Gabriel

L. Kalifa,

MD

#{149} Catherine
S

Chiron, MD Gerard Ponsot,

#{149} Nicolas
MD
#{149}

Sellier,

MD

#{149} Philippe
MD
#{149} Olivier

Guy

Lalande,

Demange, Robain,

MD MD

MR

Hemimegalencephaly: Imaging in Five Children

Hemimegalencephaly is a rare brain malformation characterized by cerebral asymmetry and cortical dysplasia. Infants with the condition present with early seizures and Severe encephalopathy. Five patients were studied with computed tomography and magnetic resonance (MR) imaging. MR imaging was the most efficient diagnostic method for this rare entity. It demonstrated brain hemispheric hypertrophy with lateral ventricle dilatation, abnormal gyral pattern, and a thick cortex on the enlarged side. The images correlate well with the known pathologic data.
Index terms: Brain, enlarged, abnormalities, 10.144 #{149} Brain, #{149} Brain, MR studies, 10.1214 nervous system, 10.144 Radiology 1987; 165:29-33 10.144 diseases, #{149} Infants, Brain, 10.144 central

is a rare malformation consisting of unilateral hypertnophy of the brain. The gyri are enlarged, and there is diffuse enlargement of the cortex with disapEMIMEGALENCEPHALY pearance of horizontal layering of

saturation) sequences (400 or 600/25) (repetition time [TRI msec/echo time [TE] msec) and long (spin-echo) sequences (2,000/40 or 80) were used. First, sagittal views were obtained with short sequences. Then were obtained coronal and axial views with Ti- and T2-weighted reviewed and the separate!y results were data. proof on (pa-

#{149}

the neurons. Glial abnormalities can be observed. These features are nestnicted to one hemisphere. Until now, the diagnosis was usually made only at autopsy. New imaging modalities, especially magnetic nesonance (MR) imaging, now allow necognition of this entity early in life. In addition, similar, but more localized, lesions have been discovered with MR imaging in patients presenting with the same symptoms. We describe five patients with congenital unilateral brain hypertrophy who presented with partial and very early epilepsy. Focal neurologic signs were present, and the circumference of the head was larger than normal. We discuss the diagnostic possibibities of MR imaging and consider the classification of this malformation.

spin-echo sequences. Examinations were by three radiologists, correlated with the All of our patients of the pathologic but correlation similar cases As indicated tient with therapy. followed 3) has several

clinical and EEC are alive, so no condition is avai!ab!e,

was made with data found in the literature. in Table 1, one chi!d severe seizures menta! each retardation day despite

Two patients up for 9 and retardation. crises followed 4 and

(patients 7 years, each

1 and respective-

2),

ly, have mild or two epileptic other months patients, (patients have

They have one month. Two only a few moderate A!l

up for 5), have with drugs.

retardation, patients and several

but no been antiepileptic

recurrent treated

seizures. steroids

RESULTS
In all cases
of

CT

scans shift

disclosed
responsi-

hy-

MATERIALS

AND

METHODS

pertrophy

a hemisphere

Clinical data for our five patients are summarized in Tab!e 1. All children were admitted for early seizures. The convulsions were partial, motor, and intractab!e. Clinical examination head circumference nial rologic Mental finding. ic limb asymmetry signs There or trunk were retardation in showed in five three present was an cases in all a constant enlarged and craneuearly of somatskin or there was electroenabnormal side. computed and MR necessary cases.

ble
of

for
the

a midline
lateral

and
on

dilatation
the hyper-

ventricle

trophic
a tumor.

side.
The

These
ventricular

signs

can

exclude in the ocsulci apthe oc-

dilatation

cases.

