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1.
Clin Endosc ; 57(3): 402-406, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38605688

RESUMO

Cronkhite-Canada syndrome is a rare gastrointestinal polyposis syndrome with distinctive clinical features and endoscopic findings. Diagnosis can be challenging without suspicion, and the disease carries high mortality due to complications such as infection, gastrointestinal bleeding, and malignancies. This paper presents two cases of Cronkhite-Canada syndrome occurring after coronavirus disease 2019 (COVID-19) mRNA vaccination. Both cases exhibited typical clinical findings, including hypogeusia, onychodystrophy, alopecia, and weight loss. Typical polyposis in the gastrointestinal tract was confirmed through endoscopies. As symptomatic treatment did not improve the symptoms, corticosteroids were administered, and symptoms and laboratory test results improved immediately. The patients improved upon corticosteroids tapering. These cases illustrate typical presentations of Cronkhite-Canada syndrome and the course of the disease following corticosteroid treatment. Additionally, they suggest the possibility that Cronkhite-Canada syndrome may be triggered by COVID-19 mRNA vaccination.

2.
Eur J Neurol ; 31(5): e16242, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38344918

RESUMO

BACKGROUND AND PURPOSE: Diagnosis of lymphoma involving the central nervous system (CNS) is challenging. This study aimed to explore the abnormal vestibular and ocular motor findings in CNS lymphoma. METHODS: A retrospective search of the medical records identified 30 patients with CNS lymphoma presenting ocular motor and vestibular abnormalities from four neurology clinics of university hospitals in South Korea (22 men, age range 14-81 years, mean 60.6 ± 15.2). The demographic and clinical features and the results of laboratory, radiological and pathological evaluation were analyzed. RESULTS: Patients presented with diplopia (13/30, 43%), vestibular symptoms (15/30, 50%) or both (2/30, 7%). In 15 patients with diplopia, abnormal ocular motor findings included ocular motor nerve palsy (n = 10, 67%), internuclear ophthalmoplegia (n = 2, 13%), external ophthalmoplegia (n = 2, 13%) and exophoria (n = 1, 7%). The vestibular abnormalities were isolated in 14 (82%) of 17 patients with vestibular symptoms and included combined unilateral peripheral and central vestibulopathy in three from lesions involving the vestibular nuclei. CNS lymphoma involved the brainstem (53%), cerebellum (33%), leptomeninges (30%), deep gray nuclei (23%) or cranial nerves (17%). Two patients showed the "double-panda" sign by involving the midbrain. CONCLUSIONS: This study expands the clinical and radiological spectra of CNS lymphoma. Neuro-ophthalmological and neuro-otological evaluation may guide the early diagnosis of CNS lymphoma.


Assuntos
Diplopia , Transtornos da Motilidade Ocular , Masculino , Humanos , Adolescente , Adulto Jovem , Adulto , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais , Estudos Retrospectivos , Transtornos da Motilidade Ocular/diagnóstico , Movimentos Oculares , Cerebelo , Paralisia
3.
J Clin Neurol ; 20(3): 315-320, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38330419

RESUMO

BACKGROUND AND PURPOSE: The neutrophil-to-lymphocyte ratio (NLR) and platelet-to-lymphocyte ratio (PLR) have been identified as useful biomarkers for assessing the inflammatory response and for predicting the prognosis of various diseases. This study aimed to determine the clinical significance and effects on prognostic prediction of NLR and PLR in acute unilateral vestibulopathy (AUV). METHODS: We retrospectively recruited 128 patients who were diagnosed with AUV from July 2016 to April 2021, and compared NLR and PLR values between these patients with AUV and age- and sex-matched healthy subjects. We also analyzed the correlations of various clinical parameters with NLR and PLR. RESULTS: NLR and PLR in the AUV group were 3.41±2.80 (mean±standard deviation) and 128.86±67.06, respectively, with only NLR being significantly higher than that in the control group (1.55±0.60, p<0.001). The gain asymmetry of the horizontal vestibulo-ocular reflex (VOR) was slightly larger in patients with high NLR (n=52) than in those with normal NLR (n=76) (41.9%±20.2% vs. 33.6%±17.4%, p=0.048). However, the hospitalization period, preceding infection, canal paresis, and absolute horizontal VOR gain did not differ between patients with high and normal NLR and PLR values. The correlation analyses also revealed that none of the clinical parameters were significantly correlated with NLR or PLR. At 3-month follow-up examinations, NLR and PLR did not differ significantly between patients with and without function recovery of the horizontal VOR. CONCLUSIONS: This study found a high NLR in AUV, suggesting an acute inflammatory status in the vestibular organ. However, the usefulness of NLR and PLR as prognostic markers remains unclear.

