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aicardi_syndrome [2015/06/20 16:33] – created adminaicardi_syndrome [2025/04/18 20:40] (current) – external edit 127.0.0.1
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 ====== Aicardi Syndrome ====== ====== Aicardi Syndrome ======
-=====Main Features====+====Main Features====
   * Classic Triad:    * Classic Triad: 
-  - Infantile Spasms +    - Infantile Spasms 
-  - Agenesis of the corpus callosum +    - Agenesis of the corpus callosum 
-  - Chorioretinal lacunae +    - Chorioretinal lacunae 
- +====Eye Findings====
-=====Eye Findings====== +
   * Anterior Segment: Microcornea with or without coloboma   * Anterior Segment: Microcornea with or without coloboma
   * Posterior Segment: Chorioretinal Lacunae radiating from optic disc most often   * Posterior Segment: Chorioretinal Lacunae radiating from optic disc most often
Line 15: Line 12:
   * Visual prognosis better without lacunae   * Visual prognosis better without lacunae
   * Gross motor and language worse if many large lacunae   * Gross motor and language worse if many large lacunae
-=====Etiology=====+====Etiology====
   * Xp22 mutation   * Xp22 mutation
   * X-Linked Dominant   * X-Linked Dominant
   * Lethal in Males   * Lethal in Males
- +====Other Findings====
-=====Other Findings====+
   * Neuro: Mental retardation, Characteristic EEG   * Neuro: Mental retardation, Characteristic EEG
   * ENT: Cleft Lip and Palate   * ENT: Cleft Lip and Palate
   * Skin: Scalp lipomas, cavernous hemangiomas   * Skin: Scalp lipomas, cavernous hemangiomas
   * Bone: Vertebral and rib abnormalities   * Bone: Vertebral and rib abnormalities
- +====Reference====
-=====Reference=====+
   * [[http://omim.org/entry/304050|OMIM 304050]]   * [[http://omim.org/entry/304050|OMIM 304050]]
   * McMahon RG, Bell RA, Moore GRW, Ludwin SK. Aicardi's Syndrome, A Clinicopathologic Study. Arch Ophthalmol 102; 250-53,1984   * McMahon RG, Bell RA, Moore GRW, Ludwin SK. Aicardi's Syndrome, A Clinicopathologic Study. Arch Ophthalmol 102; 250-53,1984
  
 {{tag>syndrome}} {{tag>syndrome}}