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1.
Optom Vis Sci ; 101(4): 211-223, 2024 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-38684064

RESUMO

SIGNIFICANCE: This multicenter study assessed clinical and psychological aspects of infantile nystagmus syndrome (INS) focusing on its management and nonsurgical treatment. PURPOSE: This study aimed to assess clinical features, management, relationship life, and psychological impact in a group of patients with nystagmus onset in pediatric age. METHODS: This observational study included patients diagnosed with INS referred to two Italian centers from January 1, 2017, to December 31, 2020. Ophthalmologic and orthoptic features and impact of visual function on quality of life, according to nystagmus-specific nystagmus quality of life questionnaire, were analyzed within the overall sample and in any of INS subgroups. RESULTS: Forty-three patients were included; 65.1% of them had idiopathic INS (IINS), and 34.9% had INS associated with ocular diseases (INSOD). The median age was 15.4 years (interquartile range [IQR], 10.4 to 17.3 years), significantly different between groups (median, 15.8 years among those with IINS vs. 12.3 years among those with INSOD; p<0.001). In the INSOD subgroup, strabismus was significantly more prevalent (93.3 vs. 57.1%; p=0.017). Binocular distance best-corrected visual acuity in primary position was significantly higher in the IINS subsample (p<0.001). Such behavior was further confirmed at anomalous head position evaluation (p<0.001). At near best-corrected visual acuity assessment, differences between groups were more remarkable in primary position (p<0.001) than in anomalous head position. Contrast sensitivity showed significantly higher values in the IINS subgroup (p<0.001). The nystagmus quality of life questionnaire disclosed a significantly lower score in IINS as compared with INSOD (median total score, 90.5 [IQR, 84 to 97] vs. 94 [IQR, 83.0 to 96.5]; p<0.001). CONCLUSIONS: The IINS group showed significantly better ophthalmologic and orthoptic outcomes than the INSOD group. The psychological and quality-of-life impact was instead significantly greater in the IINS group. To the best of our knowledge, this is the first multicenter study investigating the clinical features of IIN and comparing the two main subgroups, IINS and INSOD.


Assuntos
Nistagmo Congênito , Qualidade de Vida , Acuidade Visual , Humanos , Masculino , Feminino , Adolescente , Estudos Retrospectivos , Criança , Acuidade Visual/fisiologia , Nistagmo Congênito/fisiopatologia , Inquéritos e Questionários , Nistagmo Patológico/fisiopatologia
2.
J Vestib Res ; 34(2-3): 103-112, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38457163

RESUMO

BACKGROUND: The upright head roll test (UHRT) is a recently introduced diagnostic maneuver for lateral semicircular canal benign paroxysmal positional vertigo (LSC-BPPV). OBJECTIVE: This study aimed to evaluate the reliability and validity of the UHRT. METHODS: Two separate studies were conducted. Study 1 analyzed 827 results of videonystagmography (VNG) to assess UHRT reliability, and Study 2 analyzed 130 LSC-BPPV cases to evaluate UHRT validity. RESULTS: The inter-test reliability between UHRT and the supine head roll test (SHRT) showed substantial agreement (Cohen's kappa = 0.753) in direction-changing positional nystagmus (DCPN) and almost perfect agreement (Cohen's kappa = 0.836) in distinguishing the direction of DCPN. The validity assessment of UHRT showed high accuracy in diagnosing LSC-BPPV (80.0%) and in differentiating the variant types (74.6%). UHRT was highly accurate in diagnosing the canalolithiasis type in LSC-BPPV patients (Cohen's kappa = 0.835); however, it showed only moderate accuracy in diagnosing the cupulolithiasis type (Cohen's kappa = 0.415). The intensity of nystagmus in UHRT was relatively weaker than that in SHRT (P < 0.05). CONCLUSION: UHRT is a reliable test for diagnosing LSC-BPPV and distinguishing subtypes. However, UHRT has a limitation in discriminating the affected side owing to a weaker intensity of nystagmus than SHRT.


Assuntos
Vertigem Posicional Paroxística Benigna , Canais Semicirculares , Testes de Função Vestibular , Humanos , Vertigem Posicional Paroxística Benigna/diagnóstico , Vertigem Posicional Paroxística Benigna/fisiopatologia , Masculino , Feminino , Reprodutibilidade dos Testes , Pessoa de Meia-Idade , Canais Semicirculares/fisiopatologia , Idoso , Adulto , Testes de Função Vestibular/métodos , Testes de Função Vestibular/normas , Movimentos da Cabeça/fisiologia , Idoso de 80 Anos ou mais , Nistagmo Patológico/diagnóstico , Nistagmo Patológico/fisiopatologia , Adulto Jovem , Nistagmo Fisiológico/fisiologia
3.
J Neurol ; 271(5): 2844-2849, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38353747

