Article
Biochemistry & Molecular Biology
Lina-Marielle Krysewski, Nicole Power Guerra, Annika Glatzel, Carsten Holzmann, Veronica Antipova, Oliver Schmitt, Libo Yu-Taeger, Huu Phuc Nguyen, Andreas Wree, Martin Witt
Summary: This study investigated olfactory deficits in a transgenic rat model of Huntington's disease (HD). The results suggest that the main olfactory epithelium (MOE) is temporarily affected in HD, while the vomeronasal (VNE) remains relatively stable.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Review
Pharmacology & Pharmacy
Jessie S. Gibson, Daniel O. Claassen
Summary: This article reviews the medications used to treat HD chorea in the USA and emphasizes the importance of considering patient comorbidities when treating chorea. Prospective studies evaluating the long-term effectiveness and safety of VMAT-2 inhibitors and antipsychotics in HD are recommended.
EXPERT OPINION ON PHARMACOTHERAPY
(2021)
Article
Clinical Neurology
Erin Furr Stimming, Daniel Claassen, Elise Kayson, Jody Goldstein, Raja Mehanna, Hui Zhang, Grace S. Liang, Dietrich Haubenberger
Summary: Valbenazine was evaluated as a treatment for chorea associated with Huntington's disease, and it was found to be more effective than placebo in improving chorea symptoms and well tolerated.
Article
Clinical Neurology
Anna Heinzmann, Sabrina Sayah, Francois-Xavier Lejeune, Valerie Hahn, Marc Teichmann, Marie-Lorraine Monin, Enrica Marchionni, Fleur Gerard, Perrine Charles, Jeremie Pariente, Alexandra Durr
Summary: The study investigated the phenotype of CAG(36-38) repeat carriers and found that they had a similar cognitive profile to those with the more common CAG(40-42) expansions, but exhibited differences in motor function. This finding should encourage neurologists to consider Huntington's disease in cognitively impaired elderly patients without typical chorea and anticipate consequences for genetic counseling in their offspring.
MOVEMENT DISORDERS
(2023)
Review
Clinical Neurology
Roberta Bonomo, Antonio E. Elia, Giulio Bonomo, Luigi M. Romito, Caterina Mariotti, Grazia Devigili, Roberto Cilia, Riccardo Giossi, Roberto Eleopra
Summary: In patients with Huntington's disease, deep brain stimulation in the globus pallidus internus can lead to significant improvements in disease rating and motor symptoms, while effectively reducing chorea scores. Further studies are needed to identify reliable criteria to guide patient selection for DBS therapy.
NEUROLOGICAL SCIENCES
(2021)
Review
Biochemistry & Molecular Biology
Amy Kim, Kathryn Lalonde, Aaron Truesdell, Priscilla Gomes Welter, Patricia S. Brocardo, Tatiana R. Rosenstock, Joana Gil-Mohapel
Summary: Huntington's disease is a neurodegenerative disorder caused by a CAG expansion in the HD gene, with symptoms typically appearing in mid-life involving cognitive deficits and motor disturbances. Despite known genetic cause, multiple mechanisms are believed to contribute to neurodegeneration, leading to various pre-clinical and clinical studies testing therapeutic approaches.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Clinical Neurology
Jaroslaw Dulski, Anna Sulek, Magdalena Krygier, Wiktoria Radziwonik, Jaroslaw Slawek
Summary: This study reports a 42-year-old patient with clinical phenotype suggestive of HD, who was repeatedly negative on genetic testing until a new set of primers enabled successful diagnosis of HD. The study suggests incorporating these new primers into routine genetic testing for patients suspected of HD displaying homoallelism in the standard protocol.
