Abstract Details

Title Spontaneous, Episodic, Hyperhidrosis in Transthyretin Related Amyloid Neuropathy

Topic Peripheral Nerve

Presentation(s) P01 - (-)

Poster/Presentation
Number 143

Objective

Background BACKGROUND: Hereditary amyloidosis due to TTR gene mutations manifests with somatic and autonomic neuropathy. The autonomic dysfunction has a major impact in the quality of life on some patients. In this series of patients we typify a kind of non-thermoregulatory, sudomotor dysfunction, presenting as spontaneous, episodic hyperhidrosis.

Design/Methods DESIGN/METHODS: Patients with amyloid neuropathy and TTR gen mutations were asked for the occurrence of spontaneous, episodic hyperhidrosis, not related to exercise or hot weather. They were also asked to quantify the frequency and duration of episodes, and to describe any triggering factors or accompanying symptoms. The proportion of patients with episodic hyperhidrosis was analyzed for associations with nerve conduction abnormalities, cardiovagal dysfunction and sensory-motor disability.

Results RESULTS: Seventeen symptomatic patients were evaluated, eight of which reported episodic hyperhidrosis (47%). None was taking any drug known to cause hyperhidrosis. The frequency of episodes spanned 3/year to 3/day. Most of them lasted less than 15 minutes and no triggering or ameliorating factors were recognized. In three patients the sweating was generalized, in three it was segmental and the other two were unable to characterize it. Only two patients recognized accompanying symptoms, which included hunger, lightheadedness and diarrhea. The group with sweating episodes was more likely to show abnormalities in nerve conduction studies and some degree of sensory-motor disability. On the other hand, cardiovagal function tests were abnormal in most patients and they did not discriminate between patients with sweating episodes or without.

Conclusions CONCLUSIONS: Spontaneous, episodic hyperhidrosis was reported by almost half the patients with peripheral neuropathy due to TTR related amyloidosis. We found a significant association among episodic sweating, nerve conduction abnormalities and sensory-motor disability. Cardiovagal function tests did not discriminate between patients with and without hyperhidrosis.

Authors/Disclosures
PRESENTER	No disclosure on file
Phillip Kucab, MD (John D. Dingell VA Medical Center-A2062)	Dr. Kucab has received personal compensation in the range of $500,000-$999,999 for serving as a Neurology Chief with US Department of Veteran Affairs.
Brian C. Healy	The institution of Mr. Healy has received research support from Analysis Group. The institution of Mr. Healy has received research support from Bristol-Myers Squibb. The institution of Mr. Healy has received research support from Verily Life Sciences. The institution of Mr. Healy has received research support from Novartis. The institution of Mr. Healy has received research support from Merck Serono. The institution of Mr. Healy has received research support from Genzyme.

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