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Not for further distribution unless allowed by the License or with the express written permission of Cambridge University Press.
https://doi.org/10.1017/S0317167100006764
https://doi.org/10.1017/S0317167100006764 Published online by Cambridge University Press
2022-04-12T19:44:34+00:00
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[(Kwong-Kum Liao, Department of Neurology)65(, )18(T)70(aipei )17(V)111(eterans)]TJ
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20.04148 0.00001 TD
[(T)70(aipei, )18(T)70(aiwan 1)36(1217, R.O.C.)]TJ
ET
Q
/GS2 gs
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BT
/F1 1 Tf
9 0 0 9 39.4306 741.3389 Tm
0 0 0 1 k
0.0013 Tc
(O)Tj
0.77732 0.00001 TD
0.001 Tc
[(RIGINAL)-164(A)-1(R)36(TICLE)]TJ
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 309.5354 247.5035 Tm
0 0 0 1 k
0.2 Tw
(visualized by magnetic resonance imaging (MRI), but are of)Tj
0 -1.1579 TD
0.02 Tc
0.455 Tw
(great importance in causing disability in patients with)Tj
0 -1.15789 TD
0 Tc
0.001 Tw
[(neurological disorders, such as mitochondrial disease. )18(Though it)]TJ
T*
0.08 Tw
(is generally agreed that genetic testing is very important in the)Tj
0 -1.1579 TD
0.353 Tw
(diagnosis of KD, EPs might still help in revealing subtle)Tj
ET
0.5 w
0 0 0 1 K
159.3038 751.2395 m
39.3998 751.2395 l
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/F0 1 Tf
9.5 0 0 9.5 40.024 717.0228 Tm
0 0 0 1 k
0.285 Tw
(neurological dysfunction. Here, we used multimodal EPs to)Tj
0 -1.15789 TD
0.079 Tw
(investigate systemically the central sensory and motor pathway)Tj
T*
0.025 Tw
(function of KD patients. )Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F1 1 Tf
9.5 0 0 9.5 40.024 673.0228 Tm
0 0 0 1 k
(M)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F1 1 Tf
7.125 0 0 7.125 48.9906 673.0228 Tm
0 0 0 1 k
0.0015 Tc
(E)Tj
0.6675 -0.00001 TD
0.001 Tc
(THODS)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F2 1 Tf
9.5 0 0 9.5 40.024 658.0228 Tm
0 0 0 1 k
(Subjects)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 52.024 643.0228 Tm
0 0 0 1 k
0.253 Tw
[(W)80(e included six KD patients, two from the same family)]TJ
-1.26316 -1.15789 TD
0.122 Tw
[((patients 2 and 3). )18(The demographics and clinical findings are)]TJ
0 -1.1579 TD
0.372 Tw
[(listed in )18(T)70(able 1. Neurological examination showed motor)]TJ
0 -1.15789 TD
0.197 Tw
(weakness of each subject and sensory deficits of tactile with)Tj
T*
0.281 Tw
(glove-and-stocking patterns in three subjects. None of them)Tj
0 -1.1579 TD
0.101 Tw
[(complained of hearing or visual problems. )55(All the KD patients)]TJ
0 -1.15789 TD
0.086 Tw
(had increased values of serum creatinine kinase \(KD 248-1654)Tj
T*
0.02 Tc
0.405 Tw
(U/L; control 27-168 U/L\) and normal values of serum)Tj
0 -1.1579 TD
0 Tc
-0.001 Tw
(testosterone (KD 4.58-9.37 NG/ML; control 2.36-9.96 NG/ML).)Tj
0 -1.15789 TD
0.025 Tw
(They all fit the diagnostic criteria of KD and were diagnosed by)Tj
T*
0.119 Tw
[(genetic testing. )18(The control included ten healthy men matched)]TJ
0 -1.1579 TD
0.048 Tw
[(with age and height. )18(They were studied after informed consents)]TJ
0 -1.15789 TD
0.013 Tc
0.362 Tw
[(were given. Besides multimodal EP)-588(studies, the protocol)]TJ
T*
0 Tc
0.166 Tw
(included standard nerve conduction studies (NCS) and needle)Tj
0 -1.1579 TD
0 Tw
(electromyography)Tj
7.20947 0 TD
(.)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F2 1 Tf
9.5 0 0 9.5 309.16 717.0228 Tm
0 0 0 1 k
[(EP)-238(studies)]TJ
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 321.16 702.0228 Tm
0 0 0 1 k
0.037 Tw
(The parameters of EPs were all based upon the guidelines of)Tj
-1.26316 -1.15789 TD
0.338 Tw
[(the International Federation of Clinical Neurophysiology)65(.)]TJ
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
6.27 0 0 6.27 543.5547 694.1578 Tm
0 0 0 1 k
-0.0001 Tc
(8)Tj
0.49989 -0.00001 TD
0 Tc
[(-1)56(1)]TJ
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 309.16 680.0228 Tm
0 0 0 1 k
0.114 Tw
(Pattern visual EPs (VEPs) were obtained to specifically assess)Tj
0 -1.1579 TD
0.152 Tw
(the function of optic nerve pathways. Brainstem auditory EPs)Tj
0 -1.15789 TD
0.05 Tw
((BAEPs) were done by 10 Hz click and were averaged for total)Tj
T*
-0.001 Tw
[(1024 stimuli, focusing on the interpeak latencies of I-V)129(, I-III and)]TJ
0 -1.1579 TD
0.098 Tw
[(III-V)129(. BAEPs were done with binaural stimulators. )17(When click)]TJ
0 -1.15789 TD
0.292 Tw
(stimuli were applied at one side, white noise masking was)Tj
T*
0.02 Tc
0.521 Tw
(employed at the intensity of 40 dB at another side.)Tj
0 -1.1579 TD
0 Tc
-0.017 Tw
(Somatosensory evoked potentials (SEPs) were obtained from the)Tj
0 -1.15789 TD
0.055 Tw
(upper and lower limbs by electrical stimulation delivered to the)Tj
T*
0.141 Tw
(median nerve at the wrist and the posterior tibial nerve at the)Tj
0 -1.1579 TD
0.062 Tw
[(ankle. Responses were recorded from the ipsilateral Erb\325)55(s point)]TJ
0 -1.15789 TD
0.139 Tw
((N9), above the 5th cervical spine (N13) and the contralateral)Tj
T*
0.086 Tw
(scalp (N20) with reference to Fz for median nerve stimulation;)Tj
0 -1.1579 TD
0.033 Tw
(and the 1st lumbar spine \320contralateral iliac crest (N22), the 5th)Tj
0 -1.15789 TD
0.375 Tw
(cervical spine (P30), and the contralateral scalp (P37) for)Tj
T*
0.09 Tw
