STURGE-WEBER-DIMITRI
DISEASE
Report
of
Case
with
Necropsy
By 1. W. Falkinburg, M.D., M. L. Silver, M.D., M. N. Kay, M.D., and J. Stoll, M.D.
J)(./)(Jrtlll(’llts- of Patliologi,, Neurosurgery and Pediatrics, Roger WTillia,ns General Hospital,
Proeideuce, Rhode Island
(Accepted February 28, 1958; stmblilitte(i January 11.)
ADDRESS: (L.\\.F.) 825 Chalkstone Avenile, Providemice 8, Rhode Island.
319
PEDIATRICS, August 1958
TZ NOWLEDCE of the cerebral angiomas
1,. (bites 1)ack to the mniddlle of the
Nine-teenthi Cemittmrv, and! omie of the first
publica-tiomis Oh this subject was that of Luschka’ iii 1854.
Kalischer was the first to 1)Oiflt out the association of skimi nevi and hemangiomna of
the brain, dmldl to comifirmn this associatiomi hv necrO)sy.
Shinner’ first mioted the association of
facial nevims amid! congenital glaucoma.
Sturge ili 1879 iresented the case of a 63-vear-ohd girl, with pronounced!
right-s:(le(! facial iievtis, right buphithalmos, and!
eI)ilel)tic fits begiminimig in the left hiand. He
comiclnde(! that the right side of the brain
was the site of a nevus. This conclusion was
l)ased upon chimiical observation tlidi ‘Vt15 not confirmed either hw iiecropsv or operation. \Veber,h Dimnitri , Krahbe, \I arque,
Vol-land,’#{176} amid HeboldPm ill pul)lishied cases of
facial amigioma, associated! with cerebral
angionia, im.i which mineral deposits couldl
be demm)mistrated in the 1)rain hy
roentgeno-gram, thereby emphasizing amiother
char-acteristic sign, useful fri the clinical
diagmio-sis of this con(!itiomi. Krahbe shio\ved! that
thfs mineral , which lie regarded as calciun,
was (le1)Osite(I withiin the cerebral cortex,
amid! not within the overlying (h’splastic pial vessels. Sturge-\Veber’s dlisease was first
die-scribed imi the Americami literature by
Ctmsh-ing.12
This comiil)hicated historic background has
resulted! imi an umiusual proliferation of
names, 1)0th eponymnic andi descriptive. The
disease has heemi at one time or anothier designatedl Sturge-W7eber’s disease,
Sturge-\Veber-Dimnitri synd!rome, Sturge’s disease,
Krabbe’s disease, Kalischer’s disease,
Brush-field-Wyatt syndrome, neurocutaneous
svn-d!rome, encephalotrigeminal angiomatosis, nevoid amentia, and cerebrocutaneous
syn-dlrome.
\Vith this background we wish to present
the following case of Sturge-\Veher’s
dhi5-ease, confirmed by necropsy.
History
CASE REPORT
J.B., a white female child 23 years old, ‘as ad!mitte(l to the Roger Williams General
Hospital on April 19, 1956, with a chief
corn-plaint of convulsions.
The 1)arelits, three brothers, and! one sister
were Sai(l to I)e healthy. One uncle was mcmi-tally retarded, and another uncle was said to
have suffered! from convulsions since 5 years
of age. The mother’s pregnancy was unevent-ful. The child! was born 14 (lays overdue but
dleliverv was natural. Birth ‘eight was 4,0:30 gin. At delivery a congenital hemamigioma in-volving both sides of the face (Fig. 1) war
noted. The Child! ‘as l)ottle-fed until the age of
9 momiths. Her dlevelopment was normal: she
recognized her mother at 4 months of age, sat
Uj) at 6 months, stood at 10 months, walkedl at 14 months, and Could1 talk in small sentences at 2 years of age. The mother describedl her as
an active child! who 1)laved normally with other
children.
At 9 months of age she suffered from an
attack of acute bronchitis andl at 18 months of age had chickenpox. In December, 1955, she ‘as ill for 2 weeks with acute tonsillitis,
dur-ing which time her temperature reached 40#{176}C. A week before admission to the hospital she l)umped her head on the crib, sustainimig a
slight scratch over the right temple. Until this time there was no history of convulsions.
