Analysis of spontaneous nystagmus and the frequency characteristics of affected semicircular canals in patients with vestibular neuritis / 中华内科杂志

Objective: To investigate the spontaneous nystagmus (SN) and the frequency characteristics of affected semicircular canals in patients with vestibular neuritis (VN). Methods: This is a cross-sectional study. A total of 61 patients with VN admitted to the Department of Neurology of Shanxi Bethune Hospital from June 2020 to October 2021, 39 were male and 22 were female, with a mean age of (46±13) years old and male to female ratio of 1.77∶1. According to SN characteristics, 61 patients were divided into non-nystagmus group(nSN), horizontal nystagmus group(hSN) and horizontal-torsional nystagmus group (htSN). Clinical data were collected, and SN, unilateral weakness (UW), directional preponderance (DP), and video head impulse test (vHIT) gain were used as observation indicators. Statistical analysis by SPSS23.0 software. Normal distributed quantitative data (age, semicircular canal gain, SN intensity) were expressed by x¯±s, non-normal distributed quantitative data (disease course, UW, DP) were expressed by M(Q1,Q3), qualitative data were expressed by rate and composition ratio, difference analysis by one-way ANOVA, rank sum test, Chi-square test or Fisher's exact probability method, considered by P value<0.05. Results: (1)The disease course of nSN, hSN and htSN was 7.0 (4.0, 12.5), 6.0 (3.5, 11.5), and 3.0 (2.0, 6.5) days respectively, and there were statistical differences (χ2=7.31,P=0.026).(2)The horizontal nystagmus intensity of htSN was (16.8±8.6)°/s, which was significantly higher than that of (9.8±4.7)°/s in hSN (t=3.71, P<0.001). There was no significant difference in the positive rate of UW between the three groups (P=0.690), and there was a significant difference in the positive rate of DP in the three groups (χ2=12.23, P=0.002). The horizontal nystagmor intensity in the htSN was positively correlated with the vertical nystagmus intensity (r=0.59, P=0.001).(3)The gain of the affected horizontal canal of the three groups was statistically different (F=8.28, P=0.001), and the gain of the horizontal canal of hSN and htSN was significantly lower than that of nSN (t=2.74, P=0.008; t=4.05, P<0.001); The gain of the affected anterior canal in the three groups was statistically different (F=5.32, P=0.008). The gain of the anterior canal in both nSN and hSN was significantly higher than that in htSN (t=3.09, P=0.003; t=2.15, P=0.036). The horizontal canal gain of htSN is positively correlated with the anterior canal gain (r=0.74, P<0.001).(4)The affected semicircular canals in the two groups with no-vertical-component nystagmus (nSN and hSN) and the htSN were counted. The composition ratio of the affected semicircular canals in the two groups was different (χ2=8.34, P=0.015). Conclusion: The occurrence of SN in patients with VN is related to many factors, such as the disease course, low and high frequencies, and the severity of the condition in the affected semicircular canal.

Further exploration of the classification and clinical value of head-shaking nystagmus / 临床耳鼻咽喉头颈外科杂志

