Two-muscle surgical treatment of a compensatory head tilt in an adult with acquired downbeat nystagmus.

Kestenbaum-Anderson-like operations have proven beneficial in treatment of compensatory head tilt in patients with infantile nystagmus. However, their use in acquired vertical nystagmus in adults with head tilt has rarely been reported. Presented here is a case of a 52-year-old woman with acquired downbeat nystagmus with a significant head tilt who responded to a simple two-muscle surgery involving the superior recti. Cyclovertical muscle surgery should be considered a viable option in such patients who are refractory to medical intervention. Additionally, it appears that four-muscle vertical muscle recessions (two muscles per eye) may not be necessary to dampen vertical nystagmus since good results can be obtained with a single muscle recession bilaterally.

Vertical Nystagmus Recognition Based on Deep Learning.

Vertical nystagmus is a common neuro-ophthalmic sign in vestibular medicine. Vertical nystagmus not only reflects the functional state of vertical semicircular canal but also reflects the effect of otoliths. Medical experts can take nystagmus symptoms as the key factor to determine the cause of dizziness. Traditional observation (visual observation conducted by medical experts) may be biased subjectively. Visual examination also requires medical experts to have enough experience to make an accurate diagnosis. With the development of science and technology, the detection system for nystagmus can be realized by using artificial intelligence technology. In this paper, a vertical nystagmus recognition method is proposed based on deep learning. This method is mainly composed of a dilated convolution layer module, a depthwise separable convolution module, a convolution attention module, a Bilstm-GRU module, etc. The average recognition accuracy of the proposed method is 91%. Using the same training dataset and test set, the recognition accuracy of this method for vertical nystagmus was 2% higher than other methods.

Vestibular Disorders after Kidney Transplantation: Focus on the Pathophysiological Mechanisms Underlying the Vertical Nystagmus Associated with Tacrolimus-Related Hypomagnesamia.

The purpose of this paper is to present the case of a patient undergoing kidney transplantation who developed limb tremor dizziness and vertical nystagmus (ny) during Tacrolimus (TAC) therapy and to investigate the pathophysiological mechanisms underlying the balance disorder. This case study regards a 51-year old kidney transplant male patient with hand tremors and lower limbs asthenia associated with dizziness and nausea. The symptoms started two months after the beginning of intravenous TAC for renal transplantation. The pure-tone audiometry showed a mild symmetrical high-frequencies down-sloping sensorineural hearing loss. Acoustic emittance measures showed a normal tympanogram; stapedial reflexes were normally elicited. The Auditory Brainstem Responses (ABR) and Cervical Vestibular Evoked Myogenic Potentials (c-VEMPs) were bilaterally normally evoked. The bedside vestibular examination showed spontaneous down-beating stationary persistent, omni-positional nystagmus, not inhibited by fixation. The Head-Shaking Test accentuates the spontaneous ny. The horizontal clinical head impulse test was negative, bilaterally. A biochemical blood test revealed a decrease in Magnesium (Mg) levels (0.8 mg/dL; normal range 1.58-2.55). The integration of Mg induced both a plasma levels normalization and an improvement of clinical symptoms. This case suggests that TAC treatment can induce a Mg depletion that caused the transient cerebellar lesion. Therefore, the monitoring of serum electrolytes during immunosuppressive treatment appears to be a useful tool in order to reduce the central system symptomatology.

Spontaneous Upbeat Nystagmus and Selective Anterior Semicircular Canal Hypofunction on Video Head Impulse Test: A New Variant of Canalith Jam?