:320 STURGE-\VEBER-DIMITRL DISEASE
The concentration of hemoglobin was 10.6 lI( . 1. Ph otograpli of j.B.. shaaving h)ilateral
congel iital facial lieluangioula.
small 111101111t of vaterv fltIi(lail her eves ‘ere
deviated towards the right. About 2 hours later she VOIiiite(l again. ga\e a little er, aix! tried iiiisuccessfullv to StaII(l up. She became limp,
(.0111(1not stand, valk, oi Imold up her head. The
e\ es were deviate(l towar(ls the right, amid! she (11(1not res1)OI1(I to (1lIeStiOllS, talk, or cr. She ‘as l)athe(l in a cold sveat. an(l all of the
cx-tremities t1)I)Irl )(trimlyz((l. After a third!
epi-SO(le of volluting she 1(!uiitte(I to the
hos-Pitill.
Physical Findings
the child \VJS ell iionrished JI1(h Stllpol)us.
res)on(hing oiil’ a little to viriotms stimuli. She turned her head heii cahle(l, opened her eyes hien ordered hit vould nut talk. She Criedl
unIv on 1)aillflll StilTIlIli.
ihere as a congenital nevus vith telangiec-tdsia 011 both sides of the face, (..OrrespOlidilIg
to the (liStribiltion of the fifth cramiial mierve (Fig. 1). The skull was otherwise not
remarka-1)le. The tipi1s ere e( 1td, round, reacted to light, all(l the evel)Lhls (hisplav(.dl 110 lilliltatiOli
of inotioii . EXamination of the ocular fundii
vielde(l norual findiligs on both sides; a slight nvstagnius was liote(I 01) 11U)V1l1 the evel)ahls to the extreuii.’ right.
ihie ITI)SC aIKl thiott \VeIe I orulal . The
phar-ynx was slightly illj(Ct(.’(l and the tonsils ere
I1TI)deratelv enlarge(l. The contents of the thorax and alxlolnell were not remarkable. All extremities were flacid. The reflexes were miliinial Oh the left Si(lean(h cOul(l not be
dem-ollstrate(1 011 the right.
Laboratory Findings
gui I 00 nil and time ervthirocvte count :3,900,00() rum . The leukocvte (OtlIlt was
12,000 mm with 10 11Ol1-Segniehlte(l aII(! 79
seghiiehited forms, 9( lymphocytes and 2%
inoiiocvtes.
Examination of the urine ‘as negative.
Culture of the UOSC revealed staphlococCi
and streptococcus viridlalis.
Roentgenograrns of chest and skull did not reveal an al)norrnahity.
Lumbar l)uliCture yielded! slightly clOudI\ fluid, containing 1 68 ervthrocvtes ali(l 1
hvmpho-cvtc ‘mni. The concentratiomi of total protein ‘as 28 mg,. 100 ml aiid that of sugar ‘as 100
mg .100 ml. Culture and! smear of
cerebro-spinal fluid were negative.
The electroencephalogram revealed no
clear-cut I)aroxysmal seizure discharges. Definite dlifferehiCes ‘ere noted in the teinpero-occipital
regions of both sides, but it was hard to
(Ic-cidle from these findlimigs which coul(I be con-sidered the al)miornial side. It was felt that the abnormality ‘as 1)rl)1il\ 01) the left Si(le.
Course
Treatinent consisted! of bed rest and a(lfliifl-istration of sodlium phenobarbital, 64 nig ever 8 hr and, later, dliphenylhvdantoiu, 50 nig
ever 8 hr. Acetvlsahicvlic acid! was given as needled!.
During the next 10 (layS several i(lehltical convulsions occurred, and 2 days after
ad-I11i55i011 there was a rise in teniperattire to
:39. 1 #{176}C,vhichi was colisidleredl of tipper
respira-tory origin . For this complication tetracycline, acetysahicylic acid amid Dichrysticin “ were
given, as vell as ether to control the
convul-sions.