Objective:To investigate the classification of head shaking nystagmus（HSN） and its clinical value in vestibular peripheral diseases. Methods:Clinical data of 198 patients with peripheral vestibular disorders presenting with HSN were retrospectively analyzed. Video Nystagmograph（VNG） was applied to detect spontaneous nystagmus（SN）, HSN, and Caloric Test（CT）. The intensity and direction of SN and HSN as well as the unilateral weakness（UW） and direction preponderance（DP） values in caloric test was analyzed in patients. Results:Among the 198 patients with vestibular peripheral disease, there were 105 males and 93 females, with an average age of（49.1±14.4） years （range: 14-87 years）. One hundred and thirty seven patients were diagnosed as Vestibular Neuritis（VN）, 12 as Meniere's Disease（MD）, 41 as sudden deafness（SD） and 8 as Hunt's syndrome accompanied by vertigo. Among them, there were 116 patients in the acute phase, including 68 cases（58.6%） with decreased HSN, 4 cases（3.4%） with increased HSN, 5 cases（4.3%） with biphasic HSN, 38 cases（32.8%） with unchanged HSN, and 1 case（0.9%） with perverted HSN. There were 82 cases in the non-acute phase, 51 cases（62.2%） with decreased HSN, 3 cases（3.6%） with increased HSN, 9 cases（11.0%） with biphasic HSN, and 19 cases（23.2%） with unchanged HSN. In biphasic HSN, the intensity of phase I nystagmus was usually greater than that of phase II, and the difference was statistically significant（P<0.01）. There was no correlation between HSN type and course of disease or DP value. The intensity of HSN was negatively correlated with the course of disease（r=-0.320, P<0.001） and positively correlated with DP value（r=0.364, P<0.001）, respectively. The intensity of unchanged nystagmus and spontaneous nystagmus were（8.0±5.7） °/s and（8.5±6.4）°/s, respectively. There was no statistically significant difference in the intensity of nystagmus before and after shaking the head. Conclusion:HSN can be classified into five types and could be regarded as a potential SN within a specific frequency range （mid-frequency）. Similarly, SN could also be considered as a common sign of unilateral vestibular impairment at different frequencies. HSN intensity can reflect the dynamic process of vestibular compensation, and is valuable for assessing the frequency of damage in peripheral vestibular diseases and monitoring the progress of vestibular rehabilitation.

Esotropia, nystagmus and optic disk staphyloma in Donnai-Barrow syndrome / Esotropia, nistagmo e estafiloma de disco óptico na síndrome de Donnai-Barrow

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.

A rare case of spasmus nutans in an infant

@#We report a case of an otherwise healthy 23-month-old boy who presented with nystagmus, head shaking, and abnormal head posture suggestive of spasmus nutans. Neuro-ophthalmologic exam revealed bilateral, low-amplitude, high-frequency, horizontal, disconjugate nystagmus that was more prominent in one eye along with head shaking and a head tilt or face turn. The rest of the exam and the systemic physical examination were normal. Magnetic resonance imaging of the brain did not disclose optic pathway glioma, which has been reported to cause spasmus nutans-like disease. Electroretinogram (ERG) was also recommended to rule out occult retinopathies. However, it was not done due to unavailability of the appropriate corneal electrode for his age. Instead, close follow-up was advised to monitor spontaneous improvement or resolution, or until the child comes of age that he can undergo ERG. This case highlights the management approach and rationale of patients with presumed spasmus nutans. Recognition of the triad of spasmus nutans allows for quick diagnosis and more focused and efficient investigation.

Interpretación de resultados de las pruebas oculomotoras, posicionales y calóricas de la videonistagmografía. Revisión documental / Interpretation of the results of the oculomotor, positional and caloric test of the videonistagmography. Documentary review

Introducción: en la actualidad, se reconocen cuadros vestibulares periféricos y centrales que pueden ser diagnosticados mediante la videonistagmografía (VNG). Los avances en la tecnología han provocado en los profesionales una constante actualización en el uso e interpretación de las distintas pruebas que conlleven, en su lectura cruzada, un diagnóstico acertado y a tratamientos de rehabilitación exitosos. El objetivo fue describir las interpretaciones de los resultados de las pruebas oculomotoras, posicionales y calóricas de la VNG para lograr un diagnóstico detallado de las disfunciones vestibulares. Materiales y métodos: revisión documental obtenida de 40 fuentes reportadas en la literatura científica entre 2010 a 2020 tomadas de bases de datos, tesis de grado y libros. Discusión: dentro de la revisión se encontraron tres categorías (pruebas oculomotoras, posicionales y calóricas) y siete subcategorías (nistagmo espontáneo, nistagmo evocado por la mirada, rastreo, sacadas, optocinético, Dix-Hallpike y roll test). Conclusión: los diversos elementos encontrados en la presente revisión son relevantes ya que precisan no solo el tipo de vértigo, sino también su localización topográfica, lo que favorece el proceso de evaluacióndiagnóstico en la población en general.

