Spontaneous nystagmus with an upbeat component: Central or peripheral vestibular disorders?

Objective: To determine the topical diagnosis and etiologies of spontaneous nystagmus (SN) with an upbeat component. Methods: We retrospectively recruited 43 patients with SN with an upbeat component at a university hospital in China from 2020 to 2022. SN with an upbeat component was divided into pure upbeat nystagmus (UBN), SN with a predominant upbeat component, and SN with a non-predominant upbeat component. We analyzed their clinical and neurotologic findings and the final diagnosis. Results: Fourteen (32.6%) of them showed pure UBN, while 29 (67.4%) exhibited SN mixed with an upbeat component, mixed upbeat-horizontal in 15, mixed upbeat-horizontal-torsional in 13, and upbeat-torsional in the remaining one. Pure UBN and SN with a predominant upbeat component were more common in central than in peripheral vestibular disorders [16 (80.0%) vs. 0 (0%), Chi-Square test, p < 0.001]. Central vestibular disorders were diagnosed in 20 (46.5%) patients, peripheral in 14 (32.6%), and undetermined in nine (20.9%) patients. The underlying causes mainly included acute unilateral peripheral vestibulopathy (n = 11), posterior circulation infarction (n = 9), benign recurrent vertigo (n = 4), vestibular migraine (VM, n = 3), and VM of childhood (n = 2). Conclusion: SN with an upbeat component can be seen in both central and peripheral vestibular disorders. Pure UBN was a characteristic sign of central vestibular dysfunction. Central vestibular disorders should be highly suspected when patients show pure UBN or SN with a predominant upbeat component.

Generalized vestibular hyporeflexia and chronic upbeat nystagmus due to thiamine deficiency.

BACKGROUND: Ocular motor and vestibular manifestations of Wernicke's thiamine deficiency (WTD) are frequent and heterogeneous. Previous neuropathological and neuroimaging findings identified brainstem and cerebellar lesions responsible for these findings, however, peripheral vestibular lesions are probably uncommon in human WTD, though noted on an avian thiamine deficient study. MATERIAL: Single case study of a WTD patient post-gastric bypass who developed ataxia, oscillopsia and nystagmus, with low serum thiamine, and increased MRI T2 signal in the thalami, but normal brainstem and cerebellum. Vestibular evaluation showed significant vestibular hyporreflexia affecting all six canals, and a chronic upbeat nystagmus, now for 14 months after WTD onset. METHODS: Serial clinical, video head impulse, nystagmus analysis, cervical and ocular vestibular evoked responses. She is undergoing treatment with Memantine, Clonazepam and vestibular rehabilitation, and feels improvement. CONCLUSION: This report shows a novel combination of central and peripheral vestibular findings, of relevance for diagnosis and treatment, in addition to the development of a coherent hypothesis on the ocular motor and vestibular findings in WTD.

Creutzfeldt-Jakob disease with dizziness initially presenting to the otolaryngology department.

Creutzfeldt-Jakob disease is a fatal transmissible prion disease of the central nervous system. Dizziness as an initial manifestation of Creutzfeldt-Jakob disease is rare. However, patients with Creutzfeldt-Jakob disease and dizziness may initially visit the otolaryngology department, but this is uncommon. We report the case of a 56-year-old woman with Creutzfeldt-Jakob disease who initially presented with dizziness as an emergency patient to the otolaryngology department. Primary position upbeat nystagmus was identified using a charge-coupled device camera with infrared illumination. Electronystagmography revealed impaired smooth pursuit and diminished optokinetic nystagmus. Based on these findings, we immediately suspected an intracranial cause of dizziness and reached a presumptive diagnosis of sporadic Creutzfeldt-Jakob disease, thus preventing severe transmission. This case emphasizes that Creutzfeldt-Jakob disease should be included as a differential diagnosis for patients with dizziness and abnormal eye movements, such as primary position upbeat nystagmus, which might be caused by intracranial disease.

Upbeat Nystagmus with an Unusual Velocity-Decreasing and Increasing Waveform: a Sign of Gaze-Holding Dysfunction in the Paramedian Tracts in the Medulla?