(posterior tibial nerve stimulation. Five hundred responses were)Tj
0 -1.1579 TD
0.16 Tw
(averaged for each trial of median nerve stimulation and 1000)Tj
0 -1.15789 TD
0.025 Tw
(responses for tibial nerve stimulation. )Tj
1.26316 -1.1579 TD
0.104 Tw
[(T)35(ranscranial magnetic stimulation (TMS) with recording on)]TJ
-1.26316 -1.15789 TD
0.178 Tw
(the abductor pollicis brevis of the upper limb was performed)Tj
0 -1.1579 TD
0.103 Tw
[(with a magnetic stimulator \(MagStim 200, Magstim Company)65(,)]TJ
T*
0.114 Tw
(UK\) to measure the latency of motor evoked potential \(MEP\).)Tj
0 -1.15789 TD
0.169 Tw
(Motor threshold (MT) was usually estimated at complete rest)Tj
0 -1.1579 TD
0.038 Tw
(and was defined as a reliable MEP)Tj
14.30755 0 TD
(response with a minimal 50-)Tj
-14.30755 -1.1579 TD
-0.033 Tw
[(100 \265)-76(V)-199(in five of ten consecutive trials.)]TJ
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
6.27 0 0 6.27 457.8937 419.1578 Tm
0 0 0 1 k
(1)Tj
0.44414 0 TD
(1)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 465.7076 416.0228 Tm
0 0 0 1 k
-0.033 Tw
(When there were active)Tj
-16.4787 -1.15789 TD
0.14 Tw
(muscle activities in the background, the MT)Tj
18.84606 0 TD
(was defined as a)Tj
-18.84606 -1.1579 TD
0.161 Tw
(minimal response size around 200-300 )Tj
16.54921 0 TD
Tj
0.57594 0 TD
(V)Tj
0.59301 0 TD
(.)Tj
0.643 0 TD
(The motor central)Tj
-18.36116 -1.15791 TD
0.008 Tc
0.367 Tw
(conduction time (CCT) was calculated by subtracting the)Tj
0 -1.15789 TD
0 Tc
0.117 Tw
(peripheral motor conducting time from the total latency of the)Tj
0 -1.1579 TD
0.158 Tw
[(MEP)111(, i.e. motor CCT)-390((ms) = cortical MEP)-371(latency - \(F wave)]TJ
T*
0.109 Tw
(latency + distal latency of compound motor action potentials -)Tj
0 -1.15789 TD
0.025 Tw
(1\)/2. )Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F2 1 Tf
9.5 0 0 9.5 309.16 317.0228 Tm
0 0 0 1 k
0.025 Tw
(Statistical analysis)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 321.16 302.0228 Tm
0 0 0 1 k
(The )Tj
2.13343 0 TD
0.347 Tw
[(W)40(ilcoxon Rank Sum test was used to analyze the)]TJ
-3.39659 -1.1579 TD
0.165 Tw
[(dif)18(ference between the KD and control groups. )54(All data were)]TJ
0 -1.15789 TD
0.025 Tw
(considered significant when the )Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F3 1 Tf
9.5 0 0 9.5 433.0326 280.0228 Tm
0 0 0 1 k
(p)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 440.3951 280.0228 Tm
0 0 0 1 k
0.025 Tw
(value was < 0.05. )Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F1 1 Tf
9.5 0 0 9.5 309.16 258.0228 Tm
0 0 0 1 k
(R)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F1 1 Tf
7.125 0 0 7.125 316.0183 258.0228 Tm
0 0 0 1 k
0.001 Tc
[(ESUL)93(T)-1(S)]TJ
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F2 1 Tf
9.5 0 0 9.5 309.16 243.0228 Tm
0 0 0 1 k
0.025 Tw
(Routine electrophysiological studies)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 321.16 228.0228 Tm
0 0 0 1 k
0.041 Tw
(The data of electrophysiological studies were summarized in)Tj
-1.26316 -1.15789 TD
(T)Tj
0.54101 0.00001 TD
0.126 Tw
(able 2. Sensory NCS showed decreased sensory nerve action)Tj
-0.54101 -1.15791 TD
0.286 Tw
(potentials. Motor NCS showed decreased compound muscle)Tj
0 -1.15789 TD
0.349 Tw
(motor action potentials but normal conduction time in the)Tj
T*
0.02 Tc
0.356 Tw
(forearm or leg segments in the nerves sampled. Needle)Tj
0 -1.1579 TD
0 Tc
0.025 Tw
(electromyography revealed denervation in the muscles sampled.)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F2 1 Tf
9.5 0 0 9.5 309.16 151.0228 Tm
0 0 0 1 k
0.025 Tw
(Sensory EPs)Tj
0 -1.57895 TD
0 Tw
(VEPs)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 321.16 121.0228 Tm
0 0 0 1 k
0.131 Tw
[(Our data \(mean \261)-366(standard deviation; SD\) showed that our)]TJ
-1.26316 -1.1579 TD
0.019 Tw
(KD patients had significant delayed P100 in the study of pattern)Tj
0 -1.15789 TD
0.025 Tw
(VEPs \(KD 1)Tj
5.06734 0.00001 TD
[(13.6 \261)-260(1)37(1.4 ms; control 98.4 \261)-260(4.9 ms; )]TJ
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F3 1 Tf
9.5 0 0 9.5 506.9935 99.0228 Tm
0 0 0 1 k
(p)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 514.356 99.0228 Tm
0 0 0 1 k
(<)Tj
0.83894 0 Td
(0.0001\).)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F1 1 Tf
9.5 0 0 9.5 40.024 413.0902 Tm
0 0 0 1 k
0.001 Tc
0.025 Tw
[(T)92(able 1: Demography of patients with Kennedy\325)37(s disease)]TJ
ET
1 w
0 0 0 1 K
39.024 105.3289 m
286.2 105.3289 l
S
39.024 426.651 m
286.2 426.651 l
S
0.5 w
39.024 393.651 m
286.2 393.651 l
S
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
8.5 0 0 8.5 40.024 93.9201 Tm
0 0 0 1 k
0.025 Tw
(CAG: cytosine-adenine-guanine; NCS: nerve conduction study; EMG:)Tj
0 -1.29412 TD
(electromyography; ++: the initial symptom at onset; + : yes; -: no; s:)Tj
T*
(single; N: within normal limits; )Tj
12.9297 0.00001 TD
(A: abnormal)Tj
ET
/GS2 gs
q
39.024 104.934 m
39.024 394.28 l
286.2 394.28 l
286.2 104.934 l
W n
BT
/F8 1 Tf
7.992 0 0 7.548 41.177 381.4789 Tm
0 g
3.498 Tc
0 Tw
[(P)3489(a)3487(t)3505(i)3504(e)3487(n)3487(t)3505(s)-975(123456)]TJ
ET
39.881 377.6029 456.192 1.224 re
f
BT
/F8 1 Tf
7.992 0 0 7.548 41.177 370.4629 Tm
0.011 Tc
[(A)2(ge)-1(/)19(S)2(ex)-4273(36)]TJ
/F9 1 Tf
7.992 0 0 7.548 115.049 370.4629 Tm
0 Tc
<00F2>Tj
/F8 1 Tf
7.992 0 0 7.548 138.377 370.4629 Tm
0.011 Tc
(47)Tj
/F9 1 Tf
7.992 0 0 7.548 147.449 370.4629 Tm
0 Tc
<00F2>Tj
/F8 1 Tf
