Article
Clinical Neurology
Sicong Tu, Mengjie Huang, Jashelle Caga, Colin J. Mahoney, Matthew C. Kiernan
Summary: The study investigated the association between pseudobulbar affect and pathological laughter and crying episodes among ALS patients. It found that the presence of pseudobulbar affect was significantly higher in ALS patients with bulbar onset. Additionally, the frequency of pathological laughter episodes was differentially associated with cognitive performance and brainstem integrity.
FRONTIERS IN NEUROLOGY
(2021)
Article
Clinical Neurology
Julian Klingbeil, Max Wawrzyniak, Anika Stockert, Max-Lennart Brandt, Hans-Ralf Schneider, Moritz Metelmann, Dorothee Saur
Summary: The study of pathological laughter and crying provides insights into the neural basis of these behaviors, which are triggered by CNS disorders. Lesions causing PLC are linked to dysfunction in a cortico-limbic-subcortico-thalamo-ponto-cerebellar network, involving both emotional and volitional systems for controlling facial movements.
Article
Clinical Neurology
Qian-Qian Wei, Ruwei Ou, Junyu Lin, Lingyu Zhang, Yanbing Hou, Bei Cao, Yongping Chen, Tianmi Yang, Huifang Shang
Summary: The study aimed to explore the prevalence and clinical correlates of pathological laughter and crying (PLC) in patients with amyotrophic lateral sclerosis (ALS). Results showed that PLC was not uncommon in ALS, especially in bulbar-onset and female patients. Emotional state and disease stage were found to be important factors associated with PLC in ALS.
FRONTIERS IN NEUROLOGY
(2021)
Article
Neuroimaging
Francesca Trojsi, Federica Di Nardo, Giulia D'Alvano, Giuseppina Caiazzo, Carla Passaniti, Antonella Mangione, Minoo Sharbafshaaer, Antonio Russo, Marcello Silvestro, Mattia Siciliano, Mario Cirillo, Gioacchino Tedeschi, Fabrizio Esposito
Summary: This study explored the brain functional connectivity (FC) correlates of pseudobulbar affect (PBA) in patients with amyotrophic lateral sclerosis (ALS). The findings suggest that alterations of fronto-tempo-parietal-cerebellar circuits could be related to PBA in ALS patients. Abnormal FC between the cerebellum and posterior cingulate cortex and left middle frontal gyrus highlight the crucial role of the cerebellum in regulating emotion expression in ALS patients with PBA.
BRAIN IMAGING AND BEHAVIOR
(2023)
Article
Behavioral Sciences
Volker A. Coenen, Bastian E. A. Sajonz, Trevor A. Hurwitz, Marlies Boeck, Jonas A. Hosp, Peter C. Reinacher, Horst Urbach, Ganna Blazhenets, Philipp T. Meyer, Marco Reisert
Summary: This article reports two cases of stimulation induced pathological laughter (PL) in patients undergoing thalamic deep brain stimulation (DBS) for essential tremor and interprets the effects based on a modified neuroanatomy of positive affect display (PAD). The researchers found that a co-stimulation of parts of a fiber structure called motorMFB can lead to a non-volitional modulation of PAD, resulting in PL.
FRONTIERS IN BEHAVIORAL NEUROSCIENCE
(2022)
Article
Neurosciences
Zuzanna Z. Balewski, Thomas W. Elston, Eric B. Knudsen, Joni D. Wallis
Summary: During decision-making, neurons in the orbitofrontal cortex (OFC) switch between representing the value of different options, influencing the encoding of choice response in the anterior cingulate cortex (ACC). By studying simultaneous recordings from OFC and ACC in nonhuman primates, it was found that ACC neurons encoding the choice response steadily increased their firing rate throughout the decision-making process, with OFC value dynamics affecting the ramping of ACC activity. The interaction between OFC and ACC explains how the more valuable response is selected.