We report a patient with spontaneous upbeat nystagmus (UBN) due to an ischemic lesion involving the paramedian tract (PMT) in the medulla. Eye movement recordings, using an infrared video-oculography (VOG) system, showed that the slow phase of the nystagmus was initially velocity-decreasing but gradually became velocity-increasing. Simulation of the nystagmus with a mathematical model supports a role for the PMT in relaying premotor signals for vertical gaze holding to the cerebellum. Our model shows that the disruption in cerebellar input from PMT can lead to the velocity-increasing waveform of the nystagmus, whereas the velocity-decreasing waveform could be related to a mismatch between the innervational commands to the ocular muscles (the pulse and step) needed to hold gaze steady.

Primary Position Upbeat Nystagmus with Low Serum Vitamin B12.

A 38-year-old man presented with primary position upbeat nystagmus accompanied by peripheral neuropathy. The serum vitamin B12 level was low along with high plasma homocysteine level, indicating vitamin B12 deficiency. Cyanocobalamin supplementation showed partial clinical and electrophysiological improvement. Although brain magnetic resonance imaging did not show any abnormal intensity lesions, the electrophysiological findings suggested that a pontomedullary medial lesion was responsible for the upbeat nystagmus. To our knowledge, this is the first case of upbeat nystagmus with low serum vitamin B12. Physicians need to recognize the possibility of vitamin B12 deficiency as a cause of upbeat nystagmus.

The Use of Video-Head Impulse Test in Different Head Positions in Vertical Nystagmus and Ataxia Associated with Probable Thiamine Deficiency.

Upward and downward bias of eye movement signals in the semicircular canals (SCC)- and/or otolith-related central pathways have been proposed to explain the occurrence of vertical nystagmus (downbeat nystagmus [DBN] and upbeat nystagmus [UBN]) and its frequent modulation with head position. Video-head impulse test (VHIT), usually performed in upright position, is a recent development for measuring SCC function. We performed longitudinal nystagmus and VHIT assessments in different head positions in a patient with probable thiamine deficiency, in order to explore a possible relationship between the positional behavior of vertical nystagmus and SCC function. Initially, UBN in upright position changed to DBN in prone position and remained relatively unchanged in supine position. This was associated with both anterior and posterior SCC hyperactive responses in upright position, and a relative enhancement of the anterior SCC responses in prone position and the posterior SCC responses in supine position. Over 1 year, in prone position, change from UBN to DBN and the enhancement of anterior SCC responses remained, while in supine position, UBN either decreased or changed to DBN, when compared to upright position. This was associated with a relative enhancement of the anterior SCC responses in supine position, albeit inconsistently, and the presence of posterior SCC hypoactive responses in all positions, including prone. While not contradicting a primary otolithic dysfunction in the genesis of UBN change to DBN with head position, we provide evidence for positional modulation of SCC function in thiamine deficiency and a possible relationship with nystagmus positional behavior.

[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

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.

Don't Miss This! Red Flags in the Pediatric Eye Examination: Acquired Nystagmus.

When a new patient presents with a chief complaint of nystagmus, the clinician must know what further action is necessary in order to identify vision or life-threatening diseases. Some types of acquired nystagmus include upbeat nystagmus, downbeat nystagmus, see-saw nystagmus, and period alternating nystagmus. By identifying the characteristics and causes, acquired nystagmus can be recognized as a "red flag." In contrast, children diagnosed with infantile or physiological nystagmus generally will not need neurological testing. As crucial as it is to recognize acquired nystagmus, it is equally important to differentiate among the specific subtypes and to be familiar with the key features of infantile nystagmus.

Acute thrombosis of the carotid artery associated with positional nystagmus at multiple planes.

OBJECTIVE: An acute onset central pathology without any clear neurological symptoms may mimic peripheral vestibular problem in an emergency setting. A 54-year-old man suddenly developed dizziness without any cranial nerve symptoms, paresis, cerebellar signs or sensory disturbances except upbeat positional nystagmus at multiple provoked positions which alerted for a possible acute central pathology. FINDINGS: An instantaneous magnetic resonance imaging and angiography studies further showed obstruction of the left internal carotid artery above the bifurcation. The patient's subsequent prognosis was consistent with good recovery following anti-coagulant therapy. A follow-up MRI and angiography showed resolution of thrombosis. CONCLUSION: It should be kept in mind that positional nystagmus is likely to occur in central pathologies. Differentiation between benign paroxysmal positional vertigo and central positioning nystagmus is critical.