RESUMO

BACKGROUND: Disconjugate eye movements are essential for depth perception in frontal-eyed species, but their underlying neural substrates are largely unknown. Lesions in the midbrain can cause disconjugate eye movements. While vertically disconjugate eye movements have been linked to defective visuo-vestibular integration, the pathophysiology and neuroanatomy of horizontally disconjugate eye movements remains elusive. METHODS: A patient with a solitary focal midbrain lesion was examined using detailed clinical ocular motor assessments, binocular videooculography and diffusion-weighted MRI, which was co-registered to a high-resolution cytoarchitectonic MR-atlas. RESULTS: The patient exhibited both vertically and horizontally disconjugate eye alignment and nystagmus. Binocular videooculography showed a strong correlation of vertical and horizontal oscillations during fixation but not in darkness. Oscillation intensities and waveforms were modulated by fixation, illumination, and gaze position, suggesting shared visual- and vestibular-related mechanisms. The lesion was mapped to a functionally ill-defined area of the dorsal midbrain, adjacent to the posterior commissure and sparing nuclei with known roles in vertical gaze control. CONCLUSION: A circumscribed region in the dorsal midbrain appears to be a key node for disconjugate eye movements in both vertical and horizontal planes. Lesioning this area produces a unique ocular motor syndrome mirroring hallmarks of developmental strabismus and nystagmus. Further circuit-level studies could offer pivotal insights into shared pathomechanisms of acquired and developmental disorders affecting eye alignment.


Assuntos
Mesencéfalo , Humanos , Mesencéfalo/diagnóstico por imagem , Mesencéfalo/fisiopatologia , Mesencéfalo/patologia , Masculino , Transtornos da Motilidade Ocular/fisiopatologia , Transtornos da Motilidade Ocular/etiologia , Feminino , Movimentos Oculares/fisiologia , Pessoa de Meia-Idade , Nistagmo Patológico/fisiopatologia , Nistagmo Patológico/etiologia , Nistagmo Patológico/diagnóstico por imagem , Adulto
4.
Rev. bras. oftalmol ; 82: e0014, 2023. graf
Artigo em Inglês | LILACS | ID: biblio-1431666

RESUMO

ABSTRACT A 12-year-old boy with Donnai-Barrow syndrome diagnosed intra-uterus presented esotropia, high myopia, nystagmus, and optic disk staphyloma in an ophthalmologic examination. The patient had associated Fanconi syndrome and sensorineural hearing loss as well as facial manifestations as hypertelorism, downward slanting of palpebral fissures and low ear implantation. Magnetic resonance imaging revealed agenesis of the corpus callosum. To our knowledge, this is the first reported case associated with esotropia, nystagmus, and optic disk staphyloma.


RESUMO Paciente do sexo masculino, 12 anos, com diagnóstico intrauterino de síndrome de Donnai-Barrow, apresentava ao exame oftalmológico esotropia, alta miopia, nistagmo e estafiloma de disco óptico. Associado ao quadro, apresentava síndrome de Falconi e perda auditiva neurossensorial, além de alterações faciais, como hipertelorismo, inclinação inferior das fissuras palpebrais e implantação baixa das orelhas. Ressonância magnética revelou agenesia de corpo caloso. Ao nosso conhecimento, este é o primeiro caso relatado associando esotropia, nistagmo e estafiloma de disco óptico.


Assuntos
Humanos , Masculino , Criança , Anormalidades Múltiplas , Doenças do Nervo Óptico/fisiopatologia , Esotropia/fisiopatologia , Nistagmo Patológico/fisiopatologia , Miopia/fisiopatologia , Erros Inatos do Transporte Tubular Renal , Síndrome , Acidose Tubular Renal , Descolamento Retiniano , Criptorquidismo , Síndrome de Fanconi/fisiopatologia , Agenesia do Corpo Caloso/fisiopatologia , Hérnias Diafragmáticas Congênitas , Perda Auditiva Neurossensorial , Hipertelorismo/fisiopatologia
5.
Invest Ophthalmol Vis Sci ; 63(2): 33, 2022 02 01.
Artigo em Inglês | MEDLINE | ID: mdl-35212720

