63ournalof N e urolog y , N e uros urg e ry , and P s y c h i at ry 1 9 9 6; 61 : 638 - 64 3

B e h av i ouraland e le c t rop h y s i olog i c alc h rom at i c

and ac h rom at i c c ont ras t s e ns i t i v i t y i nan
ac h rom at op s i c p at i e nt
C h arle s A H e y w ood , J uli anJ N i c h olas , AlanC ow e y
Ab s t rac t
O b j e c t i v e s - I nc as e s of i nc om p le t e ac h ro-
m at op s i ai t i s unc le arw h e t h e rre s i d ual
v i s ualf unc t i oni s m e d i at e d b y i nt ac t s t ri -
at e c ort e x orre s ult s f rom i nc om p le t e
le s i ons t oe x t ras t ri at e c ort i c alv i s ual
are as . A p at i e nt w i t h c om p le t e c e re b ral
ac h rom at op s i aw as t e s t e d t oe s t ab li s h t h e
nat ure of h i s re s i d ualv i s i onand t od e t e r-
m i ne t h e i nt e g ri t y of s t ri at e c ort e x f unc -
t i on.
Me t h od s - B e h av i ouralc ont ras t s e ns i t i v -
i t y , us i ng t h e m e t h od of ad j us t m e nt , and
av e rag e d v i s ually e v ok e d c ort i c alp ot e n-
t i als w e re m e as ure d t os i nus oi d ally m od -
ulat e d c h rom at i c and ac h rom at i c
g rat i ng s i nanac h rom at op s i c p at i e nt and
anorm alob s e rv e r. Ey e m ov e m e nt s w e re
m e as ure d i nt h e p at i e nt us i ng aS k alar
i nf rare d m oni t ori ng s y s t e m .
Re s ult s - Th e p at i e nt ' s c h rom at i c c on-
t ras t s e ns i t i v i t y w as norm al, i nd i c at i ng
t h at d e s p i t e h i s d e ns e c olourb li nd ne s s h i s
oc c i p i t alc ort e x s t i llp roc e s s e d i nf orm a-
t i onab out s p at i alv ari at i ons i nh ue . H i s
s e ns i t i v i t y t oac h rom at i c g rat i ng s w as
d e p re s s e d p art i c ularly at h i g h s p at i alf re -
q ue nc i e s , p os s i b y b e c aus e of h i s j e rk ny s -
t ag m us . Th e s e b e h av i ouralre s ult s w e re
re i nf orc e d b y t h e nat ure of v i s ually
e v ok e d re s p ons e s t oc h rom at i c and
ac h rom at i c g rat i ng s , i nw h i c h t ot alc olour
b li nd ne s s c oe x i s t e d w i t h analm os t nor-
m alc ort i c alp ot e nt i alt oi s olum i nant
c h rom at i c g rat i ng s .
C onc lus i ons - Th e re s ult s s h ow t h at i nf or-
m at i onab out c h rom at i c c ont ras t i s p re -
s e nt i ns om e c ort i c alare as , and c od e d i n
ac olour- op p one nt f as h i on, i nt h e ab s e nc e
of any p e rc e p t uale x p e ri e nc e of c olour.
De p art m e nt of
Ex p e ri m e nt al
P s y c h olog y , U ni v e rs i t y
of O x f ord , S out h P ark s
Road , O x f ord
O XI 3U D, U K
J J N i c h olas
A C ow e y
De p art m e nt of
P s y c h olog y , S c i e nc e
L ab orat ori e s , S out h
Road , Durh am
DH 1 3L E, U K
C A H e y w ood
C orre s p ond e nc e t o:
DrC A H e y w ood ,
De p art m e nt of P s y c h olog y ,
U ni v e rs i t y of Durh am ,
S c i e nc e L ab orat ori e s , S out h
Road , Durh am , DH 1 3L E,
U K.
( 7 N e urolN e uros urg P s y c h i at ry 1 9 9 6; 61 : 638 - 64 3)
Ke y w ord s : ac h rom at op s i a; c h rom at i c c ont ras t s e ns i t i v -
i t y ; v i s uale v ok e d p ot e nt i als
C e re b ralac h rom at op s i ai s as e v e re d i s t ur-
b anc e of c olourv i s i onc aus e d b y v e nt rom e d i al
oc c i p i t alb rai nd am ag e . - 3Th e i m p ai rm e nt i s
not , i nd i re c t ly , of re t i nalori g i nas m e as ure -
m e nt of t w oc olouri nc re m e nt alt h re s h old s 4
s h ow s i nt ac t t ri c h rom at i c m e c h ani s m s 5 and
t h e c h arac t e ri s t i c s h ap e of t h e s p e c t rals e ns i t i v -
i t y f unc t i oni nd i c at e s re s i d ualc olourop p one nt
p roc e s s i ng . 6At ne c rop s y t h e b rai ns of ac h ro-
m at op s i c p at i e nt s i nv ari ab ly s h ow d am ag e i n
t h e re g i onof t h e li ng ualand c aud alf us i f orm
g y rus i nt h e v e nt rom e d i alas p e c t of t h e oc c i p i -
t allob e s . 7 P os i t rone m i s s i ont om og rap h y s t ud -
i e s i nnorm als ub j e c t s s h ow anare ai nt h e
s am e re g i on, w h e re t h e re i s as e le c t i v e i nc re as e
i nre g i onalb lood f low w h e ns ub j e c t s p as s i v e ly
v i e w ac oloure d Mond ri anp at t e rn. 8 H ow e v e r,
ot h e rs t ud i e s i nv olv i ng f unc t i onalne uroi m ag -
i ng b y P ET s ug g e s t t h at t h e p roc e s s i ng of
c olouri s not re s t ri c t e d t oas i ng le e x t ras t ri at e
v i s ualare a. 9 1 0 S uc h f i nd i ng s h av e le d t ot h e
h y p ot h e s i s t h at c e re b ralac h rom at op s i are s ult s
f rom d am ag e t oone orm ore e x t ras t ri at e v i s ual
are as w h i c h are s p e c i ali s e d f ort h e p roc e s s i ng
of c olour. 3' 1
Analt e rnat i v e h y p ot h e s i s , w h i c h s t e m s f rom
s t ud i e s of m onk e y s , ' 2 i s t h at ac h rom at op s i a
c anre s ult f rom d am ag e any w h e re along a
c h anne lof p roc e s s i ng t h at ori g i nat e s i nt h e
c olourop p one nt P B re t i nalg ang li onc e lls t h at
i nne rv at e anat om i c ally and f unc t i onally s e g re -
g at e d re g i ons of s t ri at e c ort e x ( V 1 ) v i at h e p ar-
v oc e llularlay e rs of t h e d ors allat e ralg e ni c ulat e
nuc le us ( d L G N ) . Th i s p at h w ay , c alle d t h e P
c h anne l, t h e nb e c om e s e lab orat e d i ns om e
e x t ras t ri at e c ort i c alare as ( V 2 and V 4 ) w h i c h
p roj e c t t oi nf e ri orp ort i ons of t h e t e m p oral
lob e . Th i s p at h w ay c anb e d i s t i ng ui s h e d f rom
i t s p art ne r, t h e M c h anne l, w h i c h ari s e s f rom
P re t i nalg ang li onc e lls t h at p roj e c t t om ag no-
c e llularlay e rs of t h e d L G N w h i c h , i nt urn,
p roj e c t t od i s t i nc t re g i ons of V lw h i c h c on-
t i nue t h i s s t re am of p roc e s s i ng i nt ot h e p ari e t al
lob e v i ac ort i c alare as V 2 , V 3, and MT, as w e ll
as , le s s i nt e ns i v e ly , i nt ot h e t e m p orallob e .
