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151 Spastic Cerebellar Ataxia of Charlevoix-SaguenayThe patient is a 19 year old high school student who carried the diagnosis of autosomal recessive spastic cerebellar ataxia of Charlevoix-Saguenay. He was born at term, walked at 9 months and developed well with normal milestones. At age 10 he began to have difficulty speaking, occasional involuntar...Image/MovingImage
152 Pendular Vertical OscillationsThis 36 year old woman had a nine year history of multiple sclerosis (MS) with onset in 1980. In 1989 she was seen in the neurovisual clinic for evaluation of oscillopsia, (an illusion of movement of the visual world). This symptom was accompanied by difficulty in reading because the print was "ju...Image/MovingImage
153 Downbeat NystagmusThis 58 year old engineer was referred by his neurologist for evaluation of periodic episodes of difficulty focusing and blurred vision for 8 years. In 1981 whilst sightseeing in Newport, he became acutely aware of difficulty focusing and blurry vision. The symptoms lasted for twenty minutes and t...Image/MovingImage
154 Bilateral Internuclear OphthalmoplegiaThis 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 ...Image/MovingImage
155 Supranuclear Vertical Gaze PalsyThis 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...Image/MovingImage
156 Pendular Horizontal OscillationsThis 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. ...Image/MovingImage
157 Saccadic Initiation Deficit of Horizontal SaccadesThe 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 ...Image/MovingImage
158 Slow Saccade SyndromeThe patient is a 62 year old woman referred from Ireland with a one year history of unsteadiness walking which she first noted following a fall from a chair. Her gait disorder was insidious in onset and may have pre-dated the fall. Since then she had become increasingly unsteady with a tendency to f...Image/MovingImage
159 Palatal TremorThe patient is a 44 year old left handed man with a history of IV drug abuse (heroin and cocaine) alcoholism, hypertension, and rheumatic fever. In March 1990, at 3 a.m. on the day of admission, he had acute onset of dizziness, slurred speech, left sided weakness and difficulty walking. He was ...Image/MovingImage
160 Duane's SyndromeThe patient is a 7 year old boy born two weeks premature with transposition of the major arteries of the heart, four holes in the heart, and an absent spleen. He had cardiac surgery at age 2 days and at age one year and his development was excellent thereafter. At age 6 months, it was noted that th...Image/MovingImage
161 Progressive External OphthalmoplegiaThe patient is an 18 year old girl, first seen in 1990 with a 6 year history of progressive ptosis. In 1986, at age 14, she was seen by an ophthalmologist and pediatric neurologist and investigated. Myasthenia Gravis was ruled out by a negative Tensilon test, negative anti-acetylcholine receptor ...Image/MovingImage
162 Alexia Without AgraphiaThe patient is a 69 year old left handed man with a history of hypertension, insulin dependent diabetes mellitus and atrial fibrillation. Treated with coumadin, adjusted to keep the INR between 2 and 3. On the morning of admission he awoke at 4 a.m., sat momentarily on the side of the bed and then...Image/MovingImage
163 Upbeat NystagmusThe 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-ophth...Image/MovingImage
164 Psychogenic BlepharospasmThe patient is a 57 year old woman who presented in 1989, with a 3 year history of frequent eyelid blinking, twitching and twinking. She was under a good deal of stress and anxiety attributed to moving house after 26 years and losing touch with her friends and social activities. She was also conce...Image/MovingImage
165 Fisher's One and a Half SyndromeThe patient is a 56 year old woman who was seen by a Stroke Consult on the cardiac unit because she had difficulty opening her left eye and diplopia looking to the right. On examination she had ptosis OS and "a partial ophthalmoplegia" sparing the pupil. Diagnosis Partial left third nerve palsy....Image/MovingImage
166 Gaucher's DiseaseThis little boy has Gaucher's disease. Gaucher's disease is an autosomal recessive disorder, linked to chromosome 1q21, due to glucocerebroside β-glucosidase deficiency. There are three phenotypic variances of Gaucher's disease. Type I is the most common and lacks neurological features. Type 2 is...Image/MovingImage
167 Bilateral Ptosis Facial DiplegiaThe patient is a 74 year old woman who one month prior to admission suffered from a non-productive cough, right ear pain, and pharyngitis treated with amoxicillin. On the day prior to admission she awoke with blurred vision and horizontal and vertical diplopia that persisted all day. The next m...Image/MovingImage
168 Restrictive OrbitopathyThis 71 year old woman was referred with bilateral optic neuropathy and thyroid associated ophthalmopathy (TAO) of Graves' Disease. She had been treated for primary hyperthyroidism on three occasions with radioactive iodine and was taking Tapazole 5 mg daily. Neuro-ophthalmological examination: ...Image/MovingImage
169 Lid LagThe classical eye signs of thyroid associated ophthalmopathy (TAO) of Graves' Disease is illustrated by case ID925-4. This 50 year old woman with TAO is included in the collection because she illustrates very well lid lag (persistent elevation of the upper eyelid in downgaze) - von Graefe sign E...Image/MovingImage
170 Palatal TremorThe patient is a 60 year old woman with insulin dependent diabetes mellitus. She was legally blind as a result of diabetic retinopathy and bilateral vitreous hemorrhage. In 1987, she was admitted to her local hospital with slurred speech and a right hemiparesis progressing over a period of three d...Image/MovingImage
171 Supranuclear Paralysis of UpgazeThe 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 t...Image/MovingImage
172 Progressive External OphthalmoplegiaIn 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...Image/MovingImage
173 Progressive External OphthalmoplegiaThis 48 year old woman, who was first seen by a neurologist in March 1989, with a 7 year history of progressive unilateral ptosis, mild facial weakness and generalized fatigue. On examination she had ptosis of the right eye and bilateral limitation of upward gaze. Myasthenia Gravis was ruled out by...Image/MovingImage
174 Progressive External OphthalmoplegiaThe patient is a retired physician, age 70, who recalls having eye muscle exercises as a child way back in 1924. Years later, she noted difficulty in focusing her eyes on horizontal gaze to the right and left which preceded the onset of bilateral ptosis. She presented in 1985, at age 65, with mark...Image/MovingImage
175 Downbeat NystagmusThe patient is a 59 year old woman who carried a diagnosis of: 1. Congenital anomaly of the occipito-cervical junction with occipitalization of the C1 ring 2. Basilar invagination with Chiari Type I 3. A syrinx in the center of the cervical-thoracic spinal cord extending from C2 to T4 In 1957, at ag...Image/MovingImage
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