Blink reflex in newly diagnosed and treated patients with Wilson’s disease

Abstract

Abnormal blink reflex (BR) results mainly from the dysfunction of reticular brainstem pathways and is one of the features of degenerative brain disorders. We aimed to investigate whether patients with Wilson’s disease (WD) have abnormal BR. This was a prospective, observational, single-center study. BR was assessed in accordance with generally accepted standards in 44 newly diagnosed treatment-naïve and 66 treated patients with WD. Any abnormal parameters in BR were observed in 45.5% treatment-naïve patients and 37.9% treated patients (p = 0.429). We also did not observe significant differences in BR parameters and frequency of abnormal findings between treated and treatment naïve patients. Abnormal findings in any of the BR parameters were more frequent in patients with neurological vs. non-neurological presentation (57.5 vs. 28.6%, p = 0.002), present vs. absent Kayser–Fleischer ring (73 vs. 21.5%, p < 0.001), and typical vs. no typical WD abnormalities in brain MRI (50% vs. 24.4%, p = 0.009). In addition, longer median R1 and R2 latencies, both ipsilateral and contralateral, were significantly more frequent in neurological than non-neurological WD patients, those with Kayser–Fleischer rings, and those with abnormal MRI findings typical of WD. Our results confirm frequent BR abnormalities in WD, which may be explained by the pathological influence of copper deposits in the circuit linking the basal ganglia, cerebellum and brainstem.

Olfactory dysfunction in patients with Wilson’s Disease

“…Although patients with a neurological presentation of WD typically develop extrapyramidal symptoms [8], other subclinical abnormalities have also been reported in motor-evoked potentials reflecting pyramidal tracts damage [20], somatosensory, auditory and visually-evoked potentials [21], visual pathways [22] and blink reflex [23]. Our study supports other reports which have suggested that, additionally, olfactory tracts may be affected in WD patients [11][12][13].…”