Upbeat nystagmus is a useful sign in the regional diagnosis of trigeminal nerve disorder with multiple sclerosis.

Trigeminal nerve disorder is an important neurological sign that is often seen with multiple sclerosis (MS). We investigated eye movements in three MS patients with trigeminal disorder due to pontine lesions near the trigeminal root entry zone (REZ). Upbeat nystagmus was observed in all MS patients with trigeminal REZ lesions. We conjecture that trigeminal nerve disorder and upbeat nystagmus appeared due to simultaneous damage to both the trigeminal nerve and the vestibulo-ocular reflex pathway. If upbeat nystagmus appears in MS patients exhibiting a trigeminal nerve disorder, such as trigeminal neuralgia, and paralysis, pontine lesions near the trigeminal REZ should be considered. Upbeat nystagmus can be understood as a useful sign for the clinical regional diagnosis of trigeminal nerve disorder.

Upbeat nystagmus after bilateral superior canal plugging: A peripheral cause of vertical nystagmus.

Vertical nystagmus without a torsional component is generally considered a finding indicative of central nervous system pathology. We report two cases of purely upbeat nystagmus elicited with mastoid vibration after bilateral superior canal plugging, to highlight the vestibular pathophysiology involved in this unusual peripheral cause for upbeat nystagmus. Laryngoscope, 127:1698-1700, 2017.

[The vestibular craniovertebral joints]. / Die vestibulären Kopfgelenke.

INTRODUCTION: The significance of cervical proprioception for human balance has thus far not been sufficiently elucidated. The aim of this study was to provoke selective cervico-vestibular stimulation using the trunk excursion test (TET) we ourselves constructed. This chair is designed to enable evaluation of cervico-ocular reactions during isolated trunk excursion and possible effects of aging. METHODS: The previously used head excursion test (HET) was statistically compared to the TET. In both methods, 100 healthy subjects of two age groups (group(26): median age = 26 years, n = 50; group(50): median age = 50 years, n = 50) were randomized for comparison of similar neck-to-trunk-positions. RESULTS: HET enabled detection of significant nystagmus modulation in horizontal and vertical dimensions; whereas in pure cervical stimulation using the new TET, this was only evident in the horizontal dimension and only during trunk torsion. Comparison of the two methods confirmed significantly stronger nystagmus modulation through head excursion. In terms of the HET, group(50) showed significantly more vertical nystagmus activity than group(26). However, no significant difference was found between the groups in terms of their reactions to trunk excursion in the TET. In a group-specific comparison of the methods, group(26) showed a significant increase in horizontal nystagmus in head compared to trunk excursion, whereas group(50) generally displayed a significantly greater response to provocation by head excursion in HET. Analysis of the significant vertical nystagmus modulation produced with the TET method showed predominance of upbeat- (UBN) over downbeat-nystagmus (DBN). Through head excursion with the HET, DBN was more frequently evoked in group(50) than in group(26). No significant age-dependent difference could be derived in UBN. CONCLUSION: The results of the pilot study indicate that head-to-trunk provocation is a suitable means of evaluating cervicotonic provocation nystagmus. Only by evaluation of adequate excursion limits and consistent analysis of patients with cervical deficiency can the effects of the method be further assessed.

The intercalatus nucleus of Staderini.

Rutilio Staderini was one of the leading Italian anatomists of the twentieth century, together with some scientists, such as Giulio Chiarugi, Giovanni Vitali, and others. He was also a member of a new generation of anatomists. They had continued the tradition of the most famous Italian scientists, which started from the Renaissance up until the nineteenth century. Although he carried out important studies of neuroanatomy and comparative anatomy, as well as embryology, his name is rarely remembered by most medical historians. His name is linked to the nucleus he discovered: the Staderini nucleus or intercalated nucleus, a collection of nerve cells in the medulla oblongata located lateral to the hypoglossal nucleus. This article focuses on the biography of the neuroanatomist as well as the nucleus that carries his name and his other research, especially on comparative anatomy and embryology.

Benign intermittent upbeat nystagmus in infancy: a new clinical entity.