Th e c ort i c als e g re g at i onof t h e P and M c h an-
ne ls h as b e e ns h ow nw i t h c y t oc h rom e ox i d as e
s t ai ni ng , w h e nt h e P c h anne li s as s oc i at e d w i t h
t h e c y t oc h rom e ox i d as e ri c h " b lob s " and
" i nt e rb lob s " of V l, and t h e t h i nand p ale
i nt e rs t ri p e s of V 2 . Th e M c h anne li s p ri m ari ly
re s t ri c t e d t olay e r4 B of V i and t h e t h i c k
s t ri p e s of V 2 . Re s p ons e p rop e rt i e s of s i ng le
ne urons along t h e t w op at h w ay s are c ons i s t e nt
w i t h t h e not i onof af unc t i onals e g re g at i on
s uc h t h at t h e P c h anne li s c onc e rne d w i t h p ro-
c e s s i ng c h rom at i c i nf orm at i oni nad d i t i ont o
h i g h s p at i aland low t e m p oralf re q ue nc i e s .
C e lls ont h e M c h anne lare e s p e c i ally re s p on-
s i v e t om ot i onand are m ore s e ns i t i v e t h an
t h os e of t h e P c h anne lt olow c ont ras t .
S e ns i t i v i t y t ot h e ori e nt at i onof av i s uals t i m u-
lus i s not ane x c lus i v e p rop e rt y of e i t h e rp at h -
w ay and h e nc e i t m ay b e s up p os e d t h at
i nf orm at i onab out f orm i s c arri e d b y b ot h
p at h w ay s .
I f ac h rom at op s i ai s t h e re s ult of s e le c t i v e
d i s t urb anc e of t h e P c h anne lb e f ore i t s i nne r-
v at i onof s t ri at e c ort e x t h e ni t s h ould b e
638
group.bmj.com on January 15, 2014 - Published by jnnp.bmj.com Downloaded from
H e y w ood , N i c h olas , C ow e y
ac c om p ani e d b y i m p ai rm e nt s of t h e d i s c ri m i -
nat i onof f orm , t e x t ure , and d ot p os i t i on.
N e urot ox i c le s i ons c onf i ne d t ot h e p arv oc e llu-
larlay e rs of t h e d L G N re s ult i s s e v e re d i s rup -
t i onof t h e p roc e s s i ng of t h e s e , orc om p arab le ,
at t ri b ut e s . ' 2 Th e d e m ons t rat i ont h at ac h ro-
m at op s i ac us t om ari ly e x i s t s i nt h e ab s e nc e of
s uc h ac c om p any i ng d i s ord e rs arg ue s ag ai ns t
t h i s v i e w .
Ac h rom at op s i c p at i e nt s p e rf orm p oorly ,
e v e nrand om ly , ont h e F arns w ort h - Muns e ll
1 0 0 h ue t e s t , w h i c h re q ui re s t h e ord e ri ng of
s om e i s olum i nant c oloure d c h i p s ont h e b as i s
of t h e i rc h rom at i c i t y . H ow e v e r, t h e s e v e ri t y of
t h e c olourd i s t urb anc e i s v ari ab le and m any
p at i e nt s are d y s c h rom at op s i c - f ore x am p le ,
s h ow i ng are lat i v e ly g re at e rd i s t urb anc e f or
b lue / g re e ns t h anf orre d s ' 1 31 4 and s om e p re s e r-
v at i onof c olournam i ng . Th e ab i li t y t onam e
f i g ure s ort rac e t h e f i g ure s e m b e d d e d i nt h e
I s h i h arap s e ud oi s oc h rom at i c p lat e s i s als o
v ari ab le . S om e p at i e nt s f ai lt oi d e nt i f y any of
t h e f i g ure s ' 5- ' 7 w h e re as ot h e rs i d e nt i f y m any of
t h e m . ' ' 4 I t h as b e e np rop os e d t h at t h e s e v e ri t y
of c e re b ralac h rom at op s i ai s re lat e d t ot h e
e x t e nt t ow h i c h t h e s t ri at e c ort e x , p art i c ularly
i t s c h rom at i c c om p art m e nt s , h as b e e nc om -
p rom i s e d . ' 4 Th e lat t e raut h ors re p ort ap at i e nt
i nw h om p oorp e rf orm anc e of c olourord e ri ng
and i d e nt i f i c at i onw as ac c om p ani e d b y norm al
c olourc ont ras t s e ns i t i v i t y , i nt ac t ac ui t y , a
c le arv i s i b le v i s uale v ok e d p ot e nt i alt oani s o-
lum i nant c h rom at i c c h e q ue rb oard , and an
i nt ac t ab i li t y t od e t e c t c olourd i f f e re nc e s i nan
od d i t y t as k . More ov e r, e i g h t out of ni ne
I s h i h arap lat e s w e re i d e nt i f i e d . Th e y c on-
c lud e d t h at re s i d ualc h rom at i c ab i li t i e s w e re
m e d i at e d b y i nt ac t s t ri at e c ort e x . Analt e rna-
t i v e e x p lanat i on3i s t h at t h e le s i ont ot h e c ru-
c i alare as of t h e li ng ualand f us i f orm g y ri i s
i nc om p le t e . Th i s i nt e rp re t at i onw ould b e
s t re ng t h e ne d b y e x am i nat i onof t h e i nt e g ri t y of
f unc t i onof t h e s t ri at e c ort e x i nap at i e nt w i t h
c om p le t e ac h rom at op s i a, w h os h ould lac k a
norm al, ore v e nany , v i s ually e v ok e d p ot e nt i al
i nre s p ons e t oi s olum i nant s i nuos oi d ally m od -
ulat e d c h rom at i c g rat i ng s . I t i s t h e re s ult s of
s uc h ane x am i nat i ont h at w e re p ort h e re .
C as e h i s t ory
Th i s h as b e e np ub li s h e d i nd e t ai le ls e w h e re 6
1 8 1 9
and i s s um m ari s e d h e re . Th e p at i e nt i s a4 4
y e arold m an, w h ow as f i t and w e llunt i lh e
h ad i d i op at h i c e nc e p h ali t i s as a2 2 y e arold
p oli c e c ad e t . As are s ult , h e h ad p e rm ane nt
s e v e re b i lat e ralc e re b rald am ag e . H e h as s e v e re
ob j e c t ag nos i a, c annot re c og ni s e f am i larf ac e s
and h as t op og rap h i c d i s ori e nt at i onand ad i s -
t urb anc e i ns e m ant i c m e m ory . Rout i ne p h y s i c al
e x am i nat i ons h ow e d norm alS ne lle nv i s ual
ac ui t y i ne ac h e y e and ad e ns e le f t h om ony -
m ous h e m i anop i aw i t h m ac ulars p ari ng .
