C0003803 - Arnold-Chiari malformation consists of a downward displacement of the cerebellar tonsils and the medulla through the foramen magnum, sometimes causing hydrocephalus as a result of obstruction of CSF outflow. [HPO:curators] 2/10
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Terms, descriptions
CUI    C0003803
RussianMedical Subject Headings Russian D001139 L3339420preferred S3866901 Y АРНОЛЬДА-КИАРИ СИНДРОМ
RussianMedical Subject Headings Russian D001139 L0890442no S1094259 Y ARNOL'DA-KIARI SINDROM
RussianMedical Subject Headings Russian D001139 L1509279no S1805188 Y ARNOL'DA-KIARI MAL'FORMATSIIA
RussianMDRRUS 10003101 L15747643no S19045772 N Мальформация Арнольда-Киари
RussianMDRRUS 10003101 L15747643no S19045772 Y Мальформация Арнольда-Киари
RussianMDRRUS 10008503 L15747644no S19045774 Y Мальформация Киари
RussianMedical Subject Headings Russian D001139 L3339419no S3866900 Y АРНОЛЬДА-КИАРИ МАЛЬФОРМАЦИЯ
Medical Subject Headings A1634676 AT53894320 A group of congenital malformations involving the brainstem, cerebellum, upper spinal cord, and surrounding bony structures. Type II is the most common, and features compression of the medulla and cerebellar tonsils into the upper cervical spinal canal and an associated MENINGOMYELOCELE. Type I features similar, but less severe malformations and is without an associated meningomyelocele. Type III has the features of type II with an additional herniation of the entire cerebellum through the bony defect involving the foramen magnum, forming an ENCEPHALOCELE. Type IV is a form a cerebellar hypoplasia. Clinical manifestations of types I-III include TORTICOLLIS; opisthotonus; HEADACHE; VERTIGO; VOCAL CORD PARALYSIS; APNEA; NYSTAGMUS, CONGENITAL; swallowing difficulties; and ATAXIA. (From Menkes, Textbook of Child Neurology, 5th ed, p261; Davis, Textbook of Neuropathology, 2nd ed, pp236-46)
NCI Thesaurus A17692115 AT197999109 A rare genetic brain malformation characterized by displacement of the brain stem and cerebellum through the foramen magnum. It may result in hydrocephalus.
Medical Subject Headings Czech A20032691 AT204250433 Malformace mozku s posunem cerebella a prodloužené míchy do páteřního kanálu. Je způsobena chybným utvářením polohy flexury pontu v průběhu embryogeneze, která elonguje do IV. komory mozkové. Malformace se sdružuje s meningomyelokélou. Vyvíjí se hydrocefalus, dráždění mozkového kmene může vyvolat Chiariho krize - obtížné polykání, hromadění sekretu v dutině ústní, dušení se stridorem, apnoické pauzy a spasticitu horních končetin. Bez adekvátní léčby krize končí smrtí, která je způsobena mozkovou herniací; je nutná včasná dekomprese neurochirurgickým zákrokem. (cit. Velký lékařský slovník online, 2013 http://lekarske.slovniky.cz/ )
MEDLINEPLUS A23169949 AT220105812

Chiari malformations (CMs) are structural defects in the cerebellum. The cerebellum is the part of the brain that controls balance. With CM, brain tissue extends into the spinal canal. It can happen when part of the skull is too small, which pushes the brain tissue down. There are several types of CM. One type often happens in children who have neural tube defects. Some types cause no symptoms and don't need treatment. If you have symptoms, they may include

  • Neck pain
  • Balance problems
  • Numbness or other abnormal feelings in the arms or legs
  • Dizziness
  • Vision problems
  • Difficulty swallowing
  • Poor hand coordination

Doctors diagnose CM using imaging tests. Medicines may ease some symptoms, such as pain. Surgery is the only treatment available to correct or stop the progression of nerve damage.

NIH: National Institute of Neurological Disorders and Stroke

HPO A24675419 AT206291214 Arnold-Chiari malformation consists of a downward displacement of the cerebellar tonsils and the medulla through the foramen magnum, sometimes causing hydrocephalus as a result of obstruction of CSF outflow. [HPO:curators]