4.
Intest Res ; 2023 Nov 09.
Artigo em Inglês | MEDLINE | ID: mdl-37939723

RESUMO

Background/Aims: We investigated the incidences of overall and site-specific malignancies and chemopreventive effects of statin, metformin, and aspirin in patients with ulcerative colitis. Methods: We collected data using the Health Insurance Review and Assessment claims database from January 2007 to April 2020. Results: The overall malignancy risk among the 35,189 ulcerative colitis patients was similar to that of the general population (standardized incidence ratio, 0.94; 95% confidence interval, 0.88-1.00). In male patients, standardized incidence ratios were high for thyroid cancer and low for stomach cancer, colorectal cancer, liver cancer, and lung cancer. Concurrently, standard incidence ratios were high for liver cancer and central nervous system cancer in female patients. While 122 cases of colorectal cancer occurred in the study patients, the standardized incidence ratio was 0.83 (95% confidence interval, 0.69-0.99). Treatment for ulcerative colitis was not associated with an increased adjusted hazard ratio, while comorbidities increased it for all malignancies. Treatment for ulcerative colitis was associated with an increased adjusted hazard ratio, while comorbidities did not increase it for colorectal cancer. After adjusting for age, sex, comorbidities, and ulcerative colitis treatment, statins showed a dose-dependent chemopreventive effect for all malignancies (P=0.002), while metformin and aspirin did not show any. Conclusions: In ulcerative colitis patients, standardized incidence ratios for all malignancies and colorectal cancer did not increase. Adjusted hazard ratios for all malignancies increased with comorbidities and those for colorectal cancer with ulcerative colitis treatment. Statins have a dose-dependent chemopreventive effect for all malignancies.

5.
Turk J Gastroenterol ; 34(9): 932-942, 2023 09.
Artigo em Inglês | MEDLINE | ID: mdl-37565797

RESUMO

BACKGROUND/AIMS: The number of endoscopic procedures and related adverse events is increasing. We investigated South Korean endoscopists' awareness and experience of endoscopic adverse events. MATERIALS AND METHODS: We used Google Forms to conduct an online questionnaire survey among South Korean endoscopists from December 11 to 29, 2020. The survey comprised 30 questions developed by members of the Quality Management Committee of the Korean Society of Gastrointestinal Endoscopy. RESULTS: In total, 475 endoscopists participated in the survey. Of these, 454 (95.6%) were board-certified gastroenterologists and 255 (53.7%) had >10 years of endoscopy experience. Most participants had experienced serious adverse events requiring hospitalization (80.4%, 382/475); however, only 100 (21.1%) were aware of programs for the prevention and management of adverse endoscopic events in their affiliated endoscopy centers. Most participants (98.5%, 468/475) agreed with the need for education on medical accidents for healthcare workers. Responses were inconsistent regarding the definition of adverse events formulated by the 2010 American Society for Gastrointestinal Endoscopy Workshop. Most participants were not aware of the minimal standard terminology (76.6%, 364/475) and had not used it when writing endoscopy reports (88.8%, 422/475). Responses were inconsistent regarding which events to record in endoscopy records. CONCLUSION: Further discussion on the nationwide adverse-event reporting system and education program for adverse events related to endoscopy is needed to ensure the safety of patients and endoscopists.


Assuntos
Endoscopia Gastrointestinal , Gastroenterologistas , Humanos , Estados Unidos , Endoscopia Gastrointestinal/métodos , Inquéritos e Questionários , República da Coreia
6.
Front Neurol ; 14: 1223996, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37475735