7.992 0 0 7.548 170.777 370.4629 Tm
0.011 Tc
(44)Tj
/F9 1 Tf
7.992 0 0 7.548 179.849 370.4629 Tm
0 Tc
<00F2>Tj
/F8 1 Tf
7.992 0 0 7.548 203.177 370.4629 Tm
0.011 Tc
(51)Tj
/F9 1 Tf
7.992 0 0 7.548 212.249 370.4629 Tm
0 Tc
<00F2>Tj
/F8 1 Tf
7.992 0 0 7.548 235.577 370.4629 Tm
0.011 Tc
(41)Tj
/F9 1 Tf
7.992 0 0 7.548 244.649 370.4629 Tm
0 Tc
<00F2>Tj
/F8 1 Tf
7.992 0 0 7.548 267.977 370.4629 Tm
0.011 Tc
(71)Tj
/F9 1 Tf
7.992 0 0 7.548 277.049 370.4629 Tm
0 Tc
<00F2>Tj
/F8 1 Tf
7.992 0 0 7.548 41.177 360.4669 Tm
0.011 Tc
[(H)3(ei)17(gh)-1(t)-252(\()20(c)-2(m)-21(\))-2873(1)-1(69)-2352(1)-1(72)-2352(1)-1(63)-2352(1)-1(63)-2352(1)-1(72)-2352(1)-1(65)]TJ
0 -1.16216 TD
0.012 Tc
[(C)4(A)4(G)-264(N)4(o)1(.)-4062(4)1(7)-2919(48)-2918(48)-2918(50)-2918(53)-2918(46)]TJ
T*
[(A)3(g)1(e)-270(o)1(ns)-1(et)-3521(3)1(0)-2919(30)-2918(32)-2918(37)-2918(30)-2918(29)]TJ
T*
0.011 Tc
[(S)2(l)17(ur)20(r)19(ed)-271(s)-2(p)-1(ee)-1(c)-2(h)-1299(-)-3710(-)-3710(-)-3710(++)-2864(-)-3710(+)]TJ
0 -1.16217 TD
[(D)3(y)-2(s)-3(pha)-1(gi)17(a)-3299(-)-3710(+)-3459(-)-3710(+)-3459(-)-3710(+)]TJ
0 -1.16216 TD
3.47 Tc
[(W)3495(e)3459(a)3458(k)3457(n)3458(e)3459(s)3456(s)3186(\()3479(M)3465(R)3463(C)3435(\))3343(+++++)3459(+)595(+)]TJ
T*
[(F)3459(a)3459(t)3477(i)3476(g)3459(u)3458(e)-1271(++++-)-251(+)]TJ
0 -1.18919 TD
0.011 Tc
[(Fas)-3(c)-2(i)17(c)-3(ul)17(a)-1(t)19(i)17(o)-1(n)]TJ
4.05405 -1.16216 TD
3.47 Tc
[(L)3459(i)3475(m)3466(b)3458(s)2105(++-)-251(+-)-251(+)]TJ
T*
[(F)3459(a)3459(c)3456(e)1675(-)-251(+++++)]TJ
-4.05405 -1.16216 TD
0.011 Tc
[(M)6(us)-3(c)-2(l)17(e)-271(w)3(as)-3(t)19(i)17(ng)-1191(-)-3710(+)-3459(-)-3710(+)-3459(+)-3459(+)]TJ
T*
[(C)3(r)20(am)6(p)-5082(++)-2864(+)-3459(+)-3459(-)-3710(-)-3710(-)]TJ
T*
[(T)27(r)20(em)6(o)-28(r)-4845(+)-3459(++)-2864(++)-2864(++)-2864(-)-3710(+)]TJ
T*
[(G)5(ai)17(t)-252(d)-1(i)17(s)-2(o)-1(r)20(de)-28(r)-2305(-)-3710(+)-3459(+)-3459(+)-3459(++)-2864(+)]TJ
T*
-0.006 Tc
[(C)-14(lim)-10(b)-18(in)-17(g)-288(d)-17(i)-28(f)2(f)-25(i)-1(c)-19(u)-17(lt)1(y)-264(+)-3476(+)-3476(-)-3727(+)-3476(+)-17(+)-2881(+)]TJ
0 -1.18919 TD
0.011 Tc
[(S)2(en)-1(s)-2(or)19(y)]TJ
1.35135 -1.16216 TD
3.721 Tc
[(N)3713(u)3710(m)3716(b)3710(n)3709(e)3710(s)3707(s)1815(+)251(----+)]TJ
0 -1.16217 TD
[(S)3712(i)3727(g)3710(n)3709(s)-509(-+)251(-+)251(-+)]TJ
-1.35135 -1.16216 TD
0.011 Tc
[(G)5(y)-2(n)-1(ec)-3(om)6(as)-3(t)19(i)17(a)-1623(+)-3459(++)-2864(-)-3710(++)-2864(-)-3710(++)]TJ
T*
-0.008 Tc
[(In)-20(fe)-20(r)1(t)-27(i)-2(l)-3(i)-29(ty)-4373(+)-3478(+)-3478(-)-3729(-)-3729(s)-3562(-)]TJ
T*
3.721 Tc
[(D)3713(y)3708(s)3707(u)3710(r)3730(i)3727(a)-967(-+)251(---+)]TJ
T*
[(D)3713(i)3727(a)3710(b)3710(e)3709(t)3729(e)3709(s)-401(-----+)]TJ
T*
0.011 Tc
[(C)3(r)20(ea)-1(t)19(i)17(n)-1(i)17(ne)-271(k)-2(i)17(n)-1(as)-3(e)]TJ
/F9 1 Tf
7.992 0 0 7.548 105.977 167.0749 Tm
3.054 Tc
<00F500F500F500F500F500F5>Tj
/F8 1 Tf
7.992 0 0 7.548 41.177 157.0789 Tm
0.011 Tc
[(C)3(hol)17(e)-1(s)-2(t)18(er)19(ol)-2984(N)]TJ
/F9 1 Tf
7.992 0 0 7.548 138.377 157.0789 Tm
0 Tc
<00F5>Tj
/F11 1 Tf
7.992 0 0 7.548 170.777 157.0789 Tm
3.332 Tc
(NNNN)Tj
/F8 1 Tf
-16.21622 -1.32432 TD
0.036 Tc
[(AL)79(T)-6245(N)]TJ
/F9 1 Tf
7.992 0 0 7.548 138.377 147.0829 Tm
3.054 Tc
<00F500F5>Tj
/F11 1 Tf
7.992 0 0 7.548 203.177 147.0829 Tm
3.332 Tc
(NNN)Tj
/F8 1 Tf
-20.27027 -1.32433 TD
[(T)3375(e)3294(s)3318(t)3340(o)3321(s)3318(t)3340(e)3320(r)3341(o)3320(n)3321(e)1104(NNNNNN)]TJ
0 -1.16216 TD
3.387 Tc
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T*
[(S)3378(e)3376(n)3376(s)3373(o)3376(r)3395(y)3103(N)3380(C)3379(S)1405(AAAAAA)]TJ
T*
[(E)3378(M)3382(G)-2429(AAAAAA)]TJ
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[(LE JOURNAL)-238(CANADIEN DES SCIENCES NEUROLOGIQUES)]TJ
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Q
/GS2 gs
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/F3 1 Tf
9 0 0 9 39.024 29.4312 Tm
0 0 0 1 k
0.025 Tw
[(V)111(olume 34, No. 3 \320 )18(August 2007)]TJ
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9 0 0 9 540.524 29.4312 Tm
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(329)Tj
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[(CANADIAN JOURNAL)-238(OF NEUROLOGICAL)-238(SCIENCES)]TJ
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(330)Tj
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/F2 1 Tf
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0 0 0 1 k
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/GS2 gs
/GS1 gs
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(The stimuli, i.e. the hearing threshold level (HL), was at 70)Tj
-1.26316 -1.1579 TD
-0.017 Tw
(dB HL)Tj
2.92839 0 TD
(for each control subject but increased in KD patients \(KD)Tj
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0.284 Tw
[(91.3 \261)-519(1)37(1.3 dB HL, range: 75 - 1)37(10 dB HL\). Inconsistent)]TJ
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0.175 Tw
(waveforms were noted in each KD subject. )Tj
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(Therefore, it was)Tj
-18.69569 -1.1579 TD
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[(dif)18(ficult to measure the responses after superimposition. )]TJ
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0 0 0 1 k
(SEPs)Tj
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Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
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0 0 0 1 k
