Neurology Book

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Chiari MalformationAka: Chiari I Malformation

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  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 (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)
ConceptsCongenital Abnormality (T019)
MSHD001139
EnglishACM - Arnold-Chiari malformation, Arnold - Chiari syndrome, Arnold Chiari Deformity, Arnold Chiari Malformation, Arnold Chiari Syndrome, Arnold-Chiari Deformity, Arnold-Chiari malformation, Arnold-Chiari Syndrome, CEREBELLOMEDULLARY MALFORMATION SYNDROME, Chiari malformation, Chiari's malformation
Spanishmalformacion da Arnold - Chiari, malformacion de Chiari, sindrome de Arnold - Chiari, sindrome de Arnold-Chiari
Parent ConceptsNeural Tube Defects (C0027794), Congenital Abnormality (C0000768), Congenital anomaly of brain (C0266449), Ambiguous concept (C1274012)
SourcesCOSTAR, DXP, MEDLINEPLUS, MSH, MTH, NDFRT, OMIM, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)


Chiari I malformation (C1857474)

ConceptsFinding (T033)
EnglishChiari I malformation
SourcesOMIM
Derived from the NIH UMLS (Unified Medical Language System)



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