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Oral Diseases Jan 2023The objective of this systematic literature review was to provide a complete panorama of the oral manifestations of Huntington's disease (HD). (Review)
Review
OBJECTIVES
The objective of this systematic literature review was to provide a complete panorama of the oral manifestations of Huntington's disease (HD).
MATERIALS AND METHODS
Databases were searched, and original research studies or case report manuscripts up to May 2021 were included using keywords that describe HD combined with words related to oral health; MeSH terms were used exclusively. No time or language restrictions were applied.
RESULTS
Twenty-two investigations (12 original articles and 10 case reports) regarding oral manifestations of HD were included. The subjects examined in the selected research articles were dental health, coordination of oral structures, speech, dysphagia, and swallowing alterations. The case reports described dental treatment procedures, oromandibular dyskinesia, dysphagia, and speech alterations.
CONCLUSIONS
The oral manifestations of HD were found to be associated with the advance of the disease in that the more severe the HD, the worse the alterations affecting the oral cavity. Dysphagia, dysarthria, masticatory problems, oral health impairment, and choreiform movements involving the tongue and other orofacial muscles were the main manifestations of HD in the oral cavity. The PROSPERO systematic review registration number of this study is CRD42021238934.
Topics: Humans; Huntington Disease; Deglutition Disorders; Prevalence; Deglutition
PubMed: 34773332
DOI: 10.1111/odi.14076 -
Nihon Shokakibyo Gakkai Zasshi = the... 2023Metronidazole (MNZ) is a widely used drug for protozoan and anaerobic infections. The continuous use of MNZ causes various neurological symptoms, such as cerebellar...
Metronidazole (MNZ) is a widely used drug for protozoan and anaerobic infections. The continuous use of MNZ causes various neurological symptoms, such as cerebellar ataxia, visual disturbance, vestibulocochlear symptoms, gait disturbance, dysarthria, and epileptic seizures of unknown cause, named MNZ-induced encephalopathy (MIE), in rare cases. MIE is a reversible disease that often improves within a few days of MNZ discontinuation, but irreversible neurological symptoms rarely remain. Herein, we report a case of MIE that developed during MNZ administration for a liver abscess, causing prolonged unconsciousness and death even after drug discontinuation. An 85-year-old female patient complained of fever, elevated liver enzymes, and a multifocal abscess in the right hepatic lobe, as seen on computed tomography. Percutaneous transhepatic abscess drainage and antibiotic therapy were initiated. The causative agent of the liver abscess could not be identified, thus meropenem was started, which demonstrated no inflammation improvement, thus oral MNZ was added. The inflammation recurred when MNZ was discontinued, and the patient continued taking MNZ. Vomiting, upper limb tremors, consciousness disturbance, and convulsions appeared on day 46 (total dose of MNZ 73.5mg), and the patient was hospitalized. T2-weighted, diffusion-weighted, and FLAIR head magnetic resonance imaging (MRI) revealed symmetrical abnormal high-signal areas in the cerebellar dentate nucleus, corpus callosum, cerebral white matter, and periventricular areas. MIE was diagnosed based on the patient's course and MRI images, and MNZ was discontinued. The patient continued to suffer from impaired consciousness and convulsions after MNZ discontinuation and died due to aspiration pneumonia. Suggestively, MIE development is related to long-term MNZ administration, poor nutrition, liver disease, underlying diseases (such as advanced cancer), and serious complications. A systematic review of MIE cases revealed that 4.8-5.9% of the patients demonstrated little improvement of symptoms after MNZ discontinuation, and some deaths were reported. Patients with poor prognosis were often suffering from impaired consciousness and convulsions. Furthermore, impaired consciousness was the most common residual symptom. Abnormal signals in characteristic areas, such as the dentate nucleus cerebri and corpus callosum, on head MRI are useful for MIE diagnosis, especially in patients with abnormal findings in the cerebral white matter, which is associated with a poor prognosis. We should pay close attention to the onset of MIE when MNZ is administered.