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(Abnormal SEPs were noted during stimuli at the tibial nerve)Tj
-1.26316 -1.1579 TD
0.245 Tw
[(but not at median nerve. )18(The chief findings were decreased)]TJ
0 -1.15789 TD
0.086 Tw
[(amplitudes of the cortical potentials \(KD 0.9 \261)-321(0.5 \265)-76(V)74(; control)]TJ
0 -1.1579 TD
0.025 Tw
[(1.9 \261)-260(0.4 \265)-76(V)74(; )]TJ
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F3 1 Tf
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0 0 0 1 k
(p)Tj
ET
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/F0 1 Tf
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0 0 0 1 k
(=)Tj
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(0.0002\). )Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F2 1 Tf
9.5 0 0 9.5 40.024 227.3823 Tm
0 0 0 1 k
(TMS)Tj
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Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 52.024 212.3823 Tm
0 0 0 1 k
0.275 Tw
(Increased motor threshold was noted in our KD patients)Tj
-1.26316 -1.1579 TD
-0.014 Tw
[(\(TMS intensity)65(, KD 59.2 \261)-221(7.9%; control 45.4 \261)-222(7.2%; )]TJ
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F3 1 Tf
9.5 0 0 9.5 248.7999 201.3823 Tm
0 0 0 1 k
(p)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 255.7955 201.3823 Tm
0 0 0 1 k
(<)Tj
0.80031 0 Td
(0.05\).)Tj
-23.5131 -1.15789 TD
-0.035 Tw
[(The central motor conduction time was not dif)18(ferent between KD)]TJ
T*
0.049 Tw
[(and control groups \(KD 6.8 \261)-284(0.2 ms; control 6.9 \261)-284(0.4 ms; )]TJ
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F3 1 Tf
9.5 0 0 9.5 272.61 179.3823 Tm
0 0 0 1 k
(p)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 280.2022 179.3823 Tm
0 0 0 1 k
(>)Tj
-25.28192 -1.1579 TD
(0.05\).)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F1 1 Tf
9.5 0 0 9.5 40.024 146.3823 Tm
0 0 0 1 k
(D)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F1 1 Tf
7.125 0 0 7.125 46.8823 146.3823 Tm
0 0 0 1 k
0.001 Tc
(ISCUSSION)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 52.024 131.3823 Tm
0 0 0 1 k
0.02 Tc
0.426 Tw
(Androgen modulates primary sensory signals via the)Tj
-1.26316 -1.1579 TD
0 Tc
0.06 Tw
(androgen receptors.)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
6.27 0 0 6.27 115.4963 123.5174 Tm
0 0 0 1 k
(12)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 124.1912 120.3823 Tm
0 0 0 1 k
0.06 Tw
(Androgen receptors are widespread in the)Tj
-8.85971 -1.15789 TD
0.236 Tw
[(brain. Using immunohistochemical technique, dif)18(fuse nuclear)]TJ
T*
0.086 Tw
(accumulation of mutant androgen receptors has been proved to)Tj
0 -1.1579 TD
0.262 Tw
(be far more frequent and extensive in the brain of the KD)Tj
0 -1.15789 TD
0.03 Tw
(patients than thus far believed.)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
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0 0 0 1 k
(13)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 166.2669 76.3823 Tm
0 0 0 1 k
0.03 Tw
(The mutant androgen receptors)Tj
15.04138 31.78949 TD
0.034 Tw
(are distributed not only in the neural tissues but also in the non-)Tj
0 -1.1579 TD
0.177 Tw
[(neural tissues. )18(The neural tissues included cerebrum, superior)]TJ
T*
0.17 Tw
(colliculus, pons, medulla, cerebellum, spinal cord, dorsal root)Tj
0 -1.15789 TD
0.034 Tw
(ganglia, and the sympathetic ganglia; and the non-neural tissues)Tj
0 -1.1579 TD
0.193 Tw
[(included liver)40(, kidney)65(, pancreas, testis, prostate gland, scrotal)]TJ
0 -1.15789 TD
0.025 Tw
(skin, and skin.)Tj
1.26316 -1.1579 TD
0 Tw
(The )Tj
1.82698 0 TD
0.04 Tw
(VEPs represent a mass response of cortical and possible)Tj
-3.09014 -1.1579 TD
0.176 Tw
(subcortical visual areas.)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
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0 0 0 1 k
(8)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 410.4981 301.3825 Tm
0 0 0 1 k
0.176 Tw
(VEPs are employed routinely in the)Tj
-10.66717 -1.15789 TD
-0.004 Tw
(assessment of the functional integrity of the visual pathways, i.e.)Tj
0 -1.1579 TD
0.084 Tw
[(the optic nerve or optic pathways. However)40(, the identity of the)]TJ
0 -1.15789 TD
0.045 Tw
[(cortical generators of pattern )18(VEPs is neither known nor agreed)]TJ
T*
0 Tw
(upon.)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
6.27 0 0 6.27 330.535 260.5175 Tm
0 0 0 1 k
(8)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
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0 0 0 1 k
-0.016 Tw
(Androgen receptors exist in the optic nerve head)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
6.27 0 0 6.27 518.6626 260.5175 Tm
0 0 0 1 k
(14)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 527.1531 257.3825 Tm
0 0 0 1 k
-0.016 Tw
(and the)Tj
-22.94664 -1.1579 TD
0.028 Tw
(primary visual cortex.)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
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0 0 0 1 k
(15)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 401.6942 246.3824 Tm
0 0 0 1 k
0.028 Tw
(Although eyes and optic nerve were not)Tj
-9.74044 -1.15789 TD
0.089 Tw
[(selected for analysis in the study of )55(Adachi et al,)]TJ
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
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0 0 0 1 k
(13)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 510.348 235.3825 Tm
0 0 0 1 k
0.089 Tw
(we inferred)Tj
-21.17768 -1.15789 TD