Introduction: At present, peripheral and central vestibular frames are recognized that can be diagnosed by videonystagmography (VNG). Advances in technology have caused professionals to constantly update the use and interpretation of the different tests that lead, in their cross-reading, to an accurate diagnosis and successful rehabilitation treatments. The objective was to describe the interpretations of the results of the oculomotor, positional and caloric tests of the VNG, for a detailed diagnosis of the vestibular dysfunctions. Materials and method: Documentary review obtained from 40 sources reported in the scientific literature between 2010 and 2020, taken from databases, thesis and books. Discussion: Within the review, three categories were found (oculomotor, positional and caloric tests) and seven subcategories (spontaneous nystagmus, gaze-evoked nystagmus, tracking, saccades, optokinetic, Dix-Hallpike and roll test). Conclusion: The various elements found in this review are relevant in that they specify not only the type of vertigo but also its topographic location, favoring the evaluation-diagnosis process in the general population.

Clinical features and CACNA1A gene mutation in a family with episodic ataxia type 2 / 中南大学学报(医学版)

Episodic ataxia (EA) is a group of disorders characterized by recurrent spells of vertigo, truncal ataxia, and dysarthria. Episodic ataxia type 2 (EA2), the most common subtype of EA, is an autosomal dominant disease caused by mutation of the CACNA1A gene. EA2 has been rarely reported in the Chinese population. Here we present an EA2 family admitted to Xiangya Hospital in October 2018. The proband was a 22-year-old male who complained of recurrent spells of vertigo, slurred speech, and incoordination for 4 years. Brain magnetic resonance imaging (MRI) showed cerebellar atrophy. He had neuropsychological development disorder in childhood, and cognitive assessment in adulthood showed cognitive impairment. The proband's mother and grandmother had a similar history. Peripheral blood samples from the proband and family members were collected, and genomic DNA was isolated. Whole exome sequencing of the proband detected a heterozygous frameshift mutation c.2042_2043del (p.Q681Rfs*100) of CACNA1A gene. This mutation was verified in the proband and 2 family members using Sanger sequencing. One family member carrying this mutation was free of symptoms and signs, suggesting an incomplete penetrance of the mutation. We reported a variant c.2042_2043del of CACNA1A gene as the pathogenic mutation in a Chinese EA2 family for the first time. This case enriched the clinical spectrum of CACNA1A related EA2, and contributed to the understanding of clinical and genetic characteristics of EA2 to reduce misdiagnosis.

Nystagmus may be the first neurological sign in early stages of spinocerebellar ataxia type 3 / Nistagmo pode ser o primeiro sinal neurológico nos estágios precoces da ataxia espinocerebelar tipo 3

Abstract Background: Spinocerebellar ataxia type 3 (SCA3) is the most common autosomal dominant spinocerebellar ataxia worldwide. Almost all patients with SCA3 exhibit nystagmus and/or saccades impairment. Objective: To investigate the presence of nystagmus as an early neurological manifestation, before ataxia, in some patients with SCA3 in the first six months of the disease. Methods: We evaluated a series of 155 patients with clinically and molecularly proven SCA3 between 2013 and 2020. Data regarding sex, age, age at onset, disease duration, CAG repeat expansion length, first symptom, presence of ataxia, scores on SARA and ICARS scales, and presence and characteristics of nystagmus were collected. Results: We identified seven patients with symptomatic SCA3 who presented with isolated nystagmus. In these seven individuals the age at onset ranged from 24 to 57 years, and disease duration from four to six months. Conclusions: Our study showed that nystagmus may be the first neurological sign in SCA3. This clinical observation reinforces the idea that the neurodegenerative process in SCA3 patients may start in vestibular system connections or in flocculonodular lobe. This study adds relevant information about pre-symptomatic features in SCA3 that may work as basis for a better understanding of brain degeneration and for future therapeutic clinical trials.

