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1 Crowded Disc - FamilyWilliam F. Hoyt, PhDLeft eye. PP3 a & b: sister; PP4 a&b: brother; Congenital disc margin blurring with crowded discs. Excellent example of pseudo papilledema. Pathology: Normal variation of the optic disc. Disease/Diagnosis: Normal variation of the optic disc. Crowded disc. Clinical notes: Appearance due to too many f...Pseudopapilledema; Congenital Blurred Disc
2 Optic Atrophy (PowerPoint)William F. Hoyt, PhDa) Evolution of optic disc pallor after optic nerve transection. Normal Right eye. Photo taken December 9, 1978. b) Injury on December 8, 1978. Evolution of optic disc pallor after optic nerve transection. Woman having rhinoplasty suffered optic nerve transection. One day after nerve transection. N...Optic Disc Atrophy from Retrobulbar Causes (Retrograde Optic Nerve Degeneration); Severe Atrophy; Optic Atrophic
3 Temporal Atrophy (PowerPoint)William F. Hoyt, PhDSegmental Atrophy - Temporal pallor - Nutritional amblyopia (alcoholic). 1985.Temporal Atrophy; Optic Disc Atrophy from Retrobulbar Causes (Retrograde Optic Nerve Degeneration); Severe Atrophy; Alcohol
4 Carotid Cavernous Fistulas (PowerPoint)AAO/NANOS - American Academy of Ophthalmology / North American Neuro-Ophthalmology SocietyThis 76-year-old woman has a 7-month history of redness and pressure sensation in both eyes that is worse in the morning. She has noted intermittent horizontal diplopia during this time. Angiography demonstrated a right dural cavernous sinus fistula, which was successfully occluded with direct injec...Dural Arteriovenous Malformation; Carotid Cavernous Fistulas
5 Central/Branch Retinal Artery Occlusion (PowerPoint)AAO/NANOS - American Academy of Ophthalmology / North American Neuro-Ophthalmology SocietyOcclusion of a branch or central retinal artery may result in acute visual loss. The ophthalmoscopic findings are retinal whitening due to ischemic retina in the distribution of the occluded artery. Sparing or selective involvement of cilioretinal artery branches may occur. Patients with a central r...Central/Branch Retinal Artery Occlusion
6 Chest CT Thymoma (Guest Lecture)Robert A. Novelline, MDThe patient is a 46 year old woman who presented in July 1977 with horizontal double vision lasting two weeks. Three weeks later the left upper eyelid started to droop and by the end of the day the eye was closed. She had no ptosis of the right eye and no generalized fatigue. She consulted an intern...Unilateral Ptosis; Unilateral Lid Retraction; Myasthenic Lid Twitch; External Ophthalmoplegia; Ocular Myasthenia Gravis; Tensilon Test; Thymolipoma; Generalized Myasthenia Gravis; Unilateral Myasthenia Gravis; Myasthenic Ptosis; Lid Retraction; Lid Twitch
7 Chiasmal Herniation (PowerPoint)AAO/NANOS - American Academy of Ophthalmology / North American Neuro-Ophthalmology SocietyThis woman was 61 years old when she underwent initial neuro-ophthalmologic evaluation. Twenty-three years earlier, she had undergone removal of a pituitary adenoma followed by radiation therapy. At that time, she had noted a preoperative visual field defect that improved somewhat after the surgery....Chiasmal Herniation
8 Chiari I MalformationShirley H. Wray, MD, PhD, FRCPSee also: http://content.lib.utah.edu/cdm/ref/collection/ehsl-shw/id/295, http://content.lib.utah.edu/cdm/ref/collection/ehsl-shw/id/323, and http://content.lib.utah.edu/cdm/ref/collection/ehsl-shw/id/61Downbeat Nystagmus; Oscillopsia; Chiari-1 Malformation; Primary Position Downbeat Nystagmus; Vertical Saccadic Dysmetria; Horizontal Saccadic Dysmetria; Ataxia; Dysmetria; Chiari Malformation
9 Paraneoplastic Disease (PowerPoint)AAO/NANOS - American Academy of Ophthalmology / North American Neuro-Ophthalmology SocietyThis 46-year-old woman noted progressive bilateral visual loss over a 10-month period. She had a malignant melanoma removed from her right thigh 2 years ago and excisional biopsy of an inguinal node metastasis 8 months ago. She also complained of poor night vision and rare intermittent ""sparkles"" ...Paraneoplastic Disease
