OBJECTIVE Normal pressure hydrocephalus (NPH) is clinically characterized by gait disturbance, cognitive impairment, and urinary incontinence, as well as enlargement of the ventricles. To the best of the authors' knowledge, there have been no previous publications regarding the correlation between bulbar dysfunction and NPH. The primary objective of this study was to compare preoperative and postoperative prevalence of bulbar dysfunction in patients with NPH. Secondary objectives included assessing the results of surgery for swallowing, speech, gait, cognition, and urination, and evaluating the correlation between bulbar dysfunction and triad symptoms. METHODS Fifty-three patients with NPH who underwent shunt placement surgery at Siriraj Hospital were included in the study. Patients were evaluated for gait, cognition, urination, swallowing, and speech before and 6 months after shunt placement. Triad symptoms were assessed using standard methods. Bulbar dysfunctions were assessed using the Swallowing Problem Questionnaire, Thai Articulation Test, Resonation Screening Test (RST), and Thai Nasality Test. The Thai Speech Assessment Program and nasometer were used for objective speech measurement. RESULTS Preoperatively, 86% (43/50) of patients had swallowing problems and 75% (37/49) had speech problems, as measured by the RST. Postoperatively, there was significant improvement in swallowing (p < 0.001), speech problems by RST (p = 0.008), and voice volume (p = 0.009), but no significant change in the nasometer test. All triad symptoms were improved. There were significant correlations between swallowing impairment and gait disturbance (r = 0.358, p = 0.009), and RST and cognitive impairment (r = -0.502, p < 0.001). CONCLUSIONS This is the first study of bulbar dysfunction in patients with NPH. The results showed that the prevalence of bulbar dysfunction is very high. The correlation between bulbar dysfunction and the classic NPH triad has been documented and published. These bulbar symptoms also significantly improved after surgery. As such, bulbar dysfunction should be regarded as a core symptom that should be considered along with the classic triad in the clinical diagnosis and management of NPH.
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http://dx.doi.org/10.3171/2016.6.FOCUS16183 | DOI Listing |
Brain Behav
January 2025
Department of Neurology, Rambam Medical Center, Haifa, Israel.
Background: Myasthenia gravis (MG) is strongly associated with thymic tumors, but whether it is also associated with extrathymic cancers is debatable or whether MG can be considered a paraneoplastic disorder for extrathymic cancers.
Methods: This is a retrospective analysis of the MG cohort for 23 years' time (January 2000 to May 2023), extracting cancer rates with clinical, electrophysiological, and biochemical cancer associations and the effect of chronic medications.
Results: We identified 436 patients with MG and 3924 controls.
PLoS Genet
January 2025
Department of Pediatric and Adolescent Medicine, Mayo Clinic, 200 1st St. SW, Rochester, Minnesota 55905, United States of America.
Motor neuron diseases, such as amyotrophic lateral sclerosis (ALS) and progressive bulbar palsy, involve loss of muscle control resulting from death of motor neurons. Although the exact pathogenesis of these syndromes remains elusive, many are caused by genetically inherited mutations. Thus, it is valuable to identify additional genes that can impact motor neuron survival and function.
View Article and Find Full Text PDFOrphanet J Rare Dis
January 2025
Department of Voice, Speech and Hearing Disorders, University Dysphagia Center, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
Background: Bulbar function is frequently impaired in patients with spinal muscular atrophy (SMA). Although extremely important for the patient's quality of life, it is difficult to address therapeutically. Due to bulbar dysfunction, maximum mouth opening (MMO) is suspected to be reduced in children with SMA.
View Article and Find Full Text PDFJ Neurol
January 2025
Department of Circuit Theory, Faculty of Electrical Engineering, Czech Technical University in Prague, Technická 2, Praha 6, 16000, Prague, Czech Republic.
Background And Objectives: Patients with synucleinopathies such as multiple system atrophy (MSA) and Parkinson's disease (PD) frequently display speech and language abnormalities. We explore the diagnostic potential of automated linguistic analysis of natural spontaneous speech to differentiate MSA and PD.
Methods: Spontaneous speech of 39 participants with MSA compared to 39 drug-naive PD and 39 healthy controls matched for age and sex was transcribed and linguistically annotated using automatic speech recognition and natural language processing.
Cornea
January 2025
Department of Ophthalmology and Visual Sciences, Escola Paulista de Medicina, Universidade Federal de São Paulo, São Paulo, SP, Brazil.
Purpose: To evaluate the efficacy and safety of intense pulsed light (IPL) combined with meibomian gland expression (MGX) for the treatment of dry eye disease and meibomian gland dysfunction associated with chronic Stevens-Johnson syndrome and toxic epidermal necrolysis.
Methods: This prospective noncomparative interventional study included 29 patients (58 eyes) who underwent 3 sessions of IPL and MGX at 2-week intervals. Subjective symptoms (ocular surface disease index score) and objective dry eye tests: matrix metalloproteinase 9, tear meniscus height, bulbar redness score, tear film lipid layer thickness (LLT), Schirmer I test, conjunctival and corneal staining, meibomian gland loss, MGX score [meibomian gland score (MGS)], and tear break-up time were assessed at the baseline and after 4, 8, and 12 weeks.
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