| The exercise test in Andersen syndrome. | |
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MedLine Citation:
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PMID: 10190827 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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BACKGROUND: Andersen syndrome is a rare form of periodic paralysis (PP) associated with dysmorphic features and potentially fatal cardiac dysrhythmias. To date, no electrodiagnostic abnormalities have been reported that can be used to confirm the presence of PP in this condition. OBJECTIVES: To determine if the exercise test could be used to confirm the diagnosis of PP in Andersen syndrome. To evaluate the exercise test as a means to assess neuromuscular status during treatment. METHODS: We performed the exercise test on 2 patients with Andersen syndrome. In 1 patient, we used a modified version of the test to document responsiveness to treatment with tocainide. RESULTS: Studies in both patients demonstrated a progressive decline in the compound muscle action potential amplitude after exercise that was characteristic of the phenomenon seen in other forms of PP. In 1 patient, improvement in interattack strength and a reduction in the number of attacks of weakness correlated with improvement in the test results. CONCLUSIONS: Our cases demonstrate that the exercise test can confirm the diagnosis of PP in Andersen syndrome. A modified version of exercise testing may also be considered as an objective method for documenting treatment responses in PP. |
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Authors:
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J S Katz; G I Wolfe; S Iannaccone; W W Bryan; R J Barohn |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Archives of neurology Volume: 56 ISSN: 0003-9942 ISO Abbreviation: Arch. Neurol. Publication Date: 1999 Mar |
Date Detail:
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Created Date: 1999-04-14 Completed Date: 1999-04-14 Revised Date: 2004-11-17 |
Medline Journal Info:
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Nlm Unique ID: 0372436 Medline TA: Arch Neurol Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 352-6 Citation Subset: AIM; IM |
Affiliation:
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Department of Veterans Affairs, Department of Neurology, Stanford University School of Medicine, Palo Alto, Calif 94304, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Abnormalities, Multiple Action Potentials Adolescent Adult Anti-Arrhythmia Agents / therapeutic use Exercise Test Facial Bones / abnormalities Female Humans Long QT Syndrome / diagnosis*, drug therapy, etiology Male Muscle Weakness Paralyses, Familial Periodic / diagnosis* Tocainide / therapeutic use |
| Chemical | |
Reg. No./Substance:
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0/Anti-Arrhythmia Agents; 41708-72-9/Tocainide |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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