0.045 Tw
(that delayed P100 latencies in our KD patients were also due to)Tj
0 -1.1579 TD
0.154 Tw
(the accumulation of mutant androgen receptors in the eyes or)Tj
0 -1.15789 TD
0.025 Tw
(optic related pathways. )Tj
1.26316 -1.15789 TD
0.044 Tw
(Abnormal BAEPs were noted in our patients despite the fact)Tj
-1.26316 -1.15789 TD
0.003 Tc
0.372 Tw
(that none of them complained of hearing loss. Brainstem)Tj
0 -1.1579 TD
0 Tc
0.061 Tw
(auditory evoked potential (BAEP) is a simple method to screen)Tj
0 -1.15789 TD
0.162 Tw
[(hearing function and its related brainstem function. )55(Abnormal)]TJ
T*
0.302 Tw
(BAEPs had been reported in patients with KD)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
6.27 0 0 6.27 504.6683 150.5176 Tm
0 0 0 1 k
(7)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 513.0464 147.3825 Tm
0 0 0 1 k
0.302 Tw
(and spinal)Tj
-21.46172 -1.1579 TD
0.299 Tw
(muscular atrophy)Tj
7.20333 0.00001 TD
(.)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
6.27 0 0 6.27 379.9666 139.5175 Tm
0 0 0 1 k
(16 )Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 389.6776 136.3825 Tm
0 0 0 1 k
0.299 Tw
(In KD patients, the main findings were)Tj
-8.47554 -1.15789 TD
0.099 Tw
(increased thresholds, delayed wave I latencies and inconsistent)Tj
T*
0 Tw
(waveforms.)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
6.27 0 0 6.27 353.733 117.5176 Tm
0 0 0 1 k
(7)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 361.8675 114.3825 Tm
0 0 0 1 k
(T)Tj
0.54102 0.00001 TD
0.294 Tw
(one audiometry further proved that these KD)Tj
-6.08918 -1.15791 TD
0.134 Tw
[(patients had mild hearing loss at the higher frequency)65(.)]TJ
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
6.27 0 0 6.27 524.7475 106.5175 Tm
0 0 0 1 k
(7)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 531.5307 103.3825 Tm
0 0 0 1 k
0.134 Tw
(In the)Tj
-23.40744 -1.15789 TD
0.064 Tw
(animal studies, androgen receptor mRNA-containing cells were)Tj
T*
0.283 Tw
(found in the cochlear nuclei, the lateral lemniscus, and the)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F1 1 Tf
9.5 0 0 9.5 71.3877 710.3332 Tm
0 0 0 1 k
0.001 Tc
0.025 Tw
[(T)92(able 2: Evoked potentials of Kennedy\325)37(s disease)]TJ
ET
1 w
0 0 0 1 K
70.388 487.841 m
523.364 487.841 l
S
70.388 723.894 m
523.364 723.894 l
S
0.5 w
70.388 690.894 m
524.34 690.894 l
S
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
8.5 0 0 8.5 71.3877 476.4312 Tm
0 0 0 1 k
0.025 Tw
[(BAEP: brainstem auditory evoked potentials; CCT)50(: central motor conduction time; HL)-239((dB): hearing level (decibels); DR: dif)18(ficult)]TJ
0 -1.29412 TD
[(to reproduce; MEP: motor evoked potentials to transcranial magnetic stimulation; MT)50(: motor threshold; SEP: somatosensory)]TJ
T*
(evoked potentials; )Tj
7.58565 0 TD
(VEP: pattern visual evoked potentials; (): standard deviation; \264: > 3 standard deviation; * : )Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F3 1 Tf
8.5 0 0 8.5 449.0481 454.4312 Tm
0 0 0 1 k
(p)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
8.5 0 0 8.5 455.6356 454.4312 Tm
0 0 0 1 k
0.025 Tw
(value < 0.05. )Tj
ET
/GS2 gs
q
70.388 472.631 m
70.388 691.523 l
523.364 691.523 l
523.364 472.631 l
W n
BT
/F12 1 Tf
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0 g
-0.008 Tc
0 Tw
[(VE)-24(P)]TJ
ET
111.1187 673.7499 33.36 0.72 re
f
BT
/F12 1 Tf
9.84 0 0 9.84 219.1187 675.4299 Tm
0.016 Tc
(BAEP)Tj
ET
180.4787 673.7499 77.52 0.72 re
f
BT
/F12 1 Tf
9.84 0 0 9.84 329.9987 675.4299 Tm
(SEP)Tj
ET
288.4787 673.7499 110.88 0.72 re
f
BT
/F12 1 Tf
9.84 0 0 9.84 468.4787 675.4299 Tm
0.021 Tc
[(ME)5(P)]TJ
ET
432.4787 673.7499 57.6 0.72 re
f
BT
/F12 1 Tf
9.84 0 0 9.84 72.4787 663.4299 Tm
0.005 Tc
[(P)13(a)-24(t)15(i)7(e)1(n)-25(t)-498(P)14(100)-292(\()-4(m)9(s)-7(\))-2931(H)-29(L)-244(\()-3(d)-24(B)13(\))-28(/)15(w)-5(a)1(v)-8(e)-24(f)15(or)-4(m)-2528(P)13(37)-292(\()-4(m)9(s)-7(\))-28(/)15(P)-11(37-)-4(N)-4(45)]TJ
/F13 1 Tf
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0 Tc
Tj
/F12 1 Tf
9.84 0 0 9.84 380.8787 663.4299 Tm
0.267 Tc
[(V)275(\))-3985(M)246(T\()234(%)279(\))258(/)-284(C)257(C)233(T\()234(m)271(s)255(\))]TJ
ET
71.0387 657.1899 506.88 1.44 re
f
BT
/F12 1 Tf
9.84 0 0 9.84 72.4787 648.0699 Tm
0.005 Tc
[(1.)-254(L)-2000(1)1(34)]TJ
6.48 0 0 6.48 125.0387 653.1099 Tm
0 Tc
Tj
9.84 0 0 9.84 183.1187 648.0699 Tm
0.005 Tc
[(85)-5927(D)-4(R)-2200(38.)15(3)-5366(0.)14(9)-5926(70)]TJ
6.48 0 0 6.48 443.5187 653.1099 Tm
0 Tc
Tj
9.84 0 0 9.84 476.6387 648.0699 Tm
0.005 Tc
[(6.)-10(6)]TJ
-39.97561 -1.17074 TD
[(R)-1834(139)]TJ
6.48 0 0 6.48 125.0387 641.5899 Tm
0 Tc
Tj
9.84 0 0 9.84 183.1187 636.5499 Tm
0.005 Tc
[(85)-5927(D)-4(R)-2200(36.)15(7)-5366(0.)14(4)]TJ
6.48 0 0 6.48 374.1587 641.5899 Tm
0 Tc
Tj
9.84 0 0 9.84 432.4787 636.5499 Tm
0.005 Tc
(70)Tj
6.48 0 0 6.48 443.5187 641.5899 Tm
0 Tc
Tj
9.84 0 0 9.84 476.6387 636.5499 Tm
0.005 Tc
[(6.)-10(8)]TJ
-41.07317 -1.17073 TD
[(2.)-254(L)-2000(1)1(05)-5903(100)-5366(D)-4(R)-2200(40.)15(4)-5366(0.)14(8)-5926(65)-3366(6.)-10(9)]TJ
1.09756 -1.17073 TD
[(R)-1834(104)-5902(100)-5366(D)-5(R)-2200(3)1(9.)14(6)-5366(0.)15(7)-5927(60)-3366(6)1(.)-10(7)]TJ
-1.09756 -1.17073 TD
[(3.)-254(L)-2000(1)49(1)-24(0)-5927(85)-5927(D)-4(R)-2200(35.)15(1)-5366(0.)14(8)-5926(60)-3366(7.)-10(0)]TJ
1.09756 -1.17072 TD
[(R)-1834(1)49(1)-24(3)-5927(90)-5927(D)-5(R)-2200(3)1(5.)14(4)-5366(0.)15(8)-5927(65)-3366(6)1(.)-10(7)]TJ
