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26 163-155Bilateral Internuclear Ophthalmoplegia (INO)The patient is a 48 year old woman who presented with horizontal double vision looking to the right and to the left. She also noted blurring of vision with the image jerking horizontally back and forth. Neuro-ophthalmological examination: Visual acuity 20/20 OU corrected Visual fields, pupils, and f...Bilateral Internuclear Ophthalmoplegia; Abducting Nystagmus; Upbeat Nystagmus on Upgaze; Oscillopsia; Multiple Sclerosis
27 163-2See-saw NystagmusThe patient is a 21 year old woman who was referred to an endocrinologist for evaluation of amenorrhea. She was found to have bitemporal hemianopia and compression of the chiasm. CT Brain showed: A partially cystic, partially solid suprasellar mass with focal calcification consistent with a crani...Pendular See-saw Nystagmus; Bitemporal Hemianopia; Craniopharyngioma
28 163-21Nuclear Third Nerve PalsyThe following case is the first patient I saw with a nuclear third nerve palsy. The patient is a 52 year old man with hypertension and hyperlipidemia. He awoke one morning unable to open his eyes. To walk he tilted his head backwards and looked down. He came to the emergency room of the Massach...Nuclear Third Nerve Palsy; Bilateral Ptosis; Bilateral Superior Rectus Palsy; Normal Pupils; Midbrain Infarct; Oculomotor Nerve; Nuclear; Thalamic Infarct
29 163-3Bilateral Sixth Nerve PalsyThis patient was known to be a chronic alcoholic. He presented with acute onset of unsteadiness walking which he attributed to double vision. He was unable to give an accurate history and was disoriented for time and place. Neurological examination: Significant for impaired memory and cognitive ...Bilateral Sixth Nerve Palsy; Esotropia; Supranuclear Paralysis of Downgaze; Absent Convergence; Upbeat Nystagmus on Upgaze; Wernicke's Disease; Supranuclear Paralysis of Downgaze Encephalopathy; Gaze Evoked Upbeat Nystagmus; Wernicke Encephalopathy; Thiamine Deficiency
30 163-6Bilateral Internuclear OphthalmoplegiaThis 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 signific...Bilateral Internuclear Ophthalmoplegia; Upbeat Nystagmus; Medulloblastoma; Abducting Nystagmus
31 163-9-1Congenital NystagmusThis young boy has oculocutaneous albinism. In 1979 he presented for evaluation of oscillations of his eyes present since birth. He had no head turn or head tremor. Diagnosis: Albinism Congenital sensory nystagmus Ocular Albinism: Infants with albinism of all types are typically slow to see, ofte...Congenital Nystagmus; Horizontal Pendular Nystagmus; Optokinetic Nystagmus Absent; Infantile Nystagmus Syndrome; Oculocutaneous Albinism
32 163-9-2Congenital Nystagmus; Latent NystagmusThis little girl has had nystagmus since birth. She has the classical constellation of signs, initially diagnosed as congenital motor nystagmus with: • Horizontal jerk nystagmus • A slight head turn to the right to place the eyes in the null position • Latent nystagmus • Inability to superi...Congenital Nystagmus; Horizontal Jerk Nystagmus; Optokinetic Nystagmus absent; Infantile Nystagmus Syndrome Latent Nystagmus; Fusional Maldevelopment Nystagmus Syndrome
