(gene) dbr:CYP7B1 dbr:Pallesthesia dbr:ZFR dbc:Cytoskeletal_defects dbr:CYP2U1 dbr:Locus_(genetics) dbr:White_matter dbr:Hereditary_sensory_neuropathy dbr:Heredity dbr:Botulinum_toxin dbr:ARSI dbr:BSCL2 dbr:Autosomal_dominant dbr:Autosomal_recessive dbr:Norway dbr:SPG20 dbr:ZFYVE27
dbo:wikiPageExternalLink: n14:br.fcgi%3Fbook=gene&part=hsp n26: n14:br.fcgi%3Fbook=gene&part=spg3a n35:ACNR_JF07_neurogenetics.pdf
owl:sameAs: n6:4077650-5 wikidata:Q657516 dbpedia-es:Síndrome_de_Strumpell_Lorrain dbpedia-zh:遺傳性痙攣性下半身麻痺 dbpedia-ca:Paraplegia_espàstica_hereditària n17:4qPBr dbpedia-fr:Paraplégie_spastique_familiale dbpedia-ru:Спастическая_спинальная_параплегия dbpedia-de:Spastische_Paraplegie dbpedia-pl:Choroba_Strümplla dbpedia-ar:شلل_نصفي_تشنجي_موروث n31:Umurtqa_spastik_paraplegiyasi yago-res:Hereditary_spastic_paraplegia dbpedia-da:Arvelig_spastisk_paraplegi dbpedia-pt:Paraparesia_espástica_familiar dbpedia-nl:Hereditaire_spastische_paraparese dbpedia-it:Paraparesi_spastica_ereditaria n40:1153 freebase:m.01hzg9
dbp:wikiPageUsesTemplate: dbt:OMIM dbt:Reflist dbt:Dunno dbt:Cn dbt:CNS_diseases_of_the_nervous_system dbt:Cite_journal dbt:Citation_needed dbt:PNS_diseases_of_the_nervous_system dbt:Authority_control dbt:Inherited_disorders_of_trafficking dbt:Infobox_medical_condition_(new) dbt:Medical_resources
dbo:thumbnail: n19:Overview_of_HSP_pathogenesis_on_cellular_level._Identified_affected_genes_in_each_pathway_are_depicted.jpg?width=300
dbp:diseasesdb: 33207
dbp:icd: G11.4 334.1
dbp:meshid: D015419
dbp:omim: 312920
dbp:orphanet: 685
dbo:abstract: Choroba Strümplla (dziedziczna paraplegia spastyczna, ang. HSP hereditary spastic paraplegia) – choroba neurodegeneracyjna, charakteryzująca się nasiloną paraparezą i dodatkowymi objawami ze strony nerwu wzrokowego, móżdżku i kory mózgowej. Chorobę jako pierwszy opisał niemiecki neurolog Adolf von Strümpell (1853-1925). Hereditaire spastische paraparese (HSP), ook wel de ziekte van Strümpell of ziekte Strümpell-Lorrain genoemd, is een dominant-erfelijke spastische paraparese. De symptomen, de intensiteit en de leeftijd waarop de eerste symptomen beginnen, kunnen sterk uiteenlopen, zelfs binnen één familie. De ziekte is niet te genezen. De aandoening moet niet worden verward met spastische diplegie / ziekte van Little, waarvan de symptomen overeenkomen maar waarbij de oorzaak in de hersenen is gelegen. Evenmin moet het verward worden met de niet-erfelijke aandoening primaire laterale sclerose, waarvan de klachten eveneens sterke overeenkomsten vertonen. Hereditary spastic paraplegia (HSP) is a group of inherited diseases whose main feature is a progressive gait disorder. The disease presents with progressive stiffness (spasticity) and contraction in the lower limbs. HSP is also known as hereditary spastic paraparesis, familial spastic paraplegia, French settlement disease, Strumpell disease, or Strumpell-Lorrain disease. The symptoms are a result of dysfunction of long axons in the spinal cord. The affected cells are the primary motor neurons; therefore, the disease is an upper motor neuron disease. HSP is not a form of cerebral palsy even though it physically may appear and behave much the same as spastic diplegia. The origin of HSP is different from cerebral palsy. Despite this, some of the same anti-spasticity medications used in spastic cerebral palsy are sometimes used to treat HSP symptoms. HSP is caused by defects in transport of proteins, structural proteins, cell-maintaining proteins, lipids, and other substances through the cell. Long nerve fibers (axons) are affected because long distances make nerve cells particularly sensitive to defects in these mentioned mechanisms. The disease was first described in 1880 by the German