EUROPEAN JOURNAL OF NEUROLOGY
(2021)
Article
Biochemistry & Molecular Biology
Evelini Placido, Priscilla Gomes Welter, Ana Wink, Gabriela Duarte Karasiak, Tiago Fleming Outeiro, Alcir Luiz Dafre, Joana Gil-Mohapel, Patricia S. Brocardo
Summary: Huntington's disease (HD) is a neurodegenerative genetic disorder without effective therapy, but evidence suggests that environmental enrichment (EE) can modulate disease progression. In this study, exposure to EE had an antidepressant-like effect and slowed motor deficits in HD mice. It also reduced monoamine levels and modulated neuronal differentiation in the hippocampus, suggesting that EE can be a therapeutic strategy for modulating neuroplasticity deficits in HD.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Biochemistry & Molecular Biology
In K. Cho, Faye Clever, Gordon Hong, Anthony W. S. Chan
Summary: Huntington's Disease (HD) is an incurable autosomal dominant disease characterized by severe neurodegeneration. The expansion of CAG trinucleotide repeat on the Huntingtin gene (HTT) is responsible for the disease. Our study focused on tissue-specific CAG repeat instability in transgenic nonhuman primate models of HD, and found a correlation between CAG repeat expansion and symptom severity and tissue susceptibility. Additionally, we observed a correlation between CAG repeat expansion and the expression profile of certain genes.
Article
Pharmacology & Pharmacy
Kendall Curtis, Victor Sung
Summary: Huntington disease (HD) is a hereditary neurodegenerative disorder characterized by chorea. Deutetrabenazine is an effective and well-tolerated treatment for HD-associated chorea, with a longer half-life and reduced plasma fluctuations compared to tetrabenazine.
JOURNAL OF CLINICAL PHARMACOLOGY
(2023)
Review
Clinical Neurology
Quang Tuan Remy Nguyen, Juan Dario Ortigoza Escobar, Jean-Marc Burgunder, Caterina Mariotti, Carsten Saft, Lena Elisabeth Hjermind, Katia Youssov, G. Bernhard Landwehrmeyer, Anne-Catherine Bachoud-Levi
Summary: This study examines the diagnosis of HD phenocopies and proposes guidance that combines the detection of clinical red flags with the classification of paraclinical testing options. The study utilizes a literature review and physician surveys to provide a diagnostic approach for HD phenocopies.
FRONTIERS IN NEUROLOGY
(2022)
Article
Clinical Neurology
Daniel O. Claassen, Rajeev Ayyagari, Viviana Garcia-Horton, Su Zhang, Jessica Alexander, Sam Leo
Summary: This study evaluated real-world adherence patterns with tetrabenazine and deutetrabenazine in patients diagnosed with Huntington's disease. Results showed that patients treated with deutetrabenazine had higher adherence and lower discontinuation rates compared to those treated with tetrabenazine. However, potential limitation includes overestimated adherence due to claims for prescription fills may not capture actual use. Further research is needed to explore differences in adherence patterns between these treatments for informed decision-making.
NEUROLOGY AND THERAPY
(2022)
Review
Pharmacology & Pharmacy
Robert Wiggins, Andrew Feigin
Summary: Huntington's disease is characterized by motor dysfunction, behavioral symptoms, and cognitive impairment, with current clinical practice focusing mainly on symptomatic treatment. Recent advancements in disease modifying therapeutics have created optimism in the field, with diverse and promising emerging therapies expected to have a meaningful impact on patients' lives.
EXPERT OPINION ON EMERGING DRUGS
(2021)
Article
Public, Environmental & Occupational Health
Ernesto Solis-Anez, Philippe A. Salles, Natalia Rojas, Olga Benavides, Pedro Chana-Cuevas
Summary: The study investigated the sociodemographic, genotype, and neuropsychiatric features of patients with Huntington's disease (HD) in Chile. The minimal prevalence of HD in Chile was found to be 0.72/100,000 inhabitants, with a mean CAG repeat of 47.2 and a mean age of onset of 41.39 years. 79.6% of patients had a family history of HD.
Article
Biotechnology & Applied Microbiology
Melanie Alpaugh, Maria Masnata, Aurelie de Rus Jacquet, Eva Lepinay, Helena L. Denis, Martine Saint-Pierre, Peter Davies, Emmanuel Planel, Francesca Cicchetti
Summary: This study suggests that attenuating tau pathology could mitigate behavioral and molecular hallmarks associated with Huntington's disease.