RESUMO Antecedentes: A ataxia espinocerebelar tipo 3 (SCA3) é a ataxia espinocerebelar de herança autossômica dominante mais comum em todo o mundo. Quase todos os pacientes com SCA3 têm nistagmo e/ou comprometimento das sácades. Objetivo: Investigar a presença de nistagmo como manifestação neurológica precoce, antes do surgimento da ataxia, em alguns pacientes com SCA3 nos primeiros seis meses de doença. Métodos: Foram avaliados 155 pacientes com diagnóstico clínico e molecular de SCA3, entre 2013 e 2020, em relação a sexo, idade, idade de início, duração da doença, expansão da repetição CAG, primeiro sintoma, presença de ataxia, pontuações nas escalas SARA e ICARS, e presença e caracterização de nistagmo. Resultados: Identificamos sete pacientes com SCA3 que apresentavam nistagmo isolado. A idade de início da doença nesses pacientes variou de 24 a 57 anos e a duração da doença variou de quatro a seis meses. Conclusões: O nosso estudo mostrou que o nistagmo pode ser o primeiro sinal neurológico na SCA3. Essa observação clínica reforça a ideia de que o processo neurodegenerativo nos pacientes com SCA3 pode se iniciar nas conexões do sistema vestibular ou no lobo floculonodular. Este estudo adiciona informações relevantes sobre características pré-sintomáticas na SCA3 e que podem servir de base para melhor entendimento da degeneração cerebral e para futuras terapias.

Visual outcomes of audio-luminous biofeedback training for a child with idiopathic nystagmus / Desfechos visuais de treinamento de biofeedback audioluminoso em criança com nistagmo idiopático

ABSTRACT Microperimetry biofeedback training is a vision rehabilitation method that involves the training of attention and oculomotor control, and the rehabilitation of poorly located and non-functional preferred retinal loci. It can significantly improve distance and near visual acuity in age-related macular degeneration. Previous studies have shown that biofeedback training using electrical nystagmography can reduce nystagmus amplitude and increase foveation time. However, these improvements have not been sustained following training sessions. We hereby report a pediatric case of idiopathic nystagmus in an 11-year old patient treated with microperimetric biofeedback to improve visual acuity and fixation stability. The training had a beneficial impact, positively affecting fixation stability as well as distance and near reading vision. Subjectively, improvement in quality of life was also reported. Conversely to previous studies, the positive effects in this case were maintained for as long as twelve months following therapy. To the best of our knowledge, this is the first case with long-term benefits to be reported in the literature.(AU)

RESUMO O treinamento de biofeedback por microperimetria é um método de reabilitação da visão que envolve treinamento de atenção, controle oculomotor e reabilitação do locus preferencial de fixação da retina. Esse treinamento pode melhorar significativamente a acuidade visual para longe e perto na degeneração macular relacionada à idade. Estudos anteriores mostraram que o treinamento de biofeedback usando a nistagmografia elétrica pode reduzir a amplitude do nistagmo e aumentar o período de foveação. Entretanto, os resultados não se mantiveram após o término das sessões. Aqui é relatado um caso de tratamento com biofeedback por microperimetria para melhorar a acuidade visual e a estabilidade de fixação em uma criança de 11 anos de idade. O treinamento teve impacto benéfico e afetou positivamente a estabilidade da fixação e a visão para longe, para perto e de leitura. Subjetivamente, foi relatada melhoria da qualidade de vida. Em contraste com estudos anteriores, os efeitos positivos foram mantidos até 12 meses após a terapia. Até onde sabemos, este é o primeiro caso na literatura que relata benefícios de longo prazo.(AU)

Effects of vestibular spontaneous nystagmus on visual smooth pursuit function / 中华耳鼻咽喉头颈外科杂志