B e f ore h i s i llne s s h e h ad norm alc olourv i s i on
as as s e s s e d b y t h e I s h i h arap lat e s . S i nc e h i s i ll-
ne s s h e i s unab le t om at c h ornam e c olours
p re s e nt e d i nh i s re m ai ni ng v i s ualh e m i f i e ld .
H i s c olourord e ri ng ont h e F arns w ort h -
Muns e ll1 0 0 h ue t e s t i s rand om , y i e ld i ng an
e rrors c ore of 1 2 4 5.
B rai nMRI s h ow s t h e e x t e nt of t h e d am ag e . 6
Th e re i s e x t e ns i v e v e nt rom e d i ald am ag e i n
b ot h h e m i s p h e re s w h i c h i nc lud e s t h e li ng ual
and f us i f orm g y ri . I nt h e ri g h t h e m i s p h e re t h e
s e c ond , t h i rd , and f ourt h t e m p oralg y ri are
c om p le t e ly d e s t roy e d as i s t h e p ole of t h e t e m -
p orallob e . Th e re i s als od am ag e t ot h e
p arah i p p oc am p alg y rus . Th e re i s c ons i d e rab le
d am ag e t ot h e oc c i p i t allob e w i t h s p ari ng of
t h e c aud alt i p of t h e c alc ari ne c ort e x ( t h i s
c ould e x p lai nt h e m ac ulars p ari ng i nh i s le f t
v i s ualf i e ld ) . I nt h e le f t h e m i s p h e re t h e p ole of
t h e t e m p orallob e , t h e p arah i p p oc am p aland
t h e f ourt h t e m p oralg y ri are t ot ally d e s t roy e d
as i s t h e are aof t h e m e s i aloc c i p i t ot e m p oral
j unc t i on. Th e d ors alp art of b ot h h e m i s p h e re s
s h ow s re lat i v e ly li t t le d am ag e c om p are d w i t h
t h e v e nt ralp art . I nt h e ri g h t h e m i s p h e re t h e
w h i t e m at t e rund e rt h e i nf e ri orh alf of t h e i nf e -
ri orp ari e t allob e i s d am ag e d . I nt h e le f t h e m i -
s p h e re t h e re i s noe v i d e nc e of d am ag e e i t h e ri n
t h e w h i t e ort h e g re y m at t e rof t h e p ari e t al
lob e .
Me t h od s
C O N TRAS T S EN S I TI V I TY
H ori z ont als i ne w av e g rat i ng s , s ub t e nd i ng
50
x 5 of v i s ualang le f rom av i e w i ng d i s -
t anc e of 1 m , w e re p re s e nt e d i nt h e c e nt re of a
h i g h re s olut i on( Ei z o, 1 0 2 4 x 1 0 2 4 p i x e ls ,
f ram e rat e 1 0 0 H z ) , d i s p lay , w h i c h s ub t e nd e d
2 2 0 x 1 60 of v i s ualang le . G am m ac orre c t i on,
t oe q uat e t h e i s olum i nant rat i oof t h e re d and
g re e ng uns , w as c arri e d out i ns of t w are i n
w h i c h lum i nanc e w as m e as ure d w i t h a
Mi nolt alum i nanc e m e t e rL S - 1 1 0 . I s o-
lum i nant c h rom at i c g rat i ng s w e re d i s p lay e d b y
m od ulat i ng t h e re d and g re e ng uns i n
ant i p h as e w i t h 1 2 b i t p re c i s i onag ai ns t auni -
f orm y e llow b ac k g round of t h e s am e m e an
lum i nanc e ( 33C / d M2 ) . Ac h rom at i c ( s t ri c t ly
s p e ak i ng , m onoc h rom at i c ) y e llow / b lac k g rat -
i ng s w e re d i s p lay e d b y m od ulat i ng t h e g uns i n
p h as e . Ac h rom at i c c ont ras t i s d e f i ne d as t h e
d i f f e re nc e b e t w e e nt h e m ax i m um and m i ni -
m um lum i nanc e of t h e g rat i ng , d i v i d e d b y
t h e i rs um . C h rom at i c c ont ras t i s d e f i ne d as
e q ualt ot h e lum i nanc e c ont ras t of e ac h c on-
s t i t ue nt g rat i ng , i f t h e t w og rat i ng s are m ono-
c h rom at i c and of e q ualc ont ras t .
Me as ure m e nt s of c ont ras t t h re s h old s w e re
m ad e us i ng t h e m e t h od of ad j us t m e nt i nt h e
p at i e nt and anag e / s e x m at c h e d c ont rols ub -
j e c t w h oh ad norm alc olourv i s i onand a
S ne lle nac ui t y of 6/ 6. Th e p roc e d ure w as as
f ollow s . Th e s ub j e c t s at i nad ark e ne d room ,
t ow h i c h h e w as ad ap t e d , f ac i ng t h e s c re e n
and w as i ns t ruc t e d t om ai nt ai nf i x at i onona
s m allc ros s w h i c h w as p e rm ane nt ly d i s p lay e d .
A b ri e f t one i nd i c at e d t h e ap p e aranc e of a
g rat i ng , p re s e nt e d at 1 0 0 % c ont ras t i m m e d i -
at e ly t ot h e ri g h t of t h e f i x at i onp oi nt . Th i s
e ns ure d t h at t h e g rat i ng w as not p re s e nt e d i n
t h e le f t h e m i anop i c f i e ld of t h e p at i e nt . and
t h e re f ore s ub t e nd e d t h e s am e v i s i b le v i s ual
ang le i nt h e ri g h t h e m i f i e ld of b ot h s ub j e c t s .
B y ad j us t i ng ah and h e ld s w i t c h t h e s ub j e c t
c ould d e c re as e t h e c ont ras t i ns t e p s of 0 9
t i m e s t h e c urre nt c ont ras t , ori nc re as e i t b y a
639
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B e h av i ouraland e le c t rop h y s i olog i c alc h rom at i c and ac h rom at i c c ont ras t s e ns i t i v i t y i nanac h rom at op s i c p at i e nt
f ac t orof 1 1 . Th e s ub j e c t w as as k e d t om ak e
t h e ad j us t m e nt unt i lt h e g rat i ng b e c am e i nv i s -
i b le . G rat i ng s w e re p re s e nt e d at s e v e rals p at i al
f re q ue nc i e s ( s i ne w av e m od ulat e d g rat i ng
re v e rs al) . F ore ac h c ond i t i onof s t at i c or
d y nam i c g rat i ng s , t h e s p at i alf re q ue nc y of t h e
g rat i ng w as rand om ly s e le c t e d f rom t h e s t i m u-
lus s e t f rom t ri alt ot ri al. At e ac h s p at i alf re -
q ue nc y t h re e e s t i m at e s of t h re s h old w e re
m ad e s uc h t h at ont h e s e c ond and t h i rd p re -
s e nt at i ons t h e i ni t i alc ont ras t w as e i t h e rt w o
ort h re e t i m e s h i g h e rt h ant h e t h re s h old d e t e r-
m i ne d ont h e p re v i ous p re s e nt at i on- t h at i s ,
t h e g rat i ng i s alw ay s v i s i b le at t h e b e g i nni ng of
t h e s e ri e s . S e ns i t i v i t y t os t at i onary c h rom at i c
g rat i ng s of 0 - 5, 1 , 2 , 4 , 8 , and 1 0 c y c le s / !
w as m e as ure d . F orac h rom at i c m e as ure m e nt s
anad d i t i onalf re q ue nc y of 1 6c y c le s / ! w as
us e d .
