Abstract
In order to evaluate autonomic function in primary amyloidosis (AL), five non invasive tests (Valsalva ratio, heart rate variation during deep breathing, 30/15 ratio, blood pressure response to standing and blood pressure response to sustained handgrip) were performed in 16 patients with positive fat tissue aspirates (FTA) for amyloid (11 females, 5 males, aged 48-85 years). Three of 16 were asymptomatic and without signs of organ involvement. Ten normal subjects and 10 patients with monoclonal gammopathy of undetermined significance (MGUS) of similar age were studied as controls. Abnormal autonomic function was found in 15 of the 16 patients with AL amyloidosis. A severe autonomic dysfunction was observed in only 4 cases. Isolated sympathetic damage was found in 4 cases, parasympathetic damage in 5 and combined (parasympathetic and sympathetic) damage in 5. (One patient showed parasympathetic damage but he was unable to complete the sympathetic tests). No alteration was observed in the controls. Thirty months following the autonomic function investigation, 14 patients were unchanged clinically, one was worse and 2 died because of other disease. In conclusion, our results indicate that at least in some patients with less severe damage, autonomic neuropathy does not seem to carry a poor prognosis with respect to that previously reported in the literature. Since autonomic damage was also observed in biopsy positive asymptomatic patients, we suggest that autonomic neuropathy may represent an early marker of systemic AL amyloidosis.
| Original language | English |
|---|---|
| Pages (from-to) | 167-172 |
| Number of pages | 6 |
| Journal | Amyloid |
| Volume | 3 |
| Issue number | 3 |
| Publication status | Published - 1996 |
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Keywords
- AL amyloidosis
- Autonomic neuropathy
- Dysautonomia
- Monoclonal gammopathy of undetermined significance
- Plasma cell dyscrasia
ASJC Scopus subject areas
- Medicine(all)
- Pathology and Forensic Medicine
Cite this
Functional study of autonomic neuropathy in primary (AL) amyloidosis. / Gemmi, Fabrizio; Miliani, Anna; Bergesio, Franco; Salvadori, Maurizio; Merlini, Giampaolo.
In: Amyloid, Vol. 3, No. 3, 1996, p. 167-172.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Functional study of autonomic neuropathy in primary (AL) amyloidosis
AU - Gemmi, Fabrizio
AU - Miliani, Anna
AU - Bergesio, Franco
AU - Salvadori, Maurizio
AU - Merlini, Giampaolo
PY - 1996
Y1 - 1996
N2 - In order to evaluate autonomic function in primary amyloidosis (AL), five non invasive tests (Valsalva ratio, heart rate variation during deep breathing, 30/15 ratio, blood pressure response to standing and blood pressure response to sustained handgrip) were performed in 16 patients with positive fat tissue aspirates (FTA) for amyloid (11 females, 5 males, aged 48-85 years). Three of 16 were asymptomatic and without signs of organ involvement. Ten normal subjects and 10 patients with monoclonal gammopathy of undetermined significance (MGUS) of similar age were studied as controls. Abnormal autonomic function was found in 15 of the 16 patients with AL amyloidosis. A severe autonomic dysfunction was observed in only 4 cases. Isolated sympathetic damage was found in 4 cases, parasympathetic damage in 5 and combined (parasympathetic and sympathetic) damage in 5. (One patient showed parasympathetic damage but he was unable to complete the sympathetic tests). No alteration was observed in the controls. Thirty months following the autonomic function investigation, 14 patients were unchanged clinically, one was worse and 2 died because of other disease. In conclusion, our results indicate that at least in some patients with less severe damage, autonomic neuropathy does not seem to carry a poor prognosis with respect to that previously reported in the literature. Since autonomic damage was also observed in biopsy positive asymptomatic patients, we suggest that autonomic neuropathy may represent an early marker of systemic AL amyloidosis.
AB - In order to evaluate autonomic function in primary amyloidosis (AL), five non invasive tests (Valsalva ratio, heart rate variation during deep breathing, 30/15 ratio, blood pressure response to standing and blood pressure response to sustained handgrip) were performed in 16 patients with positive fat tissue aspirates (FTA) for amyloid (11 females, 5 males, aged 48-85 years). Three of 16 were asymptomatic and without signs of organ involvement. Ten normal subjects and 10 patients with monoclonal gammopathy of undetermined significance (MGUS) of similar age were studied as controls. Abnormal autonomic function was found in 15 of the 16 patients with AL amyloidosis. A severe autonomic dysfunction was observed in only 4 cases. Isolated sympathetic damage was found in 4 cases, parasympathetic damage in 5 and combined (parasympathetic and sympathetic) damage in 5. (One patient showed parasympathetic damage but he was unable to complete the sympathetic tests). No alteration was observed in the controls. Thirty months following the autonomic function investigation, 14 patients were unchanged clinically, one was worse and 2 died because of other disease. In conclusion, our results indicate that at least in some patients with less severe damage, autonomic neuropathy does not seem to carry a poor prognosis with respect to that previously reported in the literature. Since autonomic damage was also observed in biopsy positive asymptomatic patients, we suggest that autonomic neuropathy may represent an early marker of systemic AL amyloidosis.
KW - AL amyloidosis
KW - Autonomic neuropathy
KW - Dysautonomia
KW - Monoclonal gammopathy of undetermined significance
KW - Plasma cell dyscrasia
UR - http://www.scopus.com/inward/record.url?scp=0011979228&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0011979228&partnerID=8YFLogxK
M3 - Article
AN - SCOPUS:0011979228
VL - 3
SP - 167
EP - 172
JO - Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis
JF - Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis
SN - 1350-6129
IS - 3
ER -