After 10 (layS in the hospital there was
niarked ITIC1Ital amid! physical imnproveinent . and
the coiiviilsiomms cease(!. She was discharged on May 8, 1956, and ‘as referredl to the
Outpa-tient l)epartnieiit vhiere she was se(’II several
tunes. After discharge she had three
convul-SiOlIS in 3 weeks, and! as sent to the Children’s
Nledlical Center, Boston, for eonslhlt:ttion aII(l reviev. It vas the Opinion of the consultants
that a 1)rest11pti%’e diagnosis of Stnrge-\Veber svndrolne \Vas justified! although the findings vere not suffIcient to allo a definite (liagnosis.
The childi \Vas readmittec! to the Roger \Vil-hams General Hospital on August 24, 1956,
ccmnplain:ng of voniiting of 6 (lays (luration,
ARTICLES :321
FIG. 2#{149}Pian umoencephalograln demonstrating cortical atrophy on the left side.
Flie l)atiellt no \aste(I and al)atlietic,
vith a re(ldish-1)urple discoloration of the
en-tire face and left Sidle of the foreheadi.
Other-yise the iiIisicml examination shoved nothing
new.
Additional Laboratory Findings
Occasional coarse granular casts were found in the urine. There was also a trace of acetone.
The concentration of hemoglobin was 14.2
gn 100 InI. TIme ervthrocvte count was 4 900,00() mm and the leukocyte count 15,000/
mm. The concentration of chiloridle in the
1)100(1 \\S c:)6 inEq I, that of sodijuirl 1:37
IflE(1 I, aiicI that of potassium 4.2 inEq I. The cerel)rospinal fluid vas clear and had a
specific gravity of 1 .003. Lymphocytes nuni-bered 23 mm. Coimceiitritions of sugar, ebb-ridle and tOtal protein of thie cerebrospinal fluid
were 65, 1 18 and 35 rng 100 ml, respectively.
Serologic test for svphihis was negative. The concentration of iron of the fluidlWaS 8 ig 100 ml.
Roentgenograms of skull and! chest were again negative.
cvi-:322 STUIIGE-\VEBER-DIMITRI DISEASE
(heIICe of he1 t-si(led cortical atrophy and niiii-11101 enbargenient of the v(Iitricllbar svsteni,
right imiome than left ( Fig. 2).
Later Course
Sonic improvement was IlOted during tile first Ptrt of the second hospital aduiissioim. The
(OlmytlIsiOlLs ceased, tPI)etit’ increased!, an(l she \\iLS iiiore active.
Angiograni shoved a questionable filling
die-f(:ct of the left l)oSteriOr parietal branchmes of tIme Ifli(l(lle cerel)ral artery.
Despite initial iul1)rovIneIIt, the j)atients
(OllditiOlI deteriorated 1)rogressivehv. There
ere OcCLSiOI1ll tvitchies of the arnis, face, sluulders. (CS, iriouth, llaIldlS and legs. She l)ecalne febrile, PPtrelmtlY froln all tipper res-1)i1atrY infection, vith coryza, conjunctivitis, 00(1 cough. Physical signs suggeste(i
broncho-pIletIITIOlIiiL of increasing severity, aceoui1)anied
liv repeated coiivtilsions, volniting. an(l evano-sis. She (lie(i On November 2 1
,
I 9.56.Necropsy
GRoss FINDINGs: The body was that of a
poorly 11011 rishied
,
but e11 developed femaleinfant weighing about 18 kg. On 1)0th) sideS of
the face were numerous large audi SImiall
pur-phishl-redl blotches ranging in (!iaulieter from 1 to
4 These macules corres1ionded to the
dis-tribution of all the raIni of the fifth cranial
nerve. On both legs there ‘as excessive growth
of hair.
The lungs ‘ere ell aerated! an(l \(‘re not
grossly remarkable.
The calvarium iii situ was not remarkable.