Topics: Female; Humans; Aged, 80 and over; Metronidazole; Brain Diseases; Anti-Bacterial Agents; Seizures; Liver Abscess
PubMed: 37821376
DOI: 10.11405/nisshoshi.120.858 -
American Journal of Speech-language... May 2022Speech-language pathologists (SLPs) often advise adult patients to complete at-home programs in order to improve outcomes. Despite this widespread practice, relatively...
PURPOSE
Speech-language pathologists (SLPs) often advise adult patients to complete at-home programs in order to improve outcomes. Despite this widespread practice, relatively little is known about treatment adherence. The purposes of this systematic review were to identify adherence strategies and adherence tracking methods used by adult populations that are commonly treated by SLPs (i.e., dysphagia, aphasia, traumatic brain injury, dysphonia, dysarthria), and to identify the efficacy of these strategies.
METHOD
The systematic review was conducted in accordance with A Measurement Tool to Assess Systematic Reviews guidelines. A comprehensive literature search was performed in three databases (CINAHL, PubMed, and Web of Science).
RESULTS
Of the 679 articles found, 18 were selected for analysis. Two thirds of the included articles received the second highest rating on the 5-point JAMA Quality Rating Scheme. Interventions designed to alter treatment adherence included (most to least frequent) computer programs, portable devices/phone apps, alarm reminders, instructional DVDs, check-ins from a clinician/volunteer, and wearable device. Adherence reporting methods included (most to least frequent) self-report diaries, computer program/app-aided collection, wearable device, and clinician/volunteer observation. Of the articles that reported practice frequency, 58% found that adherence strategies improved practice frequency as compared to control. Of the articles that reported treatment outcomes, 66% found that adherence strategies were associated with improved treatment outcomes as compared to control.
CONCLUSIONS
The paucity of publications reviewed suggests that treatment adherence is considerably understudied in speech-language pathology. A clearer understanding of how to improve the design of adherence strategies could yield highly valuable clinical outcomes.
SUPPLEMENTAL MATERIAL
https://doi.org/10.23641/asha.19393793.
Topics: Adult; Aphasia; Humans; Pathologists; Speech-Language Pathology
PubMed: 35320678
DOI: 10.1044/2022_AJSLP-21-00255 -
Journal of Clinical Neuroscience :... Mar 2021Hereditary spastic paraplegia type 11 (SPG11) is the most common subtype of autosomal recessive hereditary spastic paraplegia (HSP), to date, there are more than 181...
Hereditary spastic paraplegia type 11 (SPG11) is the most common subtype of autosomal recessive hereditary spastic paraplegia (HSP), to date, there are more than 181 different KIAA1840 gene mutations detected, and yet the genetic landscape of SPG11 is far from complete. To find the clinical and genetic characteristics of SPG11, we performed a reanalysis of the clinical features and genotype-phenotype correlations in all reported studies exhibiting SPG11 mutations. A total of 339 patients were collected, their mean age at onset was 13.10 ± 3.65 years, with initial symptoms like gait disturbance (107/195, 54.87%) and mental retardation (47/195, 24.10%). Cognitive decline (228/270, 84.44%) was the most common complex manifestation stepped by dysarthria (134/195, 68.72%), neuropathy (112/177, 63.28%), amyatrophy, sphincter disturbance (60/130, 46.15%) and ataxia (90/194, 46.39%). The most common brain MRI abnormality is thinning of the corpus callosum (TCC) (173/190, 91.05%), followed by periventricular white matter changes (130/158, 82.28%), cerebral or cerebellar cortical atrophy (55/107, 51.40%). The mutational spectrum associated with KIAA1840 gene is wide, and frameshift mutations are the most common type followed by nonsense mutations. Our reanalysis demonstrated that SPG11 exhibited significant clinical and genetic heterogeneity, and no clear genotype-phenotype correlation was observed. There is no mutational hot spot in the KIAA1840 gene, which emphasizes the need to analyse the whole gene in clinical practice. In addition to conventional genetic testing methods, further mRNA analysis should be conducted on some cases to yield a definitive diagnosis.
