| Identifier |
Spinocerebellar_Ataxias_1080p |
| Title |
Spinocerebellar Ataxias |
| Creator |
Andrew G. Lee, MD; Nicole Weber |
| Affiliation |
(AGL) Chairman, Department of Ophthalmology, The Methodist Hospital, Houston, Texas; Professor of Ophthalmology, Weill Cornell Medicine, New York City, New York; (NW) Baylor College of Medicine, Houston, Texas |
| Subject |
Spinocerebellar Ataxia; SCA; Machado-Joseph; Saccade |
| Description |
Dr. Lee lectures medical students on the subject of spinocerebellar ataxias. |
| Transcript |
So, we had a request to talk a little bit about the spinocerebellar ataxias. These are disorders that can present to ophthalmologists, but not because of the ataxia, because of the cerebellar component. So, the cerebellar one is going to produce cerebellar eye signs and so these SCA's, they're numbered, and the ones that we are interested in are numbers 1 through 7. And so, the cerebellar eye signs that are common to all of the spinocerebellar ataxia syndromes are going to be saccadic abnormalities. So, as you know, the cerebellum is in charge of controlling the fine movement in your body, but also the fine movement in your eyes. So for the saccade, it will get this cerebellar saccadic abnormality and those are going to be most prominent in SCA number 1 and number 2. All of them are going to be characterized by the typical features of spinocerebellar ataxia: the cerebellar body signs and the ataxia. This talk is mostly going to be about the eye component. So, the neurologist is going to be dealing with the ataxia and the cerebellar findings, the imaging, and the other work up for the other etiologies other than the ‘SCA's' as we call them, and the ophthalmologist's job is to look at the eye. So, when we're looking at the eye we have both the afferent system and the efferent system. And so, on the efferent pathway the ‘SCA' number 3 (SCA number 3) has a lot of eye findings. So, the problems in the ‘SCA'3 are lid retraction and ophthalmoparesis. So, this is the one that we really should be paying attention to. So, if you have to pick a ‘SCA' (SCA), you cannot use the cerebellar ataxia or the cerebellar signs to differentiate them. The differentiating feature from the ophthalmology side is on the efferent side-that's lid retraction and ophthalmoparesis. It's not pathognomonic, but it's suggestive that it's got 3,that is the Machado-Joseph variety. The next one that we're going to be worried about then is 6 and 7. So, 6 and 7 are the two other ones that come to us. 6is going to come to us as a downbeat nystagmus. And the 7is the one that's going to be associated with the pigmentary retinopathy and the optic atrophy. So, the reasons that an ophthalmologist would be called in a patient who has ataxia with cerebellar signs and is being considered for a ‘SCA'(spinal cerebellar ataxia)are to document the saccadic abnormalities (which are cerebellar eye signs), problems with the saccade (the pursuit and the fine movement control, nystagmus).We're going to be looking for the lid retraction and ophthalmoparesis in the ‘SCA' number 3.We're going to be looking for downbeat nystagmus in ‘SCA'6. And in ‘SCA' 7 a pigmentary retinopathy or optic atrophy (fundus findings that would suggest that it's ‘SCA' number 7). So, this is the way that we, as ophthalmologists, can help neurologists figure out which of these numbers is causing the spinocerebellar ataxia |
| Date |
2021-06 |
| Language |
eng |
| Format |
video/mp4 |
| Type |
Image/MovingImage |
| Collection |
Neuro-Ophthalmology Virtual Education Library: Andrew G. Lee Collection: https://novel.utah.edu/Lee/ |
| Publisher |
North American Neuro-Ophthalmology Society |
| Holding Institution |
Spencer S. Eccles Health Sciences Library, University of Utah, 10 N 1900 E SLC, UT 84112-5890 |
| Rights Management |
Copyright 2019. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
| ARK |
ark:/87278/s6hx7cd2 |
| Setname |
ehsl_novel_lee |
| ID |
1701587 |
| Reference URL |
https://collections.lib.utah.edu/ark:/87278/s6hx7cd2 |
