This article reviews four of the involuntary hyperkinetic motor disorders that affect the orofacial region: bruxism, orofacial dystonia, oromandibular dyskinesia, and medication-induced extrapyramidal syndrome-dystonic reactions. It discusses and contrasts the clinical features and management strategies for spontaneous, primary, and drug-induced motor disorders in the orofacial region. The article provides a list of medications that have been reported to cause drug-related extrapyramidal motor activity, and discusses briefly the genetic and traumatic events that are associated with spontaneous dystonia. Finally, it presents an approach for management of the orofacial motor disorders. The contraindications, side effects, and usual approach for medications and injections are covered. An overview of the indications, contraindications, and complications of using botulinum toxin as a therapeutic modality is discussed briefly.
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http://dx.doi.org/10.1016/j.cden.2006.09.002 | DOI Listing |
Mov Disord Clin Pract
January 2025
University of Rochester Medical Center, Center for Health + Technology (CHeT), Rochester, New York, USA.
Background: Limited evidence exists regarding the meaningfulness of symptoms experienced in early Parkinson's disease (PD).
Objectives: To identify the most bothersome symptoms experienced by people with early PD, leveraging data from the Parkinson's Disease Patient Report of Problems (PD-PROP) questionnaire within the Fox Insight Study.
Methods: Individuals with a self-reported diagnosis of PD completed the PD-PROP questionnaire, reporting up to five most bothersome symptoms.
J Neurosci
January 2025
Arizona State University, Department of Psychology, Tempe, AZ, 85287 USA.
The cerebellum, identified to be active during cognitive and social behavior, has multisynaptic connections through the cerebellar nuclei (CN) and thalamus to cortical regions, yet formation and modulation of these pathways are not fully understood. Perineuronal nets (PNNs) respond to changes in local cellular activity and emerge during development. PNNs are implicated in learning and neurodevelopmental disorders, but their role in the CN during development is unknown.
View Article and Find Full Text PDFAm J Hum Genet
January 2025
Institute of Medical Genetics and Applied Genomics, University of Tübingen, 72076 Tübingen, Germany; Center for Rare Disease, University of Tübingen, 72076 Tübingen, Germany; Genomics for Health in Africa (GHA), Africa-Europe Cluster of Research Excellence (CoRE).
Inborn errors of selenoprotein expression arise from deleterious variants in genes encoding selenoproteins or selenoprotein biosynthetic factors, some of which are associated with neurodegenerative disorders. This study shows that bi-allelic selenocysteine tRNA-specific eukaryotic elongation factor (EEFSEC) variants cause selenoprotein deficiency, leading to progressive neurodegeneration. EEFSEC deficiency, an autosomal recessive disorder, manifests with global developmental delay, progressive spasticity, ataxia, and seizures.
View Article and Find Full Text PDFPhys Life Rev
December 2024
Community Healthcare Center Dr. Adolf Drolc Maribor, Ulica talcev 9, 2000 Maribor, Slovenia; Faculty of Natural Sciences and Mathematics, University of Maribor, Koroška cesta 160, 2000 Maribor, Slovenia; Complexity Science Hub, Metternichgasse 8, 1080 Vienna, Austria; Department of Physics, Kyung Hee University, 26 Kyungheedae-ro, Dongdaemun-gu, Seoul 02447, Republic of Korea. Electronic address:
Synchrony in neuronal networks is crucial for cognitive functions, motor coordination, and various neurological disorders. While traditional research has focused on pairwise interactions between neurons, recent studies highlight the importance of higher-order interactions involving multiple neurons. Both types of interactions lead to complex synchronous spatiotemporal patterns, including the fascinating phenomenon of chimera states, where synchronized and desynchronized neuronal activity coexist.
View Article and Find Full Text PDFJ Neurol Sci
December 2024
Department of Neurology, Seoul National University Hospital, Seoul, Republic of Korea; Department of Translational Medicine, Seoul National University College of Medicine, Seoul, Republic of Korea; Biomedical Research Institute, Seoul National University Hospital, Seoul, Republic of Korea; Wide River Institute of Immunology, Seoul National University, Hongcheon, Republic of Korea; Neuroscience Research Institute, Seoul National University College of Medicine, Seoul, Republic of Korea. Electronic address:
Facial onset sensory and motor neuronopathy (FOSMN) syndrome is a rare neurodegenerative disorder initially characterized by facial sensory deficits, which later progress to motor deficits in a rostral-caudal distribution. This study investigated the prevalence, clinical features, and prognosis of FOSMN syndrome and compared these aspects with those of bulbar-onset amyotrophic lateral sclerosis (ALS) within a single institutional cohort of motor neuron diseases. We identified four patients with FOSMN syndrome who had been misclassified as having bulbar-onset ALS, representing approximately 2 % of such ALS cases.
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