10 Leber's Optic Neuropathy (PowerPoint)AAO/NANOS - American Academy of Ophthalmology / North American Neuro-Ophthalmology SocietySeveral Primary Mutations result in Leber's hereditary optic neuropathy, including mitochondrial deletions at positions 11778, 3460, and 14484. Although the 11778 is the most common mutation, the 11484 has the best prognosis for spontaneous recovery. This case exhibits the 3460 mutation.Leber's Optic Neuropathy
11 Evolution of Optociliary Veins with Perioptic Nerve SheathWilliam F. Hoyt, PhDSeries of images showing progression of disc swelling and macular degeneration. Pathology: Optociliary Vein. Disease/Diagnosis: Perioptic nerve sheath meningioma evolution. Clinical notes: Visual Loss.Optic Disc Atrophy with Special Features; Optociliary Veins; Shunt Vessels (Meningioma)
12 Exposed Drusen (PowerPoint)William F. Hoyt, PhDPP25a: Left eye: Severe visual field defect. PP25b: right eye with exposed drusen and field loss: visual field defects; PP25c: right eye visual field PP25d: left eye visual field.Pseudopapilledema; Exposed Drusen
13 AneurysmsAAO/NANOS - American Academy of Ophthalmology / North American Neuro-Ophthalmology SocietyAneurisms may result in neuro-ophthalmologic sign and symptoms by direct compression of the afferent or efferent systems or by the secondary effects of hemorrhage. Basilar aneurisms may result in ocular motor deficits such as a unilateral or bilateral third nerve palsy.Aneurysm
14 Eyebrow Spasm (Guest Lecture)Shirley H. Wray, MD, PhD, FRCPThis case is published courtesy of Daniel J. Costello, M.D., Department of Neurology, Massachusetts General Hospital, Boston. The patient is a 32-year-old right-handed man with an established diagnosis of Tuberous Sclerosis Complex characterized by: -medically intractable epilepsy -developmental del...Rhythmic Eyebrow Spasm; Torsional Nystagmus; Primary Position Left Beating Nystagmus; Epileptic Seizures; Tuberous Sclerosis Complex (TSC-2 DNA sequence variant); Bipolar Affective Disorder
15 Brain MRI in Multiple Sclerosis (Guest Lecture)Anne G. Osborn, MDThe patient is a 25 year old woman who was in excellent health until 4 days prior to admission when she noted blurred vision and horizontal double vision on lateral gaze to right and left. Past History: Negative for strabismus as a child. No previous episodes of transient neurological symptoms. Fami...Bilateral Internuclear Ophthalmoplegia; Abducting Nystagmus; Normal Convergence; Gaze Evoked Upbeat Nystagmus; Gaze Evoked Downbeat Nystagmus; Saccadic Dysmetria; Multiple Sclerosis; Horizontal Saccadic Dysmetria
16 Blepharospasm Round-Up (Guest Lecture)Shirley H. Wray, MD, PhD, FRCPThe patient is a 60 year old estate manager with a history of retinal laser therapy, dry eyes and age related bilateral ptosis. He carries a diagnosis of hilar lymphadenopathy due to sarcoid and has had cancer of the kidney. He presented in 1995 with a 6 month history of frequent blinking and spasms...Benign Essential Blepharospasm; Focal Dystonia; Blepharospasm
17 Pendular Vertical OscillationsShirley H. Wray, MD, PhD, FRCPSee also: http://content.lib.utah.edu/cdm/ref/collection/ehsl-shw/id/68, http://content.lib.utah.edu/cdm/ref/collection/ehsl-shw/id/247, http://content.lib.utah.edu/cdm/ref/collection/ehsl-shw/id/111, http://content.lib.utah.edu/cdm/ref/collection/ehsl-shw/id/312, http://content.lib.utah.edu/cdm/ref...Palatal Tremor (Myoclonus); Pendular Vertical Oscillations; Unilateral Horizontal Gaze Palsy; Facial Palsy; Pontine Infarct; Degenerative Hypertrophy of the Inferior Olivary Nucleus; Lesion in the Guillain-Mollaret Triangle; Oculopalatal Myoclonus; Oculopalatal Tremor Lid Nystagmus; Bilater...