RESUMO

Purpose: Patients with amblyopia are known to have fixation instability, which arises from alteration of physiologic fixation eye movements (FEMs) and nystagmus. We assessed the effects of monocular, binocular, and dichoptic viewing on FEMs and eye alignment in patients with and without fusion maldevelopment nystagmus (FMN). Methods: Thirty-four patients with amblyopia and seven healthy controls were recruited for this study. Eye movements were recorded using infrared video-oculography during (1) fellow eye viewing (FEV), (2) amblyopic eye viewing (AEV), (3) both eye viewing (BEV), and (4) dichoptic viewing (DcV) at varying fellow eye (FE) contrasts. The patients were classified per the clinical type of amblyopia and FEM waveforms into those without nystagmus, those with nystagmus with and without FMN. Fixational saccades and intersaccadic drifts, quick and slow phases of nystagmus, and bivariate contour ellipse area were analyzed in the FE and amblyopic eye (AE). Results: We found that FEMs are differentially affected with increased amplitude of quick phases of FMN observed during AEV than BEV and during DcV at lower FE contrasts. Increased fixation instability was seen in anisometropic patients at lower FE contrasts. Incomitance of eye misalignment was seen with the greatest increase during FEV. Strabismic/mixed amblyopia patients without FMN were more likely to demonstrate a fixation switch where the AE attends to the target during DcV than patients with FMN. Conclusions: Our findings suggest that FEM abnormalities modulate with different viewing conditions as used in various amblyopia therapies. Increased FEM abnormalities could affect the visual function deficits and may have treatment implications.


Assuntos
Ambliopia/fisiopatologia , Movimentos Oculares/fisiologia , Fixação Ocular/fisiologia , Nistagmo Patológico/fisiopatologia , Visão Binocular/fisiologia , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Gravação em Vídeo , Acuidade Visual
6.
Invest Ophthalmol Vis Sci ; 63(2): 28, 2022 02 01.
Artigo em Inglês | MEDLINE | ID: mdl-35195683

RESUMO

Purpose: Children with Down's syndrome (DS) are known to have poorer visual acuity than neurotypical children. One report has shown that children with DS and nystagmus also have poor acuity when compared to typical children with nystagmus. What has not been established is the extent of any acuity deficit due to nystagmus and whether nystagmus affects refractive error within a population with DS. Methods: Clinical records from the Cardiff University Down's Syndrome Vision Research Unit were examined retrospectively. Binocular visual acuity and refraction data were available for 50 children who had DS and nystagmus and 176 children who had DS but no nystagmus. Data were compared between the two groups and with published data for neurotypical children with nystagmus. Results: The study confirms the deficit in acuity in DS, compared to neurotypical children, of approximately 0.2 logMAR and shows a deficit attributable to nystagmus of a further 0.2 logMAR beyond the first year of life. Children with both DS and nystagmus clearly have a significant additional impairment. Children with DS have a wide range of refractive errors, but nystagmus increases the likelihood of myopia. Prevalence and axis direction of astigmatism, on the other hand, appear unaffected by nystagmus. Conclusions: Nystagmus confers an additional visual impairment on children with DS and must be recognized as such by families and educators. Children with both DS and nystagmus clearly need targeted support.


Assuntos
Síndrome de Down/fisiopatologia , Nistagmo Patológico/fisiopatologia , Erros de Refração/fisiopatologia , Transtornos da Visão/fisiopatologia , Acuidade Visual/fisiologia , Astigmatismo/fisiopatologia , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Nistagmo Patológico/diagnóstico , Refração Ocular/fisiologia , Estudos Retrospectivos , Testes Visuais , Visão Binocular/fisiologia
7.
Invest Ophthalmol Vis Sci ; 63(1): 31, 2022 01 03.
Artigo em Inglês | MEDLINE | ID: mdl-35072688

RESUMO

Purpose: Research on infantile nystagmus syndrome (INS) and motion perception is limited. We investigated how individuals with INS perform coherent motion tasks. Particularly, we assessed how the null position affects their performance. Methods: Subjects with INS and controls identified the direction of coherent motion stimuli (22 subjects with INS and 13 controls) in a two-alternative forced-choice design. For subjects with INS, testing was done at the null position and 15 degrees away from it. If there was no null, testing was done at primary gaze position and 15 degrees away from primary. For controls, testing was done at primary gaze position and 20 degrees away from primary. Horizontal and vertical motion coherence thresholds were determined. Results: Subjects with INS showed significantly higher horizontal and vertical motion coherence thresholds compared with controls at both gaze positions (P < 0.001). Within the INS group, for 12 subjects with INS who had an identified null position, no differences in coherence thresholds were found between their null and 15 degrees away from it (P > 0.05). Conclusions: Coherent motion perception was impaired in subjects with INS. The null position did not significantly influence motion coherence thresholds for either horizontal or vertical motion.