NATURE NEUROSCIENCE
(2023)
Article
Clinical Neurology
Marlene Tahedl, Ee Ling Tan, We Fong Siah, Jennifer C. Hengeveld, Mark A. Doherty, Russell L. McLaughlin, Orla Hardiman, Eoin Finegan, Peter Bede
Summary: PBA is a symptom of neurological conditions, characterized by disproportionate emotional responses. This study used neuroimaging techniques to explore the neuroanatomical basis of PBA and found associations between white matter degeneration and PBA. These findings are consistent with the classical model of PBA.
JOURNAL OF THE NEUROLOGICAL SCIENCES
(2023)
Article
Neurosciences
Toshio Shimizu, Yuki Nakayama, Kota Bokuda, Kazushi Takahashi
Summary: The study found enhanced cortical responses in somatosensory evoked potentials (SEP) in patients with amyotrophic lateral sclerosis (ALS), with no significant differences in sensory gating between patients and controls. The baseline amplitudes of certain components of SEP were significantly correlated with their gating ratios in ALS patients, indicating preserved sensory gating in the early stages of the disease.
Review
Multidisciplinary Sciences
Karin Roelofs, Bob Bramson, Ivan Toni
Summary: Emotion regulation is crucial for survival in a rapidly changing world. The ability to switch between different emotional control strategies is important for dealing with various demands. The Flexible Emotion Control Theory (FECT) integrates insights from decision and affective sciences to explain how individuals can adapt their emotion-regulatory behavior. FECT proposes that rapid switching between emotional control strategies requires evaluating both current and alternative options, and involves specific prefrontal structures such as the frontal pole.
ANNALS OF THE NEW YORK ACADEMY OF SCIENCES
(2023)
Review
Neurosciences
Longhong Zhu, Shihua Li, Xiao-Jiang Li, Peng Yin
Summary: Researchers have created animal models to study ALS, a complex neurodegenerative disease. However, most genetically modified rodent models lack key pathological features. Recent studies using large animals like pigs and non-human primates have provided important insights into the disease.
TRANSLATIONAL NEURODEGENERATION
(2023)
Article
Clinical Neurology
Hiroshi Yaguchi, Kenichi Sakuta, Taiji Mukai, Shinji Miyagawa
Summary: This study aimed to characterize the pharyngeal findings in amyotrophic lateral sclerosis (ALS) using a fiberoptic laryngoscope and compare them with the findings of general neurological examination. The specific findings of laryngoscopy in ALS patients, such as speech-swallow dissociation (SSD) in velopharyngeal closure and laterality in pharyngeal constriction, could not be evaluated by general neurological examination via the mouth.
JOURNAL OF THE NEUROLOGICAL SCIENCES
(2022)
Article
Biochemistry & Molecular Biology
Debora Lanznaster, Clement Bruno, Jerome Bourgeais, Patrick Emond, Ilyess Zemmoura, Antoine Lefevre, Pascal Reynier, Sebastien Eymieux, Emmanuelle Blanchard, Patrick Vourc'h, Christian R. Andres, Salah Eddine Bakkouche, Olivier Herault, Luc Favard, Philippe Corcia, Helene Blasco
Summary: This study investigates the relationship between biomarkers, pathological alterations, and energetic metabolism dysfunction in the muscle of ALS patients. The findings suggest that there is a global dysfunction in the early-stage ALS patients' muscles. Novel metabolites have been identified as potential biomarkers for the diagnosis and prognosis of ALS patients.
Article
Multidisciplinary Sciences
Keith A. Bush, G. Andrew James, Anthony A. Privratsky, Kevin P. Fialkowski, Clinton D. Kilts
Summary: This study used a combination of engineering control theory, machine learning, and human neuroimaging to investigate the role of the dorsal anterior cingulate cortex (dACC) in goal-directed performance monitoring during an emotion regulation task. The findings suggest that the dACC plays a role in affect processing, arousal, attention, and regulation resources. The study also introduced a novel analytical framework for understanding emotion processing and regulation.