We describe a rare case of spontaneous upbeat nystagmus (UBN) attributable to a canalith jam involving the anterior semicircular canal (ASC) in a patient in whom comprehensive vestibular assessment was useful to identify the underlying pathomechanism. A 56-year-old woman with unsteadiness following repositioning procedures for left-sided benign paroxysmal positional vertigo (BPPV) presented with spontaneous UBN that showed slight right torsional components. A vestibular test battery detected isolated left ASC hypofunction on a video-head impulse test (Video-HIT). We postulated a persistent utriculopetal deflection of the left ASC cupula, which was attributable to entrapment of debris in a narrow canal tract, with consequent sustained inhibition of the ampullary afferents. Although spontaneous UBN receded after impulsive physical therapy, unsteadiness deteriorated into positional vertigo secondary to canalolithiasis involving the ipsilateral posterior canal. In our view, physical therapy possibly fragmented the canalith jam and released free-floating otoconia that eventually settled into the ipsilateral posterior canal. Video HIT revealed normalization of ASC hypofunction, and leftsided posterior canal canalolithiasis was successfully treated using appropriate repositioning procedures. We propose that a canalith jam involving the ASC should be considered in the differential diagnosis of spontaneous UBN, particularly in patients with a history of BPPV and isolated ASC hypofunction detected on video HIT.

[Vertical nystagmus]. / Vertikal'nyi nistagm.

The review article provides a definition and classification of different nystagmus types, a comparative description of the central and peripheral vestibular nystagmus. The pathogenetic patterns of up-beating and down-beating nystagmus are accurately described. The features of nystagmus formation in various diseases are discussed, such as Wernicke encephalopathy, Arnold-Chiari anomaly, spinocerebellar ataxia and vestibular migraine. The authors provide their own data on oculomotor disorders in 100 patients with vestibular migraine and migraine with a brain stem aura. This article considers approaches to treatment: surgical and conservative. In conclusion, was noted the possibility of differentiating the central and peripheral vestibular nystagmus by means of clinical study. As well, the differences between vertical nystagmus associated with organic lesions of the brain stem or cerebellum and transient nystagmus with vestibular migraine are highlighted. The authors note the need for in-depth studies of nystagmus in vestibular migraine patients and methods of dealing with it.

Vertical nystagmus as isolated presentation in a patient with new diagnosis of multiple sclerosis.

Multiple sclerosis (MS) is a progressive demyelinating disease of the central nervous system with a wide array of symptoms. We present a healthy young woman who came to the Emergency Department with two days of isolated vertical nystagmus and was subsequently diagnosed with MS on imaging. Although bilateral vertical nystagmus is not a common presentation of MS, its presence should prompt inclusion of this disease process in the differential diagnosis.

Acquired nystagmus.

Nystagmus is the repetitive to and fro movement of the eyes, which may be physiological or pathological. The movements can be horizontal, vertical, torsional or a combination of these movements. It starts by a slow movement of the eye away from the visual target. The second movement brings the eye back to the visual target. If this second movement is quick, the nystagmus is called jerk nystagmus. If the second movement is slow, the nystagmus is said to be pendular. Maintaining steady gaze is dependent upon visual fixation, the vestibulo-ocular reflex and the gaze-holding neural integrator system. Pathological nystagmus typically presents with the symptom of oscillopsia, which is the illusory movement of the surrounding environment. Nystagmus that develops outside of early infancy is termed acquired nystagmus. There may be serious underlying pathology that will require further investigation and management. This article reviews the terminology, pathophysiology, causes and treatment of acquired nystagmus.

Vertical nystagmus as a feature of PIGN-related glycosylphosphatidylinositol biosynthesis defects.

Vertical nystagmus is a known clinical feature, is however rarely observed in a specific neurodevelopmental disorder. Based on our experience with Polish patients with glycosylphosphatidylinositol biosynthesis defects (GPIBD) due to PIGN variants, supported by literature review, we have verified the clinical significance of this feature in PIGN-related disorder. We hope to underline the clinical implication of vertical nystagmus in the evaluation of patients with developmental encephalopathy with epilepsy, which may accelerate the neurological diagnosis process by orientating it towards PIGN-GPIBD.

[Research advances on mechanism and lesion location of vertical nystagmus].

Vertical nystagmus is a vertical nystagmus caused by structural abnormalities and/or dysfunction of the central vestibular system and observed in situ in the center of the eyeball. There are two kinds of nystagmus （UBN） and downbeat nystagmus （DBN） according to the direction of nystagmus. The diagnosis of UBN is mainly made by naked eye or electronystagmography/viewer. It is a common neuro-ophthalmologic sign in the field of vestibular medicine. In this paper, the mechanism of vertical nystagmus formation and the location of lesions were briefly introduced, in order to provide help for the diagnosis and treatment of Vertigo.