Objective: The aim of the study is to analyze the effects of vestibular spontaneous nystagmus(SN) on the smooth pursuit function of visual ocularmotor system. Methods: A total of 46 patients with acute unilateral peripheral vestibular syndrome with SN (26 cases of vestibular neuritis, 6 cases of Ramsay Hunt Syndrome (RHS) with vertigo, 14 cases of sudden deafness with vertigo) were included in this work. In the study group, the results of SPT and SN test with videonystagmography(VNG) were also reviewed. Taking SPT parameters, the influence of SN intensity on SPT gain, asymmetry and waveform and their correlation were analyzed.SPSS19.0 software was used for statistical analysis. Results: Among the 46 patients, there were 36 cases of SN pointing to the healthy side(SN intensity range of 2.68°/s-32.53°/s), and 10 cases of SN pointing to the affected side (SN intensity range of 2.66°/s-16.54°/s). SN intensity was divided into 3 groups, including light(0.50°/s-5.00°/s), medium(5.01°/s-10.00°/s) and strong(>10.01°/s), accounting for 14 cases(30.4%), 18 cases(39.1%) and 14 cases(30.4%), respectively. The differences of the gain of SPT to the fast phase and slow phase direction in the overall groups and light, medium and strong groups of SN intensity respectively were statistically significant(ttotal=13.338, tlight=6.184, tmedium=8.436, tstrong=8.477, all of P<0.001). The difference of SPT gain in SN fast phase direction between groups with different SN intensity was statistically significant(F=9.639, P<0.001),there was no statistically significant difference in SPT gain between the groups on the SN slow phase direction(F=1.137, P=0.330).The SN intensity significantly negatively correlated with the SPT gain of the fast phase direction of SN (r=-0.433, P=0.003), that was, the SPT gain on the fast phase direction of SN decreased with the increase of SN intensity. There was no significant correlation between SN intensity and the gain of SPT on the slow phase direction of SN (r=-0.061, P=0.687). SPT waveform analysis showed that type I, type II and type III accounted for 8 cases(17.4%), 21 cases(45.6%) and 17 cases(37.0%), respectively. The corresponding mean values of SN intensity were (3.71±0.69)°/s, (7.44±1.88)°/s, (20.04±5.53)°/s, respectively, without type IV wave. The intensity of SN was positively correlated with the asymmetric value of the gain of SPT left and right(r=0.450,P=0.002). That was, with the increase of SN strength, the asymmetric value also increased, and the worse the asymmetry of the gain of SPT left and right pursuit was, the worse the SPT waveform was. Conclusion: SPT gain, asymmetry and SPT waveforms are all affected by SN, and the greater the intensity of SN, the greater the influence on the three. When SN is strong, type III waves may occur, suggesting that acute peripheral vestibular syndrome can also affect the visual ocularmotor systems.

Acute Vertical Pendular Nystagmus and Delayed Oculopalatal Tremor in a Patient with Bilateral Horizontal Gaze Palsy

No abstract available.

Nistagmo vertical hacia abajo / Downbeat nystagmus

RESUMEN El downbeat nystagmus o nistagmo vertical hacia abajo es el nistagmo de fijación adquirido más frecuente, que en la mayoría de los casos es causado por patología a nivel del sistema nervioso central que genera disrupción del control inhibitorio del flóculo y paraflóculo cerebeloso sobre los núcleos vestibulares. Entre sus causas se encuentran enfermedades neurodegenerativas y vasculares de cerebelo o tronco cerebral, tumores y traumas, pero cerca del 40% de los casos son idiopáticos y hasta la mitad de los pacientes presentan estudio imagenológico negativo. En este artículo presentamos dos casos que consultaron en el Servicio de Otorrinolaringología del Hospital Clínico de la Universidad de Chile.

ABSTRACT Downbeat nystagmus is the most frequent acquired fixation nystagmus and it is generally caused by central pathology disrupting the inhibitory control of the cerebellar flocculus and paraflocculus over the vestibular nuclei. Among its causes are neurodegenerative and vascular diseases of cerebellum or brainstem, tumors and trauma, but nearly 40% of the cases are idiopathic and up to half the patients have negative imaging study. In this article we present two cases that were evaluated in the Otolaryngology Department of the Clinical Hospital of the University of Chile.

Evaluation of vestibular and oculomotor functions in individuals with dizziness after stroke / Avaliação das funções vestibular e oculomotora em indivíduos com tontura após acidente vascular encefálico

ABSTRACT Changes in postural balance and visual complaints are frequent consequences of stroke. We aimed to investigate the symptoms and the vestibular and oculomotor functions of patients with dizziness post ischemic and hemorrhagic stroke and compare the results among them. Methods: Fifty patients with dizziness after stroke were evaluated through a clinical anamnesis and computerized vector electronystagmography: calibration of ocular movements, spontaneous nystagmus, semi-spontaneous nystagmus, pendular tracking, optokinetic nystagmus, rotary chair testing, and the caloric test. Results: All patients complained of dizziness, especially imbalance. Ischemic stroke in the carotid territory was the prevalent type. Visual complaints were reported by 56% of the sample and were related to abnormalities in oculomotor and caloric tests. Conclusion: The occurrence of visual symptoms was related to some abnormalities in the vector electronystagmography tests, being more frequent in cases of stroke in the vertebrobasilar system, and with oscillopsia and reduced visual acuity as symptoms.