Assuntos
Movimentos Oculares/fisiologia , Percepção de Movimento/fisiologia , Nistagmo Patológico/fisiopatologia , Acuidade Visual , Adolescente , Adulto , Feminino , Humanos , Masculino , Adulto Jovem
8.
Acta Otolaryngol ; 142(1): 43-47, 2022 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-34955085

RESUMO

BACKGROUND: Pseudo-spontaneous nystagmus (PSN) can be detected in patients with lateral canal benign paroxysmal positional vertigo (LC-BPPV). Its frequency, and correlation with the therapeutic outcome have been less described and conflicting results have been reported. OBJECTIVE: This study aims to investigate its clinical and prognostic significance. MATERIAL AND METHOD: One hundred and eighty-four patients with LC BPPV (98 apogeotropic, 86 geotropic type) were enrolled for the study. Clinical parameters were reviewed in patients with or without PSN. The Chi-square and one way ANOVA tests were used to compare the difference between study groups. Statistical significance was set at p < .05. RESULTS: Twenty-two patients with apogeotropic (22.4%; 98/22) and 17 patients with geotropic nystagmus (19.7%; 86/17) had PSN. The incidence, age, male-female ratio, mean slow phase velocity (SPV), duration of BPPV and the rate of recurrence were not significant in patients with LC-BPPV whether they have PSN or not. CONCLUSION: Prognostic role of PSN in patients with LC BPPV seems to be questionable. SIGNIFICANCE: Appearance and disappearance of PSN with regard to head position helps to differentiate BPPV from other acute vestibular disorders. Additionally, direction of nystagmus assists to determine the site of the affected canal. However, prognostic significance is obscure.


Assuntos
Vertigem Posicional Paroxística Benigna/fisiopatologia , Nistagmo Patológico/fisiopatologia , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade
9.
Clin Neurophysiol ; 134: 43-49, 2022 02.
Artigo em Inglês | MEDLINE | ID: mdl-34971940

RESUMO

OBJECTIVE: The purpose of this study was to investigate the effects of excessive cerebrospinal fluid (CSF) retention on the peripheral vestibular function and the inner ear fluid in patients with idiopathic normal pressure hydrocephalus (iNPH). METHODS: In 25 patients with iNPH (14 females, age 65-88 years), cervical vestibular evoked myogenic potential (cVEMP) was measured before the spinal tap test. The asymmetry ratios (ARs) and tuning properties in 500 Hz and 1,000 Hz short-tone burst stimuli of cVEMP were evaluated. Furthermore, cVEMP was measured in an age-matched control group of 12 non-iNPH patients. RESULTS: Seven (28%) iNPH patients exhibited a cVEMP asymmetry (AR > 33%). cVEMP tuning was significantly shifted to a higher frequency in the iNPH group than in the age-matched control group. CONCLUSIONS: One-fourth of patients with iNPH had obvious saccular dysfunction. A high rate of a shift in cVEMP tuning in the iNPH group indicated that excessive CSF accumulation propagated to the endolymph and perilymph. SIGNIFICANCE: Saccular dysfunction might be one of the possible causes of imbalance in iNPH, and the shift in cVEMP tuning may be a determining factor in the diagnosis and treatment strategy.


Assuntos
Hidropisia Endolinfática/fisiopatologia , Hidrocefalia de Pressão Normal/fisiopatologia , Nistagmo Patológico/fisiopatologia , Sáculo e Utrículo/fisiopatologia , Potenciais Evocados Miogênicos Vestibulares/fisiologia , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino
11.
J Neurol Neurosurg Psychiatry ; 92(11): 1181-1185, 2021 11.
Artigo em Inglês | MEDLINE | ID: mdl-34285066

RESUMO

OBJECTIVE: Cochleovestibulopathy is a distinguishable paraneoplastic phenotype. In this study, we evaluate clinical presentation, serological/cancer associations and outcomes of paraneoplastic cochleovestibulopathy. METHODS: Retrospective chart review of patients with hearing impairment and/or vestibulopathy who underwent serological evaluations for paraneoplastic antibodies between January 2007 and February 2021 was performed. RESULTS: Twenty-six patients were identified (men, n=23; median age, 45 years, range: 28-70). Biomarkers detected included: KLHL11-IgG| |(n=20,| |77% (coexisting LUZP4-IgG, n=8)),| ||ANNA1-IgG| | |(n=3,| |12%),| |amphiphysin-IgG|| |(n=2,| |8%)| |and| |LUZP4-IgG|| |(n=1,| |4%). Most common neoplastic association was |testicular|/|extra-testicular| |seminoma| | (n=13,| |50%).|| Hearing| impairment (bilateral, 62%) was |present| |in| |all| |patients.| |Fifteen patients (58%) had cochleovestibular dysfunction as their initial presentation before rhombencephalitis/encephalomyelitis manifestations (hearing loss, four; acute vertigo, eight; both, three). |Brain| |MRI| |demonstrated| |internal| |auditory| |canal| |enhancement| |in| |four |patients.| Audiometry commonly revealed severe-profound bilateral sensorineural hearing loss. Most patients |had| a refractory course |despite| |immunotherapy| |and/or| |cancer| |treatment|. CONCLUSION: Cochleovestibulopathy commonly presents with rapidly progressive bilateral hearing loss and/or acute vertigo. However, in some patients, these symptoms present along with or following brainstem/cerebellar manifestations. KLHL11-IgG and seminoma are the most common serological and cancer associations, respectively. Recognition of this phenotype may aid in earlier diagnosis of paraneoplastic autoimmunity and associated cancer.