V I S U AL EV O KED P O TEN TI AL S
V i s uale v ok e d p ot e nt i als ( V EP s ) w e re
re c ord e d i nre s p ons e t ore v e rs i ng ac h rom at i c
and c h rom at i c s i ne w av e g rat i ng s i nt h e
p at i e nt and t h e c ont rol. Th e g rat i ng s s ub -
t e nd e d 50 x 50 , w e re v i e w e d f rom ad i s t anc e
of 9 0 c m , and w e re p rod uc e d b y aP lut oI I
g rap h i c s d e v i c e c onne c t e d t oaP C and d i s -
p lay e d onaMi c rov i t e c m oni t or( 7 68 x 57 6
p i x e ls , 50 H z f ram e rat e ) . Th e re d and g re e n
g uns w e re m od ulat e d w i t h 8 b i t p re c i s i onand
g am m ac orre c t i onw as ag ai nc arri e d out i n
s of t w are . C h rom at i c and ac h rom at i c g rat i ng s
w e re c ons t ruc t e d b y m od ulat i ng t h e re d and
g re e ng uns i nant i p h as e and i np h as e re s p e c -
t i v e ly . Th e m e ans c re e nlum i nanc e w as m ai n-
t ai ne d at 1 3- 2 C / d M2 . Toe ns ure t h at t h e
g rat i ng s w e re i s olum i nant t ot h e p at i e nt , t h e
p e ak lum i nanc e s of t h e re d and g re e ng uns
w e re s e le c t e d ont h e b as i s of f li c k e rp h ot om e t ry
and w e re s h ow nt ob e i nd i s t i ng ui s h ab le t ot h e
p at i e nt i nat h re e c h oi c e od d i t y t as k 6. Ev ok e d
p ot e nt i als w e re re c ord e d i nre s p ons e t o6- 2 5
H z ( 1 2 - 5 re v e rs als / s ) p at t e m re v e rs alof g rat -
i ng s of 0 5, 1 , 2 , and 4 c y c le s / ! e ac h at ac on-
t ras t of 0 - 4 8 .
S t e ad y s t at e e v ok e d p ot e nt i als w e re
re c ord e d f rom 9 m m Ag / Ag C lc up e le c t rod e s .
Th e s ourc e and re f e re nc e e le c t rod e s w e re
p lac e d i nt h e m i d li ne 5 c m ab ov e t h e i ni onand
1 2 c m ab ov e t h e nas i onre s p e c t i v e ly , and a
f ore h e ad e le c t rod e ac t e d as t h e s ub j e c t ' s e art h .
Ele c t rod e i m p e d anc e w as le s s t h an5 k W. Th e
s i g nalw as am p li f i e d b y 1 0 0 0 0 b y ad i f f e re nt i al
am p li f i e r( Di g i t i m e r, N L 1 0 4 ) and f i lt e re d
b e low 0 1 H z and ab ov e 2 5 H z ( 4 0 d B / d e c ad e
at t e nuat i on) . Th e f i lt e re d s i g nalw as d i g i t i s e d
( C ED, 1 4 0 1 ) and av e rag e d ov e rap e ri od of
one s e c ond b y aP C , s am p li ng at 2 56s am -
p le s / s . A m i ni m um of 1 2 8 s w e e p s c ont ri b ut e d
t oe ac h av e rag e . Th e e x p e ri m e nt e rc ould v i e w
b ot h t h e runni ng av e rag e and t h e raw d at at o
allow f oronli ne art e f ac t re j e c t i on. Th e am p li -
t ud e of t h e s e c ond h arm oni c re s p ons e w as
m e as ure d b y F ouri e ranaly s i s , i nw h i c h t h e
b and w i d t h of e ac h s p e c t ralc om p one nt w as
1 - 0 H z .
Th e s ub j e c t s w e re i ns t ruc t e d t om ai nt ai n
t h e i rf i x at i onalong av e rt i c alli ne at t h e
e x t re m e le f t of t h e d i s p lay . Th i s re s t ri c t e d t h e
s t i m ulus t ot h e ri g h t v i s ualf i e ld and e ns ure d
t h at t h e norm als ub j e c t ' s re s ult s w e re c om p a-
rab le w i t h t h os e of t h e p at i e nt , w h os e le f t
v i s ualf i e ld i s h e m i anop i c .
EYE MO V EMEN T REC O RDI N G S
Th e p at i e nt ' s e y e m ov e m e nt s w e re m e as ure d
w i t h t h e S k alari nf rare d m oni t ori ng s y s t e m
and re c ord e d b y ac om p ut e rw i t h a2 50 H z
m e t rab y t e analog ue - t o- d i g i t alc onv e rt e r.
Analy s i s w as p e rf orm e d of f li ne . Ey e m ov e -
m e nt s w e re re c ord e d i nv e ry d i m s urround -
i ng s . Th e p at i e nt w as s e at e d 1 1 4 c m f rom an
array of L EDs arrang e d i nani s ov e rg e nt p lane
( b ot h e y e s and allL EDs lay ont h e c i rc um f e r-
e nc e of ani m ag i nary c i rc le of rad i us 57 c m ) .
B ot h e y e s w e re op e n. C ali b rat i onw as p e r-
f orm e d w i t h s e v e nc ali b rat i onp oi nt s . Th e
p at i e nt f i rs t f i x at e d ac e nt ralt arg e t f or30 s e c -
ond s , f ollow e d b y at arg e t at 1 2 i nh i s ri g h t
v i s ualf i e ld . C ali b rat i onw as re p e at e d w i t h a
c e nt ralt arg e t f ollow e d b y at arg e t ap p e ari ng at
1 2 0 i nh i s le f t h e m i anop i c f i e ld . Th i s w as i nv i s -
i b le b ut h e k ne w i t w as t h e m i rrori m ag e
arrang e m e nt of t h e f i rs t c ali b rat i on. O t h e r,
m ore e c c e nt ri c p os i t i ons w e re t h e nus e d .
F i g ure 1 C ont ras t
s e ns i t i v i t y t oac h rom at i c
( le f t ) and c h rom at i c
( ri g h t ) g rat i ng s f ort h e
p at i e nt ( t ri ang le s ) and t h e
c ont rols ub j e c t ( c i rc le s ) .