The brain w’eighedi 655 gn (about one-half the
nornial weig!it for this age). The dora niater
was natural. The entire left cerebral herni-sphere was covered
by
a thmick, iiiushv, soggy, l)turl)lisl)-redi 1liiISS, which represented aITlark-cdlv alteredi leptomeuiiuix (Fig. 3). On time right
side the leptomeninx displayed! moderate comi-gestion over the frontal lobe. The cerebellar
surfaces appeared! normal. The left frontal andl temporal lobes exhibited several areas in vhich
there was marked widening of the sulci and flattening of the gvri. Post-morteni stludiies of
the brain by roentgenograrns (lisplavedi fine l)lluictate areas of mineral (le1)OSitS, arranged! in
serpeuitine or tortuous configuration, jim the left
occipital lobe.
The pituitary I)0(i\ and sella turcica ‘ere grossly negative.
After fixation, the braiui was Sectioned!
seri-ally. There was a varying (legree of atrophy of the left cerebral cortex over which the
al-tered dlvSplaStiC lel)tonieniuix rested like a thin
black spomige. No gritting against the seCtioulilig knife was noted.
The spinal cord was grossly negative. All other organs were within normal gross limits.
1’ic. :1. Gross appearance of cut section of brain showing thick, soggy,
ARTICLES 323
Fu;. 4. Brain, showing congenital hemangioma of leptomeniux eXteli(hihig
deeply clown between cerebral convolutions. (x 32.)
Mmc ROSCOPIC FINDINGS : The bronchioles
were 1)acked ithi polvmorphlonuclear
leuko-cvtes and! ill some fieldls there was a patchy
filling of some of tile adljacemit pulmoIitry alveoli
with these cells.
In the brain there was congestion of the
right I(Pt0meIm inges. The leptomeninges on the
left side were the site of a well-diefined
con-genital anomaly. This consisted of innumerable
\Vi(ielV d!ilate(i vascular Spilces, found h)Oth on the surface of the cerel)ral hemisphere and
(upping deeply down between the convolutiomis ( Fig. 4). These (l\51)ltstic vascular
malforma-tions ranged! in thickmiess from three to four layers to as man as 14 or 15 layers over the left occipital lol)e, whiere the lesion was the thickest. The abnormal blood vessels
thlem-selves consisted for the most part of simple (‘Ii(lOt!lel ial-linedi vascular spaces packed vith
erthrocvtes. These spaces varied! markedly iii Size audi shape, ranging from tiny to (fuite
large. The spaces were separated by more or
l(’SS cellular conmiective tissue, in which there
\‘as occasional evidence of chronic
inflamma-tion.
Iost of these siaces were lined only
by
en-dot!ielitmni. However, a fe of the larger ones had thin walls in vhich a miumber of smooth nitusele fibers could be unadle out (Fig. 5).The cerebral cortex beneath this dvsplastic vascular mass was affected iii varying dlegrees. %I05t pathologic changes were foundl in the
occipital cortex, where there was an almost comnplete (lisappearance of neurons vith marked! proliferation of glial cells. Here there
was a promu)unced mineral deposition, which we regard as composed of both calcium and
iron salts, on the l)asis of histochemical studies
(Fig. 6). These deposits appeared! in two dis-tinct forms and were encountered! chiefly in the
secondi audi third cellular layers of the left
occi-pital cortex. In one form large, (lark, round or OVOid1, irregularly shaped and! sized! granules
were scattered! tt ramidom in the cortex. In the other form there as a diffuse scatteriuig or
dusting of mvriads of mimiute, deeply basophihic
granules. Dr. F. \Vohlwill kindllv reviewed this slide and it was his impression that the mineral deposi ts were extraordinarily abuiidant,
con-sidering the short diuration of the d!isease. Both h)asal ganglia displayed! occasiomial
simi-lar granules, commonly in close proximity to 1)100(1 vessels.
Other sections of brain and! spinal cord were miegative microscopically.
Pathologic diagmioses were
memiingoemiceph-alotrigeunimial amigiomatosis (Sturge-Weber’s dis-ease), with atrophy of the left cerebral cortex
and bilateral h)ronciioplieumonia.