Assuntos
Perda Auditiva Neurossensorial/patologia , Síndromes Paraneoplásicas do Sistema Nervoso/patologia , Doenças do Nervo Vestibulococlear/patologia , Adulto , Idoso , Feminino , Perda Auditiva Neurossensorial/diagnóstico por imagem , Perda Auditiva Neurossensorial/fisiopatologia , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Exame Neurológico , Nistagmo Patológico/diagnóstico por imagem , Nistagmo Patológico/patologia , Nistagmo Patológico/fisiopatologia , Síndromes Paraneoplásicas do Sistema Nervoso/diagnóstico por imagem , Síndromes Paraneoplásicas do Sistema Nervoso/fisiopatologia , Estudos Retrospectivos , Doenças do Nervo Vestibulococlear/diagnóstico por imagem , Doenças do Nervo Vestibulococlear/fisiopatologia
12.
Ophthalmic Genet ; 42(5): 539-552, 2021 10.
Artigo em Inglês | MEDLINE | ID: mdl-34251969

RESUMO

Purpose: To correlate clinical features, molecular genetic findings, and visual acuity in a cohort of patients clinically diagnosed with oculocutaneous albinism.Design: Retrospective chart reviewMethods: 58 charts met the inclusion criteria. Clinical examination, ancillary testing, and molecular genetic diagnoses were extracted. A novel clinical albinism score (CAS) was developed.Results: A least one likely pathogenic mutation was found in 44/58 (75.9%) patients. Mutations in the OCA1 gene were the most common (52.3%), followed by OCA2 (34%), OCA4 (2.3%), OA1 (6.8%), and HPS (4.5%). Thirty-four percentage of patients had a complete genotype, 41% had one mutation found and 24% had negative genetic testing. CAS was statistically significantly higher in patients with complete genotype, versus patients with one or no mutations found (p < .01). Better visual acuity was associated with lower CAS and fewer disease-causing mutations (p < .01). Foveal defects and iris transillumination were associated with a higher number of mutations (p < .01). Patients with nystagmus or anomalous optic nerves had worse visual acuity than those who did not (p < .01, p < .05).Conclusions: Patients with a complete genotype were more likely to have higher CAS. Vision loss correlated with complete phenotype and higher CAS, the presence of nystagmus and anomalous optic nerves. Patients with features of albinism in whom an incomplete genotype was found had better vision than those with complete genotype, suggesting a mild occult mutation or modifier variant. Genetic diagnosis is vital for complete diagnosis, counseling, and family planning.


Assuntos
Albinismo Oculocutâneo/diagnóstico , Nistagmo Patológico/diagnóstico , Doenças do Nervo Óptico/diagnóstico , Acuidade Visual/fisiologia , Albinismo Oculocutâneo/genética , Albinismo Oculocutâneo/fisiopatologia , Antígenos de Neoplasias/genética , Criança , Proteínas do Olho/genética , Feminino , Testes Genéticos , Genótipo , Humanos , Masculino , Glicoproteínas de Membrana/genética , Proteínas de Membrana/genética , Proteínas de Membrana Transportadoras/genética , Mutação , Nistagmo Patológico/fisiopatologia , Doenças do Nervo Óptico/fisiopatologia , Estudos Retrospectivos , Tomografia de Coerência Óptica , Tirosina/genética
13.
Neurology ; 97(1): e42-e51, 2021 07 06.
Artigo em Inglês | MEDLINE | ID: mdl-33986142