Topics: Adolescent; Age of Onset; Child; Female; Genetic Association Studies; Humans; Male; Mutation; Proteins; Spastic Paraplegia, Hereditary
PubMed: 33581793
DOI: 10.1016/j.jocn.2020.11.036 -
Journal of Medical Systems Feb 2015The release of smartphones and tablets, which offer more advanced communication and computing capabilities, has led to the strong emergence of mHealth on the market.... (Review)
Review
The release of smartphones and tablets, which offer more advanced communication and computing capabilities, has led to the strong emergence of mHealth on the market. mHealth systems are being used to improve patients' lives and their health, in addition to facilitating communication between doctors and patients. Researchers are now proposing mHealth applications for many health conditions such as dementia, autism, dysarthria, Parkinson's disease, and so on. Usability becomes a key factor in the adoption of these applications, which are often used by people who have problems when using mobile devices and who have a limited experience of technology. The aim of this paper is to investigate the empirical usability evaluation processes described in a total of 22 selected studies related to mHealth applications by means of a Systematic Literature Review. Our results show that the empirical evaluation methods employed as regards usability could be improved by the adoption of automated mechanisms. The evaluation processes should also be revised to combine more than one method. This paper will help researchers and developers to create more usable applications. Our study demonstrates the importance of adapting health applications to users' need.
Topics: Humans; Mobile Applications; Research Design; Software Design; Telemedicine; User-Computer Interface
PubMed: 25600193
DOI: 10.1007/s10916-014-0182-2 -
Brain Stimulation 2018The cerebellum is involved in the pathophysiology of many movement disorders and its importance in the field of neuromodulation is growing.
BACKGROUND
The cerebellum is involved in the pathophysiology of many movement disorders and its importance in the field of neuromodulation is growing.
OBJECTIVES
To review the current evidence for cerebellar modulation in movement disorders and its safety profile.
METHODS
Eligible studies were identified after a systematic literature review of the effects of cerebellar modulation in cerebellar ataxia, Parkinson's disease (PD), essential tremor (ET), dystonia and progressive supranuclear palsy (PSP). Neuromodulation techniques included transcranial magnetic stimulation (TMS), transcranial direct current stimulation (tDCS) and deep brain stimulation (DBS). The changes in motor scores and the incidence of adverse events after the stimulation were reviewed.
RESULTS
Thirty-four studies were included in the systematic review, comprising 431 patients. The evaluation after stimulation ranged from immediately after to 12 months after. Neuromodulation techniques improved cerebellar ataxia due to vascular or degenerative etiologies (TMS, tDCS and DBS), dyskinesias in PD patients (TMS), gross upper limb movement in PD patients (tDCS), tremor in ET (TMS and tDCS), cervical dystonia (TMS and tDCS) and dysarthria in PSP patients (TMS). All the neuromodulation techniques were safe, since only three studies reported the existence of side effects (slight headache after TMS, local skin erythema after tDCS and infectious complication after DBS). Eleven studies did not mention if adverse events occurred.
CONCLUSIONS
Cerebellar modulation can improve specific symptoms in some movement disorders and is a safe and well-tolerated procedure. Further studies are needed to lay the groundwork for new researches in this promising target.
Topics: Cerebellum; Clinical Trials as Topic; Deep Brain Stimulation; Humans; Movement Disorders; Parkinson Disease; Transcranial Direct Current Stimulation; Transcranial Magnetic Stimulation; Treatment Outcome
PubMed: 29191439
DOI: 10.1016/j.brs.2017.11.015 -
Pediatric Neurosurgery 2019The purpose of this study was to explore the clinical features and risk factors of outcomes in pediatric posterior cranial fossa ependymoma. We aim to provide...
