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1 + HIT, + Skew, Unidirectional Nystagmus: Central Acute Vestibular Syndrome Due to Wallenberg SyndromeThis is a 45-year-old woman who presented to the ED with acute prolonged vertigo and vertical diplopia. She was seen as an outpatient 1 month after her ED visit, and double vision and balance were improving by that time. Her HINTS testing showed the following (seen in the video): 1) Head Impulse - A...Image/MovingImage
2 A Comparison of Nystagmus and Saccadic Intrusions/OscillationsNystagmus can be classified into pendular and jerk waveforms, where both are generated by a slow, pathologic phase. Corrective phase (the position reset mechanism) differs. In pendular nystagmus, the eyes move back and forth with about the same velocity and amplitude, similar to that of a pendulum...Image
3 Abnormal Visually-Enhanced VOR in Cerebellar Ataxia, Neuropathy, Vestibular Areflexia Syndrome (CANVAS)A 67 year old woman presented with 1 year of progressive numbness, gait instability, and oscillopsia when walking or with head movements. Examination showed excessive square-wave jerks, bilateral horizontal gaze-evoked nystagmus, impairment of the visually-enhanced vestibular ocular reflex (vVOR - s...Image/MovingImage
4 Acute Vestibular Syndrome with Ocular Tilt Reaction Due to Bacterial LabyrinthitisThis is a patient who initially presented with the acute vestibular syndrome (AVS, e.g., acute prolonged vertigo, spontaneous nystagmus) and right sided hearing loss, and was diagnosed with bacterial labyrinthritis. Her HINTS (Head Impulse, Nystagmus, Test of Skew) testing indicated a central etiolo...Image/MovingImage
5 Acute Vestibular Syndrome With Skew Deviation and Positive Head Impulse Test Due to a Demyelinating LesionThis is a patient who initially presented with the acute vestibular syndrome (AVS, e.g., acute prolonged vertigo, spontaneous nystagmus). ; See https://collections.lib.utah.edu/details?id=187730 for additional history. ; Her HINTS (Head Impulse, Nystagmus, Test of Skew) testing indicated a central e...Image/MovingImage
6 Anti-GAD Associated Cerebellopathy and Bilateral VestibulopathyThis is a 70-year-old woman with the subacute onset of severe imbalance and dizziness. On her initial examination, she had prominent gaze-evoked nystagmus and bilateral vestibular loss. Smooth pursuit was saccadic, although her vestibulo-ocular reflex (VOR) suppression was much smoother. Usually pur...Image/MovingImage
7 Atypical Ocular Motor Features (Gaze-evoked Nystagmus) in PSPThis is a 70-yo-woman who met clinical and radiologic diagnostic criteria for progressive supranuclear palsy (PSP). Typical ocular motor features of PSP include square wave jerks, hypometric saccades, choppy pursuit/VORS, impaired down>upgaze (supranuclear in origin) and impaired down>upward saccade...Image/MovingImage
8 Bilateral 6th Nerve Palsies Due to Idiopathic Intracranial HypertensionThis is a 25-year-old woman who presented with diplopia and blurry vision. On exam, she was found to have papilledema and bilateral 6th nerve palsies. Her opening pressure was >40 cm of water with a normal CSF analysis, and neuroimaging was unremarkable aside from subtle findings that have been asso...Image/MovingImage
9 Bilateral INOs and Partial 3rd Nerve PalsiesThis is a 45-year-old man with progressive ptosis and ophthalmoparesis. 10 years prior to presentation, he experienced diplopia and had a hyperintense lesion involving the medial longitudinal fasciculus (MLF) per report. Over time, he developed bilateral adduction paresis, ptosis and upgaze paresis ...Image/MovingImage
10 Brainstem Ocular Motor MachinerySeen here is a sagittal view of the brainstem. The medulla has a significant role in gaze-holding, and the nucleus prepositus hypoglossi (NPH, along with the medial vestibular nucleus ) is the horizontal neural integrator. The abducens (6th) nucleus is located in the dorsal pons, and sends off the 6...Image/MovingImage
11 Bruns Nystagmus Due to a Cerebellopontine Angle Tumor𝗢𝗿𝗶𝗴𝗶𝗻𝗮𝗹 𝗗𝗲𝘀𝗰𝗿𝗶𝗽𝘁𝗶𝗼𝗻: This is a 15-yo-girl who experienced headache and imbalance leading to an MRI which showed a left sided cerebellopontine angle (CPA) tumor. Because of involvement of the left brainstem/cerebellum (e.g., dysfunction of the...Image/MovingImage
12 Central Acute Vestibular Syndrome Due to Posterior Fossa HemorrhageThis is a patient presenting with the acute vestibular syndrome (AVS, e.g., acute prolonged vertigo, spontaneous nystagmus) whose HINTS (Head Impulse, Nystagmus, Test of Skew) testing indicated a central etiology based on negative (normal) head impulse testing (HIT). Nystagmus was unidirectional and...Image/MovingImage