RESUMO

OBJECTIVE: Failure of fixation suppression of spontaneous nystagmus is sometimes seen in patients with vestibular strokes involving the cerebellum or brainstem; however, the accuracy of this test for the discrimination between peripheral and central causes in patients with an acute vestibular syndrome (AVS) is unknown. METHODS: Patients with AVS were screened and recruited (convenience sample) as part of a prospective cross-sectional study in the emergency department between 2015 and 2020. All patients received neuroimaging, which served as a reference standard. We recorded fixation suppression with video-oculography (VOG) for forward, right, and left gaze. The ocular fixation index (OFI) and the spontaneous nystagmus slow velocity reduction was calculated. RESULTS: We screened 1,646 patients reporting dizziness in the emergency department and tested for spontaneous nystagmus in 148 patients with AVS. We analyzed 56 patients with a diagnosed acute unilateral vestibulopathy (vestibular neuritis) and 28 patients with a confirmed stroke. There was a complete nystagmus fixation suppression in 49.5% of patients with AVS, in 40% of patients with vestibular neuritis, and in 62.5% of patients with vestibular strokes. OFI scores had no predictive value for detecting strokes; however, a nystagmus reduction of less than 2 °/s showed a high accuracy of 76.9% (confidence interval 0.59-0.89) with a sensitivity of 62.2% and specificity of 84.8% in detecting strokes. CONCLUSIONS: The presence of fixation suppression does not rule out a central lesion. The magnitude of suppression was lower compared to patients with vestibular neuritis. The nystagmus suppression test predicts vestibular strokes accurately provided that eye movements are recorded with VOG. CLASSIFICATION OF EVIDENCE: This study provides Class II evidence that in patients with an AVS, decreased fixation suppression recorded with VOG occurred more often in stroke (76.9%) than in vestibular neuritis (37.8%).


Assuntos
Tontura/fisiopatologia , Nistagmo Patológico/fisiopatologia , Acidente Vascular Cerebral/diagnóstico , Acidente Vascular Cerebral/etiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Estudos Transversais , Tontura/diagnóstico por imagem , Feminino , Fixação Ocular , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Nistagmo Patológico/diagnóstico por imagem , Valor Preditivo dos Testes , Estudos Prospectivos , Reprodutibilidade dos Testes , Acidente Vascular Cerebral/diagnóstico por imagem , Doenças Vestibulares/fisiopatologia , Neuronite Vestibular/fisiopatologia , Adulto Jovem
15.
Sci Rep ; 11(1): 8208, 2021 04 15.
Artigo em Inglês | MEDLINE | ID: mdl-33859270

RESUMO

The vestibular receptor of cupula acts an important role in maintaining body balance. However, the cupula buried in the semicircular canals (SCCs) will be destroyed if it is detached from the relevant environment. The mechanical properties of human cupula still remain ambiguous. In this paper, we explored the cupula responses changing with temperature by experiments and numerical simulation of SCCs model. We obtained 3 volunteers' nystagmus induced by constant angular acceleration when the temperature of volunteers' SCCs was 36 °C and 37 °C respectively. The slow-phase velocity of 3 volunteers decreased by approximately 3°/s when the temperature of SCCs reduced by 1 °C, which corresponded to the reduction of cupula deformation by 0.3-0.8 µm in the numerical model. Furthermore, we investigated the effects of the variation of endolymphatic properties induced by temperature reduction on cupula deformation through numerical simulation. We found that the decrease of cupula deformation was not caused by the change of endolymphatic properties, but probably by the increase of cupula's elastic modulus. With the temperature reducing by 1 °C, the cupula's elastic modulus may increase by 6-20%, suggesting that the stiffness of cupula is enhanced. This exploration of temperature characteristic of human cupula promotes the research of alleviating vestibular diseases.


Assuntos
Fenômenos Biomecânicos/fisiologia , Canais Semicirculares/fisiologia , Temperatura , Aceleração , Adulto , Temperatura Corporal/fisiologia , Endolinfa/fisiologia , Humanos , Masculino , Nistagmo Patológico/fisiopatologia , Reflexo Vestíbulo-Ocular/fisiologia , Rotação , Doenças Vestibulares/etiologia , Doenças Vestibulares/patologia
16.
J Neuroophthalmol ; 41(2): 261-265, 2021 Jun 01.
Artigo em Inglês | MEDLINE | ID: mdl-33630775

RESUMO

ABSTRACT: A 45-year-old man with a history of testicular seminoma treated 8 years earlier presented with chronic progressive truncal and limb ataxia, progressive sensorineural hearing loss, and episodic vertigo. Eye movement and neuro-otology examinations showed localizing abnormalities to the bilateral cerebellar flocculus, vermis, and bilateral cerebellar hemispheres. Audiometric testing showed bilateral symmetric sensorineural hearing loss. There was a normal MRI of the brain. Cerebrospinal fluid (CSF) showed modest lymphocytic pleocytosis, and there was an elevated serum choriogonadotrophic hormone. An abdominal CT scan showed a solitary, large retroperitoneal lymph node, and histopathologic examination of the node biopsy showed granulomatous inflammation without microorganisms; eventually, immunohistochemical markers confirmed the diagnosis of metastatic seminoma. Although normal neuroimaging and inflammatory CSF reaction suggested a paraneoplastic etiology, the initial paraneoplastic antibody testing was negative. Subsequent investigation identified a positive kelch-11 protein antibody, thus confirming the paraneoplastic connection between the metastatic seminoma and the subacute neurologic-cochleovestibular syndrome.


