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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

Fundus photograph and fluorescein angiography of both eyes. Cherry-red spots are visible on both fundus images. Fluorescein angiography is normal.
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f2-medscimonit-17-10-cs129: Fundus photograph and fluorescein angiography of both eyes. Cherry-red spots are visible on both fundus images. Fluorescein angiography is normal.

Mentions: Around the cherry-red spots in the macula we observed a slightly oval, pale perimacular area, about 1.5 disc diameters in size. Besides this finding, the clinical examination was normal. Fluorescein angiography was also performed and no abnormal leakage was observed. Neurological examination at age 38 years revealed severe myoclonus and dystonic movements of the trunk, exaggerated by voluntary movements and emotion, in all 4 extremities, head and face. Generalised ataxia was present. Her speech was dysarthric and explosive. Horizontal nystagmus was observed. Deep tendon reflexes were hyperactive. Because of involuntary movements and ataxia she was unable to feed and dress herself and she was wheelchair-bound. Neuropsychological evaluation revealed mild impairment of cognitive functions and adaptive depressive reaction. The brain MRI was normal. EEG occasionally showed discharges of sharp waves and diffuse theta waves. Visual acuity dropped to 0.1 in both eyes at that time. During the fundus examination, the cherry-red spots on the macula and a normal optic nerve head were observed. Fluorescein angiography was unremarkable (Figure 1). Spectral optical coherence tomography (SD-OCT, Spectralis, Heidelberg Engineering, Germany) was additionally performed (Figure 2). SD-OCT is a novel technique providing information about the internal structure of an object by measuring the interferometric signal detected as a function of optical frequencies. This allows for high speed and high resolution cross-sectional imaging of the human retina, enabling more exact visualization of retinal layers, especially photoreceptors and external limiting membrane. The SD-OCT used in this study provides 3.9 μm axial and 12–18 μm transverse resolution, and 3-dimensional scanning is also possible. The examination revealed increased paracentral retinal thickness with normal central retinal thickness. All retinal layers seemed normal except the thickened nerve fibre layer, including an intact photoreceptor layer and external limiting membrane.


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)

Fundus photograph and fluorescein angiography of both eyes. Cherry-red spots are visible on both fundus images. Fluorescein angiography is normal.
© Copyright Policy
Related In: Results  -  Collection

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

f2-medscimonit-17-10-cs129: Fundus photograph and fluorescein angiography of both eyes. Cherry-red spots are visible on both fundus images. Fluorescein angiography is normal.
Mentions: Around the cherry-red spots in the macula we observed a slightly oval, pale perimacular area, about 1.5 disc diameters in size. Besides this finding, the clinical examination was normal. Fluorescein angiography was also performed and no abnormal leakage was observed. Neurological examination at age 38 years revealed severe myoclonus and dystonic movements of the trunk, exaggerated by voluntary movements and emotion, in all 4 extremities, head and face. Generalised ataxia was present. Her speech was dysarthric and explosive. Horizontal nystagmus was observed. Deep tendon reflexes were hyperactive. Because of involuntary movements and ataxia she was unable to feed and dress herself and she was wheelchair-bound. Neuropsychological evaluation revealed mild impairment of cognitive functions and adaptive depressive reaction. The brain MRI was normal. EEG occasionally showed discharges of sharp waves and diffuse theta waves. Visual acuity dropped to 0.1 in both eyes at that time. During the fundus examination, the cherry-red spots on the macula and a normal optic nerve head were observed. Fluorescein angiography was unremarkable (Figure 1). Spectral optical coherence tomography (SD-OCT, Spectralis, Heidelberg Engineering, Germany) was additionally performed (Figure 2). SD-OCT is a novel technique providing information about the internal structure of an object by measuring the interferometric signal detected as a function of optical frequencies. This allows for high speed and high resolution cross-sectional imaging of the human retina, enabling more exact visualization of retinal layers, especially photoreceptors and external limiting membrane. The SD-OCT used in this study provides 3.9 μm axial and 12–18 μm transverse resolution, and 3-dimensional scanning is also possible. The examination revealed increased paracentral retinal thickness with normal central retinal thickness. All retinal layers seemed normal except the thickened nerve fibre layer, including an intact photoreceptor layer and external limiting membrane.

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