Article
Clinical Neurology
Anne E. P. Mulders, Yasin Temel, Mehmet Tonge, Frederic L. W. V. J. Schaper, Vivianne van Kranen-Mastenbroek, Linda Ackermans, Pieter Kubben, Marcus L. F. Janssen, Annelien Duits
Summary: Despite optimal improvement in motor functioning, both short and long-term studies have reported small but consistent changes in cognitive functioning following STN-DBS in Parkinson's disease. However, only few patients showed clinically relevant cognitive decline and surgical characteristics were not significantly associated with cognitive change scores. From a cognitive point of view, DBS may be considered a relatively safe procedure.
CLINICAL NEUROLOGY AND NEUROSURGERY
(2021)
Article
Medicine, Research & Experimental
Tim A. M. Bouwens van der Vlis, Megan M. G. H. van de Veerdonk, Linda Ackermans, Albert F. G. Leentjens, Marcus L. F. Janssen, Mark L. Kuijf, Koen R. J. Schruers, Annelien Duits, Felix Gubler, Pieter Kubben, Yasin Temel
Summary: This study retrospectively analyzed adverse events (AEs) of deep brain stimulation (DBS) procedures and found that surgical site infections and wire tethering were the most common AEs. However, major AEs requiring aggressive treatment were rare. A uniform reporting system for surgical and hardware-related AEs in DBS surgery would be useful.
Article
Neurosciences
Jeroen G. V. Habets, Christian Herff, Alfonso A. Fasano, Martijn Beudel, Ersoy Kocabicak, Alfons Schnitzler, Muneer Abu Snineh, Suneil K. Kalia, Carolina Ramirez-Gomez, Mojgan Hodaie, Renato P. Munhoz, Eline Rouleau, Onur Yildiz, Eduard Linetsky, Rick Schuurman, Christian J. Hartmann, Andres M. Lozano, Rob M. A. De Bie, Yasin Temel, Marcus L. F. Janssen
Summary: The study validated a predictive model (DBS-PREDICT) that considered 11 preoperative clinical characteristics and applied logistic regression to differentiate between weak and strong motor responders, predicting motor response after STN DBS. The results showed that the model effectively differentiated between weak and strong motor responders in an external multicenter cohort.
STEREOTACTIC AND FUNCTIONAL NEUROSURGERY
(2022)
Review
Medicine, General & Internal
Jana V. P. Devos, Yasin Temel, Linda Ackermans, Veerle Visser-Vandewalle, Oezguer A. Onur, Koen Schruers, Jasper Smit, Marcus L. F. Janssen
Summary: This paper provides an overview of the key aspects that need to be considered for conducting deep brain stimulation (DBS) studies.
JOURNAL OF CLINICAL MEDICINE
(2022)
Article
Neurosciences
Faris Almasabi, Marcus L. F. Janssen, Jana Devos, Michelle Moerel, Michael Schwartze, Sonja A. Kotz, Ali Jahanshahi, Yasin Temel, Jasper Smit
Summary: Tinnitus is an auditory sensation that occurs in the absence of actual external stimulation. The lack of successful treatment options for tinnitus is partly due to limited understanding of its mechanisms. Recent research has shown that the auditory thalamus plays a central role in the neuropathophysiology of tinnitus. Changes in thalamic activity and structure have been observed in both animal models and clinical studies. Based on these findings, a potential neuromodulatory treatment option for tinnitus is proposed.
Article
Geriatrics & Gerontology
M. T. H. M. Henkens, A. G. Raafs, J. A. J. Verdonschot, M. Linschoten, M. van Smeden, P. Wang, B. H. M. van der Hooft, R. Tieleman, M. L. F. Janssen, R. M. A. ter Bekke, M. R. Hazebroek, I. C. C. van der Horst, F. W. Asselbergs, F. J. H. Magdelijns, S. R. B. Heymans
Summary: Age is the main determinant of COVID-19 related in-hospital mortality, with negligible mediation effect of pre-existing comorbidities.
Article
Neurosciences
Faris Almasabi, Faisal Alosaimi, Minerva Corrales-Terron, Anouk Wolters, Dario Strikwerda, Jasper Smit, Yasin Temel, Marcus L. F. Janssen, Ali Jahanshahi
Summary: This study conducted post-mortem histopathological assessment to reveal structural changes in auditory and non-auditory regions of tinnitus patients. The findings suggest that neurodegenerative and inflammatory processes may underlie the neuropathology of tinnitus, and the raphe serotonergic system may play a role in tinnitus.