Ac h rom at i c
1 0 0
1 0
1 1 0
S p at i alf re q ue nc y ( c y c le s ! 0 )
1 0 0
C h rom at i c
1 1 0
S p at i alf re q ue nc y ( c y c le s / ! )
1 0 0
1 0
a, r
G )
C D
4 0
C . )
1
1 0 0
64 0
1
group.bmj.com on January 15, 2014 - Published by jnnp.bmj.com Downloaded from
H e y w ood , N i c h olas , C ow e y
F i g ure 2 L e f t : s t e ad y
s t at e e v ok e d p ot e nt i als t o
c ont ras t re v e rs i ng
i s olum i nant c h rom at i c
g rat i ng s f ort h e p at i e nt ( A)
and t h e c ont rols ub j e c t
( B ) . Ri g h t : Th e p ow e r
s p e c t raof t h e e v ok e d
p ot e nt i als d i s p lay e d ont h e
le f t , af t e raF ouri e r
t rans f orm . Th e arrow
i nd i c at e s t h e 2 nd h arm oni c
re s p ons e , w h i c h w as
g re at e s t i ne ac h c as e .
0 . 2
1
A
0 . 1 -
0 - 0 -
- 0 . 1 -
>
- 0 . 2
c U 0 -
E
- 0 - 1 -
0 . 1 -
0 . 0 1 0
0 - 0 0 5-
, - 2 0 . 4 0 . 6 0 . 8 1 . 0
Ti m e ( s )
0 - 0 0
0 . 0 2 -
B
0 - 0 1 -
_2
.
0 0 . 8
0 - 2 0 - 4 0 - 6 0 - 8 1 . 0 0 - 0 0
Ti m e ( s )
6- 2 5 1 2 - 50 0 1 8 - 7 5
F re q ue nc y ( H z )
6. 2 5 1 2 - 50 0 1 8 - 7 5
F re q ue nc y ( H z )
Re s ult s
C O N TRAS T S EN S I TI V I TY
F i g ure 1 s h ow s t h e ac h rom at i c and c h rom at i c
c ont ras t s e ns i t i v i t y f unc t i ons of t h e p at i e nt and
anorm alob s e rv e r. Th e s p at i alf re q ue nc y c ut
of f , i nw h i c h s e ns i t i v i t y i s e x t rap olat e d t ouni -
t ary c ont ras t , i s 1 6c y c le s / ! f ort h e p at i e nt and
4 0 c y c le s / ! f ort h e norm alob s e rv e rf orac h ro-
m at i c g rat i ng s . Th e t h re s h old v alue f ort h e
p at i e nt c orre s p ond s t oaS ne lle nac ui t y of
b e t w e e n6/ 9 and 6/ 1 2 and i s v e ry d i f f e re nt
f rom h i s m e as ure d S ne lle nac ui t y , us i ng h i g h
c ont ras t le t t e rs , of 6/ 4 - 5 + 1 ( le f t e y e ) and
6/ 4 - 5- 1 ( ri g h t e y e ) . Alt h oug h t h e s e ns i t i v i t y
f unc t i oni s b and p as s , t h e re i s are d uc t i oni n
s e ns i t i v i t y at alls p at i alf re q ue nc i e s t e s t e d . I n
re m ark ab le c ont ras t w i t h t h e re s ult s f orac h ro-
m at i c g rat i ng s , t h e g e ne rals h ap e and am p li -
t ud e of t h e c h rom at i c c ont ras t s e ns i t i v i t y
f unc t i onf ort h e p at i e nt and t h e c ont rols ub j e c t
are s i m i lar.
V I S U AL EV O KED P O TEN TI AL S
F i g ure s 2 and 3s h ow re p re s e nt at i v e av e rag e d
s t e ad y s t at e p ot e nt i als re c ord e d f rom t h e c on-
t roland t h e p at i e nt f orac h rom at i c and c h ro-
m at i c g rat i ng s of 2 c y c le s ! 0 re s p e c t i v e ly . I n
ad d i t i on, f i g ure s 2 and 3d i s p lay t h e re s ult s of
F ouri e rt rans f orm s s h ow i ng t h e f orm of p ow e r
s p e c t ra, c le arly i nd i c at i ng t h e re s p ons e t ob e
g re at e s t i nt h e s e c ond h arm oni c ( 1 2 - 5 H z ) i n
e ac h c as e . Th e re s ult s f orot h e rs p at i alf re -
q ue nc i e s t e s t e d w e re e s s e nt i ally i d e nt i c alt o
t h os e d i s p lay e d . Th e v i s uale v ok e d p ot e nt i als
re c ord e d i nt h e p at i e nt w e re s i m i lari ns h ap e
t ot h os e re c ord e d i nt h e c ont roland alt h oug h
t h e y w e re g e ne rally of d e c re as e d am p li t ud e , a
s e c ond h arm oni c re s p ons e w as e v i d e nt i n
e v e ry c as e .
EYE MO V EMEN T REC O RDI N G S
F i g ure 4 s h ow s c h arac t e ri s t i c e y e m ov e m e nt
re c ord s of t h e p at i e nt . Th e y s h ow t h at av e ry
f i ne ny s t ag m us i s p re s e nt . Th e s e are i nt h e
rang e of m i c rom ov e m e nt s of t h e e y e t h at are
norm ally s e e n. H ow e v e r, t h e y are s up e r-
i m p os e d ons q uare w av e j e rk s of 0 6 am p li -
t ud e . Th e f as t p h as e i s t ot h e ri g h t and t h e
F i g ure 3L e f t : s t e ad y
s t at e e v ok e d p ot e nt i als t o
c ont ras t re v e rs i ng
ac h rom at i c g rat i ng s f ort h e
p at i e nt ( A) and t h e c ont rol
s ub j e c t ( B ) . Ri g h t : Th e
p ow e rs p e c t raof t h e e v ok e d
p ot e nt i als d i s p lay e d ont h e
le f t , af t e raF ouri e r
t rans f orm . Th e arrow
i nd i c at e s t h e 2 nd h arm oni c
re s p ons e , w h i c h w as
g re at e s t i ne ac h c as e .
0 . 2
1 A
0 . 1 -
0 . 0 -
- 0 . 1 -
a)
E
0 u
0 . 0 1 0 -
0 . 0 0 5 -
0 . 2 0 . 4 0 . 6 0 . 8 1 . 0
Ti m e ( s )
0 . 2 1 B
0 . 1
0 . 0
- 0 . 1 -
- 0 . 2
0 . 0 0
n- nln
0 . 0 0 5 -
I . . 1 .
0 . 2 0 . 4 0 . 6 0 . 8 1 . 0 0 . 0 0
Ti m e ( s )
v
6. 2 5 1 2 . 50 1 8 . 7 5
F re q ue nc y ( H z )
2 5. 0 0
V
6. 2 5 1 2 . 50 1 8 . 7 5 2 5. 0 0
F re q ue nc y ( H z )
2 5- 0 0
2 5. 0 0
64 1
v . v I
v 1
7
group.bmj.com on January 15, 2014 - Published by jnnp.bmj.com Downloaded from
B e h av i ouraland
e le c t rop h y s i olog i c al
c h rom at i c and ac h rom at i c c ont ras t s e ns i t i v i t y i nanac h rom at op s i c p at i e nt
F i g ure
4
( A)
re p re s e nt at i v e e y e
m ov e m e nt s
of
t h e
p at i e nt
t o
t h e
le f t ( up w ard s )
and
ri g h t ( d ow nw ard s )
w h i le
h e w as
at t e m p t i ng
t o
f i x at e
a
li g h t e m i t t i ng
d i od e
d i re c t ly
i n
f ront of
h i m .