RESUMO

Objectives: This study aimed to determine the pathophysiology of recurrent benign paroxysmal positional vertigo (BPPV) in young patients using gene expression profiling combined with bioinformatics analysis. Methods: Total RNA was extracted from the whole blood of four young patients with recurrent BPPV and four controls. The differentially expressed genes (DEGs) between the groups were screened using a microarray analysis based on the cutoff criteria of |log2 fold change| > 1 and an adjusted p-value of < 0.05. Functional enrichment analysis of DEGs was performed using Gene Ontology analysis, and the protein-protein interaction (PPI) network was constructed using the Search Tool for the Retrieval of the Interacting Genes database. Results: A total of 39 DEGs were detected between the BPPV and control samples, comprising 33 upregulated DEGs and six downregulated DEGs in the BPPV group. Functional enrichment analysis indicated that the upregulated DEGs were significantly enriched in terms related to metabolic processes and the immune system. Two main pathways were extracted from the PPI network: one was associated with oxidative phosphorylation and stress and the other with the adaptive immune system and extracellular matrix degradation. Conclusion: The findings of our bioinformatics analysis indicated that oxidative stress or extracellular matrix degradation due to immune-mediated inflammatory responses may contribute to the development of recurrent BPPV in young patients.

7.
Clin Endosc ; 56(4): 499-509, 2023 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-36997186

RESUMO

BACKGROUND/AIMS: This study aimed to evaluate the characteristics of endoscopic retrograde cholangiopancreatography (ERCP) or endoscopic ultrasonography (EUS)-related adverse events (AEs) that eventually lead to medical disputes or claims on medical professional liability. METHODS: Medical disputes for ERCP/EUS-related AEs filed in the Korea Medical Dispute Mediation and Arbitration Agency between April 2012 and August 2020 were evaluated using corresponding medical records. AEs were categorized into three sections: procedure-related, sedation-related, and safety-related AEs. RESULTS: Among a total of 34 cases, procedure-related AEs were 26 (76.5%; 12 duodenal perforations, 7 post-ERCP pancreatitis, 5 bleedings, 2 perforations combined with post-ERCP pancreatitis); sedation-related AEs were 5 (14.7%; 4 cardiac arrests, 1 desaturation), and safety-related AEs were 5 (8.8%; 1 follow-up loss for stent removal, 1 asphyxia, 1 fall). Regarding clinical outcomes, 20 (58.8%) were fatal and eventually succumbed to AEs. For the types of medical institutions, 21 cases (61.8%) occurred at tertiary or academic hospitals, and 13 (38.2%) occurred at community hospitals. CONCLUSION: The ERCP/EUS-related AEs filed in Korea Medical Dispute Mediation and Arbitration Agency showed distinct features: duodenal perforation was the most frequent AE, and clinical outcomes were fatal, resulting in at least more than permanent physical impairment.

8.
eNeurologicalSci ; 30: 100447, 2023 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-36743268

RESUMO

Background: Many kinds of vaccines have been developed worldwide to bring the coronavirus disease 2019 (COVID-19) to an end. We report a case of recurrent orbital apex syndrome following the first and third doses of SARS-CoV-2 vaccination. Case presentation: A 71-year-old woman presented with acute painless diplopia and visual disturbance for two days. She had received the first dose of the COVID-19 vaccine two weeks before. She showed decreased visual acuity and ophthalmoplegia in the right eye. An orbital magnetic resonance image (MRI) revealed a hyperintense lesion with enhanced bulging in the right cavernous sinus. Following the steroid pulse therapy, she fully recovered. However, six months after the first attack, painful ophthalmoplegia with decreased visual acuity recurred in her left eye after the booster vaccination for COVID-19. MRI also showed a well-enhanced hyperintense lesion in the left orbital apex. Fortunately, her visual acuity and ocular motility returned to normal after the steroid therapy. Conclusions: Immunologic reactions from COVID-19 vaccines may cause multiple cranial neuropathies. Diverse individual immunologic states should be considered before any kind of vaccine.

9.
Intest Res ; 21(1): 3-19, 2023 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-36751042

RESUMO

The introduction of device-assisted enteroscopy (DAE) in the beginning of 21st century has revolutionized the diagnosis and treatment of diseases of the small intestine. In contrast to capsule endoscopy, the other main diagnostic modality of the small bowel diseases, DAE has the unique advantages of observing the region of interest in detail and enabling tissue acquisition and therapeutic intervention. As DAE becomes an essential procedure in daily clinical practice, there is an increasing need for correct guidelines on when and how to perform it and what technical factors should be considered. In response to these needs, the Korean Association for the Study of Intestinal Diseases developed an expert consensus statement on the performance of DAE by reviewing the current evidence. This expert consensus statement particularly focuses on the indications, choice of insertion route, therapeutic intervention, complications, and relevant technical points.

