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76 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...
77 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
78 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
79 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
80 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
81 Brain Control of Horizontal Saccadic Eye Movements (Guest Lecture)Shirley H. Wray, MD, PhD, FRCPIn 1995 I published this case alongside eleven personal cases, three with the Kearns-Sayer Syndrome (KSS) and five with Progressive External Opthalmoplegia (PEO). Am J of Neuroradiol:16 (5);1167-1173. The patient was under the care of Dr. Raymond Adams from age 13 years. In 1991, at age 40 years, I ...Bilateral Ptosis; Facial Weakness; Complete External Ophthalmoplegia; Bilateral Progressive External Ophthalmoplegia (PEO); Mitochondrial Myopathy; PEO plus Deafness; Cerebellar Degeneration with Ataxia; Chronic Progressive External Opthalmoplegia
82 Essential Palatal TremorShirley H. Wray, MD, PhD, FRCPThe patient is a 25 year old meteorologist from Tennessee who came to Boston in the summer of 1992 to vacation with his family on the Cape. His illness started with flu-like symptoms, low grade fever from 99 to 100F, sweating and episodes of light headedness associated with occasional nausea, indige...Essential Palatal Tremor (Myoclonus); Brainstem Encephalitis
83 Palatal TremorShirley 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; Bilate...
84 Myasthenia ThymomaShirley H. Wray, MD, PhD, FRCPThe 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
85 Multiple SclerosisShirley H. Wray, MD, PhD, FRCPThe patient is a 56 year old woman who presented in 1982, at the age of 48, with a one week history of painless loss of vision in the left eye. Past History: Negative for a previous attack of optic neuritis or transient neurological symptoms. Family History: Negative for CNS disease Neuro-ophthalmol...Upbeat Nystagmus; Lid Nystagmus; Square Wave Jerks; Jerk Oscillations; Rotary Nystagmus; Saccadic Pursuit; Saccadic Dysmetria; Multiple Sclerosis; Bilateral Lid Nystagmus; Primary Position Upbeat Nystagmus; Torsional Nystagmus; Horizontal Saccadic Dysmetria
86 Midbrain HemorrhageShirley H. Wray, MD, PhD, FRCPThe patient is a 49 year old woman who was in good health until January 17, 1991. When, at work one morning, she had an acute attack of light headedness and double vision and collapsed on the floor without loss of consciousness. She developed a severe retro-orbital headache. She was taken to the loc...Supranuclear Paralysis of Upgaze Hemorrhage; Convergence Retraction Nystagmus; Vertical Oculocephalic Reflex Normal; Normal Convergence; Skew Deviation; Light/Near Dissociation of the Pupils; Pretectal Syndrome; Midbrain Hemorrhage; Cavernous Angioma
87 Mitochondrial Myopathy (Guest Lecture)Shirley H. Wray, MD, PhD, FRCPSee also: http://content.lib.utah.edu/cdm/ref/collection/ehsl-shw/id/120, http://content.lib.utah.edu/cdm/ref/collection/ehsl-shw/id/121, http://content.lib.utah.edu/cdm/ref/collection/ehsl-shw/id/124, http://content.lib.utah.edu/cdm/ref/collection/ehsl-shw/id/13, and http://content.lib.utah.edu/cdm...Unilateral Ptosis; External Ophthalmoplegia Affecting Upgaze; Normal Pupils; Progressive External Ophthalmoplegia (PEO); Mitochondrial Myopathy; Unilateral Progressive External Ophthalmoplegia; Bilateral Ptosis; Facial Weakness; Complete External Ophthalmoplegia; Bilateral Progressive Exter...
88 Miller Fisher Syndrome: Guest LectureShirley H. Wray, MD, PhD, FRCPSee also: http://content.lib.utah.edu/cdm/ref/collection/ehsl-shw/id/119, http://content.lib.utah.edu/cdm/ref/collection/ehsl-shw/id/29, and http://content.lib.utah.edu/cdm/ref/collection/ehsl-shw/id/37Voluntary Ptosis; Bilateral Sixth Nerve Palsy; Esotropia; Paresis of Conjugate Upgaze; Acute Inflammatory Demyelinating Neuropathy; Guillian Barre Syndrome - Miller Fisher Syndrome; Bilateral Ptosis; Total External Ophthalmoplegia; Facial Diplegia; Areflexia; Acute Inflammatory Demyelinati...