DISCUSSION
In 1936, Greenwald and! Koota,’ fl an
re-:324 STUIIGE-WEBER-DIMITRI DISEASE
I’uc. 5. I)vsplastic meningeal vessels, in the walls of which may be seen smooth ummmscle fillers. (x4:30,
reduced 1A)
ARTICLES 325
1)orted cases of this disease and divided thiese cases into two categories.
One category included those cases,
simi-lar to the one reported! by Cohemi and Kay,14
u.kwhich
the diagnosis was unconfirmed bynecro1)sv or biopsy, I)ut was clear enough
011 clinical grounds, i.e., presence of a facial nevus, cerebral calcification, neurologic
audi/or 1)sychiatric manifestations, with or
‘ithotmt ocular symptoms.
The second! category comprised those
cases comifirmed by biopsy and/or necropsy. Includ!ing a case studied! by them, they
listed! 58 unconfirmed or presumptive cases,
and! 24 confirmed cases. Of the 24 confirmed
cases, 4 l)resented! bilateral facial nevi.
Thiese authors also presented! a
compre-liensive review of the pathology,
pathogene-sis, svinptonis aix! treatment of the (lisease. More or less uniform pathologic changes
have h)eemi rioted upon o)ening the skull.
The diuIra over the lesioii hiia’ be thickened!
andi hyl)eremic. Iii the iia mater there is a more or less extensive angioma or
angi-oiiiatouis (lYsplasia. This iesomi is a purple to black soggy mass with a spongy resilience. On the affected! sidle all lobes of the
cere-1)rumn may he covered by this lesion, hut involvement of the occipital lobe is usually
the most severe. Tile underlying cortex may
I)C atro1)hiic xvith flat ind!urated gyri. At
tunes time tissue mnay grit to the sectioning knife.
\Iicroscopicallv one sees several layers of
elid!Ot!ielial-limied vascular spaces packed
xvi th ervthirocvtes. Areas of hiemorrhage
ali(!/Or thrombosis may he present. The (Ivsplastic vascular spaces vary markedly in
size itui(I 5hlPe, and a few have thin walls coml)ose(I of fibrous an(I smooth muscle tis-sue.
The umid!erlying cortex is usually thin,
shrumiken, andl atrO1)hic. Time number of
uieuurons lTltV he diminished to a varying de-gree, or they may be missing entirely. There
is mnarked l)rliferation of gbial cells. One x’ery characteristic feature is the presemice, in
tue
affected cortex, usually in the secondamid! third cellular layer, of a more or less
ah)undault mineral d!eposit, regarded by
some as calcium (calcification), and! by
others as iron (ferrugination). The mineral
dieposit is found surrounding the walls of the precapillaries and! capillaries, or lying
free in the tissues. This deposit generally
occurs as sizeable clumps, or as minute fine particles.
In those cases with ocular involvement
thie iris and choroid are the site of
angi-omatoses.
The disease has i)een classified as one of the so-called phacomatoses (phakos, Greek: mother spot or mole), or neurocutaneous
syndromes. The diseases belonging in this
category are Sturge-Weber’s d!isease,
neturo-fihromatosis of von Reckhinghausen, and
tuheroims sclerosis. Today this angiomatous
malformation is considered a congenital
dysplasia rathier t!iami a neoplasm. This
diys-plasia, which is the fund!amental lesion of Sturge-\iVeher’s disease, is regarde(i as a
mesodermal defect, the result of Some
tin-known trauma sustained! in early fetal life.
The association of the several lesions is
at-tributed to early embryonic proximity of the areas in question. The vasculatture of these
areas is derived! from a capillary sheet
sup-plying all. In a case of angiomatous
dys-plasia, therefore, all three areas, i.e. ,
lepto-meninges, eye and skin of the face, may be
involved. Or a combination of any two
areas, or any single area may be implicated
if the fetal trauma is sustaine(! after cleav-age of the three embryonic anlagen.
Yakov-1ev15 points out that when thie facial nevus
involves all three divisions of thie trigeminal nerve, the vascular lesions of pial vessels may involve the entire cortex
(
as in the case reported here). When the occipital pole of the brain alone is involved, the facial nevusis restricted! in area (to the first division).