RESUMO Alterações no equilíbrio postural são consequências frequentes no acidente vascular cerebral (AVC). O objetivo deste estudo foi investigar os sintomas e as funções vestibular e oculomotora de sujeitos com tontura após AVC isquêmico e hemorrágico, comparando seus resultados. Métodos: Foram avaliados 50 sujeitos com tontura após AVC, por meio de anamnese clínica e vectoeletronistagmografia computadorizada (VENG): calibração dos movimentos oculares; nistagmo espontâneo e semi-espontâneo; rastreio pendular; nistagmo optocinético; prova rotatória pendular decrescente e prova calórica com estímulo a ar. Resultados: Todos relataram tontura, principalmente do tipo desequilíbrio. O AVC isquêmico e no sistema carotídeo foi o mais frequente. Sintomas visuais pós-AVC foram referidos por 56% da amostra, os quais tiveram relação com alterações nas provas oculomotoras e calórica da VENG. Conclusão: A ocorrência de sintomas visuais relacionou-se a alterações em alguns testes. Essas alterações foram mais frequentes nos casos de AVC da circulação posterior, naqueles com oscilopsia e diminuição da acuidade visual.

Benign Paroxysmal Positional Vertigo after Yoga Practice / 대한평형의학회지

A 57-year-old woman presented with sudden onset of whirling vertigo associated with nausea and vomiting. The neurological examination showed left-beating horizontal nystagmus on the lying-down test and right-beating horizontal nystagmus on the head bending test. Geotropic direction-changing horizontal nystagmus was demonstrated on both sides during the supine roll test. Benign paroxysmal positional vertigo (BPPV) was the most common vestibular disorder in patients after head trauma. The authors experienced a case of right horizontal canal BPPV occurred after a yoga practice, thereby we report the case with a review of the related literatures.

Characteristics of Nystagmus during Attack of Vestibular Migraine / 대한평형의학회지

OBJECTIVES: The purpose of this study is to investigate characteristics of nystagmus during attacks of vestibular migraine (VM), and to find a distinct clinical feature compared to other migraine and peripheral vestibular disorders. METHODS: This study is a retrospective chart review of 82 patients satisfied with VM criteria, which is formulated by the new Bárány Society. Spontaneous and positional nystagmus provoked by various head positions were examined with video-nystagmography in all patients. Bithermal caloric test and cervical vestibular evoked myogenic potential test (cVEMP) were also performed. The direction and peak slow-phase velocity (SPV) of nystagmus, unilateral caloric weakness and interaural difference of cVEMP were analyzed. Control groups were lesion side in acute VN for nystagmus results and healthy side in the patients with benign paroxysmal positional vertigo of posterior semicircular canal for caloric and cVEMP results. Chi-square test and Mann-Whitney U-test were used for statistical analysis. RESULTS: During the acute attack, nystagmus was seen in 71.9% (59 of 82) of patients. Horizontal nystagmus was the predominant type. Peak SPV in VM patients was much slower than in the control group (2.37±1.73 °/sec vs. 17.05±12.69 °/sec, p<0.0001). There was no significant difference on the result of both caloric and cVEMP test, compared to those of control groups. CONCLUSION: Nystagmus with horizontal directions and low SPV was dominant form in the attack of VM. Close observation of nystagmus can be helpful to make a correct diagnosis and to understand the pathomechanism of vertigo in VM.