Article
Neurosciences
Hannah Bernhard, Frederic L. W. V. J. Schaper, Marcus L. . F. Janssen, Erik D. Gommer, Bernadette M. Jansma, Vivianne Van Kranen-Mastenbroek, Rob P. W. Rouhl, Peter de Weerd, Joel Reithler, Mark J. Roberts
Summary: In this study, sleep spindles were investigated through thalamic recordings, revealing systematic coordination of multichannel spindle patterns at both thalamic and thalamocortical levels. Different subtypes of spindles were associated with distinct topographical patterns of thalamocortical spindle overlap.
Article
Neurosciences
Faris Almasabi, Gusta van Zwieten, Faisal Alosaimi, Jasper V. Smit, Yasin Temel, Marcus L. F. Janssen, Ali Jahanshahi
Summary: Tinnitus, the phantom perception of sound, currently lacks effective curative treatment. Deep brain stimulation has been suggested as a potential therapy, but the optimal target and stimulation regimens are still unclear. This study investigated metabolic and neuronal activity changes in a rat model of tinnitus and found alterations in multiple brain areas, including the auditory and limbic regions. The study also demonstrated that high-frequency stimulation of the medial geniculate body enhanced neuronal activity in the thalamic reticular nucleus. These findings provide further rationales for targeting the medial geniculate body with high-frequency stimulation as a symptom management tool in tinnitus.
Article
Behavioral Sciences
Aryo Zare, Gusta van Zwieten, Sonja A. Kotz, Yasin Temel, Faris Almasabi, Benjamin G. Schultz, Michael Schwartze, Marcus L. F. Janssen
Summary: This study investigated the sensory gating functions of the medial geniculate body (MGB) by recording electrophysiological evoked potentials and assessing the effect of high-frequency stimulation (HFS) on these responses. The results showed that all animals exhibited gating for pitch and grouping, while only animals not exposed to noise showed gating for temporal regularity. Furthermore, only noise-exposed animals showed restoration comparable to typical EP amplitude suppression following MGB HFS. These findings confirm adaptive thalamic sensory gating based on different sound characteristics and demonstrate the impact of temporal regularity on MGB auditory signaling.
BEHAVIOURAL BRAIN RESEARCH
(2023)
Letter
Clinical Neurology
Jana V. P. Devos, Jasper V. Smit, Erwin L. J. George, Carsten Leue, Linda Ackermans, Yasin Temel, Marcus L. F. Janssen
Letter
Clinical Neurology
Yuzhong Zhang, Marcus L. F. Janssen, Erik D. D. Gommer, Qing Zhang, Raymond van de Berg
JOURNAL OF NEUROLOGY
(2023)
Article
Clinical Neurology
Pia Brinkmann, Marit Stolte, Jana V. P. Devos, Marcus L. F. Janssen, Michael Schwartze, Sonja A. Kotz
Summary: This study designed and validated a Dutch Sensory Gating Inventory (D-SGI) to assess the capacity for sensory filtering in Dutch speaking populations. The results showed satisfactory similarity between the D-SGI and the original English SGI in terms of psychometric properties. The D-SGI demonstrated good internal consistency, discriminant validity, and test-retest reliability.
APPLIED NEUROPSYCHOLOGY-ADULT
(2023)
Article
Audiology & Speech-Language Pathology
Gusta van Zwieten, Jana V. P. Devos, Sonja A. Kotz, Linda Ackermans, Pia Brinkmann, Lobke Dauven, Erwin L. J. George, A. Miranda L. Janssen, Bernd Kremer, Carsten Leue, Michael Schwartze, Yasin Temel, Jasper V. Smit, Marcus L. F. Janssen
Summary: This study protocol aims to evaluate the safety and therapeutic effects of deep brain stimulation (DBS) in the medial geniculate body (MGB) of severe tinnitus sufferers. The study will use a double-blinded, randomized crossover design to assess safety and feasibility, as well as potential treatment effects and tinnitus severity measured using the Tinnitus Functional Index (TFI).