33 163-9-3Congenital Nystagmus: Oscillations of the EyesThis child has had nystagmus since childhood with a head turn to the left. He was seen by his pediatrician. Diagnosis: Horizontal jerk nystagmus (congenital motor nystagmus) Congenital nystagmus Classification: The Classification of Eye Movement Abnormalities and Strabismus Working Group has recomme...Congenital Nystagmus; Horizontal Jerk Nystagmus; Optokinetic Nystagmus Absent; Infantile Nystagmus Syndrome
34 163-9-4Familial NystagmusThe mother and her infant son both have conjugate horizontal pendular nysagmus. Diagnosis: Congenital sensory nystagmus Familial Infantile Nystagmus Syndrome (INS) The little boy is a bright active child with normal development. He has pendular nystagmus in central gaze without a head turn or h...Congenital Nystagmus; Horizontal Pendular Nystagmus; Infantile Nystagmus Syndrome; Familial Congenital Nystagmus
35 164-10Familial Myasthenia GravisIn 1999 Dr. A. G. Engel published a monograph on Myasthenia Gravis and Myasthenic Disorders with a chapter devoted to Congenital Myasthenic Syndromes. Dr. Engel systematically defined and classified hereditary and congenital myasthenic syndromes, delineated on the basis of their electrophysiologi...Familial Myasthenia Gravis; External Ophthalmoplegia; Ocular Myasthenia Gravis; Unilateral Ptosis; Unilateral Myasthenia Gravis
36 164-2Fisher's One and a Half SyndromeThis young patient presented with double vision and was found to have on examination the classical findings of Fisher's one-and-a-half syndrome which are: • Right internuclear ophthalmoplegia on gaze left with adduction weakness OD • Right horizontal gaze paresis with gaze evoked nystagmus • F...Unilateral Internuclear Ophthalmoplegia; Unilateral Horizontal Gaze Palsy; Upbeat Nystagmus on Upgaze; Primary Position Upbeat Nystagmus; Convergence Normal; Fisher's One-and-a-Half Syndrome; Multiple Sclerosis; Unilateral Horizontal Gaze Palsy Demyelination; Gaze Evoked Upbeat Nystagmus; Abducting ...
37 165-10Titubation OpsoclonusThis 52 year old woman presented with unsteady gait. She was admitted to an outside hospital where a diagnosis was made. 1. Paraneoplastic opsoclonus 2. Paraneoplastic cerebellar syndrome with bilateral limb and gait ataxia and titubation 3. Adenocarcinoma of the breast She was transferred to the M...Titubation; Opsoclonus; Oscillopsia; Saccadic Oscillations; Paraneoplastic Cerebellar Syndrome; Adenocarcinoma of the Breast; Anti-Ri Antibody; Paraneoplastic Opsoclonus; Divergence
38 165-2Bilateral Sixth Nerve PalsyThe patient is a 60 year old woman who consulted her ophthalmologist with a chief complaint of double vision looking to the left. He diagnosed of a left sixth nerve palsy. No investigations were done. Two years later she complained of diplopia looking to the right. A diagnosis of bilateral six...Esotropia; Bilateral Abduction Weakness; Bilateral Sixth Nerve Palsy; Clivus Meningioma; Sixth (Abducens)
39 165-9Supranuclear Vertical Gaze PalsyThe patient is a 60 year old woman who was given a diagnosis of Progressive supranuclear palsy six months before she was referred by the Movement Disorders Clinic for evaluation of difficulty reading. Family History: Negative for neurodegenerative disease. Neuro-ophthalmological examination: Sup...Global Supranuclear Vertical Gaze Palsy; Convergence absent; Prominent Stare; Slow Hypometric Horizontal Saccades; Slow Horizontal Pursuit; Vertical Oculocephalic Reflex Normal; Positive Glabella Tap; Progressive Supranuclear Palsy; Steele Richardson Olszewski Syndrome; Supranuclear Paralysis of Up ...