10.
Intest Res ; 21(1): 20-42, 2023 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-36751043

RESUMO

Colonoscopic polypectomy is effective in decreasing the incidence and mortality of colorectal cancer (CRC). Premalignant polyps discovered during colonoscopy are associated with the risk of metachronous advanced neoplasia. Postpolypectomy surveillance is the most important method for managing advanced metachronous neoplasia. A more efficient and evidence-based guideline for postpolypectomy surveillance is required because of the limited medical resources and concerns regarding colonoscopy complications. In these consensus guidelines, an analytic approach was used to address all reliable evidence to interpret the predictors of CRC or advanced neoplasia during surveillance colonoscopy. The key recommendations state that the high-risk findings for metachronous CRC following polypectomy are as follows: adenoma ≥10 mm in size; 3 to 5 (or more) adenomas; tubulovillous or villous adenoma; adenoma containing high-grade dysplasia; traditional serrated adenoma; sessile serrated lesion containing any grade of dysplasia; serrated polyp of at least 10 mm in size; and 3 to 5 (or more) sessile serrated lesions. More studies are needed to fully comprehend the patients who are most likely to benefit from surveillance colonoscopy and the ideal surveillance interval to prevent metachronous CRC.

11.
J Neurol ; 270(4): 2174-2183, 2023 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-36633670

RESUMO

The incidence and characteristics of acute vestibular symptoms, responsible structures, and lateralization of the causative lesions in supratentorial stroke remain unknown. This study aimed to determine the incidence, clinical features, and anatomical correlation of acute vestibular symptoms in supratentorial stroke. We performed a prospective, multicenter, observational study that had recruited patients with supratentorial stroke from the neurology clinics of referral-based four university hospitals in Korea. All patients received a constructed neuro-otological evaluations, and neuroimaging. We analyzed the incidence of acute vestibular symptoms, abnormal ocular motor and vestibular function tests, and stroke lesions. Of 1301 patients with supratentorial stroke, 48 (3.7%) presented with acute vestibular symptoms, and 13 of them (1%) had the vestibular symptoms in isolation. In patients with acute vestibular symptoms, abnormal findings included spontaneous nystagmus (5%), impaired horizontal smooth pursuit (41%), and abnormal tilt of the subjective visual vertical (SVV) (20%). Video head impulse and caloric tests were normal in all the patients. There was no clear correlation between acute vestibular symptoms and involvement of specific vestibular cortex. In patients with unilateral stroke, there was also no lateralization of the causative lesions of acute vestibular symptoms (left vs. right; 52 vs. 48%), even in patients with vertigo (left vs. right; 58 vs. 42%). This study demonstrates that the incidence of acute vestibular symptoms in supratentorial stroke is 3.7%, with being isolated in 1%. The widespread lesions responsible for acute vestibular symptoms implicate diffuse multisensory cortical-subcortical networks in the cerebral hemispheres without a lateralization.


Assuntos
Nistagmo Patológico , Acidente Vascular Cerebral , Humanos , Incidência , Estudos Prospectivos , Vertigem/diagnóstico , Acidente Vascular Cerebral/complicações , Acidente Vascular Cerebral/diagnóstico por imagem , Acidente Vascular Cerebral/epidemiologia , Nistagmo Patológico/diagnóstico por imagem , Nistagmo Patológico/epidemiologia , Nistagmo Patológico/etiologia
12.
Korean J Gastroenterol ; 81(1): 1-16, 2023 01 25.
Artigo em Coreano | MEDLINE | ID: mdl-36695062

RESUMO

The introduction of device-assisted enteroscopy (DAE) in the beginning of the 21st century has revolutionized the diagnosis and treatment of diseases of the small intestine. In contrast to capsule endoscopy, the other main diagnostic modality of small bowel diseases, DAE has the unique advantages of allowing the observation of the region of interest in detail and enabling tissue acquisition and therapeutic intervention. As DAE becomes an essential procedure in daily clinical practice, there is an increasing need for correct guidelines on when and how it is to be performed and what technical factors should be taken into consideration. In response to these needs, the Korean Association for the Study of Intestinal Diseases has developed an expert consensus statement on the performance of DAE by reviewing current evidence. This expert consensus statement particularly focuses on the indications, choice of insertion route, therapeutic intervention, complications, and relevant technical points.