neurologist Adolph Strümpell. It was described more extensively in 1888 by Maurice Lorrain, a French physician. Due to their contribution in describing the disease, it is still called Strümpell-Lorrain disease in French-speaking countries. The term hereditary spastic paraplegia was coined by Anita Harding in 1983. A síndrome Strumpell-Lorrain (SSL; também grafada como Strumpell Lorraine), ou Paraparesia espástica familiar, é um grupo de doenças hereditárias, cuja principal característica é a rigidez progressiva e contração (espasticidade) nos membros inferiores, como resultado de uma lesão ou disfunção dos nervos. SSL não é uma forma de paralisia cerebral, embora fisicamente possam aparecer e se comportarem da mesma forma como, por exemplo, diplegia espástica. As origens do SSL são fenômenos completamente diferentes de paralisia cerebral. Apesar disso, alguns dos mesmos medicamentos anti-espasticidade utilizados na paralisia cerebral espástica são por vezes usados para tentar tratar a sintomatologia do SSL. A condição, por vezes, também afeta o nervo óptico e a retina do olho, faz com que a catarata, ataxia (falta de coordenação muscular), epilepsia, disfunção cognitiva, neuropatia periférica, e surdez. SSL é causada por defeitos nos mecanismos que transportam as proteínas e outras substâncias através da célula. Nervos longos são afetados porque eles têm que transportar material celular através de longas distâncias, e são particularmente sensíveis a defeitos de transporte celular. A paraparesia espástica familiar foi descrita pela primeira vez em 1883 por , neurologista alemão, e mais tarde foi descrita de forma mais ampla em 1888, por Maurice Lorrain, um médico francês. 遺傳性痙攣性下半身麻痺是一種遺傳病，其會導致下肢無力與，複雜病症甚至會有痙攣、失智、等。此遺傳病的盛行率佔人口的10萬分之1.27。遺傳方面，其遺傳方式為並未確定，可能為體染色體顯性遺傳、體染色體隱性遺傳或是X染色體性聯隱性遺傳。 Спастическая спинальная параплегия — это неврологическое заболевание верхнего двигательного нейрона (motor neuron disease). La paraplegia espàstica hereditària (PEH) és un grup de malalties genètiques, la característica principal de les quals és un trastorn progressiu de la marxa. La malaltia presenta una rigidesa progressiva (espasticitat) i una contracció en les extremitats inferiors. La PEH també es coneix com a paraparèsia espàstica hereditària, paraplegia espàstica familiar o síndrome de Strumpell-Lorrain. La malaltia de Strumpell és la forma més habitual de la PEH. La paraparesi spastica ereditaria o familiare, anche chiamata sindrome o malattia di Strumpell-Lorrain, è un gruppo di malattie ereditarie caratterizzate principalmente da rigidità e spasticità della muscolatura degli arti inferiori conseguente a una lesione o a una disfunzione dei nervi. Sebbene possa clinicamente assomigliarvi, la paraparesi spastica ereditaria non è una forma di paralisi cerebrale infantile ed è completamente diversa dal punto di vista eziologico; tuttavia vi sono farmaci in comune nel trattamento di entrambe le entità nosologiche. È invece maggiormente accomunabile alla diplegia spastica. Spesso risultano coinvolti il nervo ottico e la retina, così come è comune la cataratta; si può presentare inoltre con atassia, epilessia, ritardo mentale, neuropatia periferica e sordità. La paraparesi spastica ereditaria è causata da un difetto nel meccanismo di trasporto cellulare delle proteine e di altre molecole; sono coinvolti principalmente i nervi lunghi, perché il trasporto avviene per distanze piuttosto elevate e perché tali nervi sono particolarmente sensibili alle alterazioni del trasporto cellulare. Fu identificata per la prima volta dal neurologo tedesco Adolph Strümpell nel 1883 e successivamente descritta più approfonditamente dal medico francese Maurice Lorrain, nel 1888. Die spastischen Paraplegien (SPG), auch spastische Spinalparalysen genannt, stellen eine Gruppe neurodegenerativer Erkrankungen dar, die sporadisch oder hereditär (erblich) auftreten. Die hereditären spastischen Paraplegien (kurz HSP, auch Strümpell-Lorrain-Syndrom) sind genetisch heterogen, weil Mutationen in unterschiedlichen Genen die Erkrankung auslösen können. Der Erbgang ist autosomal-dominant, autosomal-rezessiv oder X-chromosomal-rezessiv. Es handelt sich um eine Degeneration der Pyramidenseiten- und Vorderstrangbahnen. Die spastischen Paraplegien sind durch zunehmende spastische Lähmungen in den Beinen gekennzeichnet. In weit fortgeschrittenem Stadium kann die betroffene Person für die folgende Lebenszeit auf die Rollstuhlbenutzung angewiesen sein. El Síndrome de Strumpell-Lorrain", también conocido como Paraplejía Espástica Hereditaria', Paraparesia Espástica Hereditaria, Paraplejía Espástica Familiar o Síndrome del Asentamiento Francés, es un grupo de padecimientos hereditarios cuya característica principal es la rigidez progresiva y contracción (espasticidad) en los miembros inferiores, como resultado del deterioro o disfunción de los nervios. El Síndrome de Strumpell-Lorrain no es una forma de parálisis cerebral aunque tenga una apariencia y comportamiento similar al de, por ejemplo, la diplejía espástica. Los orígenes del Síndrome de Strumpell-Lorrain son un fenómeno muy diferente a los de la parálisis cerebral. A pesar de esto, algunos de los medicamentos contra la espasticidad usados para tratar la parálisis cerebral espástica se utilizan ocasionalmente para el tratamiento de esta enfermedad. Padecer este síndrome puede llegar a afectar el nervio óptico y retina, causar cataratas, ataxia (falta de coordinación muscular), epilepsia, deterioro cognitivo, neuropatía periférica, y sordera. El Síndrome de Strumpell-Lorrain es causado por defectos en el transporte de proteínas y otras sustancias a través de la célula. Los nervios largos pueden presentar posibles disfuncionalidades por ser particularmente sensibles a los defectos del transporte celular. Une paraplégie spastique familiale ou maladie de Strümpell-Lorrain est un ensemble de maladies rares, de transmission génétique, qui affecte le système nerveux central. Elle se caractérise par une faiblesse musculaire très progressive atteignant les membres inférieurs s’accompagnant d’une hypertonie musculaire entraînant une raideur du membre inférieur. Elle peut conduire à l'emploi de cannes, et, dans certains cas, d'un fauteuil roulant.Cette maladie n'est pas aujourd'hui curable. Le traitement est symptomatique. Normalement, elle n'affecte pas l'espérance de vie. Elle est nommée d'après le neurologue allemand Adolf Strümpell, qui l'a décrite en 1880, et le docteur Lorrain, qui lui a consacré sa thèse en 1888 et en a donné une nouvelle description en 1896. الشلل النصفي التشنجي الموروث (بالإنجليزية: Hereditary spastic paraplegia)‏ أو اختصارا HSP، هو مجموعة من الأمراض الوراثية النادرة، والتي تتمثل إحدى سماتها الرئيسية في اضطراب تدريجي في المشي. يظهر المرض على شكل تصلب تدريجي (التشنج) وانكماش في الأطراف السفلية. يعرف هذا المرض أيضا باسم الخزل السفلي التشنجي الوراثي، أو مرض المستوطنات الفرنسية، أو أيضا مرض سترومبل لورايين. الأعراض هي ناجة عن خلل وظيفي في محاور عصبية على مستوى النخاع الشوكي. بحيث تكون الخلايا المصابة هي الخلايا العصبية الحركية الرئيسية؛ وبالتالي، فهو مرض يصيب العصبونات الحركية العليا. بالرغم من أن الشلل النصفي التشنجي الموروث قد يبدو جسديا ويتصرف بطريقة مشابه لتلك التي يتصرف بها مرض ، إلا أنه لا يمكن اعتباره كشكل من أشكال الشلل الدماغي. حتى في ظل وجود اختلاف بين أصل المرضين، يتم في بعض الأحيان استخدام بعض الأدوية المضادة للتشنج مماثلة لتلك المستخدمة في حالات الشلل الدماغي التشنجي لعلاج أعراض الشلل النصفي التشنجي الوراثي.