OBJECTIVE
The purpose of this study was to explore the clinical features and risk factors of outcomes in pediatric posterior cranial fossa ependymoma. We aim to provide evidence-based recommendations for the improvement of prognoses.
PATIENTS AND METHODS
The clinical data, treatment modalities, approaches performed, recurrence rates and times, as well as the outcomes of 94 cases were analyzed retrospectively. The characters of neuroimaging were further studied.
RESULTS
In data from the most recent follow-up, 27 cases had tumor recurrence. The time for tumor recurrence was 13.7 ± 7.7 months. The estimated overall survival and progression-free survival, based on Kaplan-Meier analysis, was 42.2 ± 2.9 months and 38.7 ± 3.4 months, respectively. Univariate analysis showed that being free of recurrence is closely related to the high tumor sphericity (p = 0.018), homogeneity of tumor texture (p = 0.001), and gross total resection (GTR; p < 0.001). Mortality is linked to low sphericity (p = 0.017) and brain stem edema (p = 0.005). Cerebellar mutism is correlated with posterosuperior compression of the 4th ventricle roof by the tumor. The incidence rate of cerebellar ataxia, cerebellar mutism, and cerebellar dysarthria is related to the rostral extension of the tumor within the 4th ventricle. The recurrence rate is higher in subtotal resection (STR) than in GTR, and the difference is significant (p < 0.001). Although there is no significant difference between the recurrence rates in the three types, an earlier recurrence is prone with tumors located in the paramidline-lateral compared to the midline (p = 0.021) and paramidline-medial areas (p = 0.042).
CONCLUSIONS
Based on our data, GTR is indicated as the most optimal choice. Recurrence is linked to lower tumor sphericity, inhomogeneous tumor texture, and STR/partial resection. Tumor located on the lateral side might be prone for an early recurrence.
Topics: Adolescent; Child; Child, Preschool; Cranial Fossa, Posterior; Ependymoma; Female; Follow-Up Studies; Humans; Infant; Male; Prognosis; Retrospective Studies; Skull Base Neoplasms; Treatment Outcome
PubMed: 30699434
DOI: 10.1159/000495809 -
Journal of Neurology, Neurosurgery, and... Jul 2021Cognitive impairment is a common, far-reaching but imperceptible manifestation in patients with amyotrophic lateral sclerosis (ALS). We aimed to identify the risk... (Meta-Analysis)
Meta-Analysis
OBJECTIVE
Cognitive impairment is a common, far-reaching but imperceptible manifestation in patients with amyotrophic lateral sclerosis (ALS). We aimed to identify the risk factors for cognitive impairment in ALS.
METHODS
We searched PubMed and EMBASE for cross-sectional, case-control and cohort studies that reported predictors of cognitive impairment in ALS. The obtained data were meta-analysed to generate overall ORs and 95% CIs.
RESULTS
Twenty-seven eligible articles reporting on 6799 individuals were included out of 20 501 records. Nine predictors were identified: (OR 3.62, 95% CI 1.76 to 7.45), dysarthria (OR 2.25, 95% CI 1.20 to 4.22), family history of ALS (OR 1.76, 95% CI 1.18 to 2.61), predominant upper motor neuron (PUMN) phenotype (OR 1.73, 95% CI 1.09 to 2.73) and bulbar onset (OR 1.54, 95% CI 1.28 to 1.87) increased risk factors for cognitive impairment in ALS. ALS Functional Rating Scale-Revised scores, sex, age or education level were not significantly associated with cognitive impairment in ALS. In addition, (OR=5.94) and bulbar onset (OR=2.08) were strong predictors of ALS-frontotemporal dementia. Female sex conferred more susceptibility to executive cognitive impairment than male sex (OR=1.82).