-1.09756 -1.17073 TD
[(4.)-254(L)-2000(1)49(1)-24(0)-5927(90)-5927(D)-4(R)-2200(37.)15(7)-5366(2.)14(0)-5926(45)-3366(6.)-10(5)]TJ
1.09756 -1.14634 TD
1.839 Tc
[(R1)1883(1)1810(5)]TJ
6.48 0 0 6.48 125.0387 572.7099 Tm
0 Tc
Tj
9.84 0 0 9.84 183.1187 567.6699 Tm
0.005 Tc
[(85)-5927(D)-4(R)-2200(37.)15(4)-5366(1.)14(8)-5926(50)-3366(6.)-10(3)]TJ
-11.2439 -1.17072 TD
[(5.)-254(L)-2000(1)1(07)-5903(75)-5927(D)-4(R)-2200(36.)15(6)-5366(0.)14(9)-5926(55)-3366(6.)-10(8)]TJ
1.09756 -1.17073 TD
[(R)-1834(104)-5902(80)-5927(D)-5(R)-2200(3)1(7)-1(.)15(4)-5366(0.)15(8)-5927(50)-3366(7)1(.)-10(0)]TJ
-1.09756 -1.17073 TD
[(6.)-254(L)-2000(1)49(1)-24(4)]TJ
6.48 0 0 6.48 125.0387 538.1499 Tm
0 Tc
Tj
9.84 0 0 9.84 183.5987 533.1099 Tm
0.005 Tc
[(1)49(10)-5366(D)-5(R)-2200(4)1(6.)14(4)]TJ
6.48 0 0 6.48 307.6787 538.1499 Tm
0 Tc
Tj
9.84 0 0 9.84 360.4787 533.1099 Tm
0.005 Tc
[(0.)15(5)]TJ
6.48 0 0 6.48 374.1587 538.1499 Tm
0 Tc
Tj
9.84 0 0 9.84 432.4787 533.1099 Tm
0.005 Tc
[(60)-3366(7.)-10(1)]TJ
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[(R)-1834(109)-5951(1)49(10)-5366(D)-5(R)-2200(4)1(7.)14(2)]TJ
6.48 0 0 6.48 307.6787 526.6299 Tm
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Tj
9.84 0 0 9.84 360.4787 521.5899 Tm
0.005 Tc
[(0.)15(4)]TJ
6.48 0 0 6.48 374.1587 526.6299 Tm
0 Tc
Tj
9.84 0 0 9.84 432.4787 521.5899 Tm
0.005 Tc
[(60)-3366(7.)-10(0)]TJ
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[(M)9(ean)-902(1)49(13)-24(.)14(6)-292(\()-52(1)48(1)-24(.)15(4\))-4(*)-288(91.)15(3)-293(\()-52(1)49(1)-24(.)14(3\))-3(*)-6267(3)1(9)-25(.)15(9)-268(\()-4(3)-24(.)15(9\))-2419(0)1(.)-10(9)-268(\()-4(0)-24(.)14(5)1(\))-4(*)-2532(59)-25(.)15(2)-293(\()-3(7.)-10(9)1(\))-4(*)-850(6)1(.)14(8)-292(\()-4(0.)-10(2)1(\))]TJ
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[(C)-5(o)1(nt)14(r)-3(ol)-408(9)1(8.)14(4)-292(\()-4(4)1(.)14(9\))-3272(70)-268(\()-28(0\))-7199(3)1(8)-25(.)15(0)-268(\()-4(2)-24(.)14(0\))-2418(1)1(.)-10(9)-268(\()-4(0)-24(.)14(4)1(\))-3101(45)-24(.)14(2)-292(\()-4(7.)-10(0)1(\))-1151(6.)15(9)-268(\()-28(0.)15(4\))]TJ
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[(LE JOURNAL)-238(CANADIEN DES SCIENCES NEUROLOGIQUES)]TJ
ET
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/GS2 gs
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[(V)111(olume 34, No. 3 \320 )18(August 2007)]TJ
ET
Q
/GS2 gs
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/F0 1 Tf
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(331)Tj
ET
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/GS2 gs
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(medial geniculate nuclei.)Tj
ET
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/GS2 gs
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q
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/F0 1 Tf
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(17)Tj
ET
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/GS2 gs
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q
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/F0 1 Tf
9.5 0 0 9.5 145.1465 716.0964 Tm
0 0 0 1 k
0.044 Tw
(Mutant androgen receptors had been)Tj
-11.06553 -1.15789 TD
0.063 Tw
(proved in the human pontine nuclei of KD patients.)Tj
ET
Q
/GS2 gs
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q
BT
/F0 1 Tf
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(3)Tj
ET
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(Hence, in)Tj
-21.95121 -1.15789 TD
0.038 Tw
[(KD patients, inconsistent BAEP)-252(waveforms are probably due to)]TJ
0 -1.1579 TD
0.025 Tw
(dysfunction of cochlear nuclei or the 8th cranial nerve.)Tj
1.26316 -1.15789 TD
-0.03 Tw
(Somatosensory evoked potentials are well known as a method)Tj
-1.26316 -1.15789 TD
0.297 Tw
(to assess the function of the dorsal column and its related)Tj
0 -1.1579 TD
0.063 Tw
[(supraspinal pathway)65(. )55(Abnormal SEPs were reported not only in)]TJ
0 -1.15789 TD
0.152 Tw
(our patients but also in the other series.)Tj
ET
Q
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q
BT
/F0 1 Tf
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(3)Tj
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(,4,6,7,18)Tj
ET
Q
/GS2 gs
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BT
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(It is consistent)Tj
-19.76591 -1.15789 TD
0.13 Tw
(with the pathological studies of KD patients in whom there is)Tj
0 -1.1579 TD
0.043 Tw
(loss of myelinated fibers in the fasciculus gracilis and depletion)Tj
0 -1.15789 TD
0.326 Tw
(of neurons in the ventral horn throughout the same spinal)Tj
T*
0 Tw
(segments.)Tj
ET
Q
/GS2 gs
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(1)Tj
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(9)Tj
ET
Q
/GS2 gs
/GS1 gs
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/F0 1 Tf
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0.262 Tw
(It is inferred that the lesion of primary sensory)Tj
-5.14361 -1.1579 TD
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(neurons may produce retrograde degeneration of dorsal column.)Tj
ET
Q
/GS2 gs
/GS1 gs
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BT
/F0 1 Tf
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0 0 0 1 k
(4)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 52.024 573.0965 Tm
0 0 0 1 k
0.117 Tw
[(So far)40(, there were only six )17(TMS studies of KD patients. In)]TJ