Focal

was no evidence asymmetry. No noted, In a!! and cases

involved the entire hemisphere three cases and was limited to cipital horn in two cases. The were poorly seen and the gyri peared less visible, mainly in
cipital area.

visceral lesion was no fami!ia! history. cephalography activity on

I

the

(EEC) showed hypertrophic underwent (CT) scanning was always

Irom

the

Departments

of Radiology

All

patients

(G.LK., N.S., PD., G.L.). Neuropediatrics (Inserm 429) (CC., Cr.). and Neuropathology (OR.), Hospit.il Saint Vincent de Paul, 74 Ave. Denfert-Rochereau, 75674 Paris, France. 1:rom the 1986 RSNA annual meeting. Received Dccomber 3, 1986; revision requested February 17, 1987; revision received March 25; accepted Max 6. Address reprint requests to G.L.K. CRSNA, 1987

tomographic aging. Sedation

MR
mg/kg) kg)

imaging,
was used.

for

or sodium

imfor which a!imenazine (1 pentobarbital (5 mgI performed with a 1.5-

Increased density of the white matter was noted in only one case. The opposite hemisphere, the brain stem, and the cerebellum were considered normal (Fig. la). MR imaging not only showed the
same further onal lesions well but it also The hem provided ispherinformation.

ic asymmetry
views. The was

was

well
entire

seen
hemisphere

on The

corhy-

MR imaging
T magnet net in the

was

in three patients two others. Both

and 0.5-T magshort (partial

was

enlarged

in three
limited

cases.
to the

pertrophy

occipi-

29

.:.

Figure
hemispheric moderate roughly white

1. Patient 2. Images in a patient with focal right neurologic signs who first experienced seizures at 9 months. (a) On CT scan, left hypertrophy with left lateral ventricle dilatation is evident. Sulci are poorly depicted. (b) MR image (600/25 sequence) depicts left hypertrophy with left ventricle dilatation. Some compression of left side of cerebellum can be seen, and the gyri are wider and defined. (c) On MR image (600/25 sequence), a thick cortical ribbon on the left side, wide gyri, and asymmetric enlargement of can be seen.

matter

Figure 2. Patient 3. MR images in patient with clinically evident left hemispheric asymmetry who experienced seizures the 1st day of life. (a-c) Coronal views (600/25 sequence) depict the main signs, including huge left hemispheric hypertrophy and enlargement of white matter, thickened cortex, abnormal ventricle midline shift to right, and loss of norma! gyral pattern, with no sulcus seen. (d, e) 12-weighted (2,000/40-80 sequence) transverse images show abnormal white-matter signal. The volume of the white matter is increased, there are gyral abnormalities, and the cortex is thickened.

topanietab areas in two cases. The hypentrophy bed to midline shift in all cases and cerebellar compression in four cases (Fig. 2a, 2b). Otherwise, the opposite hemisphere and the cerebelbum were apparently normal
d. e. (Fig. 2c).

30

Radiology

October

1987

fo E

. .

o
5)

I)

5)

o-;i

_
#{149}-

.0

0.

a
L,

b.
3. Patient 4. (a, b) 12-weighted (2,000/40-80 sequence) images depict hypertrophy localized to the posterior segment of the left hemisphere. Enlarged left occipital horn, widened gyri in left occipital lobe, and thickened cortex are well seen on first-echo image (a). Second-echo image (b) reveals a high-intensity signal in periventricular white matter of the left posterior horn.

-5) Ia.

Figure

_0
.

4, 5,

s
.

s a,
.

dilatation was clearly demonstrated with MR imaging (Fig. 2d). Gyral pattern abnormalities were obvious with this technique; the sulci were shallow and poorly defined and the gyni excessively wide. The cortical ribbon was thicker on the hypentrophic side;
Unilateral ventricular in comparison with the opposite side,

the
tex nab

limit
was

between
poorly seen

cortex
(Figs.

and
ic,

subcor2b).

Microscopic examination shows complete disorganization of the cytoarchitecture. All lamination into honizontal layers is lost. A large number of heterotopic subcortical neurons are seen. The most singular feature is the presence of giant neurons diffuseby scattered in the cortex. Finally, glial abnormalities are less constant (fibnibbary or protoplasmic gliosis). All of these abnormalities are clearly unilateral.

