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Correspondence |

Progressive Ataxia, Palatal Tremor, and the Romberg Sign

Hélio A. Teive, MD, PhD; Renato P. Munhoz, MD
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Copyright 2009 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.

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Arch Neurol. 2009;66(2):284-285. doi:10.1001/archneurol.2008.550
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We read with great interest the article by Brinar et al1 reporting a case of progressive ataxia and palatal tremor. They described a middle-aged woman with progressive cerebellar ataxia associated with severe palatal tremor. Magnetic resonance imaging showed atrophy of the vermis and both cerebellar hemispheres as well as bilateral symmetrical hyperintensity of olivary nuclei. They noted that on examination, the patient showed no signs of pyramidal or sensory involvement but had the Romberg sign and walking instability.

In fact, the Romberg sign, described by Moritz Heinrich Romberg as a loss of balance when closing the eyes, is associated with sensory ataxia and a dorsal columns abnormality.2 Therefore, by definition, the presence of the Romberg sign indicates the implicit association with sensory dysfunction, not cerebellar disease.2 3 Rombergism, on the other hand, is a term coined by Harding4 related to cerebellar disease. This sign is present when there is axial body swaying with the eyes both open and closed and the patient demonstrates gait ataxia and dystasia but not the classic Romberg sign.4

Finally, Brinar and colleagues considered their case to be idiopathic progressive ataxia with palatal tremor and discussed similar previous case reports and spinocerebellar ataxia type 20 as another possible cause. However, there are other possible differential diagnoses in this case, such as multiple system atrophy, adult-onset Alexander disease, and Whipple disease. For instance, Kulkarni et al5 reviewed the literature of palatal tremors, in most cases associated with cerebellar ataxia, and found the most common causes to be the following: multiple system atrophy (22% of the cases), Alexander disease (22%), progressive supranuclear palsy (4.3%), spinocerebellar degeneration (4.3%), and unknown causes in the remaining cases (43.4%).

AUTHOR INFORMATION

Correspondence: Dr Teive, Rua General Carneiro 1103/102, Centro, Curitiba, Paraná, 80060-150, Brazil (hagteive@mps.com.br).

Financial Disclosure: None reported.

REFERENCES

Brinar  VV, Barun  B, Zadro  I, Ozretic  D, Habek  M. Progressive ataxia and palatal tremor. Arch Neurol 2008;65 (9) 1248- 1249
PubMed
Lanska  DJ, Goetz  CG. Romberg's sign: development, adoption, and adaptation in the 19th century. Neurology 2000;55 (8) 1201- 1206
PubMed
Meyer  MA. Romberg sign: sensory dysfunction, not cerebellar disease. Geriatrics 1998;53 (1) 10
PubMed
Harding  AE. The Hereditary Ataxias and Related Disorders.  Edinburgh, Scotland Churchill Livingstone1984;134
Kulkarni  PK, Muthane  UB, Taly  AB, Jayakumar  PN, Shetty  R, Swamy  HS. Palatal tremor, progressive multiple cranial nerve palsies, and cerebellar ataxia: a case report and review of literature of palatal tremors in neurodegenerative disease. Mov Disord 1999;14 (4) 689- 693
PubMed

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Brinar  VV, Barun  B, Zadro  I, Ozretic  D, Habek  M. Progressive ataxia and palatal tremor. Arch Neurol 2008;65 (9) 1248- 1249
PubMed
Lanska  DJ, Goetz  CG. Romberg's sign: development, adoption, and adaptation in the 19th century. Neurology 2000;55 (8) 1201- 1206
PubMed
Meyer  MA. Romberg sign: sensory dysfunction, not cerebellar disease. Geriatrics 1998;53 (1) 10
PubMed
Harding  AE. The Hereditary Ataxias and Related Disorders.  Edinburgh, Scotland Churchill Livingstone1984;134
Kulkarni  PK, Muthane  UB, Taly  AB, Jayakumar  PN, Shetty  R, Swamy  HS. Palatal tremor, progressive multiple cranial nerve palsies, and cerebellar ataxia: a case report and review of literature of palatal tremors in neurodegenerative disease. Mov Disord 1999;14 (4) 689- 693
PubMed

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Progressive ataxia, palatal tremor, and the Romberg sign.
Arch Neurol. 2009;66(2):284-5; author reply 285.