The absence of bilateral cerebral involve-ment, in the presence of bilateral facial nevus, has not been explained!.
Regarding the cortical changes, there are two schools of thought. Yakovlev and! Cuth-ne15 feel that the cortical changes are pri-mary, occurring pan passu with the
menin-geal defect. On the other hand, Alexander
326 STURGE-WEBER-DIMITRI DISEASE
Lichtenstein and Rosenberg,19 and Green,2#{176}
regard! the cerebral cortex as originally nor-mal, and feel that the observed
abnormali-ties arise as a result of incomplete
inter-ference with oxygenation of the affected
cortex, owing to the abnormal pia mater
immed!iately above.
Symptoms are usuahl’ noted! in early
in-fancy or early child!hood, andi are for the most part neurologic and! ocular:
Convulsions occur in about 75% of the
cases and are generalized! or jacksonian.
There is frequent spastic paralysis on the Sidle opposite to the effected leptomeninges.
N’Iental retardation is usually observed.
This may vary from mild! retardation to
complete amentia. However, one may
en-counter cases in which thie intelligence
re-mains intact.
Ocular abnormalities are encountered! in about 70% of the cases. The ocular condition
is usually unilateral with angiomatous al-terations in thie sclera, conjunctiva or iris.
Buphthalmos is frequent, associated with
glaucoma and! varying degrees of visual
im-pairment. Also homonymous hemianopsia,
atrophy of the iris, retinal pigmentation,
optic atrophy, heterochromnia, nystagmus,
disturbance of thie ocular reflex mechanisms,
colohoma, and! pupillarv ai)normalities have I)een dlescribe(i.
Skin nevus or angioma, usually unilateral and! rarely crossing the mid!line, is a
para-niount symptom. This lesion
characteristi-cally follows the distribution of the branches
of the fifth) cranial nerve. It generally has
a port wine color, hut at times it may be
pale and barely noticeable. The skin lesion varies in size from that of a pin head to an extensive lesion involving the head, face, neck, oral mucosa, tongue, palate, and the
greater part of the bodly. Occasionally both
sides of the face may be involved.
Roentgenographic studies show shadows of mineral 4Jeposits, usually regard!ed as
calcium salts, which are observed chiefly in
the occipital lobe. This finding is of consid-erable aid imi the diagnosis of presumptive or unconfirmed! cases.
Greenwald! and! Kootatm emphasize that
two or more of the following symptoms
were observed in every case: port wine
nevus on the face; convulsions, most fre-quently jacksonian in type; spastic hemi-plegia on the side opposite to the facial nevus; varying degrees of mental retardia-tion; serpentine shadows in the roentgeno-grams of the skull, usually in, or at least more intense in, the occipital lobe.
Greenwald and Koota stress that in every case of idiopathic epilepsy a careful search should be made for a nevus about the head, face and neck.
The immediate prognosis as to life is not bad, most patients (lying the second! or third decade. The over-all prognosis, however, is
poor and death usually results from some
intercurrent disease, resulting from general
debility.
Not much hope is offered by treatmnent,
which has consisted in attempts to remove the lesion surgically, x-ray therapy directed to the lesion, or both combined!. Otherwise treatment is symptomatic and palliative.
Although Sturge-Weber’s d!isease is very
rare, a considerable literature exists on the subject, most of which is of European on-gin, so that one is led to wonder whether
the disease is more common in Europe, or
has been more frequently recognized and
stud!ied there. Even more rare are cases con-firmed by operation or necropsy, and most
cases must be diagnosed presumptively on
clinical and roentgenographic findings
alone.
With these facts in mind, it occmmrred to
mis that it would be interesting to ascertain
how many cases of this disease have been confirmed in the United States and reported in the American literature.
Greenwald and Koota’3 listed and
sum-manized 10 confirmed American cases. Green and coworkers21 reported a case in an 8-year-old female, confirmed by right occipital lobectomy.
Lichtenstein22 presented an incomplete
case of Sturge-Weber’s disease with
me-ningeal angiomatosis without facial
he-mangioma, confirmed by necropsy. The
ARTICLES 327
article thie case of a 21-year-old! male was
1)reseuite(l, with report of necropsy.