Assuntos
Ataxia/etiologia , Autoanticorpos/sangue , Proteínas de Transporte/imunologia , Perda Auditiva Neurossensorial/etiologia , Nistagmo Patológico/etiologia , Seminoma/secundário , Neoplasias Testiculares/patologia , Ataxia/diagnóstico , Ataxia/fisiopatologia , Biomarcadores Tumorais/sangue , Biomarcadores Tumorais/imunologia , Proteínas de Transporte/sangue , Movimentos Oculares/fisiologia , Perda Auditiva Neurossensorial/diagnóstico , Humanos , Masculino , Pessoa de Meia-Idade , Metástase Neoplásica , Nistagmo Patológico/diagnóstico , Nistagmo Patológico/fisiopatologia , Síndromes Paraneoplásicas Oculares/sangue , Síndromes Paraneoplásicas Oculares/complicações , Síndromes Paraneoplásicas Oculares/diagnóstico , Seminoma/diagnóstico , Seminoma/imunologia , Neoplasias Testiculares/imunologia , Tomografia Computadorizada por Raios X
17.
Cochrane Database Syst Rev ; 2: CD013390, 2021 02 18.
Artigo em Inglês | MEDLINE | ID: mdl-33598911

RESUMO

BACKGROUND: Infantile nystagmus syndrome (INS) is a type of eye movement disorder that can negatively impact vision. Currently, INS cannot be cured, but its effects can potentially be treated pharmacologically, optically, or surgically. This review focuses on the surgical interventions for INS. Despite the range of surgical interventions available, and currently applied in practice for the management of INS, there is no clear consensus, and no accepted clinical guidelines regarding the relative efficacy and safety of the various treatment options. A better understanding of these surgical options, along with their associated side effects, will assist clinicians in evidence-based decision-making in relation to the management of INS. OBJECTIVES: To assess the efficacy and safety of surgical interventions for INS. SEARCH METHODS: We searched CENTRAL, MEDLINE Ovid, Embase Ovid, ISRCTN registry, ClinicalTrials.gov, and the World Health Organization (WHO) International Clinical Trials Registry Platform (ICTRP) to 3 July 2020, with no language restrictions. SELECTION CRITERIA: We included randomised controlled trials (RCTs) studying the efficacy and safety of surgical options for treating INS. DATA COLLECTION AND ANALYSIS: Our prespecified outcome measures were the change from baseline in: binocular best-corrected distance visual acuity; head posture; amplitude, frequency, intensity, and foveation period durations of the nystagmus waveform; visual recognition times; quality of life and self-reported outcome measures; incidence of adverse effects with a probable causal link to treatment; and permanent adverse effects after surgery. Two review authors independently screened titles and abstracts and full-text articles, extracted data from eligible RCTs, and judged the risk of bias using the Cochrane tool. We reached consensus on any disagreements by discussion. We summarised the overall certainty of the evidence using the GRADE approach. MAIN RESULTS: We only identified one eligible RCT (N = 10 participants), undertaken in India. This trial randomised participants to receive either a large retro-equatorial recession of the horizontal rectus muscle of 9 mm on the medial rectus and 12 mm on the lateral rectus, or a simple tenotomy and resuturing of the four horizontal rectus muscles. We did not identify any RCTs comparing a surgical intervention for INS relative to no treatment. In the single eligible RCT, both eyes of each participant received the same intervention. The participants' age and gender were not reported, nor was information on whether participants were idiopathic or had sensory disorders. The study only included participants with null in primary position and did not explicitly exclude those with congenital periodic alternating nystagmus. The study did not report funding source(s) or author declaration of interests. The evaluation period was six months. We judged this study at low risk for sequence generation and other sources of bias, but at high risk of bias for performance and detection bias. The risk of bias was unclear for selection bias, attrition bias, and reporting bias. There is very uncertain evidence about the effect of the interventions on visual acuity and change in amplitude, frequency, and intensity of the nystagmus waveform. We were unable to calculate relative effects due to lack of data. None of the participants in either intervention group reported adverse effects at six-month follow-up (very low-certainty evidence). There was no quantitative data reported for quality of life, although the study reported an improvement in quality of life after surgery in both intervention groups (very low-certainty evidence). Change in head posture, foveation period durations of the nystagmus waveform, visual recognition times, and permanent adverse effects after surgery were not reported in the included study. We judged the certainty of the evidence, for both the primary and secondary efficacy outcomes, to be very low. Due to a lack of comprehensive reporting of adverse events, there was also very low-certainty of the safety profile of the evaluated surgical interventions in this population. As such, we are very uncertain about the relative efficacy and safety of these interventions for the surgical management of INS. AUTHORS' CONCLUSIONS: This systematic review identified minimal high-quality evidence relating to the efficacy and safety of surgical interventions for INS. The limited availability of evidence must be considered by clinicians when treating INS, particularly given these procedures are irreversible and often performed on children. More high-quality RCTs are needed to better understand the efficacy and safety profile of surgical interventions for INS. This will assist clinicians, people with INS, and their parents or caregivers to make evidence-based treatment decisions.


