Neurology Book

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Chiari Malformation

Aka: Chiari Malformation, Chiari I Malformation
  1. See Also
    1. Chiari II Malformation
  2. Definition: Chiari I Malformation
    1. Cerebellar tonsils fall >5 mm below foramen magnum
  3. Pathophysiology
    1. Congenital disorder
  4. Epidemiology
    1. Chiari I symptom onset 30-40 years of age
  5. 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
  6. Differential Diagnosis: Chiari I
    1. Multiple Sclerosis
    2. Bony compression at craniocervical junction
    3. Syringohydromyelia
    4. Syringobulbia
  7. Radiology: Chiari I
    1. Head MRI shows ectopic cerebellar tonsils, compression
  8. Management: Chiari I
    1. Decompression of bone at craniocervical junction
  9. 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

Arnold Chiari Malformation (C0003803)

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 253184003, 156889003, 268307000, 203931009, 203938003, 204405005, 373587001, 27905003
English Arnold Chiari Deformity, Arnold Chiari Syndrome, Arnold-Chiari Deformity, Arnold-Chiari Syndrome, Deformity, Arnold-Chiari, Syndrome, Arnold-Chiari, ARNOLD-CHIARI SYNDROME, CEREBELLOMEDULLARY MALFORMATION SYNDROME, ARNOLD CHIARI MALFORMATION, 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, Chiari malformation, Chiari malformation (disorder), Arnold-Chiari; obstruction, Arnold-Chiari, obstruction; Arnold-Chiari, Arnold - Chiari syndrome, Chiari's malformation, Arnold-Chiari syndrome, Arnold-Chiari syndrome (disorder)
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, malformación da Arnold - Chiari, síndrome de Arnold - Chiari (trastorno), Arnold - Chiari syndrome, Chiari's malformation, Arnold-Chiari syndrome, Síndrome de Arnold-Chiari, Sindrome de Arnold-Chiari, malformación de Chiari (trastorno), malformación de Chiari, síndrome de Arnold-Chiari, Malformacion de Arnold-Chiari, Malformación de Arnold-Chiari
Japanese アーノルド・キアリ奇形, ｷｱﾘｷｹｲ, ｱｰﾉﾙﾄﾞｷｱﾘｷｹｲ, 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, Arnoldova-Chiariho malformace, Arnold-Chiariho malformace, Chiariho malformace
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
Sources
Derived from the NIH UMLS (Unified Medical Language System)


Chiari I malformation (C1857474)

Concepts Finding (T033)
English Chiari I malformation
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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