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TABLE 1: Clinical, neuropsychologic, and molecular data from 17 patients
Mean or % SD No. of Patients
Age at MR imaging 68 years 5.5 17
Age at tremor onset 58.3 years 3.9 15
Age at ataxia onset 61.6 years 5.6 15
Significant impairment 
 Writing 89% 17
 Walking 50% 17
Tremor 89% 17
Character of tremor 
 Kinetic > postural 100% 15
 Discrete resting tremor 58% 15
Gait ataxia 89% 17
Dyssynergia 72% 17
Tone, mild increase 47% 14
Neuropsychologic data 
 VIQ 98 16 6
 PIQ 83 17 6
 FSIQ 91 18 6
Executive function deficits 100% 6
 VIQ < 85 33% 6
 PIQ < 85 66% 6
 Impotence 100% 6
 CGG repeat 86 10 17
 FMRP level 77.5%^* 9% 6
 mRNA level 2.53-fold^† 0.48 6
* Percentage of lymphocytes that are positive for fragile X mental retardation 1 protein immuno-cytochemical staining for fragile X mental retardation 1 protein (19,46).
† 2.53-fold increase over normal (16,46).
Note.—VIQ indicates verbal intelligence quotient; PIQ, performance intelligence level; FSIQ, full scale intelligence quotient; FMRP, fragile X mental retardation 1 protein; mRNA, messenger RNA.