Article
Genetics & Heredity
Jiahao Cai, Xiong Chen, Hongxuan Wang, Zixin Wei, Mei Li, Xiaoming Rong, Xiangpen Li, Ying Peng
Summary: The study found no causal relationship between iron status and the risk of ALS. Efforts to change iron status to reduce ALS incidence may be impractical.
FRONTIERS IN GENETICS
(2021)
Article
Cardiac & Cardiovascular Systems
Jesus Sancho, Santos Ferrer, Enric Bures, Jose Luis Diaz, Teresa Torrecilla, Jaime Signes-Costa, Emilio Servera
Summary: The study showed that treatment with DM/Q in ALS patients does not prolong noninvasive respiratory management or prevent long-term deterioration of bulbar function. However, it was found to improve pseudobulbar affect during one-year follow-up.
RESPIRATORY MEDICINE
(2021)
Article
Clinical Neurology
Katharina Althaus, Martin Kasel, Albert C. Ludolph, Jan Kassubek, Rebecca Kassubek
Summary: This study retrospectively analyzed 23,948 cerebral MRIs and found that 84 images from 61 patients showed HARM-positive results. HARM was observed not only in stroke patients, but also in other neurological conditions, and it was not dependent on therapy.
INTERNATIONAL JOURNAL OF STROKE
(2022)
Article
Clinical Neurology
Angela Rosenbohm, Kelly Del Tredici, Heiko Braak, Hans-Juergen Huppertz, Albert C. Ludolph, Hans-Peter Mueller, Jan Kassubek
Summary: The study utilized DTI data to investigate specific white matter alterations in flail arm syndrome patients, showing similarities in white matter integrity with 'classical' ALS patients. The results support the hypothesis that flail arm syndrome is a phenotypical variant of ALS.
JOURNAL OF NEUROLOGY
(2022)
Article
Clinical Neurology
Florian Philipp Raber, Florian Vincent Gmeiner, Jens Dreyhaupt, Armin Wolf, Albert Christian Ludolph, Jens Ulrich Werner, Jan Kassubek, Katharina Althaus
Summary: This study aimed to investigate the feasibility, efficacy, and safety of intravenous thrombolysis (IVT) in functionally blind patients with non-arteritic central retinal artery occlusion (NA-CRAO). The results showed that early IVT therapy within 4.5 hours of symptom onset might be a potential treatment option for NA-CRAO. MRI should be performed in all patients for optimized treatment and secondary stroke prevention.
JOURNAL OF NEUROLOGY
(2023)
Editorial Material
Clinical Neurology
Jan Kassubek
FRONTIERS IN NEUROLOGY
(2022)
Article
Biology
Martin Ulrich, Katharina Heckel, Markus Koelle, Georg Groen
Summary: This study investigated the effects of methylphenidate on brain functional connectivity in responders and non-responders of ADHD treatment. Using machine learning techniques, patients were categorized into responders and non-responders based on clinical symptoms. The results showed that only responders exhibited significant changes in functional connectivity, indicating that methylphenidate may modulate the putamen for successful ADHD treatment.
Review
Clinical Neurology
Martin Regensburger, Chi Wang Ip, Zacharias Kohl, Christoph Schrader, Peter P. Urban, Jan Kassubek, Wolfgang H. Jost
Summary: Inhibitors of monoamine oxidase B (MAO-B) and catechol-O-methyltransferase (COMT) are important strategies in Parkinson's disease treatment. Several drugs have been available on the market for more than a decade, while others have been approved more recently. Various post-authorization studies have provided comprehensive data on their use and characteristics in real-life clinical practice. This article summarizes the current knowledge on these medications and discusses their clinical value in Parkinson's disease, as well as practical considerations and ongoing studies.
JOURNAL OF NEURAL TRANSMISSION
(2023)
Article
Clinical Neurology
Hans-Peter Mueller, Anna Behler, Maximilian Muench, Johannes Dorst, Albert C. C. Ludolph, Jan Kassubek
Summary: This study investigates the association between the sequential alteration pattern and disease severity in patients with ALS using diffusion tensor imaging (DTI). The results show that the progression of white matter alterations across tracts is associated with clinical disease severity, suggesting the use of staging-based DTI as a technical marker for disease progression.