-1.26316 -1.15789 TD
0.034 Tw
[(1992, )18(TMS was first applied in 12 KD patients and showed that)]TJ
T*
0.272 Tw
(they all had normal central motor conduction time.)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
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0 0 0 1 k
(6)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 257.5935 551.0964 Tm
0 0 0 1 k
(Similar)Tj
-22.90205 -1.15789 TD
0.203 Tw
[(normal results were reported in another four cases later)55(.)]TJ
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
6.27 0 0 6.27 266.7498 543.2314 Tm
0 0 0 1 k
(7)Tj
0.5 -0.00001 TD
(,18,20)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 40.024 529.0964 Tm
0 0 0 1 k
0.057 Tw
[(However)40(, Sperfeld et al)]TJ
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
6.27 0 0 6.27 131.4789 532.2315 Tm
0 0 0 1 k
(3)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 136.5409 529.0964 Tm
0 0 0 1 k
0.057 Tw
[(had done )18(TMS in 18 KD patients with)]TJ
-10.15967 -1.15789 TD
-0.021 Tw
[(dif)18(ferent results. Six of them had prolonged onset latencies to the)]TJ
0 -1.1579 TD
0.125 Tw
[(tar)18(get muscle of abductor pollicis brevis. In these six patients,)]TJ
T*
0.284 Tw
(four of them had prolonged central motor conduction time.)Tj
0 -1.15789 TD
0.067 Tw
(Although most pathological studies of KD patients did not find)Tj
0 -1.1579 TD
0.104 Tw
(any abnormal findings of the motor cortex or the corticospinal)Tj
T*
0 Tw
(tracts,)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
6.27 0 0 6.27 62.9669 466.2315 Tm
0 0 0 1 k
(19,21,22)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 87.9709 463.0965 Tm
0 0 0 1 k
0.072 Tw
(changes of corticospinal tract were reported in two)Tj
-5.04704 -1.15789 TD
-0.0001 Tc
(KD)Tj
1.79231 0 Td
0 Tc
0.099 Tw
(patients by Shaw et al)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
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0 0 0 1 k
(23)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
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0 0 0 1 k
0.099 Tw
[(in 1998. )18(This could be the reason)]TJ
-11.97036 -1.1579 TD
0.02 Tc
0.378 Tw
(why some of KD patients had prolonged central motor)Tj
0 -1.15789 TD
0 Tc
-0.025 Tw
(conduction time.)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
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0 0 0 1 k
(3,24)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 116.2168 430.0965 Tm
0 0 0 1 k
-0.025 Tw
[(Although increased MT)-207(was the only finding)]TJ
-8.02029 -1.15789 TD
-0.028 Tw
(in our series, Pachatz et al)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
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0 0 0 1 k
(24)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 146.2706 419.0965 Tm
0 0 0 1 k
-0.028 Tw
(found that all the parameters of MEP)Tj
-11.18385 -1.1579 TD
0.002 Tc
0.373 Tw
(could be altered in KD patients, such as decreased MEP)Tj
0 -1.15789 TD
0 Tc
0.022 Tw
(amplitudes, prolonged central motor conduction time, decreased)Tj
0 -1.1579 TD
0.314 Tw
(cortical silent period, reduced intracortical inhibition during)Tj
T*
-0.008 Tw
(interstimuli interval of 1-6 ms. Using proton magnetic resonance)Tj
0 -1.15789 TD
0.099 Tw
(spectroscopy (MRS), reduced metabolic activities were note in)Tj
0 -1.1579 TD
-0.011 Tw
(the motor regions and indicated neuronal dysfunction and gliosis)Tj
T*
-0.012 Tw
(in the area.)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
6.27 0 0 6.27 81.7289 345.2315 Tm
0 0 0 1 k
(25)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 90.2608 342.0965 Tm
0 0 0 1 k
-0.012 Tw
[(It is inferred that the gene ef)18(fect of the trinucleotide)]TJ
-5.28808 -1.15789 TD
0.02 Tc
0.444 Tw
(repeat mutation in KD makes the brain susceptible to)Tj
0 -1.1579 TD
0 Tc
0.212 Tw
(degeneration and subsequent development of reactive gliosis,)Tj
T*
0.065 Tw
(even though there are no pathological structural findings.)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
6.27 0 0 6.27 261.6985 312.2315 Tm
0 0 0 1 k
(25)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 270.7904 309.0965 Tm
0 0 0 1 k
(The)Tj
-24.2912 -1.15789 TD
0.254 Tw
(correlations were not significant between functional findings)Tj
T*
0.095 Tw
(\(TMS and MRS\) and clinical parameters \(age at onset, disease)Tj
0 -1.1579 TD
0.025 Tw
(duration, or CAG-repeat length\).)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
6.27 0 0 6.27 165.5006 279.2314 Tm
0 0 0 1 k
(3,25)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 52.024 265.0965 Tm
0 0 0 1 k
0.004 Tc
0.371 Tw
(Based upon the current concept, the main pathological)Tj
-1.26316 -1.15789 TD
0.007 Tc
0.368 Tw
(findings of KD were at the spinal cord and brainstem.)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
6.27 0 0 6.27 279.2437 257.2315 Tm
0 0 0 1 k
0.007 Tc
(21)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 40.024 243.0965 Tm
0 0 0 1 k
0.229 Tw
[(However)40(, a few reports showed that KD might also involve)]TJ
0 -1.15789 TD
0.272 Tw
(cerebral hemispheres. In the case of Shaw et al,)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
6.27 0 0 6.27 241.0968 235.2315 Tm
0 0 0 1 k
(23)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 252.3262 232.0965 Tm
0 0 0 1 k
(neuronal)Tj
-22.3476 -1.15789 TD
0.037 Tw
(depletion and gliosis in the hippocampus and subcortical gliosis)Tj
0 -1.1579 TD
0.352 Tw