89 Multiple Sclerosis LateropulsionShirley H. Wray, MD, PhD, FRCPThis 44 year old woman has a diagnosis of Multiple Sclerosis. See also: http://content.lib.utah.edu/cdm/ref/collection/ehsl-shw/id/281Deviation of the Eyes Under Closed Lids; Lateropulsion; Multiple Sclerosis
90 Paraneoplastic OpsocolonusShirley H. Wray, MD, PhD, FRCPThis patient is the index case of the Anti-Ri antibody, published in Annals of Neurology in 1988 (4). The Anti-Ri antibody is recognized to be a paraneoplastic marker in patients with breast and gynecological malignancies (10). The history of this case is particularly important because she was initi...Opsoclonus; Ocular Flutter; Oscillopsia; Saccadic Oscillations; Paraneoplastic Cerebellar Syndrome; Adenocarcinoma of the Breast; Anti-Ri Antibody; Paraneoplastic Opsoclonus; Paraneoplastic Ocular Flutter; Saccadomania
91 Paraneoplastic Ocular FlutterShirley H. Wray, MD, PhD, FRCPThe patient is a 58 year old woman with known hypertension. In 1994, two weeks prior to admission she had a dramatic change in behavior with insomnia, agitation and depression. This was accompanied by "ringing of hands and anxiety for no apparent reason". She became anorexic, lost 15 pounds in weigh...Ocular Flutter; Oscillopsia; Trunkal Ataxia; Paraneoplastic Cerebellar Syndrome; Small Cell Carcinoma of the Lung; Paraneoplastic Ocular Flutter; Flutter
92 Parasellar MeningiomaShirley H. Wray, MD, PhD, FRCPThis patient is a 58 year old woman from Peru who, in 1975, developed intermittent headaches and right retro-orbital eye pain. She was seen by several ophthalmologists in South America who were unable to make a diagnosis. In March 1977 she awoke one morning with vertical diplopia most marked on look...Ptosis; Third Nerve Palsy; Aberrant Reinnervation of the Third Nerve; Oculomotor Nerve; Parasellar Meningioma; Cavernous Sinus Syndrome; Unilateral Oculomotor Third Nerve Palsy; Unilateral Third Nerve Palsy
93 Parkinsons DiseaseShirley H. Wray, MD, PhD, FRCPSee also: http://content.lib.utah.edu/cdm/ref/collection/ehsl-shw/id/298 and http://content.lib.utah.edu/cdm/ref/collection/ehsl-shw/id/93Blepharoclonus; Convergence Insufficiency; Slow Hypometric Horizontal Saccades; Saccadic Breakdown of Smooth Pursuit; Parkinson's Disease- Dopamine deficiency; Basal Ganglia; Blepharoclonus; Convergence Insufficiency; Slow Hypometric Saccades; Saccadic Breakdown of Smooth Pursuit; Parkinson's Disea...
94 NeuroblastomaShirley H. Wray, MD, PhD, FRCPThe patient is Case 27-1995 Case Records of the Massachusetts General Hospital (New Eng. J Medicine 1995, 333:579-586). The discusser was Dr. Elizabeth Engle, Associate Professor of Neurology, Harvard Medical School. The baby girl was born after a 30 week gestation, with a birth weight of 1.25 kg. T...Downbeat Nystagmus; Paraneoplastic Opsoclonus; Neuroblastoma; Primary Position Downbeat Nystagmus; Ataxia; Paraneoplastic Downbeat Nystagmus
95 Brain MRI in Multiple SclerosisAnne G. Osborn, MDSlideshow describing condition.Abducting Nystagmus; Bilateral Internuclear Ophthalmoplegia; Botulinum Toxin Therapy; Gaze Evoked Downbeat Nystagmus; Horizontal Pendular Nystagmus; Horizontal Saccadic Dysmetria; Lid Nystagmus; Multiple Sclerosis; Normal Convergence; Oscillopsia; Pendular Horizontal Oscillations; Saccadic Dysmetri...
96 Cavernous Sinus MeningiomaShirley H. Wray, MD, PhD, FRCPSlideshow describing condition.Abducting Nystagmus; Cavernous Angioma; Convergence Normal; Facial Palsy; Facial Weakness; Fisher's One-and-a-Half Syndrome; Pendular Vertical Oscillations; Pontine Hemorrhage; Unilateral Gaze Palsy Hemorrhage; Unilateral Horizontal Gaze Palsy; Unilateral Internuclear Ophthalmoplegia; Upbeat Nystag...
97 Amyotrophic Lateral SclerosisRobert H. Brown Jr, D.Phil, MDSlideshow describing condition.Bulbar Palsy; CNS Degeneration; Convergence Insufficiency; Familial Amyotrophic Lateral Sclerosis; Lou Gehrig's Disease; Saccadic Breakdown of Horizontal Pursuit; Slow Hypometric Horizontal Saccades; Superoxide Dismutase (SOD1) Gene; Supranuclear Paralysis of Up and Downgaze Degeneration
98 Cavernous AngiomaShirley H. Wray, MD, PhD, FRCPSlideshow describing condition.Cavernous Angioma; Convergence Normal; Facial Palsy; Fisher's One-and-a-Half Syndrome; Pendular Vertical Oscillations; Pontine Hemorrhage; Unilateral Gaze Palsy Hemorrhage; Unilateral Horizontal Gaze Palsy; Unilateral Internuclear Ophthalmoplegia; Upbeat Nystagmus on Upgaze
99 Progressive Supranuclear PalsyShirley H. Wray, MD, PhD, FRCPSlideshow describing condition.Acquired Ocular Motor Apraxia; Apraxia of Eyelid Opening; CNS - Degeneration Convergence absent; Convergence Insufficiency; Global Supranuclear Vertical Gaze Palsy; Impaired Initiation of Horizontal Saccades; Lid Retraction; Positive Glabella Tap; Progressive Supranuclear Palsy - Tauopthay; Progres...
100 Supranuclear Paralysis of DowngazeShirley H. Wray, MD, PhD, FRCPSlideshow describing condition.Absent Convergence; Artery of Percheron; Midbrain Infarct; Ocular Tilt Reaction; Skew Deviation; Somnolence; Supranuclear Paralysis of Downgaze Infarct; Supranuclear Paralysis of Downgaze; Vertical Oculocephalic Reflex Normal
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