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Chiari type 1 deformity clinical features

Chiari type 1 deformity may present due to any of the following:

Compression of brain stem at the level of the foramen magnum.


They may cause sensory loss, ataxia, weakness, nystagmus, limb spasticity to name a few 1). Although the most common presentation of Chiari type 1 deformity is occipital headache, the association of audio-vestibular symptoms is not rare.

The headache is commonly aggravated by Valsalva and sensory and motor deficits.

see Chiari related scoliosis.

In a series of 71 patients, pain was the commonest symptom (69% of patients); other symptoms included weakness (56%), numbness (52%), and unsteadiness (40%). The presenting physical signs consisted of a foramen magnum syndrome (22%), central cord syndrome (65%), or a cerebellar syndrome (11%) 2).

Audio-vestibular manifestations

The appearance of audio-vestibular manifestations in CM-I makes it common to refer these patients to neurotologists. Unsteadiness, vertiginous syndromes and sensorineural hearing loss are frequent. Nystagmus, especially horizontal and down-beating, is not rare. It is important for neurotologists to familiarise themselves with CM-I symptoms to be able to consider it in differential diagnosis 3).

Feinberg et al present a case of Chiari malformation manifesting as isolated trismus, describe the typical symptoms associated with Chiari malformation, and discuss the potential anatomical causes for this unique presentation. A 3-year-old boy presented with inability to open his jaw for 6 weeks with associated significant weight loss. The results of medical and radiological evaluation were negative except for Chiari malformation type 1 with cerebellar tonsils 12 mm below the level of the foramen magnum. The patient underwent Chiari decompression surgery. Postoperatively, his ability to open his mouth was significantly improved, allowing resumption of a regular diet. Postoperative MRI revealed almost complete resolution of the syringobulbia. To the best of the authors' knowledge, this is the first reported case of isolated trismus from Chiari malformation with syringobulbia 4).

Singh R, Arora R, Kumar R. Clinical Notes on Chiari Malformation. J Craniofac Surg. 2018 Mar 14. doi: 10.1097/SCS.0000000000004424. [Epub ahead of print] PubMed PMID: 29543678.
Paul KS, Lye RH, Strang FA, Dutton J. Arnold-Chiari malformation. Review of 71 cases. J Neurosurg. 1983 Feb;58(2):183-7. PubMed PMID: 6848674.
Guerra Jiménez G, Mazón Gutiérrez Á, Marco de Lucas E, Valle San Román N, Martín Laez R, Morales Angulo C. Audio-vestibular signs and symptoms in Chiari malformation type i. Case series and literature review. Acta Otorrinolaringol Esp. 2015 Jan-Feb;66(1):28-35. doi: 10.1016/j.otorri.2014.05.002. Epub 2014 Sep 4. English, Spanish. PubMed PMID: 25195076.
Feinberg M, Babington P, Sood S, Keating R. Isolated unilateral trismus as a presentation of Chiari malformation: case report. J Neurosurg Pediatr. 2016 May;17(5):533-6. doi: 10.3171/2015.7.PEDS1592. Epub 2016 Jan 1. PubMed PMID: 26722762.
chiari_type_1_deformity_clinical_features.txt · Last modified: 2018/05/03 19:22 by administrador