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--- batten_disease [2025/05/30 18:24] – created Scott Larson
+++ batten_disease [2025/06/14 02:37] (current) – Scott Larson
@@ Line 10: / Line 10: @@
   * Most survive into early adulthood
+==== Fundus Exam ====
+<WRAP round box>
+{{::batten_disease_fundus.jpg|}}\\
+Mild temporal pallor right optic nerve and yellow-orange deposits\\
+photo from Jennifer L. Rossen MD's 2025 AAPOS workshop presentation
+</WRAP>
+==== OCT ====
+<WRAP round box>
+{{::batten_disease_oct.jpg|}}\\
+loss of ellipsoid zone in fovea, generalized thinning and distortion of retinal layers, loss of outer retinal layers\\
+photo from Jennifer L. Rossen MD's 2025 AAPOS workshop presentation
+</WRAP>
+==== Fundus Autofluorescence ====
+<WRAP round box>
+{{::batten_disease_fundus_autofl.jpg|}}\\
+foveal hypo-fluorescence and ring of hyper-fluorescence in macula\\
+photo from Jennifer L. Rossen MD's 2025 AAPOS workshop presentation
+</WRAP>
+===== Distinguish from Stargardt Disease =====
+  * More profound and rapid vision loss
+  * Panretinal involvement early in disease seen on ERG
+  * No flecks
+  * Optic atrophy often present early
+  * Neurological symptoms
+{{tag>syndrome}}

syndrome