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. Author manuscript; available in PMC: 2015 Apr 30.
Published in final edited form as: Sci Transl Med. 2015 Feb 25;7(276):276ra26. doi: 10.1126/scitranslmed.3010101

Fig 1. Age of onset and severity of ataxia in untreated NPC cats and NPC cats administered IC HPβCD.

Fig 1

(A) A dose-related effect on the age of onset of ataxia was noted in NPC cats receiving IC HPβCD compared to untreated NPC cats (†p<0.05). At 24 weeks of age, the severity of ataxia (B) and head tremor (C) was diminished in cats dosed presymptomatically; individual cats receiving 60 mg HPβCD or more showed no ataxia or head tremor at an age when untreated NPC cats were euthanized due to disease progression. No cats receiving 15 mg or greater HPβCD developed head tremor. At 76 weeks of age, only cats receiving 30 mg HPβCD or greater were alive, and these cats showed mild to moderate ataxia. Cats first administered HPβCD at 16 weeks of age (postsymptomatically), showed the same onset of ataxia as untreated cats (A), with individual cats showing less ataxia and head tremor at 24 weeks of age compared to untreated NPC cats (B, D). Ataxia was graded on a 0–4 scale with 0, none; +1, mild ataxia; +2, moderate ataxia resulting in falling when running; +3, severe ataxia resulting in falling when walking; +4 no longer able to stand. Head tremor graded on a 0–3 scale with 0, none; +1, mild; +2, moderate; +3, severe.