Neurocognitive evaluation of presymptomatic individuals carrying ALS-associated gene mutations using P300

ALS is a neurodegenerative disease with a poor prognosis. Recent studies have shown that degeneration in ALS is not limited to the motor system, but has a much broader and more widespread effect. The existence of a cognitive disorder in ALS has also been demonstrated in many studies. In fact, studies have shown that cognitive effects occur before motor symptoms. This information has changed the perspective on ALS to a certain extent. In our study, we examined presymptomatic individuals who carry the gene mutation known to cause ALS but who do not yet have symptoms and whose examinations are normal, neurophysiologically with event-related endogenous potentials. We evaluated the cases with tests developed for ALS in terms of cognitive involvement and neuropsychiatric conditions. 18 presymptomatic cases, 14 patients diagnosed with ALS according to the Gold Coast criteria, and 15 healthy controls participated in the study. MMSE, ACE-R, ECAS, and Beck depression scale were applied to all cases. P300 generated by auditory stimulation from event-related endogenous potentials was studied. The latency and amplitude of the resulting P300 wave were evaluated. When ALS patients were compared with healthy controls, a statistically significant difference was found in terms of ECAS executive functions and memory scores. ALS patients' P300 latencies were statistically significantly longer than healthy controls. Again, presymptomatic individuals' P300 latencies were statistically significantly longer than healthy controls. The difference between ALS and presymptomatic individuals' latencies was not statistically significant. No statistically significant difference was found in terms of P300 amplitudes. These findings support the idea that individuals carrying ALS-related gene mutations differ from normals in terms of cognition and that it may be important to monitor them for early findings, and that they can be evaluated as a candidate neurophysiological method as a biomarker.