BACKGROUND: Upbeat nystagmus in childhood is a rare phenomenon, which is classically associated with neurological or anterior visual pathway dysfunction. METHODS: We report a series of five infants with an intermittent upbeat nystagmus, without any other ocular or neurological abnormality, having spontaneously resolved before the age of 14 months. RESULTS: Nystagmus beating frequency was between 1.5 and 2.5 Hz for all patients, amplitude was remarkably large due to a baseline position in downgaze. The nystagmus mostly occurred in supine position and could be triggered by head rotations in the supine position. Age of onset was from the first weeks of life to two months; age of resolution was 5-14 months. All infants underwent complete clinical examination with ophthalmological, oto-neurological and neurological assessments and MRI-scan. CONCLUSIONS: Clinical characteristics of the nystagmus suggest an involvement of the otolithic system, with a secondary recalibration of the vestibulo-ocular pathways; however, no obvious vestibular abnormality was found. The pathophysiology of this nystagmus therefore remains unclear.

Primary Position Upbeat Nystagmus during an Acute Attack of Multiple Sclerosis.

BACKGROUND AND PURPOSE: Ocular manifestation is one of the frequent signs of an acute attack in multiple sclerosis (MS), although primary position upbeat nystagmus (PPUN) is rare. The purpose of this study is to determine the incidence of PPUN in MS and to determine the lesions that are responsible for this sign. METHODS: The medical records of 120 MS patients with acute brain lesions were reviewed over a consecutive period of 9 years; of these, 6 patients were found to have PPUN. Other ocular motor abnormalities were analyzed in combination with upbeat nystagmus, video-oculographic findings, and lesions detected on brain MRI. RESULTS: Lesions in the pontine tegmentum involving the medial longitudinal fasciculus (MLF) and ventral tegmental tract (VTT) were the most common, being observed in three of the six patients with PPUN. One patient exhibited caudal medullary lesions bilaterally affecting the paramedian portion of the posterior tegmentum, and two patients exhibited multiple lesions involving the pons with the cerebral peduncle or medulla. In five patients, other ocular motor dysfunctions, such as gaze-evoked nystagmus (n=3) and internuclear ophthalmoplegia (n=1), were found in combination with upbeat nystagmus. CONCLUSIONS: PPUN is an infrequent, ocular manifestation noted during an acute attack of MS, and was observed in 5% of the present cases. Brainstem lesions in these cases primarily involved the pontine tegmentum and the caudal medulla. These findings support the theory that upbeat nystagmus is attributable to damage to the upward vestibulo-ocular reflex pathway related to the vestibular nucleus, VTT, and interconnecting pathways.

Primary Position Upbeat Nystagmus during an Acute Attack of Multiple Sclerosis

BACKGROUND AND PURPOSE: Ocular manifestation is one of the frequent signs of an acute attack in multiple sclerosis (MS), although primary position upbeat nystagmus (PPUN) is rare. The purpose of this study is to determine the incidence of PPUN in MS and to determine the lesions that are responsible for this sign. METHODS: The medical records of 120 MS patients with acute brain lesions were reviewed over a consecutive period of 9 years; of these, 6 patients were found to have PPUN. Other ocular motor abnormalities were analyzed in combination with upbeat nystagmus, video-oculographic findings, and lesions detected on brain MRI. RESULTS: Lesions in the pontine tegmentum involving the medial longitudinal fasciculus (MLF) and ventral tegmental tract (VTT) were the most common, being observed in three of the six patients with PPUN. One patient exhibited caudal medullary lesions bilaterally affecting the paramedian portion of the posterior tegmentum, and two patients exhibited multiple lesions involving the pons with the cerebral peduncle or medulla. In five patients, other ocular motor dysfunctions, such as gaze-evoked nystagmus (n=3) and internuclear ophthalmoplegia (n=1), were found in combination with upbeat nystagmus. CONCLUSIONS: PPUN is an infrequent, ocular manifestation noted during an acute attack of MS, and was observed in 5% of the present cases. Brainstem lesions in these cases primarily involved the pontine tegmentum and the caudal medulla. These findings support the theory that upbeat nystagmus is attributable to damage to the upward vestibulo-ocular reflex pathway related to the vestibular nucleus, VTT, and interconnecting pathways.