Th e re c ord i s t ak e n
f rom
a
m uc h
long e rs e q ue nc e
t h at
las t e d
f or
one m i nut e .
( B )
A s i m i larre c ord at
h i g h e r
s p at i al
and
t e m p oral
re s olut i ont oi llus t rat e h i s
f i ne ny s t ag m us .
0 . 8
0 ,
0 - 6
c 0 . 4
0 ) -
C . 0 . 2
E)
0 -
0
DI - 0 . 2
0 )
4 ) 0 . 4
Dg '
0 . 6
i n
0 . 8
0 )
a. )
E
0
0 )
0 )
0 )
0
m ax i m u
of 2 0 / s .
Di s c us s
Th e p re
p re v i ous
c anb e
e v e r, t h (
w as d y s
c olour1
ab i li t i e s
e nc e s i n
c om p le t
i nanod (
i t y t oi
ab i li t y oi
e nc e s c a
t h e
i nt e g
f rom t h e
H ow
s i nuos oi
d i s t i ng ui
p re s e nt e
c h rom at
s p at i al:
t h re s h ol
1 0 c y c lb
s i g nali 1
i t s e lf d i
re d uc e d
t ai nc h ri
s h ow nb
nat i on.
t h at t h e
c e s s i ng .
op p one n
le s s b ri g ]
t i v e lum i
e f f e c t , 2 1
y e llow b
of t h e k i
- A
and g re e n. I nd e e d , w h e nas k e d t oi nd i c at e
h ow h e s e e s b and s i nt h e c h rom at i c g rat i ng h e
p oi nt e d t ot h e " y e llow s " .
O urp at i e nt h ad are d uc e d ac h rom at i c c on-
M A- - 2 - - l- - n
I
| s X I lAv AM ,
t ras t
s e ns i t i v i t y
at all
s p at i alf re q ue nc i e s t e s t e d ,
b ut e s p e c i ally at t h e h i g h e rf re q ue nc i e s , t h e n
h i s
p e rf orm anc e
w as
p arad ox i c ally
b ad w h e n
c om p are d w i t h h i s norm alS ne lle nac ui t y . Th e
s i m p le s t , and li k e ly , e x p lanat i oni s t h at t h e
re t i nali m ag e of g rat i ng s t i m uli i s s m e are d b y
2 4 6 8 1 0
h i s c le arj e rk ny s t ag m us i nw h i c h t h e v e loc i t y
Ti m e ( s )
of 2 0 / s i s i nt h e rang e i nw h i c h s e ns i t i v i t y t o
h i g h f re q ue nc i e s w ould b e e x p e c t e d t ob e
B d i m i ni s h e d . Th us t h e s m e ari ng w i llb e g re at e r
t h e f i ne rt h e g rat i ng . S ne lle nle t t e rs c ont ai nall
s p at i alf re q ue nc i e s and w i llt h e re f ore b e m uc h
le s s af f e c t e d . H ow e v e r, i t s h ould b e not e d t h at
t h e j e rk s are
i nt e rs p e rs e d
w i t h
p e ri od s
of re la-
t i v e ly s t ab le f i x at i onw h i c h m i g h t b e s uf f i c i e nt
f ord e t e c t i onof h i g h s p at i alf re q ue nc i e s . Th e
ori g i nof t h e ny s t ag m us i s unk now nb ut i t s
i nf lue nc e c ould b e t e s t e d b y m e as uri ng ac h ro-
m at i c c ont ras t s e ns i t i v i t y t oh i g h i nt e ns i t y ,
1 g 0 1 | 2 1 | 4 1 61 8
lt ac h i s t os c op i c ally p re s e nt e d g rat i ng s
t ore d uc e
0 0 - 2 0 - 4 0 - 60 - 8 1 - 0 1 - 2 1 - 4 1 - 61 . 8
ore v e ne li m i nat e re t i nal
s m e ari ng .
O ure le c -
Ti m e ( s ) t rog rap h i c m e as ure m e nt s of ny s t ag m us d i d
not d i s c los e i t s p ri nc i p ald i re c t i on, i f any .
S h ould t h e re b e one , t h e i m ag e of g rat i ng s t h e
i m s low
p h as e v e loc i t y
i s of t h e ord e r
ori e nt at i onof w h i c h i s p aralle lt ot h e d i re c t i on
of ny s t ag m us s h ould b e m uc h s h arp e rt h an
t h at of g rat i ng s ori e nt e d ort h og onally . Th e
ab s e nc e of any e f f e c t of h i s j e rk ny s t ag m us on
i on c h rom at i c c ont ras t s e ns i t i v i t y - at f i rs t s i g h t
! s e nt
f i nd i ng s
c onf i rm and e x t e nd a
p arad ox i c al- i s as i m p le c ons e q ue nc e of t h e
, re p ort
t h at c olourc ont ras t
s e ns i t i v i t y f ac t t h at t h e lat t e rh as t oolow as p at i alf re -
unaf f e c t e d i n
ac h rom at op s i a. ' 4 H ow - q ue nc y c ut of f t ob e d i s rup t e d b y t h e f i ne ny s -
e
p at i e nt
e x am i ne d
b y
t h os e aut h ors
t ag m us .
, c h rom at op s i c
i nt h e s e ns e t h at t h e O urf i nd i ng s s h ow t h at t h e p at i e nt h as a
os s w as
i nc om p le t e
and h i s re s i d ualnorm alc h rom at i c c ont ras t s e ns i t i v i t y f unc t i on
i nc lud e d d e t e c t i onof c olourd i f f e r- and norm alc h rom at i c V EP s . H e h as e x t e ns i v e
l
an
od d i t y
t as k . As t h e
p at i e nt
w as b i lat e ralv e nt rom e d i ald am ag e w h i c h i nc lud e s
e ly
unab le t od e t e c t c olourd i f f e re nc e s t h e li ng ualand f us i f orm g y ri . Allt h i s s ug g e s t s
d i t y t as k , 6y e t
re t ai ne d norm als e ns i t i v - t h at h i s re s i d ualab i li t y t od e t e c t c h rom at i c
i s olum i nant c h rom at i c
g rat i ng s ,
t h e
g rat i ng s d oe s not ari s e f rom ani nc om p le t e
f s om e
p at i e nt s
t od e t e c t c olourd i f f e r- le s i onof t h e li ng ualand f us i f orm g ry i and i s
nnot
ne c e s s ari ly
b e at t ri b ut e d
s ole ly
t om e d i at e d i ns t e ad e i t h e rb y e x t ras t ri at e v i s ual
J ri t y
of s t ri at e c ort e x and i s d i s s oc i ab le are as i nt h e d ors alp art of t h e c e re b ralc ort e x
d e t e c t i onof c h rom at i c b ound ari e s . w h i c h s h ow li t t le d am ag e d c om p are d w i t h t h e
d oe s our
p at i e nt
d e t e c t i s olum i nant c e nt ralp art of t h e c e re b ralc ort e x orb y i nt ac t
d al
re d / g re e ng rat i ng s
w h e nh e c annot s t ri at e c ort e x .
i s h b e t w e e nt h e s am e re d s and
g re e ns S ai t oe t al2 2 h av e s h ow nt h at t h e re are
d as
p at c h e s
ont h e s c re e n?