Neuromyelitis Optica Spectrum Disorder Presented with Upbeat Nystagmus and Intractable Vomiting / 대한평형의학회지

Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory demyelinating autoimmune disease of central nervous system characterized by relapsing attacks that target the optic nerves and spinal cord, as well as aquaporin-4 (AQP4) enriched periventricular brain regions. The area postrema (AP), located in the dorsal medulla, is the chemosensitive vomiting center and has high AQP-4 expression. The AP syndrome with unexplained hiccups, nausea, and vomiting is one of the core clinical characteristics in the NMOSD and maybe the first presenting symptom. We experienced a 25-year-old woman presented with intractable vomiting, dizziness and oscillopsia. Upbeat nystagmus detected on the bedside examination led to comprehensive neurological workups including magnetic resonance imaging, and she was diagnosed as the AP syndrome. Ten months later, she experienced a recurrence as a longitudinally extensive transverse myelitis and the diagnosis was finally compatible with NMOSD without AQP4-IgG. NMOSD, especially the AP syndrome, should be considered in any dizzy patient with intractable vomiting, and detailed neuro-otologic and neuro-ophthalmologic examinations are warranted for the correct diagnosis.

Otoneurological findings prevalent in hereditary ataxias / Alterações otoneurológicas prevalentes nas ataxias hereditárias

ABSTRACT Objective To describe and compare the vestibular findings most evident among the hereditary ataxias, as well as correlate their clinical features with the nervous structures affected in this disease. Methods Seventy-five patients were evaluated and underwent a case history, otorhinolaryngological and vestibular assessments. Results Clinically, the patients commonly had symptoms of gait disturbances (67.1%), dizziness (47.3%), dysarthria (46%) and dysphagia (36.8%). In vestibular testing, alterations were predominantly evident in caloric testing (79%), testing for saccadic dysmetria (51%) and rotational chair testing (47%). The presence of alterations occurred in 87% of these patients. A majority of the alterations were from central vestibular dysfunction (69.3%). Conclusion This underscores the importance of the contribution of topodiagnostic labyrinthine evaluations for neurodegenerative diseases as, in most cases, the initial symptoms are otoneurological; and these evaluations should also be included in the selection of procedures to be performed in clinical and therapeutic monitoring.

RESUMO Objetivo Descrever e comparar os achados vestibulares mais evidentes entre a ataxia hereditária, bem como correlacionar seus aspectos clínicos com o estudo das estruturas nervosas afetadas nesta doença. Métodos 75 pacientes foram avaliados e submetidos aos seguintes procedimentos: anamnese, avaliação otorrinolaringológica e vestibular. Resultados Clinicamente, os pacientes apresentaram sintomas de distúrbios da marcha (67,1%), tonturas (47,3%), disartria (46%) e disfagia (36,8%). No teste vestibular, as alterações foram predominantemente evidentes no teste calórico (79%), dismetria sacádicas (51%) e no teste rotatório (47%). A presença de alterações ocorreu em 87% dos pacientes. A maioria das alterações observadas foram da disfunção vestibular central (69,3%). Conclusão O estudo ressalta a importância da contribuição da avaliação labiríntica no topodiagnóstico para doenças neurodegenerativas, uma vez que, na maioria dos casos, os sintomas iniciais são otoneurológicos, e essas avaliações também devem ser incluídas na seleção de procedimentos a serem realizados no monitoramento clínico e terapêutico.

Aspectos otoneurológicos em Traumatismos Cranioencefálicos: série de casos / Otoneurological aspects in Traumatic Brain Injuries: series of cases

RESUMO Estudo retrospectivo, transversal e descritivo, aprovado pelo Comitê de Ética em Pesquisa (CEP), sob o número CAAE 16728013.0.0000.5346. Trata-se de uma série de casos que tem por objetivo investigar a presença de sintomas otoneurológicos e o equilíbrio postural de seis pacientes com tontura após traumatismo cranioencefálico (TCE). Foram submetidos a uma anamnese clínica e a avaliações: teste de organização sensorial por meio da posturografia dinâmica foam laser e provas oculomotoras e vestibulares da vectoeletronistagmografia computadorizada (VENG). Os dados foram analisados a partir dos valores de referência para cada avaliação. Na posturografia, observou-se que as maiores diferenças entre o valor obtido e o valor de referência foram nas posições VI, V e III, respectivamente. A análise sensorial indicou alteração, principalmente, nos sistemas de preferência visual e vestibular. Todos os pacientes avaliados apresentaram ao menos uma alteração nas provas da VENG. Cinco pacientes tiveram alteração na prova calórica e nenhum apresentou alteração na prova rotatória pendular decrescente, que avalia a compensação vestibular. Além das queixas vestibulares, quatro pacientes estavam em tratamento com psicotrópicos para depressão. Tais resultados ratificam a presença de alterações vestíbulo-oculares no pós-TCE, os quais devem receber atenção especial devido ao comprometimento central associado.