40 166-1Paraneoplastic Opsoclonus/FlutterIn 1975 this patient presented with oscillopsia due to opsoclonus with ocular flutter. Opsoclonus with flutter is a paraneoplastic syndrome associated with occult cancer of the breast, ovary and lung. This patient had cancer of the breast. In 1954 Cogan first used the term "ocular flutter" to d...Opsoclonus; Ocular Flutter; Paraneoplastic Cerebellar Syndrome; Adenocarcinoma of the Breast; Paraneoplastic Opsoclonus; Paraneoplastic Ocular Flutter
41 166-11Downbeat NystagmusThe patient is a 72 year old man who was found down and admitted as an emergency in coma. Neurological examination: Patient in coma failed to respond to painful stimuli. Downbeat nystagmus Lid nystagmus Pupils 2 mm OU responsive to light Corneal reflexes absent Oculocephalic reflex absent No resp...Coma; Downbeat Nystagmus; Lid Nystagmus; Bilateral Horizontal Gaze Palsy; Pontine Hemorrhage; Bilateral Horizontal Gaze Palsy Hemorrhage; Primary Position Downbeat Nystagmus
42 166-12Opsoclonus -- In the DarkN/ASaccadic Oscillations; Paraneoplastic Opsoclonus; Oscillopsia; Opsoclonus; Saccadomania
43 166-14Fisher's One and a Half SyndromeThis young man was seen in the emergency room of his local hospital following the onset of severe headache, mild confusion and diplopia. Non-contrast CT brain scan showed: A right pontine hemorrhage He was transferred to the Massachusetts General Hospital ICU. Ocular Motility: Esotropia of the r...Unilateral Internuclear Ophthalmoplegia; Unilateral Horizontal Gaze Palsy; Convergence Absent; Esotropia; Fisher's One-and-a-Half Syndrome; Facial Palsy; Abducens Nuclear Lesion; Pontine Hemorrhage; Unilateral Gaze Palsy Hemorrhage; Abducting Nystagmus; Facial Weakness
44 166-18Supranuclear Paralysis of Up and DowngazeThis patient presented to the Dementia Clinic for evaluation of forgetfulness, impaired recall memory, lack of attentiveness and spontaneity and progressive impairment of mobility over a period of one year. Neurological examination: The patient was found to have multisystem involvement with: 1. Mi...Positive Glabella Tap; Slow Hypometric Horizontal Saccades; Supranuclear Paralysis of Up and Downgaze Degeneration; Progressive Supranuclear Palsy - Tauopathy; Steele Richardson Olszewski Syndrome; CNS - Degeneration
45 166-2Ocular FlutterThis patient was seen in the Neurovisual Clinic for evaluation of a monocular attack of optic neuritis which completely resolved. Six months later, she became unsteady walking and was found to have bilateral ataxia. She was given a diagnosis of multiple sclerosis (MS). Neuro-ophthalmological examin...Ocular Flutter; Multiple Sclerosis; Lid Flutter; Multiple Sclerosis Ocular Flutter
46 166-20Fisher's One and a Half SyndromeThis 28 year old woman had severe multiple sclerosis with a spastic paraparesis due to a lesion of the spinal cord. She was admitted to the Intensive Care Unit complaining of dizziness and double vision. Neuro-ophthalmologic examination: Visual acuity 20/30 OU Pupils, visual fields and fundi normal ...Unilateral Internuclear Ophthalmoplegia; Unilateral Horizontal Gaze Palsy; Upbeat Nystagmus on Upgaze; Convergence Normal; Fisher's One-and-a-Half Syndrome; Multiple Sclerosis; Unilateral Internuclear Ophthalmoplegia; Unilateral Horizontal Gaze Palsy Demyelination; Gaze Evoked Upbeat Nystagmus; Abdu...
47 166-24Elliptical NystagmusThe patient carries a diagnosis of multiple sclerosis.Primary Position Elliptical Nystagmus; Multiple Sclerosis
48 166-25Pseudo-Internculear OphthalmoplegiaThis 32 year old man had the acute onset of double vision followed very quickly by a drooping of the left eyelid. Past History: Negative for any previous episode of double vision No history of strabismus as a child No fatigue, neck weakness, difficulty in chewing or swallowing Family History: Neg...Unilateral Ptosis; Pseudo-internuclear Ophthalmoplegia; Bilateral Weakness of Adduction; Tensilon Test; Ocular Myasthenia Gravis; Unilateral Myasthenia Gravis; Internuclear Ophthalmoplegia
49 166-26Fascicular Third Nerve PalsyThe 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; Third Nerve Dysfunction
50 166-3Neonatal OpsoclonusThis child was one of the first cases of opsoclonus that I saw with Dr. Cogan in the early 1970's. The baby is a unique case in that in addition to neonatal opsoclonus with the characteristic multidirectional conjugate back-to-back saccades, periods of large amplitude upbeat nystagmus also occurred....Neonatal Opsoclonus; Upbeat Nystagmus; Lid Nystagmus; Saccadic Oscillations
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