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TitleCreatorDescriptionSubject
876 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
877 Mitochondrial MyopathyShirley H. Wray, MD, PhD, FRCPA PowerPoint slideshow describing the condition.Bilateral Progressive External Ophthalmoplegia (PEO); Bilateral Ptosis; Cerebellar Degeneration with Ataxia; Complete External Ophthalmoplegia; External Ophthalmoplegia Affecting Upgaze; Facial Weakness; Kearns-Sayre Syndrome; Mitochondrial Myopathy; Normal Pupils; PEO plus Atypical Retinitis Pigm...
878 Transient Monocular Blindness (Guest Lecture)Shirley H. Wray, MD, PhD, FRCPSee also: http://content.lib.utah.edu/cdm/ref/collection/ehsl-shw/id/293 and http://content.lib.utah.edu/cdm/ref/collection/ehsl-shw/id/96Retinal Emboli; Transient Monocular Blindness; Ocular Stroke; Transient; Transient Visual Loss; Sunlight Provoked Transient Monocular Blindness; Low Pressure Retinopathy; Ipsilateral Internal and External Carotid Occlusive Disease; Ischemic Eye Syndrome; Retinal Stroke; Ocular Ischemic Sy...
879 Congenital Horizontal Gaze Palsy, Progressive ScoliosisShirley H. Wray, MD, PhD, FRCPThe patient is an 8 year old boy with a rare autosomal recessive disorder characterized by congenital absence of conjugate horizontal eye movements preservation of vertical gaze and convergence and progressive scoliosis (HGPPS) developing in childhood. The child was referred to Dr. Cogan with a diag...Congenital Horizontal Gaze Palsy; Progressive Scoliosis; Mutation of the ROBO 3 Gene on Chromosome 11q23-q25; Congenital Cranial Disinnervation Syndrome; Mobius Syndrome
880 Convergence InsufficiencyShirley H. Wray, MD, PhD, FRCPThe patient is a 73 year old man with a ten year history of idiopathic Parkinson's disease characterized by difficulty in walking, generalized rigidity and a mild tremor of his hands at rest with deterioration in his handwriting. He denied any memory impairment or loss of cognitive function. He was ...Basal Ganglia; Blepharoclonus; Convergence Insufficiency; Slow Hypometric Saccades; Saccadic Breakdown of Smooth Pursuit; Parkinson's Disease- Dopamine deficiency; Slow Hypometric Horizontal Saccades; Convergence
881 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
882 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
883 Cavernous Sinus MeningiomaShirley H. Wray, MD, PhD, FRCPThis patient is a 46 year old woman from Portugal who was admitted to the Massachusetts General Hospital in September 1986 with ophthalmoplegia of the left eye (OS) and signs of aberrant reinnervation of the third nerve. She presented, in August 1985, with an episode of diplopia. The diplopia was su...Ptosis; Unilateral Third Nerve Palsy; Aberrant Reinnervation of the Third Nerve; Paresis of Abduction; Sixth Nerve Palsy; Oculomotor Nerve; Cavernous Sinus Meningioma; Cavernous Sinus Syndrome; Unilateral Oculomotor Third Nerve Palsy; Unilateral Sixth Nerve Palsy
884 CNS LymphomaShirley H. Wray, MD, PhD, FRCPThe patient is a 70 year old Italian man with atrial fibrillation on long-term coumadin therapy. In October 1995, he developed generalized headache, horizontal double vision and his left eye deviated inwards (esotropia). A diagnosis of left sixth nerve palsy was made and attributed to microvascular ...Bilateral Sixth Nerve Palsy; Bilateral Esotropia; CNS Non-Hodgkin's Lymphoma; Cavernous Sinus Syndrome; Lymphoma
885 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