Assuntos
Endoscopia por Cápsula , Enteropatias , Humanos , Enteropatias/diagnóstico , Endoscopia por Cápsula/métodos , Intestino Delgado , República da Coreia
13.
Intest Res ; 21(2): 252-265, 2023 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-36453007

RESUMO

BACKGROUND/AIMS: We conducted a nationwide population-based study to investigate incidence rates of colorectal and biliary cancers according to accompanying primary sclerosing cholangitis in Korean ulcerative colitis patients. METHODS: We used the Health Insurance Review and Assessment claim database from January 2007 to April 2020. Standardized incidence ratios of colorectal and biliary cancers in ulcerative colitis patients were calculated. RESULTS: Among 35,189 newly diagnosed ulcerative colitis patients, 1,224 patients were diagnosed with primary sclerosing cholangitis. During the study period, 122 and 52 patients were diagnosed with colorectal and biliary cancers, respectively. Incidences of colorectal cancer were not higher in ulcerative colitis patients than those in the general population (standardized incidence ratios, 0.83; 95% confidence interval, 0.69-0.99), regardless of accompanied primary sclerosing cholangitis (standardized incidence ratio, 0.73; 95% confidence interval, 0.24-1.71). While incidences of biliary cancer were not higher in ulcerative colitis patients than those in the general population (standardized incidence ratio, 1.14; 95% confidence interval, 0.80-1.58), these were much higher with accompanied primary sclerosing cholangitis (standardized incidence ratio, 10.07; 95% confidence interval, 5.75-16.36). Cumulative incidences of colorectal and biliary cancers increased in patients who were diagnosed with ulcerative colitis at an older age. CONCLUSIONS: In Korean ulcerative colitis patients, colorectal cancer incidences were not higher than those in the general population regardless of accompanied primary sclerosing cholangitis. However, biliary cancer incidences were much higher in ulcerative colitis patients with primary sclerosing cholangitis than in those without, or in the general population.

14.
Front Neurol ; 13: 999419, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36438947

RESUMO

Objectives: This study aimed to describe the clinical and radiological characteristics of a cerebello-brainstem dominant form of X-linked adrenoleukodystrophy (X-ALD). Methods: Three affected members from a family with cerebellar ataxia received full neurological, laboratory and radiological examinations. Genetic diagnoses were confirmed using whole-exome sequencing and protein structural modeling. Results: All affected members presented with slurred speech, ataxia, and spasticity, but showed obvious differences in phenotypic severity and radiological findings. The levels of very long-chain fatty acids (VLCFA) were elevated in each member, while only one had adrenal dysfunction. Genetic analysis identified a hemizygous missense mutation (c.887A>G, p.Tyr296Cys) of the ATP-binding cassette subfamily D member 1 gene (ABCD1) in all affected members, which is likely to destabilize the overall structure of the ABCD1 protein. Conclusions: We report a cerebello-dominant form of X-ALD caused by a missense variant in ABCD1. This report highlights intrafamilial phenotypic variability in X-ALD.

15.
Clin Endosc ; 55(6): 703-725, 2022 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-36225130

RESUMO

Colonoscopic polypectomy is effective in decreasing the incidence and mortality of colorectal cancer (CRC). Premalignant polyps discovered during colonoscopy are associated with the risk of metachronous advanced neoplasia. Postpolypectomy surveillance is the most important method for the management of advanced metachronous neoplasia. A more efficient and evidence-based guideline for postpolypectomy surveillance is required because of limited medical resources and concerns regarding colonoscopy complications. In these consensus guidelines, an analytic approach was used to address all reliable evidence to interpret the predictors of CRC or advanced neoplasia during surveillance colonoscopy. The key recommendations state that the high-risk findings for metachronous CRC following polypectomy are as follows: (1) adenoma ≥10 mm in size; (2) 3 to 5 (or more) adenomas; (3) tubulovillous or villous adenoma; (4) adenoma containing high-grade dysplasia; (5) traditional serrated adenoma; (6) sessile serrated lesion (SSL) containing any grade of dysplasia; (7) serrated polyp of at least 10 mm in size; and (8) 3 to 5 (or more) SSLs. More studies are needed to fully comprehend the patients most likely to benefit from surveillance colonoscopy and the ideal surveillance interval to prevent metachronous CRC.

