TY  - JOUR
A1  - Üçeyler, Nurcan
A1  - Kahn, Ann-Kathrin
A1  - Kramer, Daniela
A1  - Zeller, Daniel
A1  - Casanova-Molla, Jordi
A1  - Wanner, Christoph
A1  - Weidemann, Frank
A1  - Katsarava, Zaza
A1  - Sommer, Claudia
T1  - Impaired small fiber conduction in patients with Fabry disease: a neurophysiological case–control study
JF  - BMC Neurology
N2  - Background

Fabry disease is an inborn lysosomal storage disorder which is associated with small fiber neuropathy. We set out to investigate small fiber conduction in Fabry patients using pain-related evoked potentials (PREP).

Methods

In this case–control study we prospectively studied 76 consecutive Fabry patients for electrical small fiber conduction in correlation with small fiber function and morphology. Data were compared with healthy controls using non-parametric statistical tests. All patients underwent neurological examination and were investigated with pain and depression questionnaires. Small fiber function (quantitative sensory testing, QST), morphology (skin punch biopsy), and electrical conduction (PREP) were assessed and correlated. Patients were stratified for gender and disease severity as reflected by renal function.

Results

All Fabry patients (31 men, 45 women) had small fiber neuropathy. Men with Fabry disease showed impaired cold (p < 0.01) and warm perception (p < 0.05), while women did not differ from controls. Intraepidermal nerve fiber density (IENFD) was reduced at the lower leg (p < 0.001) and the back (p < 0.05) mainly of men with impaired renal function. When investigating A-delta fiber conduction with PREP, men but not women with Fabry disease had lower amplitudes upon stimulation at face (p < 0.01), hands (p < 0.05), and feet (p < 0.01) compared to controls. PREP amplitudes further decreased with advance in disease severity. PREP amplitudes and warm (p < 0.05) and cold detection thresholds (p < 0.01) at the feet correlated positively in male patients.

Conclusion

Small fiber conduction is impaired in men with Fabry disease and worsens with advanced disease severity. PREP are well-suited to measure A-delta fiber conduction.
KW  - Fabry disease
KW  - Pain-related evoked potentials
KW  - Small fiber neuropathy
KW  - A-delta fibers
Y1  - 2013
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-96527
UR  - http://www.biomedcentral.com/1471-2377/13/47
ER  - 
TY  - JOUR
A1  - Kress, Michaela
A1  - Hüttenhofer, Alexander
A1  - Landry, Marc
A1  - Kuner, Rohini
A1  - Favereaux, Alexandre
A1  - Greenberg, David
A1  - Bednarik, Josef
A1  - Heppenstall, Paul
A1  - Kronenberg, Florian
A1  - Malcangio, Marzia
A1  - Rittner, Heike
A1  - Üçeyler, Nurcan
A1  - Trajanoski, Zlatko
A1  - Mouritzen, Peter
A1  - Birklein, Frank
A1  - Sommer, Claudia
A1  - Soreq, Hermona
T1  - microRNAs in nociceptive circuits as predictors of future clinical applications
JF  - Frontiers in Molecular Neuroscience
N2  - Neuro-immune alterations in the peripheral and central nervous system play a role in the pathophysiology of chronic pain, and non-coding RNAs – and microRNAs (miRNAs) in particular – regulate both immune and neuronal processes. Specifically, miRNAs control macromolecular complexes in neurons, glia and immune cells and regulate signals used for neuro-immune communication in the pain pathway. Therefore, miRNAs may be hypothesized as critically important master switches modulating chronic pain. In particular, understanding the concerted function of miRNA in the regulation of nociception and endogenous analgesia and defining the importance of miRNAs in the circuitries and cognitive, emotional and behavioral components involved in pain is expected to shed new light on the enigmatic pathophysiology of neuropathic pain, migraine and complex regional pain syndrome. Specific miRNAs may evolve as new druggable molecular targets for pain prevention and relief. Furthermore, predisposing miRNA expression patterns and inter-individual variations and polymorphisms in miRNAs and/or their binding sites may serve as biomarkers for pain and help to predict individual risks for certain types of pain and responsiveness to analgesic drugs. miRNA-based diagnostics are expected to develop into hands-on tools that allow better patient stratification, improved mechanism-based treatment, and targeted prevention strategies for high risk individuals.
KW  - chronic pain
KW  - biomarker
KW  - polymorphism
KW  - miRNA-based diagnostics
KW  - miRNA expression patterns
KW  - miRNA polymorphisms
KW  - antagomir
KW  - miRNA-based analgesic
Y1  - 2013
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-154597
VL  - 6
IS  - 33
ER  -