(in the prefrontal region were noted in a KD patient with)Tj
0 -1.15789 TD
0.002 Tc
0.373 Tw
(cognitive change. Immunostaining for macrophage markers)Tj
T*
0 Tc
0.058 Tw
(showed evidence for subtle corticospinal tract pathology in two)Tj
0 -1.1579 TD
-0.028 Tw
(KD patients.)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
6.27 0 0 6.27 87.7746 180.2314 Tm
0 0 0 1 k
(23)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 96.1576 177.0965 Tm
0 0 0 1 k
-0.028 Tw
(Further evidence is provided by case report of two)Tj
-5.9088 -1.15789 TD
0.102 Tw
(KD patients, one with dementia and frontal lobe atrophy)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
6.27 0 0 6.27 262.2309 169.2315 Tm
0 0 0 1 k
(26)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 271.8435 166.0965 Tm
0 0 0 1 k
(and)Tj
-24.40205 -1.15789 TD
0.014 Tc
0.361 Tw
(another with parkinsonism.)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
6.27 0 0 6.27 154.1214 158.2315 Tm
0 0 0 1 k
0.014 Tc
(27)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 166.3406 155.0965 Tm
0 0 0 1 k
0.014 Tc
0.361 Tw
(These reports indicated that)Tj
-13.29648 -1.1579 TD
0.02 Tc
0.873 Tw
(atypical pathogenetic linkage may produce unusual)Tj
0 -1.15789 TD
0 Tc
0.025 Tw
(manifestation in KD patients.)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
6.27 0 0 6.27 152.5912 136.2315 Tm
0 0 0 1 k
(26)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 52.024 122.0965 Tm
0 0 0 1 k
0.306 Tw
(Since KD is a systemic and slowly progressive disease,)Tj
-1.26316 -1.1579 TD
0.205 Tw
(sensory dysfunction develops so insidiously that KD patients)Tj
0 -1.15789 TD
0.127 Tw
(usually neglect their sensory problems. It is in part the reason)Tj
T*
0.141 Tw
(why EPs fail to establish any specific correlation between the)Tj
0 -1.1579 TD
0.019 Tc
0.356 Tw
(pattern of abnormalities and that of clinical deficits.)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
6.27 0 0 6.27 270.455 81.2314 Tm
0 0 0 1 k
0.019 Tc
(28-31)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 309.16 717.0228 Tm
0 0 0 1 k
0.001 Tw
[(However)40(, electrophysiological studies have proved that KD may)]TJ
0 -1.15789 TD
0.108 Tw
[(produce subclinical EP)-321(abnormalities and is characterized with)]TJ
T*
0.162 Tw
[(widespread involvement of the nervous system. )55(Abnormal EP)]TJ
0 -1.1579 TD
0.1 Tw
(findings are not evidence against the diagnosis of KD. If there)Tj
0 -1.15789 TD
0.083 Tw
(are potential treatments for KD patients in the future, EPs may)Tj
-0.0001 Tc
(be)'
1.21873 0 Td
0 Tc
0.025 Tw
(useful in the clinical follow-up. )Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F1 1 Tf
9.5 0 0 9.5 309.16 640.0228 Tm
0 0 0 1 k
(A)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F1 1 Tf
7.125 0 0 7.125 316.0183 640.0228 Tm
0 0 0 1 k
0.001 Tc
(CKNOWLEDGEMENT)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
9.5 0 0 9.5 321.16 625.0228 Tm
0 0 0 1 k
0.206 Tw
[(W)80(e would like to thank Miss )18(WY)-419(Shen for the statistical)]TJ
-1.26316 -1.15789 TD
0.013 Tw
[(analysis and Mr)55(. Matt Nicodemus for English editing. )18(The study)]TJ
0 -1.1579 TD
0.096 Tw
[(was in part supported by )18(T)70(aipei )17(VGH grant (93-282) and NSC)]TJ
0 -1.15789 TD
0.025 Tw
(grant (94-2314-B-075-022).)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F1 1 Tf
9.5 0 0 9.5 309.16 570.0228 Tm
0 0 0 1 k
(R)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F1 1 Tf
7.125 0 0 7.125 316.0183 570.0228 Tm
0 0 0 1 k
0.001 Tc
(EFERENCES)Tj
ET
Q
/GS2 gs
/GS1 gs
q
BT
/F0 1 Tf
8.5 0 0 8.5 309.16 557.0228 Tm
0 0 0 1 k
0.242 Tw
[(1.)-1132(Harding )54(AE, )18(Thomas PK, Baraitser M, Bradbury PG, Mor)18(gan-)]TJ
3.05882 -1.05882 TD
0.018 Tc
0.357 Tw
(Hughes JA, Ponsford JR. X-linked recessive bulbospinal)Tj
T*
0.009 Tc
0.365 Tw
[(neuronopathy: a report of ten cases. J Neurol Neurosur)18(g)]TJ
T*
0 Tc
0.025 Tw
[(Psychiatry)65(. 1982;45\(1)37(1\):1012-9.)]TJ
-3.05882 -1.05882 TD
0.24 Tw
[(2.)-1132(LaSpada )54(AR, Roling DB, Harding )55(AE, )18(W)80(arner CL, Spiegel R,)]TJ
3.05882 -1.05882 TD
0.02 Tc
0.435 Tw
(Hausmanowa-Petrusewicz I, et al. Meiotic stability and)Tj
T*
0 Tc
0.06 Tw
(genotype-phenotype correlation of the trinucleotide repeat in x-)Tj
T*
0.02 Tc
0.452 Tw
[(linked spinal and bulbar muscular atrophy)65(. Nat Genet.)]TJ
0 -1.05883 TD
0 Tc
0 Tw
(1992;2(4):301-4.)Tj
-3.05882 -1.05882 TD
(3.)Tj
1.88235 0 Td
0.205 Tw
[(Sperfeld )55(AD, Karitzky J, Brummer D, Schreiber H, Haussler J,)]TJ
1.17647 -1.05882 TD
-0.028 Tw
[(Ludolph )55(AC, et al. X-linked bulbospinal neuronopathy: Kennedy)]TJ
T*
0.025 Tw
[(disease. )55(Arch Neurol. 2002;59(12):1921-6.)]TJ
-3.05882 -1.05882 TD
(4.)Tj
1.88235 0 Td
-0.031 Tw
[(Anannontsak )55(A, Massakulpan P)111(, )55(Aksaranugraha S, Phanthumchinda)]TJ
1.17647 -1.05882 TD
0.005 Tc
0.37 Tw
(K. Somatosensory evoked potentials in X-linked recessive)Tj
T*
0 Tc
0.012 Tw
(bulbospinal neuronopathy: a case demonstration. Electromyogra)Tj
T*
0.025 Tw
(Clin Neurophysiol. 1999;39(7):393-6.)Tj
-3.05882 -1.05882 TD
(5.)Tj
1.88235 0 Td
0.189 Tw
[(Antonini G, Gragnani F)80(, Romaniello )55(A, Pennisi EM, Morino S,)]TJ
1.17647 -1.05882 TD
0.019 Tw
[(Ceschin )18(V)129(, et al. Sensory involvement in spinal-bulbar muscular)]TJ
T*
0.025 Tw
[(atrophy \(Kennedy\325)55(s disease\). Muscle Nerve. 2000;23\(2\):252-8. )]TJ
-3.05882 -1.05882 TD
(6.)Tj
1.88235 0 Td
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