JOURNAL OF NEUROLOGY
(2023)
Article
Neurosciences
Wolfgang Becker, Anna Behler, Olga Vintonyak, Jan Kassubek
Summary: The characteristics of microsaccades and square wave jerks (SWJs) have been found to follow common patterns in different neurodegenerative disorders. The amplitude of microsaccades is related to the frequency of SWJ-like patterns. This suggests that there are differences in microsaccade characteristics between different diseases, possibly due to different underlying mechanisms.
EXPERIMENTAL BRAIN RESEARCH
(2023)
Article
Neurosciences
Martin Ulrich, Alexander Rueger, Verena Durner, Georg Groen, Heiko Graf
Summary: Sexually charged stimuli dominate in engaging the brain's reward structures, while multiple brain regions are activated in processing both primary and secondary rewards. However, the effects of different stimuli are weaker in reward expectation and prediction error.
Article
Genetics & Heredity
Sarah Jesse, Hans-Peter Mueller, Hans-Juergen Huppertz, Stephanie Andres, Albert C. Ludolph, Michael Schoen, Tobias M. Boeckers, Jan Kassubek
Summary: Alterations of CSF markers in Phelan-McDermid syndrome patients were found to be correlated with structural connectivity losses. These findings have important implications for further clinical diagnostics and basic research.
ORPHANET JOURNAL OF RARE DISEASES
(2023)
Article
Clinical Neurology
Franz Marxreiter, Vera Lambrecht, Angelika Mennecke, Jannis Hanspach, Jelena Jukic, Martin Regensburger, Juergen Herrler, Alexander German, Jan Kassubek, Georg Groen, Hans-Peter Mueller, Frederik B. Laun, Arnd Doerfler, Juergen Winkler, Manuel A. Schmidt
Summary: Quantitative susceptibility mapping (QSM) on high-field MRI shows excellent diagnostic accuracy in the differential diagnosis of Parkinson's disease (PD) and multiple system atrophy (MSA). Particularly, putaminal susceptibility measures on ultra-high-field MRI can distinguish MSA patients from PD patients, allowing for an early and sensitive diagnosis of MSA.
THERAPEUTIC ADVANCES IN NEUROLOGICAL DISORDERS
(2023)
Review
Biochemistry & Molecular Biology
Anna Behler, Hans-Peter Mueller, Albert C. C. Ludolph, Jan Kassubek
Summary: Diffusion tensor imaging (DTI) can be used to visualize white matter alterations in patients with amyotrophic lateral sclerosis (ALS), and machine learning (ML) models based on DTI parameters offer new opportunities for ALS diagnosis and patient stratification. To capture the full range of neuropathological signatures, DTI can be combined with other modalities, such as T1w 3-D MRI, in ML models. Standardized DTI protocols and multi-center collaborations are needed to validate multimodal DTI biomarkers for ALS.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Clinical Neurology
Jan Kassubek, Fabrizio Stocchi, Ernest Balaguer Martinez, Rajesh Pahwa, William Ondo, Yi Zhang, Alyssa Bowling, Eric Pappert, Stuart Isaacson, Stacy Wu
Summary: Most patients with Parkinson's disease were able to optimize the dosage of sublingual apomorphine at home and find their optimal dosage.
THERAPEUTIC ADVANCES IN NEUROLOGICAL DISORDERS
(2023)
Article
Neuroimaging
Maximilian Wiesenfarth, Hans-Juergen Huppertz, Johannes Dorst, Dorothee Lule, Albert C. Ludolph, Hans-Peter Mueller, Jan Kassubek
Summary: This study explores the MRI characteristics of C9orf72-associated ALS and finds significant alterations in both white matter and gray matter in the early stages of the disease.
NEUROIMAGE-CLINICAL
(2023)