Lichitensteimi and! Rosemi!)erg#{176} rel)orted! a
case of ilicoflhl)lete Sturge-\Veber’s disease,
vithiout facial iievti, comifirmed I)y
nec-ropsy, iii t 15-year-old girl.
Hunt ami(! v1oore2 recorded a case of
Sturge-Weber’s disease in a boy 12 years of
age, vithi left frontal amigiomatosis,
con-firmed 1)\’ perati0n.
Recemitly, Wohlwill amid Yakovlev21
pre-semited three ad!ditional cases with reports of
necropsies.
As far as we can determine, the present
case is the eighteenthi comifirmed case of this
disease reported! in the American literature.
SUMMARY
A case of Sturge-\Veber’s disease,
con-firmed by miecropsy, in a female 2 years
ok!, is presented.
This case, aside from its rarity, is
note-worthy iii that it is confirmed by necropsy,
that the facial lesion is bilateral while the
brain lesiomi is unilateral, and that there is
ami unusual amount of mineral deposit in
the i)rain considering the short diuration of
the disease.
A gemieral review of this interesting
condi-tion is Preselited!.
As far as we have been able to tell, this
case is the eighteenth confirmed case of Sturge-\Veher’s d!isease recorded in the
Aunenicami literature.
REFERENCES
1. Luschka, H .: Cavern#{246}se Blutgeschwulst
des Gehirmies. Arch. path. Amiat., 6:458,
1854.
2. Kalischier, S. : Demonstration des Gehirns clues Kindes miiit Teleangiektasie der hinkseitigen Cesichts-Kopfhaut tind Hir-nol)erflachie. Berl. kim. Wchnschr., 34: 1059, 1897.
:3. Kalischier, S. : Em Fall von Teleangiektasie (Angiom) des Cesichts rind der weichen Hirnhaut. Arch. Psvchiat., 34 : 171 , 1901.
4. Schirmer, R. : Em Fall von Teleangiektasie. Arch. f. Ophth., 7:119, 1860.
5. Sturge, \V. A.: A case of partial epilepsy,
apparently due to a lesion of one of the vaso-motor centres of the brain. Tr. Climi. Soc. London, 12:162, 1879.
6. Weber, F. P. : Right-sided hemi-hypotrophv
resulting from right-sided congenital sl)astic hemiplegia, with a morbid con-dition of the left sidle of the braimi, re-vealed by radiograms.
J.
Neurol. & Psychopath., 3:134, 1922. Also Proc.Roy. Soc. Med., 22:431, 1928.
7. Dimitri, V. : Tumor cerebral cong#{233}miito
(an-gioma cavernosum). Rev. Asoc. med.
argent., Buenos Aires,
36:
1029, 1923. 8. Krabbe, K. H. : Facial amid meningealangi-omatosis associated with calcifications of the brain cortex. Arch. Neurol. & Psychiat., 32:737, 1934.
9. Marque, A. M. : Considerations sobre angi-omas en Ia infancia. Rev. oto-neuro-oftal., 1:202, 1927.
10. Volland: Uber zwei F#{228}lleVOfl zerebralem Amigiom miebst Bemerkungen ilber Hir-nangiome. Ztschr. Behandl.
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J.
:Associ-ated facial and imtracranial
hemangio-mas. Am.
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Dis. Child., 51:868, 1936. 14. Cohen, H.J.,
and Kay, M. N. : Associatedfacial hemangioma and intracranial Ic-sion (Weber-Dimitni disease). Am.
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Dis. Child., 62:606, 1941.15. Yakovlev, P. I., and Guthnie, R. H. : Con-genital ectodenmoses (neurocutaneous symidnomes) in epileptic patients. Arch. Neunol. & Psychiat., 26: 1145, 1931. 16. Alexander, L., amid Woodhall, B. : Calcified
epileptogenic lesions as caused by
in-complete interference with the blood supply of the diseased areas.