AUDIOLOGY RESEARCH
(2023)
Meeting Abstract
Medicine, General & Internal
Olena Britoa, Joao Casaca Carreirab
ANNALS OF MEDICINE
(2021)
Article
Behavioral Sciences
Radwa H. Lutfy, Sherine Abdel Salam, Haitham S. Mohammed, Marwa M. Shakweer, Amina E. Essawy
Summary: Insufficient sleep is associated with impaired hypothalamic activity and declined attentional performance. This study found that near-infrared (NIR) laser therapy can alleviate the effects of sleep deprivation on the hypothalamus, enhance antioxidant status, suppress neuroinflammation, and regulate cellular activity.
BEHAVIOURAL BRAIN RESEARCH
(2024)
Article
Behavioral Sciences
Durmus Ali Aslanlar, Emin Fatih Visneci, Mehmet Oz, K. Esra Nurullahoglu Atalik
Summary: Mood disorders caused by chemotherapy have become more important as cancer patients' survival increases. This study used methotrexate to induce mood disorders in rats and found that treatment with the antioxidant N-acetylcysteine (NAC) can alleviate anxiety and depression-like behaviors, increase antioxidant capacity, reduce oxidative stress and inflammatory response, and regulate brain chemistry. The findings suggest that NAC treatment could be an effective strategy in revising the treatment for individuals suffering from chemotherapy-induced mood disorders.
BEHAVIOURAL BRAIN RESEARCH
(2024)
Article
Behavioral Sciences
Yunfan Zhang, Yunbin Zhang, Zhuangfei Chen, Ping Ren, Yu Fu
Summary: This study systematically investigated the effects of extremely low intensity HF-rTMS on cognition in mice and found that 40 Hz rTMS significantly impaired exploratory behavior and spatial memory at both 10 mT and 1 mT conditions. Additionally, 40 Hz stimulation had remarkably different effects on exploratory behavior depending on intensity, compared to 10 Hz stimulation.
BEHAVIOURAL BRAIN RESEARCH
(2024)
Article
Behavioral Sciences
Xuan Xuan, Guangling Zheng, Wenjia Zhu, Qionghua Sun, Yawei Zeng, Juan Du, Xusheng Huang
Summary: This study examines the functional characteristics of the cerebellum in individuals with sALS and their correlation with clinical data. The results show changes in both local and global functional connectivity in the cerebellum of sALS patients, suggesting a pathophysiological role of the cerebellum in sALS.
BEHAVIOURAL BRAIN RESEARCH
(2024)
Article
Behavioral Sciences
Mehdi Rezaei, Mohammad Mahdi Shariat Bagheri
Summary: This study examined the efficacy of tDCS for PTSD and related symptoms, as well as the factors that may predict response to tDCS. The results showed that tDCS had a positive effect in reducing symptoms of PTSD, depression, anxiety, and anhedonia. The severity of symptoms at baseline may also predict the response to tDCS.
BEHAVIOURAL BRAIN RESEARCH
(2024)
Article
Behavioral Sciences
Huimin Wu, Yiqun Guo, Yaoyao Zhang, Le Zhao, Cheng Guo
Summary: Aggression can have serious consequences, but little is known about its personality and neurological origins in children. This study investigated the relationship between self-esteem, aggression, and brain structure in healthy children, and found that self-esteem was negatively associated with aggression. The study also revealed that increased cortical thickness in certain brain areas may be a potential mechanism linking low self-esteem to aggression in children.
BEHAVIOURAL BRAIN RESEARCH
(2024)
Article
Behavioral Sciences
Xinmei Deng, Kexin Chen, Xiaoming Chen, Lin Zhang, Mingping Lin, Xiaoqing Li, Qiufeng Gao
Summary: Parental involvement affects the relationship and communication between parents and adolescents. This study found that high parental involvement is associated with stronger brain-to-brain synchrony during shared positive emotional experiences, while low parental involvement is associated with stronger synchrony during shared negative emotional experiences.