ABSTRACT It is a retrospective, cross-sectional, descriptive, approved by Ethics Research Committee (ERC), under number CAAE 16728013.0.0000.5346. This is a series of cases that aims to investigate the presence of otoneurological symptoms and the postural balance of six patients with dizziness after Traumatic Brain Injury (TBI). Participants were submitted to a clinical anamnesis and the evaluations: Sensory Organization Test through Foam Laser Dynamic Posturography and oculomotor and vestibular tests of Computerized Vectoelectronystamography (VENG). The data were analyzed from the reference values for each evaluation. In posturography, it was observed that the greatest differences between the value obtained and the reference value were in positions VI, V and III, respectively. Sensory analysis indicated alteration mainly in the visual and vestibular preference systems. All the evaluated patients presented at least one alteration in the VENG tests. Five patients had alterations in the caloric test, and none presented alterations to the rotatory chair test (RCT), which evaluates the vestibular compensation. Considering vestibular complaints, four patients were on psychotropic treatment for depression. These results demonstrate the presence of vestibulo-ocular alterations in post-TBI, which should receive special attention due to associated central impairment.

A Case of Acute Vestibular Neuritis with Periodic Alternating Nystagmus / 대한이비인후과학회지

Detection of nystagmus is an important diagnostic clue in patients with acute vertigo. Patients with peripheral disorders exhibit nystagmus with a constant direction whereas those with central disorders exhibit nystagmus with changes in direction with or without gaze fixation. Periodic alternating nystagmus (PAN) is a horizontal or horizontal-rotary jerk-type nystagmus that reverses its direction with time. PAN is typically observed in patients with central disorders, such as cerebellar or pontomedullary lesions, but it is also observed in patients with peripheral disorders, albeit rarely. Here we report a rare case of a 58-year-old patient with vertigo with PAN, which was initially suspected as a central disorder, but eventually diagnosed as a peripheral vestibular disorder. We investigated the characteristics and mechanisms of peripheral PAN in this case. The absence of central disorder symptoms, visual suppression of PAN, normal oculomotor findings, and transient persistence are important diagnostic clues for differentiating peripheral from central PAN.

Simultaneous Occurrence of a Splenial Lesion and Downbeat Nystagmus Due to Lithium Neurotoxicity

No abstract available.

The manifestation of videonystamography in the patients with posterior circulation ischemia vertigo / 临床耳鼻咽喉头颈外科杂志

To investigate the changes of vedionystamography(VNG)in patients with posterior circulation ischemia vertigo(PCIV).Fifty patients who complained of vertigo and imbalance with PCI were selected as experimental group for testing of visual nystamography(VNG).Thirty normal subjects were chosen as control group.The result was analyzed.The results of VNG in PCIV group and the control group were compared.The abnormal ratio were as follows:(4%,0;>0.05)for Spontaneous nystagmus,(68%,10%;<0.01)for Saccade Test,(42.0%,6.7%;<0.01)for Tracking Test,(44%,0;<0.01)for Optokinetic Test,(78%,10%;<0.01)for Positional Test,respectively.The intensity of positional nystagmus in those patients was(4.12±3.46)°/s,which was much higher than that of the control group(<0.01).One or more abnormal findings for visual-oculomotor system examination were shown in 37 patients(74%).Both vestibular central and peripheral system can be involved in PCIV.VNG test has clinical significance in differential diagnosis and lesion location.The abnormal ratio of visual nystamography in PCIV group reaches 92%(46/50).These results suggest that VNG be used as an important accessory diagnostic tool for patients with PCIV.