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76 The Prize: An Interview with David H. Hubel Nobel Laureate Physiology or Medicine 1981David H. Hubel is the John Enders University Professor of Neurobiology at Harvard Medical School. Born in Canada of American parents, he grew up in Montreal, graduated from McGill Medical School, and received training in neurology at the Montreal Neurological Institute and Johns Hopkins Hospital. ...Image/MovingImage
77 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. This patient with KSS is still alive in 2009. In 1968, at the age of 15 he presented with a hi...Image/MovingImage
78 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
79 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
80 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
81 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
82 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
83 Release HallucinationsThe patient is a 79 year old woman with a chief complaint of visual hallucinations. She carries a diagnosis of glaucoma and cataracts. The patient was in good health until two weeks prior to admission when she noted a black cloud in her visual field in the top central area. The cloud gradually c...Image/MovingImage
84 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
85 Saccadic Initiation Deficit of Horizontal SaccadesThe patient is a 78 year old left handed woman with a diagnosis of a left parietal infarct in 1995, bilateral carotid artery stenosis and hypertension. She was first seen in August 1997 for evaluation of involuntary movements of the lower face in the setting of rapidly progressive dementia and was...Image/MovingImage
86 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
87 See-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...Image/MovingImage
88 Selective Saccadic PalsyThis case is published courtesy of Scott D.Z. Eggers, M.D., Department of Neurology, Mayo Clinic College of Medicine (1). The patient is a healthy 50 year old woman who underwent otherwise uncomplicated aortic valve replacement for an incidentally discovered ascending aortic aneurysm. Upon awakeni...Image/MovingImage
89 Sixth Nerve PalsyThis 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: ...Image/MovingImage
90 Sixth Nerve PalsyThe patient is a 45 year old pharmaceutical executive who, nine days prior to admission, flew back from San Francisco after a hectic business trip feeling jet lagged and fatigued. Inspite of this, four days later, he did a day trip to New York. On the following morning, a Saturday, he awoke with ...Image/MovingImage
91 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
92 Slow Saccade SyndromeIn 1992, this 60 year old woman realized that she was "slowing up", dragging her right foot and walking slowly, and her speech became slurred. She saw a number of physicians and was given the diagnosis of Parkinson's disease and started on Sinemet 50/200 t.i.d. In January 1994, she was seen by a n...Image/MovingImage
93 Spasm of the Near ReflexThis 17 year old boy presented with episodic eye pain. He consulted an ophthalmologist for evaluation of his symptoms and also complained of periodic difficulty focusing. On examination he had periodic spasms of abnormal eye movements, with impairment of full abduction. His ophthalmologist cons...Image/MovingImage
94 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
95 Supranuclear Paralysis of DowngazeThe patient is a 62 year old man who, on getting out of bed one morning, found his eyes did not open fully. The right eye hardly opened at all and the left just a little. He staggered to the bathroom and started to floss his teeth and dropped a piece of floss on the floor. He felt unsteady bend...Image/MovingImage
96 Supranuclear Paralysis of DowngazeThe patient is a 64 year old man with no major past medical history who, on the day of admission, suddenly developed loss of vision in both eyes and then was unable to open his eyes on his own unless he used his hands. Holding his eyelids open his vision was very blurry. Within minutes he lost con...Image/MovingImage
97 Supranuclear Paralysis of Up and DowngazeThe 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 ...Image/MovingImage
98 Supranuclear Paralysis of UpgazeThis 84 year old woman carried the following diagnoses: 1. Extrapyramidal movement disorder; Parkinson's disease vs. Progressive supranuclear palsy 2. Hypertension 3. Recurrent left Bell's palsy In 1992, the patient and her family noted that she was drifting to the right when driving. She progress...Image/MovingImage
99 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
100 Supranuclear Paralysis of UpgazeThe patient is a 71 year old woman who was admitted to the Cardiac Unit following an acute "rush to the head", diaphoresis, and palpitations. She felt she was about to faint. She described seeing gold rings in her vision for a few minutes. Past History: Partial complex seizures since 1987. Th...Image/MovingImage
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