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Neuro-path. & Exper. Neurol., 2: 1, 1943. 17. Bergstrand, H., Olivecrona, H., and Tdnnis,W. : Gefassmissbildumigen und Gef#{228}ss-geschwulste des Gehirns. Leipzig, Thieme, 1936.
18. Peters, G. : Zur Pathogenese den Sttmrge-Weberschen Krankheit. Ztschr. ges. Neurol. u. Psvchiat., 164:365, 1939. 19. Lichtcnstein, B. W., and Rosenberg, C.:
Sturge-Weber-Dimitni’s disease. Report
of an abortive case, with observations on forms, chemical nature, and pathogenesis
of cerebral cortical concretions.
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Neuropath. & Exper. Neurol., 6:369, 1947.328 STURGE-WEBER-DIMITRI DISEASE
21. Green,
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Roentgenol., 64:391, 1950. 22. Lichtenstein, B. W. : Sturge-Weber-Dimitni
syndrome. Cephalic form of neuno-cutalieous hemangiomatosis. Arch. Neunol. & Psvchiat., 71:291, 1954. 23. Hunt, H. B., amid Moore, R.C. :
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J.
Neuropath. & Exper. Neurol., 16:341, 1957.SUMMARIO IN INTERLINGUA
Morbo
De
Sturge-Weber-Dimitri
Un puera de racia blanc esseva hospitalisate al etate de 23/ 1 2 annos con Ic gravamine de convulsiones. Sn histonia l)a55Itte esseva
essen-tialmemite normnal, con Ic exceptioui did presentia
de tin congenite hemangioma l)ilaterai del facie que Corres1)Oui(ieva al dlistrii)ution dcl quinte nervo cranial. Studios interprendite al hospital
stimuiava Ic suspicion qiie illa suifreva de
an-giomatosis meningoencephialotrigemiuial (morbo de Sturge-Weber-Dimitri) e que le angioma leptomeningee esseva probabilemente fri Ic
hemisphenio sinistro-cerebral. Nuhle anormali-tates ocular esseva demonstrabile. Le
svrnpto-mas se mehiorava, e le patiente esseva dimittite. Plune menses plus tarde ilha esseva re-admittite a causa del recunrentia de sum attaccos
epilep-tiforme. Un pneumoencephalogramma indicava atrophia sinistno-cortical e allargamento mini-ma! del systema ventricular. Le condition del patiente se detenionava rapidememite, e illa mo-niva.
Le necropsia discopeniva mancate grados de atrophia sinistro-cortical con dysplasia heman-giomatose del leptomeninge sinistne. Un noent-genogramma post monte monstrava in le lobo sinistno-occipital del cerebro Ic presentia de finmente punctate areas de deposition mineral que esseva arrangiate in un configuration ton-tuose.
M icroscopicamnente Ic Ieptomeniiige sinistne esseva he sito de tin congenite amiomalia corn-ponite de innurnerabile dilatate spatios vascu-lan, le plus rnarcatc super le hobo occipital.
Le subjacente contice cerebral esseva atro-phic, con mancate gnados de proliferation glial e un mancate deposito mineral. Esseva opinate
1t1 isto consisteva de sales de ferro e de cal-cium.
Iste caso es notabilc per le facto que he lesion facial esseva bilateral e que tin quantitate in-usual de deposito mineral esseva pnesente in Ic
cenebro, ben que Ic duration del rnorbo habeva
essite breve. Es presentate tin nevista del
littena-tuna.
PnocRAris ANI) ACTIVITIES OF THE NATIONAL INSTITUTE OF MENTAL HEALTH
RELE-%‘ANT TO CHILDREN AND CHILD CARE, R. H. Felix. (Am.
J.
Pub. Health, 48:1:3:3,February, 1958.)
Pediatricians concerned with research in mental health of children should be aware
of this review of the activities of the National Institute of Mental Health. This Institute is conducting 1:39 projects on child development and behavior, including juvenile de-lin(juency, IiieIit1l retardation and emotional disturbances. Thirty-two of these are beimlg carnie(I out within the Institute and the others are supported by grants to medical schools and other nongovernmental agencies. Twenty per cent of the Institute’s grants
