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51 CANVAS (Cerebellar Ataxia, Neuropathy, and Vestibular Areflexia Syndrome) Video Head Impulse Test (vHIT) FigureCANVAS (Cerebellar Ataxia, Neuropathy, and Vestibular Areflexia Syndrome) is a genetic condition consisting of slowly progressive late-onset ataxia, bilateral vestibulopathy, sensory neuropathy, chronic cough, and autonomic dysfunction. While the term vestibular areflexia typically implies bilateral...Image
52 Caloric Testing𝗢𝗿𝗶𝗴𝗶𝗻𝗮𝗹 𝗗𝗲𝘀𝗰𝗿𝗶𝗽𝘁𝗶𝗼𝗻: Caloric testing is a peripheral vestibular test which takes advantage of the fact that the labyrinth is sensitive to temperature changes. Warm stimulation causes excitation of the semicircular canals while cold stimulatio...Image/MovingImage
53 Cavernous Sinus Mass Causing Right 3rd and 4th Nerve Palsies𝗢𝗿𝗶𝗴𝗶𝗻𝗮𝗹 𝗗𝗲𝘀𝗰𝗿𝗶𝗽𝘁𝗶𝗼𝗻: 25-yo-man who complained of diplopia and was initially found to have right 4th and 6th nerve palsies in the setting of a right cavernous sinus mass (subsequently diagnosed as Ewing's sarcoma). When seen in follow-up (this...Image/MovingImage
54 Central (Nuclear) 3rd Nerve PalsiesShown here are two patients with left sided midbrain pathology (hemorrhage and ischemia) which caused damage to the 3rd nucleus. Both of the patients have ipsilateral mydriasis, adduction, supra- and infraduction paresis. Ipsilateral>contralateral ptosis is also present, and localizes to the central...Image/MovingImage
55 Central 4th Nerve Palsy with Contralateral Horner's SyndromeThis is a 60-yo-woman who presented with a complaint of diplopia. Examination demonstrated a left hypertropia that worsened in right and down gaze as well as in left head tilt, and a left 4th nerve palsy was diagnosed. There was also evidence of a mild motility deficit in down/medial gaze OS, consis...Image/MovingImage
56 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
57 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
58 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
59 Central HINTS (With an Abnormal Head Impulse Sign) in the Acute Vestibular Syndrome Due to Lateral Pontine/Middle Cerebellar Peduncle Demyelination𝗢𝗿𝗶𝗴𝗶𝗻𝗮𝗹 𝗗𝗲𝘀𝗰𝗿𝗶𝗽𝘁𝗶𝗼𝗻: This is a 30-year-old man presenting with vertigo, diplopia and mild left facial weakness (not seen in the video). On exam, there was right-beating nystagmus (RBN) in primary gaze that increased in right gaze (in accordan...Image/MovingImage
60 Central Positional Vertigo and Nystagmus in a Posterior Fossa Tumor𝗢𝗿𝗶𝗴𝗶𝗻𝗮𝗹 𝗗𝗲𝘀𝗰𝗿𝗶𝗽𝘁𝗶𝗼𝗻: This is a 30-year old woman who presented with positional vertigo and vomiting following a concussion related to a car accident 3 months prior. She was initially diagnosed with posterior canal (PC) benign paroxysmal posit...Image/MovingImage
61 Central Vestibular Nystagmus in Anti-DPPX EncephalitisThis is a young woman who presented with oscillopsia due to spontaneous nystagmus in addition to gastrointestinal symptoms which led to the diagnosis of anti-DPP axis encephalitis. She was treated with rituximab, and experience gradual improvement over time. However, years after the onset, she con...Image/MovingImage
62 Centripetal Nystagmus ExampleA 68-year-old female reported a 2-year history of progressive gait imbalance, falls, dizziness and vertical oscillopsia. She described that dizziness and oscillopsia were worst when looking down. There was no family history of ataxia. Composite gaze with fixation was recorded with video-oculography ...Image/MovingImage
