Subthalamic and pallidal oscillatory activity in patients with Neurodegeneration with Brain Iron Accumulation type I (NBIA-I)
| Autor: | Tomasz Kmiec, Christof Brücke, Anahita Poshtiban, Rafał Rola, S. Siegert, Henryk Koziara, Julius Huebl, Tomasz Mandat, Andrea A. Kühn, Antje Bock |
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| Rok vydání: | 2019 |
| Předmět: |
Male
Deep brain stimulation Movement disorders Adolescent Neurodegeneration with brain iron accumulation medicine.medical_treatment Globus Pallidus 050105 experimental psychology Young Adult 03 medical and health sciences 0302 clinical medicine Subthalamic Nucleus Physiology (medical) Basal ganglia Humans Medicine Premovement neuronal activity 0501 psychology and cognitive sciences Child Pantothenate Kinase-Associated Neurodegeneration Dystonia business.industry Parkinsonism 05 social sciences medicine.disease Sensory Systems nervous system diseases Subthalamic nucleus nervous system Neurology Female Neurology (clinical) medicine.symptom Beta Rhythm business Neuroscience 030217 neurology & neurosurgery |
| Zdroj: | Clinical Neurophysiology. 130:469-473 |
| ISSN: | 1388-2457 |
| DOI: | 10.1016/j.clinph.2018.12.012 |
| Popis: | Objectives Neurodegeneration with Brain Iron Accumulation type I (NBIA-I) is a rare hereditary neurodegenerative disorder with pallidal degeneration leading to disabling generalized dystonia and parkinsonism. Pallidal or subthalamic deep brain stimulation can partially alleviate motor symptoms. Disease-specific patterns of abnormally enhanced oscillatory neuronal activity recorded from the basal ganglia have been described in patients with movement disorders undergoing deep brain stimulation (DBS). Here we studied oscillatory activity recorded from the internal globus pallidus (GPi) and the subthalamic nucleus (STN) to characterize neuronal activity patterns in NBIA-I. Methods We recorded local field potentials (LFP) from DBS electrodes in 6 juvenile patients with NBIA-I who underwent functional neurosurgery. Four patients were implanted in the STN and two patients in the GPi. Recordings were performed during wakeful rest. An FFT-based approach was used to analyze the power spectrum in the target area. Results In all patients we found distinct peaks in the low frequency (7–12 Hz) and in 5 out 6 also in the beta frequency range (15–30 Hz) with the largest beta peak in the patient that presented with the most prominent bradykinesia. No distinct peaks occurred in the gamma frequency range (35–100 Hz). The oscillatory pattern did not differ between STN and GPi. Conclusions Here we show for the first time the oscillatory activity pattern in the STN and the GPi in juvenile patients with dystonia plus syndrome due to NBIA-I. The low frequency peak we found is in line with previous studies in patients with isolated idiopathic dystonia. In our cohort, the pallidal beta band activity may be related to more severe motor slowing in dystonia plus syndrome such as NBIA-I. Significance Our results further support the link between hyperkinetic motor symptoms such as dystonia and enhanced basal ganglia low frequency activity irrespective of the underlying etiology of dystonia. |
| Databáze: | OpenAIRE |
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