[Neuropathic pain in Fabry's disease: heterogeneous remission in three years of enzyme replacement therapy].
BACKGROUND
Fabry's disease is associated with acute neuropathic pain (NP). When six males with classic Fabry's disease ended three years of enzyme replacement therapy (ERT), studies were conducted to analyse the progression of the NP.


METHODS
All of them received 1 mg/kg of agalsidase beta every 14 days. NP in hands and feet was evaluated at 0, 6, 12, 24 and 36 months, two modalities being considered: a) very intense pain or Fabry crises (FC), which forced the patient to rest, take analgesics and apply cold locally; b) acroparesthesias (AP), lower intensity itching, tingling and burning sensations that did not prevent them from continuing with their activities of daily living. The intensity of the FC (IFC) and the AP (IAP) were recorded using the Visual Analogue Scale, and the frequency with which both (FrFC and FrAP, respectively) appeared was measured in days. The one-tailed Wilcoxon test, binomial distribution and bootstrap method were used to carry out the analysis.


RESULTS
After six months of ERT the IFC, IAP and FrCF remained the same, although the FrAP had become worse. As shown by the NP indexes that relate IFC/FrFC and IAP/FrAP, progress was therefore favourable. At one year, IFC continued, but FrFC and IAP were lower. At two years, the four NP measurements improved in five patients, although the FC and AP indexes did not vary in one patient. At three years, all the NP variables improved in the six patients. The slope representing NP as a function of time in all the bootstrap analyses was found to be p < 0.001.


CONCLUSIONS
NP responded in a favourable, significant and heterogeneous manner and therefore justifies the early indication of ERT.
Domínguez
R O
RO
Servicio de Neurología, Hospital Sirio-Libanés, Buenos Aires, Argentina. profneuroldom@hotmail.com
Amartino
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Chamoles
N A
NA
D000328:Adult; D018450:Disease Progression; D000795:Fabry Disease / Q000503:physiopathology / Q000628:therapy; D006801:Humans; D007527:Isoenzymes / Q000627:therapeutic use*; D008297:Male; D010146:Pain / Q000503:physiopathology; D059408:Pain Management; D010147:Pain Measurement; D012074:Remission Induction; D000519:alpha-Galactosidase / Q000627:therapeutic use*
D007527:Isoenzymes; D000519:alpha-Galactosidase; C459420:agalsidase beta