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Axonal polyneuropathy in Ehlers–Danlos syndrome

✍ Scribed by Wolf Muellbacher; Josef Finsterer; Bruno Mamoli; Reginald E. Bittner; Franz Trautinger

Publisher
John Wiley and Sons
Year
1998
Tongue
English
Weight
262 KB
Volume
21
Category
Article
ISSN
0148-639X

No coin nor oath required. For personal study only.

✦ Synopsis

of motor unit action potential-like discharges (Fig. ); they disappeared during sleep. With the needle in the masseter, a spatula test 1 was performed. It was positive, and could be documented by EMG monitoring.

This case suggests that NNT should be kept in mind and always be considered in neonates because it is a treatable condition. Although the diagnosis of NNT depends on clinical features and exclusion of other causes, a positive spatula test can help. Simultaneous EMG recording from the masseter muscle makes the test more objective and sensitive. We advocate using this test in suspected cases. High-amplitude F waves and easily recordable H reflex in distal muscles of the upper extremity can support the diagnosis.

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