CONCLUSIONS
Patients with repeat expansion, dysarthria, family history of ALS, PUMN phenotype and bulbar onset had a high risk for cognitive impairment in ALS. These associations may contribute to understanding the heterogeneity of ALS.
PROSPERO REGISTRATION NUMBER
CRD42020201085.
Topics: Amyotrophic Lateral Sclerosis; Cognitive Dysfunction; Female; Humans; Male; Risk Factors; Sex Factors
PubMed: 33563800
DOI: 10.1136/jnnp-2020-325701 -
BMJ Case Reports Dec 2021Myasthenia gravis (MG) is an autoimmune condition affecting the neuromuscular junction characterised by weakness and fatiguability, carrying a high morbidity if...
Myasthenia gravis (MG) is an autoimmune condition affecting the neuromuscular junction characterised by weakness and fatiguability, carrying a high morbidity if treatment is delayed. A clear association with thymoma has led to management with thymectomy as a common practice, but MG presenting post-thymectomy has rarely been reported. We present a case of an 82- year-old woman developing fatigue, ptosis and dysarthria 3 months after thymectomy. After a clinical diagnosis of MG was made, she responded well to prompt treatment with prednisolone and pyridostigmine. Her anti-acetylcholine receptor antibody (anti-AChR) subsequently came back positive. Our systematic review reveals that post-thymectomy MG can be categorised as early-onset or late-onset form with differing aetiology, and demonstrated correlation between preoperative anti-AChR titres and post-thymectomy MG. The postulated mechanisms for post-thymectomy MG centre around long-lasting peripheral autoantibodies. Clinicians should actively look for MG symptoms in thymoma patients and measure anti-AChR preoperatively to aid prognostication.
Topics: Aged, 80 and over; Female; Humans; Autoantibodies; Myasthenia Gravis; Receptors, Cholinergic; Thymectomy; Thymoma; Thymus Neoplasms
PubMed: 34857591
DOI: 10.1136/bcr-2021-246005 -
Journal of Infection and Chemotherapy :... Feb 2021Vagococcal infections are uncommon in humans; there are limited studies on the clinical manifestations, the optimal methods for identifications, and antimicrobial...
BACKGROUND
Vagococcal infections are uncommon in humans; there are limited studies on the clinical manifestations, the optimal methods for identifications, and antimicrobial susceptibility testing for vagococcal infections. Here, we have reported a case of Vagococcus fluvialis-induced bacteremia and decubitus ulcer and have systematically reviewed other reported Vagococcus infections.
CASE PRESENTATION
A 74-year-old man presented to our emergency department with muscle weakness on his left extremities, dysarthria, and altered mental status along with fever for the past 4 days. Physical examination revealed a decubitus ulcer with foul smelling and yellowish exudative pus on his left chest wall and abdomen, forearm, thigh, and lower leg. He was empirically treated with 2.25 mg of piperacillin/tazobactam every 8 hours and 0.5 g of vancomycin every 24 hours intravenously (IV) for his decubitus ulcer. Vagococcus fluvialis was detected in both aerobic and anaerobic blood cultures (upon admission) using the VITEC 2 GP ID card (bioMérieux) and matrix-assisted laser desorption/ionization-time-of-flight mass spectrometry (MALDI-TOF MS). We continued the mentioned IV antimicrobial therapies for 4 weeks following which the patient was transferred to a long-term care facility for further rehabilitation.
CONCLUSIONS
To our best knowledge, this is the first literature review of Vagococcus infections in humans. Since it is challenging to distinguish Vagococcus from Enterococcus by a conventional method due to the similarity of its biochemical properties to those of Enterococcus, based on our literature review, 16S rRNA sequencing or analysis of bacterial protein profile using MALDI-TOF MS may be useful for the precise identification.
Topics: Aged; Bacteremia; Enterococcaceae; Humans; Male; Pressure Ulcer; RNA, Ribosomal, 16S; Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization
PubMed: 33036895
DOI: 10.1016/j.jiac.2020.09.019