Research advances on mechanism and lesion location of vertical nystagmus / 临床耳鼻咽喉头颈外科杂志

Summary@#Vertical nystagmus is a vertical nystagmus caused by structural abnormalities and/or dysfunction of the central vestibular system and observed in situ in the center of the eyeball. There are two kinds of nystagmus （UBN） and downbeat nystagmus （DBN） according to the direction of nystagmus. The diagnosis of UBN is mainly made by naked eye or electronystagmography/viewer. It is a common neuro-ophthalmologic sign in the field of vestibular medicine. In this paper, the mechanism of vertical nystagmus formation and the location of lesions were briefly introduced, in order to provide help for the diagnosis and treatment of Vertigo.

Research advances on mechanism and lesion location of vertical nystagmus / 临床耳鼻咽喉头颈外科杂志

Vertical nystagmus is a vertical nystagmus caused by structural abnormalities and/or dysfunction of the central vestibular system and observed in situ in the center of the eyeball. There are two kinds of nystagmus （UBN） and downbeat nystagmus （DBN） according to the direction of nystagmus. The diagnosis of UBN is mainly made by naked eye or electronystagmography/viewer. It is a common neuro-ophthalmologic sign in the field of vestibular medicine. In this paper, the mechanism of vertical nystagmus formation and the location of lesions were briefly introduced, in order to provide help for the diagnosis and treatment of Vertigo.

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.

Delayed endolymphatic hydrops. Special emphasis on nystagmus associated with episodes and contribution of chemical labyrinthectomy.

OBJECTIVES: The main objective was to describe spontaneous nystagmus characteristics during an episode of delayed endolymphatic hydrops (DEH), including an initial vertical upbeating nystagmus in one patient. The secondary objective was to highlight the contribution of chemical labyrinthectomy. METHODS: Episodic vertigo after a prolonged period of time of sensorineural hearing loss (profound or total) in one ear characterized ipsilateral DEH and was associated with the development of hearing loss in the opposite ear in contralateral DEH. RESULTS: Ten patients met the criteria for DEH: 7 ipsilateral and 3 contralateral. Three (all ipsilateral DEH) were examined during a vertigo episode. Two patients had a typical horizontal-torsional nystagmus beating contralaterally to the hearing loss. One patient showed atypic initial vertical upbeating nystagmus with a slight torsional component, which secondarily became horizontal-torsional beating contralaterally to the hearing loss. Four patients had disabling vertigo with unilateral total deafness (ipsilateral DEH), successfully treated by 1-3 transtympanic gentamycin (Gentalline®) injections. CONCLUSION: Nystagmus direction during vertigo episodes varies, and may initially present as vertical upbeating nystagmus, which, to our knowledge, has not been previously reported in DEH or Menière's disease. This nystagmus might reflect an inhibition of the superior semicircular canal (on the hearing-impaired side), suggesting incipient hydrops in this canal. Chemical labyrinthectomy is a simple and effective procedure in unilateral DEH, especially as the patient often suffers from total deafness.

Clinical criteria of positional vertical nystagmus in vestibular migraine.

OBJECTIVES: The goal of this study was to identify key diagnostic criteria for positional vertical nystagmus caused by vestibular migraine (VM). MATERIALS AND METHODS: The study group included a case series of 13 subjects with VM (10 females and three males with age 38.6 ± 8.9 years); they were complaining of positional vertigo. They were subjected to thorough audiovestibular examination and treated with cinnarizine or topiramate Results: The entire study group demonstrated positional vertical nystagmus (eight patients had up-beating nystagmus and five patients had down-beating nystagmus). The vertigo and nystagmus were elicited in one or more of the following positions: the right & left Dix-Hallpike, supine with head center, head right, and head left positions. The nystagmus had no latency period. It was persistent, non-fatigable and markedly reduced by visual fixation. Prophylactic therapy of VM (topiramate or cinnarizine) cured the positional vertigo in 92% of the study group. Neither the positional vertigo nor the nystagmus recurred in a 3-6 months follow-up period. CONCLUSIONS: VM can induce characteristic form of vertical positional nystagmus and vertigo, which would be treated by medications used for controlling the VM.

