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Vertical supranuclear gaze palsy in Niemann-Pick type C disease
Vertical supranuclear gaze palsy (VSGP) is a key clinical feature in patients with Niemann-Pick type C disease (NP-C), a rare, autosomal recessive, neuro-visceral disorder caused by mutations in either the NPC1 or NPC2 gene. VSGP is present in approximately 65 % of the cases and is, with gelastic cataplexy, an important risk indicator for NP-C. VSGP in
Davide Pareyson +2 more
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Movement Disorders, 2003
AbstractWe document a new oculomotor phenomenon in a patient with pathologically proven progressive supranuclear gaze palsy (PSP), namely that vertical gaze excursion improves with larger pursuit targets. We used computerised video‐oculography during vertical smooth pursuit eye movements (SPEM) of circular targets of diameter 0.16 degrees and 16 ...
Adolfo M Bronstein +2 more
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AbstractWe document a new oculomotor phenomenon in a patient with pathologically proven progressive supranuclear gaze palsy (PSP), namely that vertical gaze excursion improves with larger pursuit targets. We used computerised video‐oculography during vertical smooth pursuit eye movements (SPEM) of circular targets of diameter 0.16 degrees and 16 ...
Adolfo M Bronstein +2 more
exaly +3 more sources
Journal of Neuro-Ophthalmology, 2021
Background: Supranuclear vertical gaze palsies and slowed vertical saccades are characteristic clinic features of progressive supranuclear palsy (PSP). The “hummingbird sign,” reflective of midbrain atrophy, is a classic radiographic sign of PSP.
Karen A. Buch +6 more
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Background: Supranuclear vertical gaze palsies and slowed vertical saccades are characteristic clinic features of progressive supranuclear palsy (PSP). The “hummingbird sign,” reflective of midbrain atrophy, is a classic radiographic sign of PSP.
Karen A. Buch +6 more
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Atypical motor neuron disease with supranuclear vertical gaze palsy and slow saccades
Auris Nasus Larynx, 2009In amyotrophic lateral sclerosis (ALS), eye movements are usually preserved even after the long-term use of respirators. The present study evaluated a 57-year-old male patient who showed clinical findings compatible with ALS but exhibited disorders of eye movements before he needed to be on an artificial respiration system.
Munetaka Ushio +2 more
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Vertical supranuclear gaze palsy induced by deep brain stimulation: Report of two cases
Parkinsonism and Related Disorders, 2014Vanessa Fleury +2 more
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A patient with pantothenate kinase-associated neurodegeneration and supranuclear gaze palsy
Pantothenate kinase-associated neurodegeneration (PKAN) is a genetic disease with childhood onset characterized clinically by dystonia, parkinsonism, pyramidal signs, visual failure and mental retardation. Progression is usually relentless culminating in
Leonidas Stefanis +2 more
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The case history of a patient with a rapidly progressive spastic tetraparesis, pseudobulbar palsy, supranuclear gaze palsy, and extrapyramidal signs is presented.
Adolfo M Bronstein +2 more
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Strabismus
Purpose: Congenital supranuclear vertical gaze palsy is an extremely rare ocular motility disorder, typically presenting with vertical gaze limitation despite normal extraocular muscles and cranial nerves. The coexistence of congenital supranuclear vertical gaze palsy with large-angle horizontal and vertical deviations is scarcely reported.
Shivani Kumari, Mittali Khurana
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Purpose: Congenital supranuclear vertical gaze palsy is an extremely rare ocular motility disorder, typically presenting with vertical gaze limitation despite normal extraocular muscles and cranial nerves. The coexistence of congenital supranuclear vertical gaze palsy with large-angle horizontal and vertical deviations is scarcely reported.
Shivani Kumari, Mittali Khurana
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Progressive Supranuclear Palsy
Archives of Neurology, 1964Introduction In this report we are describing a progressive brain disease featured by supranuclear ophthalmoplegia affecting chiefly vertical gaze, pseudobulbar palsy, dysarthria, dystonic rigidity of the neck and upper trunk, and other less constant cerebellar and pyramidal symptoms. Dementia has usually remained mild. This disease would appear to be
John C, Steele +2 more
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Seminars in Pediatric Neurology, 2014
We encountered an adolescent male with cerebellar ataxia since age 11, difficulty in vertical gaze from age 2, leg weakness since age 10, and partial epilepsy since age 8. At age 14, he developed visual and auditory hallucinations, as well as mild sensorineural deafness. He was evaluated as having a mitochondrial disorder.
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We encountered an adolescent male with cerebellar ataxia since age 11, difficulty in vertical gaze from age 2, leg weakness since age 10, and partial epilepsy since age 8. At age 14, he developed visual and auditory hallucinations, as well as mild sensorineural deafness. He was evaluated as having a mitochondrial disorder.
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