886 Bilateral Internuclear OphthalmoplegiaShirley H. Wray, MD, PhD, FRCPThis patient was seen at the Yale Eye Center at the age of 37. She had a long history of multiple sclerosis. At age 22, she had an acute attack of optic neuritis in the left eye which recovered fully within three weeks. Some months later she had a recurrent episode in the same eye, which also recove...Bilateral Internuclear Ophthalmoplegia; Pendular Horizontal Oscillations; Lid Nystagmus; Upbeat Nystagmus; Botulinum Toxin Therapy; Multiple Sclerosis; Horizontal Pendular Nystagmus; Gaze Evoked Upbeat Nystagmus; Abducting Nystagmus
887 Internuclear Ophthalmoplegia in Childhood (Guest Lecture)Shirley H. Wray, MD, PhD, FRCPThis case was reported by Cogan DG and Wray SH. Internuclear ophthalmoplegia as an early sign of brain tumor. Neurology 1970; 20:629-633. The patient is Case 1. He is a 4 ½ year old boy whose parents noted that the right eye had been "drifting" for four months. On examination the only significant f...Bilateral Internuclear Ophthalmoplegia; Upbeat Nystagmus; Medulloblastoma; Abducting Nystagmus
888 Supranuclear Paralysis of Vertical GazeShirley H. Wray, MD, PhD, FRCPThis case was presented to the Clinical Eye Movement Society at the American Neurological Association Meeting in October 2007. The patient is a healthy, 36 year old Lieutenant Commander in the Coast Guard who was last seen perfectly well at 2 a.m. on the day of admission. He awoke in the morning aro...Somnolence; Global Supranuclear Paralysis of Vertical Gaze; Absent Vertical Saccades and Pursuit Movements; Intact Convergence; Convergence Retraction Nystagmus; Ocular Dysmetria; Ocular Tilt Reaction; Lateropulsion; Unilateral Midbrain and Thalamic Infarct; Supranuclear Paralysis of Up and Downgaze...
889 Supranuclear Paralysis of DowngazeShirley H. Wray, MD, PhD, FRCPJohn Trojanowski and I published this case in Neurology in 1980.(12) At that time, it was one of the first of five reports in the literature of the pathological localization of lesions in the midbrain that are responsible for selective supranuclear paralysis of downgaze. This case is of particular i...Somnolence; Supranuclear Paralysis of Downgaze; Vertical Oculocephalic Reflex Normal; Absent Convergence; Skew Deviation; Ocular Tilt Reaction; Artery of Percheron; Midbrain Infarct; Supranuclear Paralysis of Downgaze Infarct
890 Third Nerve PalsyShirley H. Wray, MD, PhD, FRCPSlideshow describing condition.Heubner's Arteritis; Meningovascular Syphilis; Oculomotor Nerve; Ptosis; Third Nerve Microinfarct; Unilateral Oculomotor Third Nerve Palsy; Unilateral Third Nerve Palsy
891 Whipples Disease (Guest Lecture)Shirley H. Wray, MD, PhD, FRCPThe patient is a 44 year old woman with a past history of ethanol abuse. She was transferred to the Massachusetts General Hospital (MGH) on 3/10/93 for evaluation of memory impairment, hypothalamic dysfunction and a one month history of diplopia and ataxia. In February 1992 (13 months PTA), she deve...Somnolence; Supranuclear Paralysis of Up and Downgaze; Vertical gaze palsy (c); Absent convergence; Pendular vergence oscillations; Bilateral lid nystagmus; Tropheryma Whippelii - Infection; CNS Whipple's Disease; Supranuclear Paralysis of Up and Downgaze Infection-Whipple's Disease
892 Progressive Supranuclear PalsyShirley H. Wray, MD, PhD, FRCPSee also: http://content.lib.utah.edu/cdm/ref/collection/ehsl-shw/id/316, http://content.lib.utah.edu/cdm/ref/collection/ehsl-shw/id/241, http://content.lib.utah.edu/cdm/ref/collection/ehsl-shw/id/32, http://content.lib.utah.edu/cdm/ref/collection/ehsl-shw/id/81, http://content.lib.utah.edu/cdm/ref/...Apraxia of Eyelid Opening; Impaired Initiation of Horizontal Saccades; Slow Hypometric Horizontal Saccades; Supranuclear Paralysis of Up and Downgaze Degeneration; Progressive Supranuclear Palsy - Tauopathy; Steele Richardson Olszewski Syndrome; CNS - Degeneration; Square wave Jerks; Supranu...
