====== Batten Disease ======
  * a.k.a. CLN3-associated disease and Juvenile Ceroid Lipofucinosis
  * Inherited CNS disorder 
    * Progressive loss of developmental milestones
    * Movement and Speech dysfunction 
    * Seizures
    * Psychosis
    * Retinal degeneration 
  * Vision loss often precedes neurological decline
  * 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}}