13 Central Anatomy of the Fourth NerveThe IVth or trochlear nucleus is located ventral to the central periaqueductal grey matter, dorsal to the medial longitudinal fasciculus (MLF) and medial to the oculosympathetic tract at the level of the inferior colliculus. The fascicles of the IVth nerve travel dorsally and caudally around the cen...Image
14 Central Anatomy of the Third NerveSeen here is an axial section of the midbrain at the level of the superior colliculus. The paired nuclei are located ventral to the periaqueductal grey, and the midline central caudal nucleus (CCN) is located between the right and left nuclei. The CCN sends projections to bilateral levator palpebrae...Image
15 Cerebellar Ataxia, Neuropathy, & Vestibular Areflexia Syndrome (CANVAS): Impaired Visually-Enhanced VOR and Abnormal Head Impulse TestingA 67 year old woman presented with 1 year of progressive numbness, gait instability, and oscillopsia when walking or with head movements. Examination showed excessive square-wave jerks, bilateral horizontal gaze-evoked nystagmus, impairment of the visually-enhanced vestibular ocular reflex (vVOR - s...Image/MovingImage
16 Chiari Malformation Causing Downbeat Nystagmus in Lateral GazeThis is a 20-yo-man who presented with oscillopsia in lateral gaze from downbeat nystagmus (DBN). In primary gaze, very subtle DBN was only noted with ophthalmoscopy, but in lateral gaze, prominent DBN was present. Other central ocular motor signs included gaze-evoked nystagmus (GEN) vertically, in ...Image/MovingImage
17 Chronic Progressive External Ophthalmoplegia (CPEO) and Cerebellar SignsThis is a 60-yo-woman who initially presented with imbalance and ophthalmoparesis. Initially, there was mild horizontal gaze limitation with mild gaze-evoked nystagmus and slow saccades, and over the years, gait ataxia and dysarthria (mainly a scanning quality to her speech) developed, and her ophth...Image/MovingImage
18 Congenital Nystagmus𝗢𝗿𝗶𝗴𝗶𝗻𝗮𝗹 𝗗𝗲𝘀𝗰𝗿𝗶𝗽𝘁𝗶𝗼𝗻: Presented here are two patients with congenital nystagmus demonstrating characteristic features including: mixed pendular and jerk nystagmus (usually gaze-evoked) waveforms, stays horizontal even in vertical gaze, suppres...Image/MovingImage
19 Convergence Insufficiency and Square Wave Jerks in PSP𝗢𝗿𝗶𝗴𝗶𝗻𝗮𝗹 𝗗𝗲𝘀𝗰𝗿𝗶𝗽𝘁𝗶𝗼𝗻: This is a 70-yo-woman with progressive supranuclear palsy with complaints of difficulty reading. Her husband noticed that she would frequently close one eye when attempting to read, and words were not clear on the page, a...Image/MovingImage
20 Coronal Section of the Brainstem Showing Ocular Motor Nuclei and Anatomy of the Vestibular Nucleus (with SCC Inputs)(A) Seen here is a coronal view of the brainstem showing the locations of the ocular motor nuclei (IIIrd, IVth, VIth) as well as the nuclei of VII and VIII (vestibular and cochlear). The vestibular nucleus (VN) is divided into the inferior, lateral, medial, and superior subnuclei, and the medial ves...Image
21 Demonstration of HINTS Examination in a Normal SubjectIn the acute vestibular syndrome - consisting of acute prolonged vertigo, spontaneous nystagmus, imbalance, nausea/vomiting, head motion intolerance which is typically due to vestibular neuritis or posterior fossa stroke - a 3 step test of ocular motor and vestibular function known as HINTS, has hig...Image/MovingImage
22 The Episodic Vestibular SyndromeThis is a 55-year-old man with 6 months of episodic vertigo without clear triggers/provocative factors, with each of his 3 previous episodes lasting less than 5 minutes. While in the clinic, he had one of his typical vertigo attacks. There was initially 30 seconds of right-beating-torsional nystagmu...Image/MovingImage
23 Examples of Patients with Saccadic Intrusions (Square Wave Jerks)Seen here are patients with saccadic intrusions that do have an intersaccadic interval. Square wave jerks are commonly seen in degenerative conditions, mainly involving the posterior fossa (e.g., cerebellar degeneration) and basal ganglia (e.g., progressive supranuclear palsy).Image/MovingImage
24 Eye Closure and Oculopalatal Tremor𝗢𝗿𝗶𝗴𝗶𝗻𝗮𝗹 𝗗𝗲𝘀𝗰𝗿𝗶𝗽𝘁𝗶𝗼𝗻: This patient suffered a traumatic brain injury with brainstem injury resulting in damage to Mollaret's triangle and palatal tremor. Inferior olivary hypertrophy was noted on her MRI, although no vertical and/or torsional ...Image/MovingImage
25 Eyelid AnatomySeen here are the major muscles of eyelid opening and closure. The levator palpebrae, which is innervated by the oculomotor nerve, inserts on the tarsus via the levator aponeurosis and directly on the skin of the upper eyelid. The superior tarsal muscle (also known as Muller's muscle, which is inner...Image
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