16.
Korean J Gastroenterol ; 80(3): 115-134, 2022 09 25.
Artigo em Coreano | MEDLINE | ID: mdl-36156035

RESUMO

Colonoscopic polypectomy is effective in decreasing the incidence and mortality of colorectal cancer (CRC). Premalignant polyps discovered during colonoscopy are associated with the risk of metachronous advanced neoplasia. Postpolypectomy surveillance is the most important method for managing advanced metachronous neoplasia. A more efficient and evidence-based guideline for postpolypectomy surveillance is required because of the limited medical resources and concerns regarding colonoscopy complications. In these consensus guidelines, an analytic approach was used to address all reliable evidence to interpret the predictors of CRC or advanced neoplasia during surveillance colonoscopy. The key recommendations state that the high-risk findings for metachronous CRC following polypectomy are as follows: 1) adenoma ≥10 mm in size; 2) 3-5 (or more) adenomas; 3) tubulovillous or villous adenoma; 4) adenoma containing high-grade dysplasia; 5) traditional serrated adenoma; 6) sessile serrated lesion (SSL) containing any grade of dysplasia; 7) serrated polyp of at least 10 mm in size; and 8) 3-5 (or more) SSLs. More studies are needed to fully comprehend the patients who are most likely to benefit from surveillance colonoscopy and the ideal surveillance interval to prevent metachronous CRC.


Assuntos
Adenoma , Pólipos do Colo , Neoplasias Colorretais , Adenoma/diagnóstico , Adenoma/patologia , Adenoma/cirurgia , Pólipos do Colo/diagnóstico , Pólipos do Colo/epidemiologia , Pólipos do Colo/cirurgia , Colonoscopia/métodos , Neoplasias Colorretais/diagnóstico , Neoplasias Colorretais/epidemiologia , Neoplasias Colorretais/cirurgia , Humanos , República da Coreia , Fatores de Risco
17.
Front Neurol ; 13: 938740, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35923832

RESUMO

Objectives: This study aimed to determine the underlying pathogenesis of Meniere's disease (MD) using transcriptome analysis. Methods: Total RNA was extracted from the peripheral blood mononuclear cells of 39 patients with MD and 39 controls. Through microarray analysis for nine patients and controls, the differentially expressed genes (DEGs) of those two groups were screened based on cut-off criteria (|fold changes| > 2.0 and adjusted p-value < 0.05). The functional enrichment analysis of DEGs was performed using Gene Ontology (GO). Results: There were 996 DEGs identified in the MD group: 415 were upregulated and 581 were downregulated. A functional enrichment analysis indicated that the downregulated DEGs were significantly enriched in terms related to immune system processes. Among them, 17 genes were enriched in terms for the major histocompatibility complex (MHC) protein complex, and the relative messenger RNA (mRNA) levels of three markedly downregulated DEGs [fold changes < -5: human leukocyte antigen (HLA)-DMA, HLA-DRB1, and HLA-DPB1] were significantly decreased in another 30 patients with MD compared with normal controls by quantitative reverse transcription-polymerase chain reaction (qRT-PCR). However, there were no correlations between the expression levels of these three genes and clinical data, such as age, onset age, time course, or hearing threshold. Conclusions: Our transcriptome analysis showed that the downregulated DEGs in MD were mainly associated with the immune system pathways including the MHC protein complex in MD. Remarkably, a breakdown in immunological tolerance mediated by MHC class II may contribute to the MD development, which has implications for targeted treatment.

18.
J Neurol ; 269(12): 6642-6647, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-35904591

RESUMO

Vertical pendular nystagmus (PN) rarely occurs with acute pontine lesions. To hypothesize a pathophysiology for acute vertical PN, we analyzed the clinical characteristics and quantitative eye-movement recordings of one new case with acute vertical PN and an additional 11 patients from the literature. Most patients had extensive pontine lesions causing either the locked-in syndrome or unresponsiveness, but two conscious patients had focal lesions restricted to the paramedian caudal pontine tegmentum. All patients presented a complete or partial horizontal gaze palsy, and about half showed ocular bobbing before or during the appearance of vertical PN. The vertical oscillations were conjugate at a frequency of 1-5 Hz, and the amplitudes were variable, ranging from 0.2° to 40°. The peak velocities were asymmetric in some patients, faster with downward movements. About half of the patients developed palatal tremor several weeks or months after presenting with acute vertical PN. Based on the location of the lesions and results of eye-movement recordings, we suggest two possible mechanisms for acute vertical PN; oscillations originating in the inferior olives due to disruption of the central tegmental tract or low-velocity saccadic oscillations caused by omnipause neuron damage.