BEHAVIOURAL BRAIN RESEARCH
(2024)
Article
Behavioral Sciences
Xin Deng, I. -Shuo Huang, Kourtlin Williams, Marcy L. Wainwright, Paul Zimba, Riccardo Mozzachiodi
Summary: Food deprivation can lead to neurological dysfunctions, including memory impairment. This study used Aplysia as an animal model to investigate the memory deficits caused by prolonged food deprivation. The results showed that 14 days of food deprivation decreased the level of 5-HT in the hemolymph, which contributed to the lack of sensitization and its cellular correlates. However, exogenous application of 5-HT partially induced sensitization in the food deprived animals.
BEHAVIOURAL BRAIN RESEARCH
(2024)
Article
Behavioral Sciences
Ihori Kobayashi, Patrick A. Forcelli
Summary: The study found that intervention with the dual orexin receptor antagonist suvorexant did not have the expected effects on extinction memory and sleep. Higher percentages of REM sleep were associated with poorer extinction memory recall and stronger fear responses. Additionally, the fear extinction training protocol used in this study did not lead to complete fear extinction.
BEHAVIOURAL BRAIN RESEARCH
(2024)
Article
Behavioral Sciences
Jiyan Xu, Xinlu Chen, Shuai Liu, Ziqi Wei, Minhui Xu, Linhao Jiang, Xue Han, Liangyu Peng, Xiaoping Gu, Tianjiao Xia
Summary: This study investigated the effects of nicotinamide mononucleotide (NMN) on oxidative stress and cognitive function in postoperative cognitive dysfunction (POCD) mice. The results showed that NMN pretreatment reduced oxidative stress damage and alleviated cognitive impairment in POCD mice.
BEHAVIOURAL BRAIN RESEARCH
(2024)
Article
Behavioral Sciences
Song Liu, Qiang Wu, Liyue Wang, Cong Xing, Junrui Guo, Baicao Li, Hongpeng Ma, Hao Zhong, Mi Zhou, Shibo Zhu, Rusen Zhu, Guangzhi Ning
Summary: In this study, a systematic assessment indicator was developed to objectively evaluate hindlimb motor function recovery in rats after thoracic contusion SCI. By screening CatWalk XT gait parameters and using exploratory factor analysis, 38 suitable parameters for assessing motor function were identified. A reliable Coordinated Function Index (CFI) was proposed based on these parameters and simplified for improved assessment efficacy.
BEHAVIOURAL BRAIN RESEARCH
(2024)
Article
Behavioral Sciences
Kyosuke Shiga, Shota Miyaguchi, Yasuto Inukai, Naofumi Otsuru, Hideaki Onishi
Summary: This study aimed to investigate the effects of transcranial alternating current stimulation (tACS) on microscale learning in implicit motor tasks. Contrary to expectations, the results showed that the stimulation protocol had no significant effects on microscale learning, revealing a novel aspect of microscale learning in implicit motor tasks.
BEHAVIOURAL BRAIN RESEARCH
(2024)
Article
Behavioral Sciences
Cahide Aslan, Rahime Aslankoc, Ozlem Ozmen, Buse Nur Suluk, Oguzhan Kavrik, Nurhan Gumral
Summary: This study examined the negative effects of high fructose corn syrup on prefrontal cortex damage in adolescent rats, as well as the protective role of vitamin D.
BEHAVIOURAL BRAIN RESEARCH
(2024)
Article
Behavioral Sciences
Matin Baghani, Arad Bolouri-Roudsari, Reyhaneh Askari, Abbas Haghparast
Summary: The study suggests that the orexinergic system in the dentate gyrus region of the brain may act as an endogenous pain control system and a potential target for treating stress-related disorders.
BEHAVIOURAL BRAIN RESEARCH
(2024)
Article
Behavioral Sciences
Sen Zhou, Yang Liu, Binbin Xue, Peigen Yuan
Summary: This study confirmed that low-dose Esketamine alleviates LPS-induced depressive symptoms by regulating the GSK-3 beta/NLRP3 pathway. Appropriate doses of Esketamine are essential for the treatment of depression in the clinical setting.
BEHAVIOURAL BRAIN RESEARCH
(2024)