893 Claude's SyndromeShirley H. Wray, MD, PhD, FRCPThe patient is a 76 year old woman who woke one morning unable to see out of her right eye because of ptosis. She came to the Massachusetts General Hospital emergency room and was admitted. Neurological Examination: Right third nerve palsy involving the pupil Ataxia of the left hand on finger-nose t...Ptosis; Fascicular Third Nerve Palsy; Fixed Dilated Pupil; Contralateral Limb Ataxia; Claude Syndrome; Midbrain Infarct; Oculomotor Nerve; Unilateral Third Nerve Palsy; Subnuclear; Fascicular Oculomotor (Third) Nerve Palsy
894 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
895 Blepharospasm Round UpShirley H. Wray, MD, PhD, FRCPSlideshow describing condition.Benign Essential Blepharospasm; Focal Dystonia; Lid Twitch; Psychogenic Blepharospasm; Voluntary Blinking
896 Amyotrophic Lateral Sclerosis (Guest Lecture)John Q. Trojanowski, MDThe patient is a 68 year old right handed retired air conditioner repair man who presented with impaired balance and slow walking. For about one year he had noted difficulty lifting his feet high enough when climbing the stairs. From that time on, his movements slowed and worsened so that he had dif...Saccadic Initiation Deficit of Unilateral Horizontal Gaze; Complete Paralysis of Voluntary Horizontal Saccades on Command to Look Left; Inability to Make a Refixation Saccade on Command to a Target Held on the Left; Normal Voluntary Horizontal Saccadic Eye Movements to the Right; Impaired Pursuit; F...
897 Clivus_ChordomaShirley H. Wray, MD, PhD, FRCPThis 46 year old patient had at age 6, a tendency for the left eye to wander out. Her face photograph at that age shows an exotropia and at age 7, a year later, the exotropia was not quite as prominent. It was assumed that the exotropia was due to a non-paralytic strabismus. Past History: At age 1 f...Esotropia; Abduction Weakness; Sixth Nerve Palsy; Clivus Chordoma; Chordoma
898 Pontine_InfarctionShirley H. Wray, MD, PhD, FRCPThe patient is a 62 year old right handed man, status post myocardial infarction in 1989 and on Coumadin. In 1993 he presented with a history of three separate TIAs 1.Instantaneous perioral tingling and/or numbness lasting less than 1 minute. 2.Episodic numbness of the right hand and foot lasting le...Unilateral Internuclear Ophthalmoplegia; Unilateral Horizontal Gaze Palsy; Upbeat Nystagmus on Upgaze; Convergence Normal; Fisher's One-and-a-Half Syndrome; Pontine Infarct; Unilateral Horizontal Gaze Palsy Infarct; Abducting Nystagmus
899 Constructional ApraxiaShirley H. Wray, MD, PhD, FRCPThe patient is a 72 year old right handed woman who presented in November 1995 with the sudden onset of impaired coordination of visual and motor skills following an inner right ear infection. One of her problems was difficulty sitting on a chair as she tended to place her body incorrectly. By late ...Dressing Apraxia; Apraxia of the Left Hand; Constructional Apraxia; Right Parietal Lobe; Progressive Lobar Atrophy; Degenerative CNS Disease; Apraxia
900 Downbeat Nystagmus PANShirley H. Wray, MD, PhD, FRCPThis patient carries a diagnosis of multiple sclerosis. See also: http://content.lib.utah.edu/cdm/ref/collection/ehsl-shw/id/317Downbeat Nystagmus; Periodic Alternating Nystagmus; Multiple Sclerosis; Primary Position Downbeat Nystagmus
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