18 Paraneoplastic Upbeat NystagmusShirley H. Wray, MD, PhD, FRCPThis case was presented to the Clinical Eye Movement Society at the American Neurological Association Meeting in October 2009. The patient is a 65 year old woman who was in good health until seven weeks prior to admission. On June 22/09 on the return flight from her daughter's wedding in Oregon she ...Upbeat Nystagmus; Lid Nystagmus; Square Wave Jerks (Saccadic Oscillations); Saccadic Dysmetria; Saccadic Pursuit; Paraneoplastic Syndrome; Paraneoplastic Upbeat Nystagmus; Pancreatic Endocrine Carcinoma; Anti-Hu - Associated Paraneoplastic Encephalitis
19 Third Nerve PalsyShirley H. Wray, MD, PhD, FRCPThe patient is a 50 year old man with Type II Diabetes who presented to an outside hospital in September 1996 with ptosis and an inability to open the right eye. Five months prior to admission (PTA), he had flu-like symptoms with mild fever, sinus congestion and diffuse myalgia in the chest, abdomen...Ptosis; Unilateral Third Nerve Palsy; Oculomotor Nerve; Meningovascular Syphilis; Heubner's Arteritis; Unilateral Oculomotor Third Nerve Palsy; Third Nerve Microinfarct; Fascicular Third Nerve Palsy
20 Familial Amyotrophic Lateral SclerosisShirley H. Wray, MD, PhD, FRCPThis 58 year old woman was referred to Dr. Robert Brown in March 1995 for evaluation of slurred speech. She remained under his care until her death. On examination she had signs of a pseudobulbar palsy: Dysarthria and dysphagia Diminished palatal movement with positive gag bilaterally Diminished rap...Supranuclear Paralysis of Up and Downgaze Degeneration; Convergence Insufficiency; Slow Hypometric Horizontal Saccades;; Saccadic Breakdown of Horizontal Pursuit; Bulbar Palsy; Familial Amyotrophic Lateral Sclerosis; Lou Gehrig's Disease; CNS -Degeneration; Superoxide Dismutase (SOD1) Gene
21 One and a Half SyndromeShirley H. Wray, MD, PhD, FRCPThis 44 year old woman presented in 1973 with an acute attack of optic neuritis in the right eye that fully recovered after a course of ACTH therapy. In 1991, 18 years later, she developed unsteadiness of gait, "walking like a chicken", stiff legs that jerked spontaneously in bed at night, and numbn...Unilateral Internuclear Ophthalmoplegia; Unilateral Horizontal Gaze Palsy; Upbeat Nystagmus on Upgaze; Convergence Normal; Fisher's One-and-a-Half Syndrome; Uhthoff's Symptom; Multiple Sclerosis; Unilateral Horizontal Gaze Palsy Demyelination; Gaze Evoked Upbeat Nystagmus; Abducting Nystagmus
22 Hemifacial SpasmShirley H. Wray, MD, PhD, FRCPThe patient is a 72 year old man with myopia, childhood exotropia, progressive age related ptosis and right hemifacial spasm. Hemifacial spasm (HS) most often begins insidiously in the orbicularis oculi muscle in the early stages, as in this man. He presented with a 2 year history of involuntary twi...Lid Twitch; Hemifacial Spasm; Neurovascular Compression Syndrome of the Facial Nerve
23 Pendular OscillationsShirley H. Wray, MD, PhD, FRCPThis 37 year old woman has had progressive multiple sclerosis (MS) affecting the cerebellum and brainstem for 6 years. Neurological examination: Titubation Dysarthria Incoordination of the extremities Ataxic gait Spastic paraparesis with generalized hyperreflexia and extensor plantar responses. Neur...Pendular Horizontal Oscillations; Left Beating Nystagmus; Obtrusive Saccades; Bilateral Gaze Evoked Nystagmus; Saccadic Dysmetria; Oscillopsia; Titubation; Ataxia; Multiple Sclerosis; Primary Position Horizontal Nystagmus; Gaze Evoked Horizontal Nystagmus; Horizontal Saccadic Dysmetria
24 LateropulsionShirley H. Wray, MD, PhD, FRCPThis 60 year old patient has Wallenberg's syndrome due to infarction of the left dorsolateral medulla. Wallenberg's syndrome is the best recognized syndrome involving the vestibular nuclei and adjacent structures. Unilateral infarcts affecting the vestibular nuclei may produce an oculomotor imbalanc...Deviation of the Eyes Under Closed Lids; Lateropulsion; Dorsolateral Medullary Infarction; Medulla Infarct
25 Alcoholic Cerebellar DegenerationShirley H. Wray, MD, PhD, FRCPThe patient is a 72 year old woman who presented with a 4 year history of progressive difficulty with balance, frequent falls and unsteadiness walking. She required a cane to steady herself. Past History: Significant for alcohol abuse. In 1980, she came to Boston for a second opinion and was seen in...Square Wave Jerks; Dysmetria; Horizontal Saccadic Hypermetria; Horizontal Gaze Evoked Nystagmus; Saccadic Pursuit; Gait Ataxia; Alcoholic Cerebellar Degeneration; Gaze Evoked Horizontal Nystagmus; Horizontal Saccadic Dysmetria; Alcohol
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