Yonsei Med J.  2009 Dec;50(6):777-783. 10.3349/ymj.2009.50.6.777.

Cheiro-Oral Syndrome: A Clinical Analysis and Review of Literature

• Chen WH

• KMID: 1777090
• DOI: http://doi.org/10.3349/ymj.2009.50.6.777

Abstract

PURPOSE
After a century, cheiro-oral syndrome (COS) was harangued and emphasized for its localizing value and benign course in recent two decades. However, an expanding body of case series challenged when COS may arise from an involvement of ascending sensory pathways between cortex and pons and terminate into poor outcome occasionally.
MATERIALS AND METHODS
To analyze the location, underlying etiologies and prognosis in 76 patients presented with COS collected between 1989 and 2007. RESULTS: Four types of COS were categorized, namely unilateral (71.1%), typically bilateral (14.5%), atypically bilateral (7.9%) and crossed COS (6.5%). The most common site of COS occurrence was at pons (27.6%), following by thalamus (21.1%) and cortex (15.8%). Stroke with small infarctions or hemorrhage was the leading cause. Paroxysmal paresthesia was predicted for cortical involvement and bilateral paresthesia for pontine involvement, whereas crossed paresthesia for medullary involvement. However, the majority of lesions cannot be localized by clinical symptoms alone, and were demonstrated only by neuroimaging. Deterioration was ensued in 12% of patients, whose lesions were large cortical infarction, medullary infarction, and bilateral subdural hemorrhage.
CONCLUSION
COS arises from varied sites between medulla and cortex, and is usually caused by small stroke lesion. Neurological deterioration occurs in 12% of patients and relates to large vessel occlusion, medullary involvement or cortical stroke. Since the location and deterioration of COS cannot be predicted by clinical symptoms alone, COS should be considered an emergent condition for aggressive investigation until fatal cause is substantially excluded.

Keyword

Cheiro-oral syndrome; infarction; hemorrhage; sensory; cortex; pons; thalamus; medulla oblongata; crossed; prognosis

MeSH Terms

Adult
Aged
Cerebrovascular Disorders/classification/complications/etiology/*pathology
Female
Humans
Male
Middle Aged
Nervous System Diseases/pathology
Prospective Studies
Syndrome

Reference

1. Sitting O. Klinische Beitrage zur Lehre von der Lokalisation der sensiblen Rindenzentren [Ger]. Prager Med Wochenschr. 1914. 45:548–550.

2. Strauss H. Über Sensibiliätsstörungen an Hand und Gesicht, Geschmacksstörungen und ihre lokalisatorische Bedeutung [Ger]. Monatsschr Psychiat Neurol. 1925. 58:265–276.

3. Garcin R, Lapresle J. [2d personal observation of a sensory syndrome of the thalamic type with cheiro-oral topography caused by localized lesion of the thalamus]. Rev Neurol (Paris). 1960. 103:474–481.

4. Kim JS. Restricted acral sensory syndrome following minor stroke. Further observation with special reference to differential severity of symptoms among individual digits. Stroke. 1994. 25:2497–2502.

5. Ngai WK, Chang YY, Liu JS, Chen SS. Cheiro-oral syndrome: identification of the lesion sites and a proposal for its clinical classification. Gaoxiong Yi Xue Ke Xue Za Zhi. 1991. 7:536–541.

6. Ten Holter J, Tijssen C. Cheiro-oral syndrome: does it have a specific localizing value? Eur Neurol. 1988. 28:326–330.

7. Nakamura M, Mizuguchi M, Momoi MY, Chou H, Masuzawa T. Transient cheiro-oral syndrome due to a ruptured intracranial dermoid cyst. Brain Dev. 2001. 23:261–263.

8. Hasegawa Y, Okada H, Okamoto S. [Neuro-Behçet disease with bilateral cheiro-oral syndrome following simultaneous multiple brain hemorrhage]. Rinsho Shinkeigaku. 1991. 31:754–759.

9. Kim JS. Cheiro-oral syndrome and vivid recollection of past in thalamic infarction. Eur Neurol. 1997. 37:253–254.

10. Mochizuki A, Eto H, Takasu M, Utsunomiya K, Schoji S. Cheiro-oral syndrome with internuclear ophthalmoplegia and cerebellar ataxia following midbrain infarction. Eur Neurol. 1994. 34:286–287.

11. Yasuda Y, Morita T, Okada T, Seko S, Akiguchi I, Kimura J. Cheiro-oral-pedal syndrome. Eur Neurol. 1992. 32:106–108.

12. Matsumoto S, Kaku S, Yamasaki M, Imai T, Nabatame H, Kameyama M. Cheiro-oral syndrome with bilateral oral involvement: a study of pontine lesions by high-resolution magnetic resonance imaging. J Neurol Neurosurg Psychiatry. 1989. 52:792–794.

13. Iwasaki Y, Kinoshita M, Ikeda K, Takamiya K, Shiojima T. Oral syndrome: an incomplete form of cheiro-oral syndrome? Int J Neurosci. 1991. 58:271–273.

14. Chang YY, Chen WH, Liu JS, Chen SS, Wu HS. Unilateral cheiro-oral syndrome in a patient with bilateral subdural hematomas. J Formos Med Assoc. 1994. 93:727–729.

15. Chen WH, Chang YY, Yin HL, Liu JS. Bilateral cheiro-oral syndrome and traumatic subdural hematoma. J Trauma. 1995. 38:826–827.

16. Chen WH, Lan MY, Chang YY, Liu JS, Chou MS, Chen SS. Bilateral cheiro-oral syndrome. Clin Neurol Neurosurg. 1997. 99:239–243.

17. Chen WH, Tseng YL, Lui CC, Liu JS. Episodic pain syndrome restricted cheiro-oral region associated with pontine lesion. Brain Inj. 2005. 19:949–953.

18. Chen WH, Lan MY, Chang YY, Lui CC, Chen SS, Liu JS. Cortical cheiro-oral syndrome: a revisit of clinical significance and pathogenesis. Clin Neurol Neurosurg. 2006. 108:446–450.

19. Chen WH, Li TH, Chen TH, Lin HS, Hsu MC, Chen SS, et al. Crossed cheiro-oral syndrome. Clin Neurol Neurosurg. 2008. 110:1008–1011.

20. Fujisawa A, Imaizumi M, Nukada T. [Clinical study of cheiro-oral syndrome (author's transl)]. Rinsho Shinkeigaku. 1979. 19:17–21.

21. Yasuda Y, Watanabe T, Tanaka H, Ogura A. Localizing value of bilateral cheiro-oral sensory impairment. Intern Med. 1998. 37:982–985.

22. Hashiguchi K, Igata A. [A case of primary pontine hemorrhage with cheiro-oral syndrome (palm-oral sensory disturbance)]. Shinkei Naika. 1976. 5:79–80.

23. Ide M, Yamamoto M, Jimbo M, Enomoto N. [Cheiro-oral syndrome with pontine infarct--report of a case]. No To Shinkei. 1985. 37:181–186.

24. Kim JS, Lee JH, Lee MC. Patterns of sensory dysfunction in lateral medullary infarction Clinical-MRI correlation. Neurology. 1997. 49:1557–1563.

25. Kumral E, Afsar N, Kirbas D, Balkir K, Ozdemirkiran T. Spectrum of medial medullary infarction: clinical and magnetic resonance imaging findings. J Neurol. 2002. 249:85–93.

26. Norrving B, Cronqvist S. Lateral medullary infarction: prognosis in an unselected series. Neurology. 1991. 41:244–248.