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Spectral optical coherence tomography in a patient with type I sialidosis.

Michalewska Z, Gajos A, Michalewski J, Nawrocki J, Pshezhetsky AV, Bogucki A - Med. Sci. Monit. (2011)

Bottom Line: Enzyme assay followed.Besides a normal angiogram, a thickened nerve fiber layer was observed on spectral optical coherence tomography.The thickened nerve fiber layer was probably caused by accumulation of metabolic products such as sialylated oligosaccharides and glycopeptides, suggesting that SD- OCT, due to its enhanced resolution, can be a useful tool for diagnosis of rare neurological conditions.

View Article: PubMed Central - PubMed

Affiliation: Ophthalmic Clinic Jasne Blonia, Lodz, Poland. zosia_n@yahoo.com

ABSTRACT

Background: The aim of our study was to analyze spectral optical coherence tomography (SD-OCT) findings in a patient with clinical signs of sialidosis.

Case report: Fluorescein angiography and spectral optical coherence tomography was performed in a 37-year-old woman using a SD-OCT device with axial resolution of 6 µm. Enzyme assay followed. The patient was diagnosed with type I sialidosis by enzymatic assay. Besides a normal angiogram, a thickened nerve fiber layer was observed on spectral optical coherence tomography.

Conclusions: The thickened nerve fiber layer was probably caused by accumulation of metabolic products such as sialylated oligosaccharides and glycopeptides, suggesting that SD- OCT, due to its enhanced resolution, can be a useful tool for diagnosis of rare neurological conditions.

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Related in: MedlinePlus

Spectral Domain Optical Coherence Tomography. Right eye on the right side and left eye on the left side. A normal fovea contour and thickened nerve fibre layer are visible.
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f1-medscimonit-17-10-cs129: Spectral Domain Optical Coherence Tomography. Right eye on the right side and left eye on the left side. A normal fovea contour and thickened nerve fibre layer are visible.

Mentions: A previously healthy 15-year-old girl developed sudden, jerky movements of the upper limbs. She was born to non-consanguineous parents and her early development and progress in school was normal. She had no siblings and her family history was unremarkable. The jerkiness gradually generalized to the lower extremities. Her gait was disturbed and her speech became slurred. At the age of 20, myoclonus of the face was present and ophthalmography revealed the opsoclonus. When she was 22-years-old generalised clinic-tonic and partial seizures occurred. The epilepsy was well controlled with valproate. Between 20 and 31 years she was several times evaluated ophthalmologically and fundus examination did not reveal any abnormalities. However, corneal opacities were noted at that time. Visual acuity dropped from 1.0 to 0.7 on Snellen charts during that time. Intraocular pressure was normal. At the age of 35 cherry-red spots in the maculae were observed for the first time (Figure 1) suggesting diagnosis of type 1 sialidosis.


Spectral optical coherence tomography in a patient with type I sialidosis.

Michalewska Z, Gajos A, Michalewski J, Nawrocki J, Pshezhetsky AV, Bogucki A - Med. Sci. Monit. (2011)

Spectral Domain Optical Coherence Tomography. Right eye on the right side and left eye on the left side. A normal fovea contour and thickened nerve fibre layer are visible.
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC3539471&req=5

f1-medscimonit-17-10-cs129: Spectral Domain Optical Coherence Tomography. Right eye on the right side and left eye on the left side. A normal fovea contour and thickened nerve fibre layer are visible.
Mentions: A previously healthy 15-year-old girl developed sudden, jerky movements of the upper limbs. She was born to non-consanguineous parents and her early development and progress in school was normal. She had no siblings and her family history was unremarkable. The jerkiness gradually generalized to the lower extremities. Her gait was disturbed and her speech became slurred. At the age of 20, myoclonus of the face was present and ophthalmography revealed the opsoclonus. When she was 22-years-old generalised clinic-tonic and partial seizures occurred. The epilepsy was well controlled with valproate. Between 20 and 31 years she was several times evaluated ophthalmologically and fundus examination did not reveal any abnormalities. However, corneal opacities were noted at that time. Visual acuity dropped from 1.0 to 0.7 on Snellen charts during that time. Intraocular pressure was normal. At the age of 35 cherry-red spots in the maculae were observed for the first time (Figure 1) suggesting diagnosis of type 1 sialidosis.

Bottom Line: Enzyme assay followed.Besides a normal angiogram, a thickened nerve fiber layer was observed on spectral optical coherence tomography.The thickened nerve fiber layer was probably caused by accumulation of metabolic products such as sialylated oligosaccharides and glycopeptides, suggesting that SD- OCT, due to its enhanced resolution, can be a useful tool for diagnosis of rare neurological conditions.

View Article: PubMed Central - PubMed

Affiliation: Ophthalmic Clinic Jasne Blonia, Lodz, Poland. zosia_n@yahoo.com

ABSTRACT

Background: The aim of our study was to analyze spectral optical coherence tomography (SD-OCT) findings in a patient with clinical signs of sialidosis.

Case report: Fluorescein angiography and spectral optical coherence tomography was performed in a 37-year-old woman using a SD-OCT device with axial resolution of 6 µm. Enzyme assay followed. The patient was diagnosed with type I sialidosis by enzymatic assay. Besides a normal angiogram, a thickened nerve fiber layer was observed on spectral optical coherence tomography.

Conclusions: The thickened nerve fiber layer was probably caused by accumulation of metabolic products such as sialylated oligosaccharides and glycopeptides, suggesting that SD- OCT, due to its enhanced resolution, can be a useful tool for diagnosis of rare neurological conditions.

Show MeSH
Related in: MedlinePlus