Assuntos
Nistagmo Patológico/cirurgia , Músculos Oculomotores/cirurgia , Viés , Humanos , Índia , Lactente , Recém-Nascido , Nistagmo Patológico/fisiopatologia , Qualidade de Vida , Ensaios Clínicos Controlados Aleatórios como Assunto , Resultado do Tratamento , Visão Binocular , Acuidade Visual
18.
Auris Nasus Larynx ; 48(5): 864-869, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-33526322

RESUMO

OBJECTIVE: Irritability is an emotional stress symptom that causes or exacerbates dizziness. Antidepressants may be helpful for some conditions that are accompanied by irritability; however, they do not completely inhibit irritability. Yokukansan (YKS), a traditional Japanese herbal medicine, has been used for neurosis, insomnia, and children's irritability and night crying. The study investigated the efficacy of YKS in nystagmus in patients with chronic dizziness and irritability. METHODS: Twenty-two cases with chronic dizziness and irritability were reviewed retrospectively. The patients were divided into two groups: control patients (0-7 days of treatment) and YKS-treated patients (YKS cases; >7 days of treatment). Dizziness before and during (after, in the controls) YKS treatment was evaluated by scoring the nystagmus intensity on a 5-point scale. The average scores were calculated within a maximum of 6 months before and during or after treatment. The normalized scores were also calculated. The optimal treatment regimen was calculated via receiver operating characteristic (ROC) curve analysis. RESULTS: There were six control cases (1 male, 5 females; mean age: 59.5 years). There were 16 YKS cases (3 males, 13 females; mean age: 61.8 years). While the group mean nystagmus intensity scores significantly decreased from 1.18 to 0.73 in the YKS cases, it did not change in the control cases. The group mean of the normalized nystagmus intensity scores during treatment was 0.73 in the YKS cases. The results of the ROC curve analysis indicated the optimal cut-off period of the YKS treatment was 10 days. CONCLUSION: The oral administration of YKS for more than 10 days was optimal. The treatments with YKS could be a good option for the treatments of vertigo.


Assuntos
Tontura/tratamento farmacológico , Medicamentos de Ervas Chinesas/uso terapêutico , Humor Irritável , Vertigem/tratamento farmacológico , Tontura/fisiopatologia , Tontura/psicologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Nistagmo Patológico/fisiopatologia , Estudos Retrospectivos , Resultado do Tratamento , Vertigem/fisiopatologia , Vertigem/psicologia
20.
Acta Otolaryngol ; 141(5): 433-439, 2021 May.
Artigo em Inglês | MEDLINE | ID: mdl-33557660

RESUMO

BACKGROUND: How to interpret the various forms of nystagmus induced by the Dix-Hallpike maneuver has been the hotspot and difficulty of research. OBJECTIVES: Analysis of the types of nystagmus induced by Dix-Hallpike maneuver, and establish a diagnosis strategy based on dynamic nystagmus observation. MATERIALS AND METHODS: We observed the otolithic movements at different locations during the Dix-Hallpike maneuver through physical virtual simulation experiments and inferred the nystagmus performance, so as to establish the nystagmus interpretation rules for the repeated Dix-Hallpike maneuver. RESULTS: There are six types of nystagmus induced by the Dix-Hallpike maneuver. Nystagmus induced by the unilateral Dix-Hallpike maneuver does not accurately locate the otolith. The typical nystagmus that is consistent before and after the repetition of the Dix-Hallpike maneuver is the outward and upbeat nystagmus, considering the ipsilateral posterior semicircular canal BPPV. CONCLUSION: The atypical nystagmus often turns negative when the Dix-Hallpike maneuver is repeated. If the repeat test is positive and consistent with the results of the first diagnostic test, the otolith can be accurately located.


Assuntos
Simulação por Computador , Técnicas de Diagnóstico Otológico , Nistagmo Patológico/diagnóstico , Membrana dos Otólitos/fisiologia , Adolescente , Adulto , Idoso , Criança , Orelha Interna , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Modelos Biológicos , Movimento , Nistagmo Patológico/fisiopatologia , Membrana dos Otólitos/diagnóstico por imagem , Canais Semicirculares , Tomografia Computadorizada por Raios X
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