II. Definition: Chiari I Malformation

  1. Cerebellar tonsils fall >5 mm below foramen magnum

III. Pathophysiology

  1. Congenital disorder

IV. Epidemiology

  1. Chiari I symptom onset 30-40 years of age

V. Symptoms (Chiari I is often asymptomatic)

  1. Headaches provoked by straining
  2. Neck Pain
  3. Lower Cranial Nerve disorders
  4. Vertigo
  5. Vertical Nystagmus (down-beating Nystagmus)
  6. Ataxia
  7. Urinary frequency
  8. Lower extremity spasticity
  9. Dissociated anesthesia

VI. Differential Diagnosis: Chiari I

  1. Multiple Sclerosis
  2. Bony compression at craniocervical junction
  3. Syringohydromyelia
  4. Syringobulbia

VII. Radiology: Chiari I

  1. Head MRI shows ectopic cerebellar tonsils, compression

VIII. Management: Chiari I

  1. Decompression of bone at craniocervical junction

IX. References

  1. Behrman (2000) Nelson Pediatrics, Saunders, p. 1810
  2. Goetz (1999) Clinical Neurology, Saunders, p. 530
  3. Goldman (2000) Cecil Medicine, Saunders, p. 2076

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Ontology: Arnold Chiari Malformation (C0003803)

Definition (MEDLINEPLUS)

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. Some 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

Definition (MSHCZE) 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/ )
Definition (NCI) A rare genetic brain malformation characterized by displacement of the brain stem and cerebellum through the foramen magnum. It may result in hydrocephalus.
Definition (MSH) 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)
Concepts Congenital Abnormality (T019)
MSH D001139
ICD10 Q07.0
SnomedCT 27905003, 203931009, 156889003, 268307000, 204405005, 203938003, 253184003, 373587001
English Arnold Chiari Deformity, Arnold Chiari Syndrome, Arnold-Chiari Deformity, Arnold-Chiari Syndrome, Deformity, Arnold-Chiari, Syndrome, Arnold-Chiari, Arnold-Chiari malformation, Arnold-Chiari malformation (diagnosis), ACM - Arnold-Chiari malformat, ACM - Arnold-Chiari malformation, Arnold Chiari Malformation, Arnold-Chiari Malformation, Malformation, Arnold Chiari, Malformation, Arnold-Chiari, Arnold-Chiari Malformation [Disease/Finding], chiari malformations, arnold chiari deformity, arnold-chiari deformity, arnold-chiari malformation, arnold chiari malformation, arnold chiari syndrome, arnold chiari malformations, arnold-chiari syndrome, chiari malformation, Arnold - Chiari syndrome, Chiari's malformation, Arnold-Chiari syndrome (disorder), Chiari Malformation, Arnold-Chiari deformity, Chiari malformation, Chiari malformation (disorder), Arnold-Chiari; obstruction, Arnold-Chiari, obstruction; Arnold-Chiari, Arnold-Chiari syndrome
Dutch Chiari-misvorming, Arnold-Chiari; obstructie, obstructie; Arnold-Chiari, Syndroom van Arnold-Chiari, Arnold-Chiari misvorming, Arnold-Chiari-syndroom, Syndroom, Arnold-Chiari-, syndroom van Arnold-Chiari
French Malformation de Chiari, Malformation d'Arnold-Chiari, Syndrome d'Arnold Chiari
German Chiari-Missbildung, Arnold-Chiari-Syndrom, Arnold-Chiari-Fehlbildung
Italian Malformazione di Chiari, Malformazione di Arnold-Chiari
Portuguese Malformação de Chiari, Síndrome de Arnold-Chiari, Má formação de Arnold-Chiari, Malformação de Arnold-Chiari
Spanish Malformación de Chiari, síndrome de Arnold - Chiari (trastorno), síndrome de Arnold - Chiari, malformación da Arnold - Chiari, Síndrome de Arnold-Chiari, malformación de Chiari (trastorno), malformación de Chiari, síndrome de Arnold-Chiari, Malformación de Arnold-Chiari
Japanese アーノルド・キアリ奇形, キアリキケイ, アーノルドキアリキケイ, アーノルド・キアリ奇形, アーノルド・キアリ症候群, Arnold-Chiari奇形, アルノルト・キアーリ奇形, アルノルド-キアリ奇形, Arnold-Chiari症候群, Chiari奇形, アルノルト-キアーリ奇形, アーノルド-キアリ奇形, アーノルド-キアリ症候群, キアリ奇形
Swedish Arnold-Chiaris missbildning
Czech Arnoldova-Chiariho deformita, Arnold-Chiariho malformace, Chiariho malformace, Arnoldova-Chiariho malformace, Arnoldův-Chiariho syndrom
Finnish Arnold-Chiarin oireyhtymä
Russian ARNOL'DA-KIARI MAL'FORMATSIIA, ARNOL'DA-KIARI SINDROM, АРНОЛЬДА-КИАРИ МАЛЬФОРМАЦИЯ, АРНОЛЬДА-КИАРИ СИНДРОМ
Korean 아놀드-키아리 증후군
Croatian ARNOLD-CHIARIJEVA MALFORMACIJA
Polish Malformacja Arnolda-Chiariego, Zespół Arnolda-Chiariego
Hungarian Arnold-Chiari malformatio, Chiari malformatio
Norwegian Arnold-Chiaris deformitet, Arnold-Chiari-malformasjon, Arnold-Chiari-misdannelse

Ontology: Chiari I malformation (C1857474)

Concepts Finding (T033)
English Chiari I malformation