dbp:emedicinesubj: pmr
dbp:emedicinetopic: 45
gold:hypernym: dbr:Group
dbp:wordnet_type: n9:synset-disease-noun-1
dbo:icd9: 334.1
dbo:eMedicineSubject: pmr
dbo:eMedicineTopic: 45
dbo:meshId: D015419
prov:wasDerivedFrom: wikipedia-en:Hereditary_spastic_paraplegia?oldid=1111635927&ns=0
dbo:wikiPageLength: 36647
dbo:diseasesDB: 33207
dbo:icd10: G11.4
dbo:omim: 312920
dbo:orpha: 685
foaf:isPrimaryTopicOf: wikipedia-en:Hereditary_spastic_paraplegia

Subject Item: dbr:Jeffrey_Macklis
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Startle-evoked_movement
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:AP4S1
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Acetyl-coenzyme_A_transporter_1
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Kate_Hood
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Eicosanoid
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Troyer
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:KIF5A
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Margaret_Robinson
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Michael_Cera
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:COX4I1
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:CYP2U1
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Yes_I_Can_(TV_series)
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:SPG
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Spinal_muscular_atrophies
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Victor_Rădulescu-Pogoneanu
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:FAM166B
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:ZFYVE27
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Spinal_cord
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Seipin
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:PLA2G6
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Phil_Parry
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Reticulon
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:SPG5B
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia
dbo:wikiPageRedirects: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Paraplegin
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:SPG15_(disease)
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Spastic_paraplegia_31
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Spastic_paraplegia_6
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Lison_syndrome
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia
dbo:wikiPageRedirects: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Struempell-Lorrain_disease
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia
dbo:wikiPageRedirects: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Strumpell-Lorrain_disease
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia
dbo:wikiPageRedirects: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Familial_spastic_paraplegia
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia
dbo:wikiPageRedirects: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Silver_syndrome
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia
dbo:wikiPageRedirects: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Spastic_ataxia
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia
dbo:wikiPageRedirects: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Spastic_paraplegia
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia
dbo:wikiPageRedirects: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Spastic_paraplegia,_familial
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia
dbo:wikiPageRedirects: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Spastic_paraplegia,_hereditary
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia
dbo:wikiPageRedirects: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Spastic_paraplegia_type_1,_X-linked
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia
dbo:wikiPageRedirects: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Spastic_paraplegia_type_2,_X-linked
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia
dbo:wikiPageRedirects: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Spastic_paraplegia_type_3,_dominant
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia
dbo:wikiPageRedirects: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Spastic_paraplegia_type_4,_dominant
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia
dbo:wikiPageRedirects: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Spastic_paraplegia_type_5A,_recessive
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia
dbo:wikiPageRedirects: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Spastic_paraplegia_type_5B,_recessive
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia
dbo:wikiPageRedirects: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Spastic_paraplegia_type_6,_dominant
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia
dbo:wikiPageRedirects: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Kjellin_syndrome
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia
dbo:wikiPageRedirects: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Troyer_syndrome
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia
dbo:wikiPageRedirects: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Strumpell-lorrain_disease
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia
dbo:wikiPageRedirects: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Strumpell_disease
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia
dbo:wikiPageRedirects: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Strumpell–Lorrain_disease
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia
dbo:wikiPageRedirects: dbr:Hereditary_spastic_paraplegia

Subject Item: dbr:Strümpell-Lorrain_disease
dbo:wikiPageWikiLink: dbr:Hereditary_spastic_paraplegia
dbo:wikiPageRedirects: dbr:Hereditary_spastic_paraplegia

Subject Item: wikipedia-en:Hereditary_spastic_paraplegia
foaf:primaryTopic: dbr:Hereditary_spastic_paraplegia