Alt h oug h num e rous c e lls i nare aMT, ap rom i ne nt p art
- i c ab e rrat i onc ould c ont ri b ut e at
h i g h of t h e s oc alle d b road b and M c h anne l, t h at are
f re q ue nc i e s any
ab e rrat i oni s s ub - not s i le nc e d at any c h rom at i c lum i nanc e rat i o.
d at low
s p at i alf re q ue nc i e s
of 0 5 and Th us i t i s p os s i b le t h at t h e ab i li t y of t h e
e s / 0 . 2 0 F urt h e rm ore , as any lum i nanc e p at i e nt t od e t e c t t h e c h rom at i c b ord e r
nt rod uc e d
b y
c h rom at i c ab e rrat i onb e t w e e ni s olum i nant c olours 6, w h i c h p re s um -
i m i ni s h e s as c h rom at i c c ont ras t i s
ab ly und e rli e s h i s ab i li t y t od e t e c t c h rom at i c
,
c h rom at i c ab e rrat i onc ould not s us -
g rat i ng s , d e p e nd s oni nt ac t e x t ras t ri at e v i s ual
om at i c c ont ras t at t h e norm alle v e ls are as i nt h e p ari e t allob e . H ow e v e r, are aMT
y
t h e
p at i e nt .
Th e re i s ab e t t e r
e x p la- h as ane g li g li b le p arv oc e llulari np ut , 2 3and i t i s
H i s
s p e c t rals e ns i t i v i t y
c urv e s h ow s d i f f i c ult t ore c onc i le t h e c om p le t e ly norm al
p at i e nt
re t ai ns
c olour- op p one nt p ro- c h rom at i c c ont ras t s e ns i t i v i t y f unc t i oni nt h e
i
O ne
c ons e q ue nc e
of
re d - g re e n
c olourp at i e nt ( at t h e t e m p oraland s p at i alf re q ue n-
i c y
i s t h at a
y e llow
s t i m ulus
ap p e ars c i e s t e s t e d ) w i t h ani nt ac t m ag noc e llularc h an-
h t t h anre d or
g re e n
of t h e s am e
ob j e c - ne li nt h e ab s e nc e of ap arv oc e lluarc h anne l
i nanc e . Th e
p at i e nt
s h ow s t h i s norm alf ort h e f ollow i ng re as ons . Mos t m ag noc e llular
w h i c h i nnorm alob s e rv e rs m ak e s t h e c e lls d oh av e anullp oi nt at i s olum i nanc e and
and s i na
re d / g re e n
s i nus oi d al
g rat i ng of t h e c e lls t h at d onot , t h e re s p ons e s at i s olu-
i nd
w e us e d look d i m m e rt h ant h e re d m i nanc e are le s s t h anat any ot h e rc olourrat i o.
64 2
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H e y w ood , N i c h olas , C ow e y
Th i s i s c ons i s t e nt w i t h t h e re s p ons e p rop e rt i e s
of s i ng le ne urons i nt h e m ag nore c i p i e nt lay e rs
of t h e s t ri at e c ort e x , w h i c h s h ow ad e e p m i ni -
m um t oi s olum i nant re d / g re e nb ord e rs and ,
li k e t h e m ag noc e llularlay e rs of t h e d L G N ,
s h ow b road b and s p e c t ralp rop e rt i e s .
Analt e rnat i v e e x p lanat i onf ort h e norm al
c h rom at i c c ont ras t s e ns i t i v i t y i nanac h ro-
m at op s i c p at i e nt s uc h as ours i s t h at t h e re i s
s t i lls ub s t ant i alc olourop p one nt p roc e s s i ng . 6
Th i s i s p re s um ab ly m e d i at e d b y t h e und am -
ag e d p art s of t h e P c h anne lw h i c h re s i d e i nt h e
i nt ac t s t ri at e c ort e x and i s c ons i s t e nt w i t h t h e
norm alc h rom at i c V EP s i nt h e s t ri at e c ort e x
w h i c h i s m e d i at e d b y t h e P p at h w ay . Th e re
h av e b e e nre c e nt d e m ons t rat i ons of ad i s s oc i a-
t i onb e t w e e nt h e ab i li t y t od e t e c t ani s olum i -
nant c h rom at i c p at t e rnand t od i s c ri m i nat e
c olourd i f f e re nc e s i nc as e s of c om p le t e 2 ' or
i nc om p le t e ac h rom at op s i a. 2 4 I t i s p os s i b le t h at
t h e " b lob " p at h w ay of V lm e d i at e s c h rom at i c
d i s c ri m i nat i onw h e re as t h e " i nt e rb lob " p at h -
w ay p roc e s s e s c olourd i f f e re nc e s t od e t e c t i s o-
lum i nant p at t e rns w i t h out c onv e y i ng t h e
nat ure of t h e h ue of w h i c h t h e p at t e rni s c om -
p os e d . Th us p art i als t ri at e i nv olv e m e nt , i n
w h i c h t h e " b lob " arm of t h e P c h anne li s
s e le c t i v e ly c om p rom i s e d w ould re s ult i n
ac h rom at op s i a. Th e i nt e g ri t y of t h e
" i nt e rb lob " p at h w ay of V l, w i t h i t s i nt ac t p ro-
j e c t i ons t oe x t ras t ri at e c ort e x , c ould t h e n
ac c ount f orre s i d ualv i s ualp roc e s s e s .
We are p re p ari ng t oe x am i ne , b y f unc t i onal
m ag ne t i c ne uroi m ag i ng , t h e s urv i v i ng e x t ras -
t ri at e are as i nv olv e d i nt h e p at i e nt ' s re s i d ual
p roc e s s i ng of c h rom at i c s i g nals .
We t h ank t h e p at i e nt f orh i s e nt h us i as t i c c oop e rat i on. Th e
re s e arc h w as s up p ort e d b y aH um anC ap i t aland Mob i li t y
G rant ( H C and M: C H RX C T 9 30 2 61 ) and t h e aw ard of a
V i s i onRe s e arc h F e llow s h i p b y t h e We llc om e Trus t t oJ J N .
1 Me ad ow s J C . Di s t urb e d p e rc e p t i onof c olours as s oc i at e d
w i t h loc ali z e d c e re b ralle s i ons . B rai n1 9 7 4 ; 9 7 ; 61 5- 32 .
