80. Small fiber neuropathy in Amyotrophic Lateral Sclerosis: Contribution of laser-evoked potentials and skin biopsy

2017 ◽  
Vol 128 (12) ◽  
pp. e434
Author(s):  
S. Maccora ◽  
V. Donadio ◽  
E. Pagliarani ◽  
R. Infante ◽  
A. Incensi ◽  
...  
2014 ◽  
Vol 125 ◽  
pp. S219
Author(s):  
C. Créac’h ◽  
J.-P. Camdessanche ◽  
F. Robert ◽  
J.-C. Antoine ◽  
R. Peyron ◽  
...  

2021 ◽  
Vol 429 ◽  
pp. 118621
Author(s):  
Silvia Giovanna Quitadamo ◽  
Eleonora Vecchio ◽  
Giuseppe Libro ◽  
Katia Ricci ◽  
Raffaella Lombardi ◽  
...  

2022 ◽  
Vol 17 (1) ◽  
Author(s):  
Jiayu Fu ◽  
Ji He ◽  
Yixuan Zhang ◽  
Ziyuan Liu ◽  
Haikun Wang ◽  
...  

Abstract Background Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder with progressive motor system impairment, and recent evidence has identified the extra-motor involvement. Small fiber neuropathy reflecting by sensory and autonomic disturbances in ALS has been reported to accompany the motor damage. However, non-invasive assessment of this impairment and its application in disease evaluation of ALS is scarce. We aim to evaluate the use of corneal confocal microscopy (CCM) to non-invasively quantify the corneal small fiber neuropathy in ALS and explore its clinical value in assessing disease severity of ALS. Methods Sixty-six patients with ALS and 64 healthy controls were included in this cross-sectional study. Participants underwent detailed clinical assessments and corneal imaging with in vivo CCM. Using ImageJ, the following parameters were quantified: corneal nerve length (IWL) and dendritic cell density (IWDC) in the inferior whorl region and corneal nerve fiber length (CNFL), nerve fiber density (CNFD), nerve branch density (CNBD), and dendritic cell density (CDC) in the peripheral region. Disease severity was evaluated using recognized scales. Results Corneal nerve lengths (IWL and CNFL) were lower while dendritic cell densities (IWDC and CDC) were higher in patients with ALS than controls in peripheral and inferior whorl regions (p < 0.05). Additionally, corneal nerve complexity in the peripheral region was greater in patients than controls with higher CNBD (p = 0.040) and lower CNFD (p = 0.011). IWL was significantly associated with disease severity (p < 0.001) and progression (p = 0.002) in patients with ALS. Patients with bulbar involvement showed significantly lower IWL (p = 0.014) and higher IWDC (p = 0.043) than patients without bulbar involvement. Conclusions CCM quantified significant corneal neuropathy in ALS, and alterations in the inferior whorl region were closely associated with disease severity. CCM could serve as a noninvasive, objective imaging tool to detect corneal small fiber neuropathy for clinical evaluation in ALS.


2010 ◽  
Vol 288 (1-2) ◽  
pp. 106-111 ◽  
Author(s):  
Isabella Laura Simone ◽  
Rosanna Tortelli ◽  
Vito Samarelli ◽  
Eustachio D'Errico ◽  
Michele Sardaro ◽  
...  

2008 ◽  
Vol 119 ◽  
pp. S87
Author(s):  
Marina de Tommaso ◽  
Michele Sardaro ◽  
Claudia Serpino ◽  
Vito Samarelli ◽  
Rosanna Tortelli ◽  
...  

2010 ◽  
Vol 16 (1) ◽  
pp. 61-63 ◽  
Author(s):  
Ales Hlubocky ◽  
Kay Wellik ◽  
Mark A. Ross ◽  
Benn E. Smith ◽  
Charlene Hoffman-Snyder ◽  
...  

Author(s):  
J. Lähdevirta ◽  
A. Rissanen ◽  
Y. Collan ◽  
E. J. Jokinen ◽  
J. Palo ◽  
...  

2019 ◽  
Vol 10 (5) ◽  
pp. 428-434
Author(s):  
Sophia C.I. Billig ◽  
Joana C. Schauermann ◽  
Roman Rolke ◽  
Istvan Katona ◽  
Jörg B. Schulz ◽  
...  

BackgroundRetrospective investigation of the somatosensory profile and prediction of histologic small fiber neuropathy (SFN) in postural orthostatic tachycardia syndrome (POTS) was performed using quantitative sensory testing (QST) as a standardized noninvasive test.MethodsIn this investigation, full data sets from 30 patients (age: 34.03 ± 10.82 years, n = 6 males), including results of autonomic function testing, norepinephrine values, skin biopsy, and QST, were retrospectively analyzed. The QST data were compared with healthy controls (HCs) (age: 34.20 ± 10.5 years, n = 6 males, t test: 0.95).ResultsThe evaluation of all QST parameters in POTS compared with HCs yielded differences in all thermal parameters (cold detection threshold: p < 0.05, warm detection threshold: p < 0.001, thermal sensory limen: p < 0.001, cold pain threshold: p < 0.05, and heat pain threshold: p < 0.001) and in paradoxical heat sensations (p < 0.05). Differences in nonpainful stimuli (mechanical detection threshold: p < 0.05 and vibration detection threshold: p < 0.001) were also detected. All patients who had clinical signs of SFN in combination with impairment of small fibers in QST also had SFN on skin biopsy.ConclusionThese results suggest that a non–region-specific SFN in POTS compared with controls can be detected by noninvasive QST that predicts histologic small fiber pathology.


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