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Maria E. Rosenberg, Timo M. T. Tervo, Juana Gallar, M. Carmen Acosta, Linda J. Müller, Jukka A. O. Moilanen, Ahti H. A. Tarkkanen, Minna H. Vesaluoma; Corneal Morphology and Sensitivity in Lattice Dystrophy Type II (Familial Amyloidosis, Finnish Type). Invest. Ophthalmol. Vis. Sci. 2001;42(3):634-641.
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purpose. To describe the corneal abnormalities and to measure different modalities
of corneal sensitivity in corneal lattice dystrophy type II (familial
amyloidosis, Finnish type, also known as gelsolin-related amyloidosis
and originally as Meretoja syndrome).
methods. Twenty eyes of 20 patients were examined by in vivo confocal microscopy
and noncontact gas esthesiometry.
results. Pleomorphism of, and dense deposits between or posterior to, the basal
epithelial cells were frequently observed, as well as a reduction of
long nerve fiber bundles in the subbasal nerve plexus. The anterior
stroma was altered in most cases, with fibrosis and abnormal
extracellular matrix. In 15 corneas, thick anterior and midstromal
filaments, corresponding to lattice lines, and in 11 corneas, thin
undulated structures were observed. The average mechanical sensitivity
threshold of 12 subjects was increased, and in the remaining 8 subjects
there was no response, even to the highest intensity of stimuli used.
Three patients did not respond to CO2, 11 to heat, and 2 to
cold, but those patients who responded had normal thresholds. Patients
with more long nerve fiber bundles per confocal microscopic image had
better mechanical and cold sensitivity than patients with fewer nerve
conclusions. Lattice lines seem to be related to amyloid material and not to corneal
nerves. However, the subbasal nerve density appears reduced, which
results mainly in a decrease in mechanical and, to a lesser extent,
thermal sensitivity. The location of stromal filaments and undulated
structures changes with increasing age.
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