Tonic Investigation Concept of Cervico-vestibular Muscle Afferents

Abstract Introduction Interdisciplinary research has contributed greatly to an improved understanding of the vestibular system. To date, however, very little research has focused on the vestibular system's somatosensory afferents. To ensure the diagnostic quality of vestibular somatosensory afferent data, especially the extra cranial afferents, stimulation of the vestibular balance system has to be precluded. Objective Sophisticated movements require intra- and extra cranial vestibular receptors. The study's objective is to evaluate an investigation concept for cervico-vestibular afferents with respect to clinical feasibility. Methods A dedicated chair was constructed, permitting three-dimensional trunk excursions, during which the volunteer's head remains fixed. Whether or not a cervicotonic provocation nystagmus (c-PN) can be induced with static trunk excursion is to be evaluated and if this can be influenced by cervical monophasic transcutaneous electrical nerve stimulation (c-TENS) with a randomized test group. 3D-video-oculography (VOG) was used to record any change in cervico-ocular examination parameters. The occurring nystagmuses were evaluated visually due to the small caliber of nystagmus amplitudes in healthy volunteers. Results The results demonstrate: no influence of placebo-controlled c-TENS on the spontaneous nystagmus; a significant increase of the vertical nystagmus on the 3Dtrunk- excursion chair in static trunk flexion with cervical provocation in all young healthy volunteers (n = 49); and a significant difference between vertical and horizontal nystagmuses during static trunk excursion after placebo-controlled c-TENS, except for the horizontal nystagmus during trunk torsion. Conclusion We hope this cervicotonic investigation concept on the 3D trunk-excursion chair will contribute to new diagnostic and therapeutic perspectives on cervical pathologies in vestibular head-to-trunk alignment.

Tonic Investigation Concept of Cervico-vestibular Muscle Afferents.

Introduction Interdisciplinary research has contributed greatly to an improved understanding of the vestibular system. To date, however, very little research has focused on the vestibular system's somatosensory afferents. To ensure the diagnostic quality of vestibular somatosensory afferent data, especially the extra cranial afferents, stimulation of the vestibular balance system has to be precluded. Objective Sophisticated movements require intra- and extra cranial vestibular receptors. The study's objective is to evaluate an investigation concept for cervico-vestibular afferents with respect to clinical feasibility. Methods A dedicated chair was constructed, permitting three-dimensional trunk excursions, during which the volunteer's head remains fixed. Whether or not a cervicotonic provocation nystagmus (c-PN) can be induced with static trunk excursion is to be evaluated and if this can be influenced by cervical monophasic transcutaneous electrical nerve stimulation (c-TENS) with a randomized test group. 3D-video-oculography (VOG) was used to record any change in cervico-ocular examination parameters. The occurring nystagmuses were evaluated visually due to the small caliber of nystagmus amplitudes in healthy volunteers. Results The results demonstrate: no influence of placebo-controlled c-TENS on the spontaneous nystagmus; a significant increase of the vertical nystagmus on the 3D-trunk-excursion chair in static trunk flexion with cervical provocation in all young healthy volunteers (n = 49); and a significant difference between vertical and horizontal nystagmuses during static trunk excursion after placebo-controlled c-TENS, except for the horizontal nystagmus during trunk torsion. Conclusion We hope this cervicotonic investigation concept on the 3D trunk-excursion chair will contribute to new diagnostic and therapeutic perspectives on cervical pathologies in vestibular head-to-trunk alignment.

One Case of Downbeat Nystagmus with Compression of Vestibulocochlear Nerve by Vertebral Arteries

A case is reported of downbeat nystagmus associated with compression of the root of vestibulocochlear nerve by vertebral arteries, which was revealed by magnetic resonance imaging. Chief complain of the patient was positional vertigo, which aggrevated at left decubitus position. Downbeat nystagmus was increased in left Dix-hall pike test. Tetsuo et al, reported downbeat nystagmus with compression of dolichoectatic vertebral arteries to the medulla oblongata and surgical neurovascular decompression of the dolichoectasia reverses the progression of symptoms if permanent neurologic damage has not already occurred.