2 P lant G T. Di s ord e rs of c olourv i s i oni nd i s e as e s of t h e ne r-
v ous s y s t e m . I n: F os t e rDH , e d . I nh e ri t e d and ac q ui re d
c olourv i s i ond e f i c i e nc e s : f und am e nt alas p e c t s and c li ni c al
s t ud i e s . V i s i onand v i s uald y s f unc t i on. V ol7 . B as i ng s t ok e :
Mac m i llanP re s s , 1 9 9 1 : 1 7 3- 9 8 .
3Z e k i S . A c e nt ury of c e re b ralac h rom at op s i a. B rai n1 9 9 0 ;
1 1 3: 1 7 2 1 - 7 7 .
4 S t i le s WS . Me c h ani s m s of c olourv i s i on. L ond on: Ac ad e m i c
P re s s , 1 9 7 8 .
5 MollonJ D, N e w c om b e F , P old e nP G , Rat c li f f e G . O nt h e
p re s e nc e of t h re e c one m e c h ani s m s i nac as e of t ot al
ac h rom at op s i a. I n: C olourv i s i ond e f i c i e nc i e s . V olV .
B ri s t ol: H i lg e r, 1 9 8 0 : 1 30 - 5.
6H e y w ood C A, C ow e y A, N e w c om b e F . C h rom at i c d i s -
c ri m i nat i oni nac ort i c ally c olourb li nd ob s e rv e r. EurJ
N e uros c i 1 9 9 1 ; 3: 8 0 2 - 1 2 .
7 Dam as i oA, Yam ad aT, Dam as i oH , C orb e t t J , Mc Ke e J .
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log i c as p e c t s . N e urolog y 1 9 8 0 ; 30 : 1 0 64 - 7 1 .
8 Z e k i S M, Wat s onJ DG , L ue c k C J , F ri s t onKJ , Ke nnard C ,
F rac k ow i ak RS J . A d i re c t d e m ons t rat i onof f unc t i onal
s p e c i ali z at i oni nh um anv i s ualc ort e x . J N e uros c i 1 9 9 1 ;
1 1 : 64 1 - 9 .
9 C orb e t t aM, Mi e z i nF M, Dob m e y e rS , S h ulm anG L ,
P e t e rs e nS E. S e le c t i v e and d i v i d e d at t e nt i ond uri ng
v i s uald i s c ri m i nat i ons of s h ap e , c olorand s p e e d : f unc -
t i onalanat om y b y p os i t rone m i s s i ont om og rap h y . J
N e uros c i 1 9 9 1 ; 1 1 : 2 38 3- 4 0 2 .
1 0 G uly i s B , Roland P E. C ort i c alf i e ld s p art i c i p at i ng i nf orm
and c olourd i s c ri m i nat i oni nt h e h um anb rai n.
N e urore p ort 1 9 9 1 ; 2 : 58 5- 8 .
1 1 Mac k ay G . Dunlop , J C . Th e c e re b ralle s i ons i nac as e of
c om p le t e ac q ui re d c olour- b li nd ne s s . S c ot t i s h Me d i c aland
S urg i c alJ ournal1 8 9 9 ; 5: 51 3- 2 .
1 2 S c h i lle rP H , L og ot h e t i s N K, C h arle s ER. F unc t i ons of t h e
c olour- op p one nt and b road - b and c h anne ls of t h e v i s ual
s y s t e m . N at ure . 1 9 9 0 ; 34 3: 68 - 7 0 .
1 3P e arlm anAL , B i rc h J , Me ad ow s J C . C e re b ralc olorb li nd -
ne s s : anac q ui re d d e f e c t i nh ue d i s c ri m i nat i on. Ann
N e urol1 9 7 9 ; 5: 2 53- 61 .
1 4 V i c t orJ D, Mai e s e K, S h ap le y R, S i d t i s J , G az z ani g aMS .
Ac q ui re d c e nt rald y s c h rom at op s i a: analy s i s of ac as e w i t h
p re s e rv at i onof c olord i s c ri m i nat i on. C li nV i s i onS c i
1 9 8 9 ; 4 : 1 8 3- 9 6.
1 5 G re e nG J , L e s s e llS . Ac q ui re d c e re b rald y s c h rom at op s i a.
Arc h O p h t h alm ol1 9 7 7 : 9 5: 1 2 1 - 8 .
1 6Alb e rt ML , Re c h e s A, S i lv e rb e rg R. H e m i anop i c c olor
b li nd ne s s . J N e urolN e uros urg P s y c h i at ry 1 9 7 5; 38 : 54 6- 9 .
1 7 H e y w ood C A, Wi ls onB , C ow e y A. A c as e s t ud y of c ort i c al
c olour" b li nd ne s s " w i t h re lat i v e ly i nt ac t ac h rom at i c d i s -
c ri m i nat i on. J N e urolN e uros urg P s y c h i at ry 1 9 8 7 ; 50 : 2 2 - 9 .
1 8 N e w c om b e F , Rat c li f f G . Ag nos i a: ad i s ord e rof ob j e c t
re c og ni t i on. I n: Mi c h e lF , S c h ot t B , e d s . L e s s y nd rom e s d e
d i s c onne x i onc alle us e c h e z l' h om m e . L y on: C olloq ue I nt e r-
nat i onald e L y on, 1 9 7 5: 31 7 - 4 1 .
1 9 Rat c li f f G , N e w c om b e F . O b j e c t re c og ni t i on: s om e d e d uc -
t i ons f rom t h e c li ni c ale v i d e nc e . I n: Elli s AW, e d .
N orm ali t y and p at h olog y of c og ni t i v e f unc t i ons . L ond on:
Ac ad e m i c P re s s , 1 9 8 2 : 1 4 7 - 7 1 .
2 0 C av anag h P , Ans t i s S M. Doop p one nt - c olorc h anne ls c on-
t ri b ut e t om ot i on? I nv e s t O p h t h alm olV i s S c i 1 9 8 6( s up p l
2 7 ) : 2 9 1 .
2 1 H e y w ood C A, C ow e y A. N e w c om b e F . O nt h e role of p ar-
v oc e llular( P ) and m ag noc e llular( M) p at h w ay s i nc e re -
b ralac h rom at op s i a. B rai n1 9 9 4 ; 1 1 7 : 2 4 5- 54 .
2 2 S ai t oH , Tanak aK, I s onoH , Yas ud aM, Mi k am i A.
Di re c t i onally s e le c t i v e re s p ons e of c e lls i nt h e m i d d le
t e m p oralare a( MT) of t h e m ac aq ue m onk e y t ot h e
m ov e m e nt of e q ui lum i nous op p one nt c olors t i m uli . Ex p
B rai nRe s 1 9 8 9 ; 7 5: 1 - 1 4 .
2 3F e rraraV P , N e ale y TA, Mauns e llJ H R. Mi x e d p arv oc e llular
and m ag noc e llularg e ni c ulat e s i g nals i nv i s ualare aV 4 .
N at ure 1 9 9 2 ; 358 : 7 56- 58 .
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64 3
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doi: 10.1136/jnnp.60.6.638
1996 60: 638-643 J Neurol Neurosurg Psychiatry

C A Heywood, J J Nicholas and A Cowey

sensitivity in an achromatopsic patient.

chromatic and achromatic contrast
Behavioural and electrophysiological
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