Assuntos
Nistagmo Patológico , Transtornos da Motilidade Ocular , Humanos , Movimentos Oculares , Ponte/diagnóstico por imagem , Ponte/patologia , Transtornos da Motilidade Ocular/complicações , Movimento
19.
Arch Pharm Res ; 45(4): 280-293, 2022 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-35441964

RESUMO

Zika virus (ZIKV), an arbovirus of the Flaviviridae family, has emerged as a significant public health concern owing to its association with congenital abnormalities and severe neurological sequelae. Thus, there is an urgent need to develop effective therapeutic approaches to efficiently treat ZIKV infections. This study used phenotypic screening to identify a series of 1,2,4-oxadiazole derivatives that possess antiviral activity against ZIKV infection. Subsequently, 28 new derivatives were designed, synthesized, and evaluated for this purpose. Among these compounds, 4-(5-phenyl-1,2,4-oxadiazol-3-yl)-N-(pyridin-3-ylmethyl)aniline (5d) had potent antiviral activity against ZIKV infections. Furthermore, a structure-activity relationship analysis indicated that a benzyl substitution on the aniline nitrogen of this compound improved potency while augmenting its drug-like properties. In addition, 5d exhibited antiviral activity against various viruses of Flaviviridae family of worldwide public health importance, such as dengue, Japanese encephalitis and classical swine fever viruses, indicating its potential as a lead compound for generating 1,2,4-oxadiazole derivatives with broad-spectrum anti-flaviviral properties.


Assuntos
Vírus da Febre Suína Clássica , Dengue , Encefalite Japonesa , Infecção por Zika virus , Zika virus , Compostos de Anilina/farmacologia , Compostos de Anilina/uso terapêutico , Antivirais/farmacologia , Antivirais/uso terapêutico , Dengue/tratamento farmacológico , Encefalite Japonesa/tratamento farmacológico , Humanos , Oxidiazóis/farmacologia , Oxidiazóis/uso terapêutico , Replicação Viral , Infecção por Zika virus/tratamento farmacológico
20.
J Neurol ; 269(9): 4742-4752, 2022 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-35394171

RESUMO

This study aimed to determine the incidence, pattern, and etiology of dissociated vestibulo-ocular reflex (VOR) function according to the stimulus frequency in dizzy patients. We retrospectively evaluated the results of bithermal caloric tests and video-head impulse tests (vHITs) in 1022 patients with dizziness or vertigo between July 2016 and April 2021. Patients were classified into concordant group (normal or abnormal results on both tests) and discordant group (dissociated results between two tests). Of 1022 patients, discordant group had 159 (16%), comprising abnormal horizontal vHITs with normal caloric responses (n = 36, 23%) and abnormal caloric tests with normal horizontal vHITs (n = 123, 77%). The former group showed similar frequency of peripheral (44%) and central (42%) causes, and more common involvement of bilateral horizontal semicircular canals in central than peripheral causes (86.7 vs 37.5%, p = 0.005). The most common peripheral causes were Meniere's disease and chronic vestibular neuritis, while central causes were variable, but mainly affecting the cerebellum. In the latter group, peripheral causes were common (67%), with the main etiologies being Meniere's disease and vestibular neuritis, whereas central causes were found in only 5%. The degree of canal paresis did not differ significantly between patients with central and peripheral causes. Discordant VOR function according to the stimulus frequency was not uncommon in dizzy patients. Specific patterns of VOR dissociation according to the disease etiologies may offer insight into underlying pathophysiology.


Assuntos
Doença de Meniere , Neuronite Vestibular , Testes Calóricos/métodos , Tontura/epidemiologia , Tontura/etiologia , Teste do Impulso da Cabeça/métodos , Humanos , Doença de Meniere/diagnóstico , Doença de Meniere/epidemiologia , Reflexo Vestíbulo-Ocular/fisiologia , Estudos Retrospectivos , Canais Semicirculares , Vertigem
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