63 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
64 Cerebellar Degeneration with Downbeat Nystagmus Provoked by ConvergenceDescription: This is a 70-yo-woman with a progressive gait disorder, diagnosed with cerebellar ataxia. She displayed typical cerebellar ocular motor signs including gaze-evoked nystagmus, choppy pursuit and VOR suppression, and there was very subtle spontaneous downbeat nystagmus, best appreciated w...Image/MovingImage
65 Cerebellar Eye Signs in SCA8𝗢𝗿𝗶𝗴𝗶𝗻𝗮𝗹 𝗗𝗲𝘀𝗰𝗿𝗶𝗽𝘁𝗶𝗼𝗻: This is a 30-yo-man with a diagnosis of SCA 8 who had appendicular and gait ataxia in addition to choppy smooth pursuit and VORS, downbeat nystagmus, saccadic hypermetria, and gaze-evoked nystagmus with rebound nystagmus....Image/MovingImage
66 Change in Pendular Nystagmus from Oculopalatal Tremor over a Four-year PeriodThis is a patient who developed oculopalatal tremor months following a pontine hemorrhage. Although it is not shown here, she also has palatal tremor. In the first video which was taken 1 year after her hemorrhage, a vertical-torsional pendular nystagmus can be seen, that is mildly dissociated giv...Image/MovingImage
67 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
68 Chronic Facial Nerve Palsy with Aberrant RegenerationThis is a 35-year-old woman who was diagnosed with right sided Bell's palsy six months prior. Contrast-enhanced MRI at that time was normal. This video demonstrates the phenomenon of aberrant regeneration (synkinesia) of the facial nerve. Due to aberrant regeneration, at rest the right palpebral fi...Image/MovingImage
69 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
70 Common Neuro-Ophthalmic Ancillary Tests to Assist in the Diagnosis and Localization of Afferent DisordersChart of the common neuro-ophthalmic ancillary tests to assist in the diagnosis and localization of afferent disorders.Text
71 Complete Microvascular 6th Nerve Palsy with Slow Abducting Saccade𝗢𝗿𝗶𝗴𝗶𝗻𝗮𝗹 𝗗𝗲𝘀𝗰𝗿𝗶𝗽𝘁𝗶𝗼𝗻: This is a 90-year-man with HTN, HLD, DM who woke up with horizontal diplopia. Two years prior, he was diagnosed with a microvascular right 6th nerve palsy that resolved over several months. There was little concern for gi...Image/MovingImage
72 Complete Peripheral Vestibulopathy & Ipsilateral Facial Palsy𝗢𝗿𝗶𝗴𝗶𝗻𝗮𝗹 𝗗𝗲𝘀𝗰𝗿𝗶𝗽𝘁𝗶𝗼𝗻: 60-yo-man who suffered the fairly abrupt onset (over hours) of right lower motor neuron facial nerve palsy (7th cranial nerve), vertigo and deafness in the right ear (8th cranial nerve). Vesicles were noted on otoscopy, a...Image/MovingImage
73 Complete Saccadic Palsy Due to Pulmonary Thrombectomy𝗢𝗿𝗶𝗴𝗶𝗻𝗮𝗹 𝗗𝗲𝘀𝗰𝗿𝗶𝗽𝘁𝗶𝗼𝗻: This is a 37-year-old woman who underwent pulmonary thrombectomy for a pulmonary embolus. Immediately following the procedure, she was unable to make normal eye movements. This video exam (she is the passenger in a car du...Image/MovingImage
74 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
75 ConvergenceConvergence: instruct the patient to focus on their thumb held at arm's length, and slowly move their thumb towards their nose. This may bring out or cause reversal of vertical nystagmus (e.g., transition from upbeat to downbeat nystagmus in Wernicke's encephalopathy [see example of transition from ...Image/MovingImage
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