0 ou:a

.itbifl t
!
-

In all
was

our
noted

cases,
in

a very
the white

intense
matter

sigon

fo
x

T2-weighted sequences. The signal was significantly more intense than that of the contralateral white matter, and the extent of this signal was roughly parallel to that of the hypertrophy (Figs. 2d, 3). MR imaging did not reveal focal areas of hetenotopia.

Radiologic

Patterns
>

DISCUSSION Literature
Fifteen hemimegalencephaly

Very few nadiologic data exist concerning hemimegalencephaly. On CT scans, hypertrophy of one hemisphere with dilatation of the lateral ventricle on the same side is observed. No description of this finding on MR images has been previously reported, to our knowledge. All the images obtained in our patients seem to correlate well with the pathologic data. The brain hypentrophy and the ventricular enlargement are well demonstrated with MR imaging, especially on coronal images. The gynal modifications, suspected with CT, are well seen with MR imaging. The contical ribbon appears thicken on pathologic and MR imaging examinations, but was not seen on CT scans. Its poorly defined
by the heterotopic
5)

0
e4 Ln

0
0

E a
0

A2
0.

A2
0.

a
N

.u

Review
well-documented have

cases been

of re-

I-.
5)

-.

0.
bC(I)

se

.-

ported in the literature (1-12). The anatomic features of the condition are distinct and fairly homogeneous. In all cases, one can note a hemispheric hypertrophy, a firm consistency, and a modified gynal pattern on the enlarged side. The sulci are
present and The posite the cortex side, but gyri is shallower are and excessively than inner on contour its thicker than normal, wide. the opis

5)

E
5) 0 0

E-o

-o
e4

-o
N

5) .5 5) LI

inner
neurons.

contour
of

may
numer-

be

0 Ln

explained ous

existence

not
ter eral

well
is thicker ventricle

delineated.
than is dilated

The
normal

white
and (Fig. 4).

matthe lat-

signal intensity on T2-weighted lated to the glial

The high of the white matter sequences may be reabnormalities. Such

5) .5

Nm

VoIumel65

Numberl

Radiology

31

mass

effect,

the

absence

of modifica-

tion at follow-up, and the association of features such as ventricular dibatation. No association with congenital hemihypertnophy, such as BeckwithWiedemann syndrome, has been
found.
I

4. Pathologic specimen shows typical findings: hypertrophic right hemisphere, enlarged white matter, thickened cortex with loss of normal layering, and abnormal neuronal migration in the white matter.

Figure

5. T2-weighted (300/28 sequence performed on model 5000 scanner, Magniscan, [CGR, Paris]) images in 6-year-old girl with agyria-pachygyria with typical history. Note thickened cortex, few sulci, widened Sylvian fissure, asymmetry of brain, and

Figure

Contralaterab atrophy can be ruled for several reasons: (a) increased head circumference, (b) abnormalities seen on the enlarged side in MR images, (c) focal clinical signs comesponding to the enlarged hemisphere, and (d) the normal pattern seen on the small side. Agynia-pachygynia (13) must be considered in the differential diagnosis. In both entities, the cortex is thickened, the gyrab pattern is abnormab, and the clinical presentation is similar. There is evidence in both
out

diseases
tion

normal

T2-weighted

signal

intensity.

of abnormal neunonal migrathe first 4 months of fetal life (14). But in agyria-pachygynia lesions are bilateral, frequently one can note
in

Table 2 Differential

Features

of Hemimegalencephaly
Hemimegalencephaly

and Similar
Cortical Focal Dysplasia

Conditions
AgyriaPachygyria Tuberous Sclerosis

Feature Clinical presentation Seizures Mental retardation Macropathology Thick cortex Unilaterality Enlarged white matter Micropathology Horizontal layers Radiate disposition Gliosis Giantneurons Note.-+ found.
=

+ + + + +
-

+
-

+ + +
-

+ +
-

+ +
-

+ + +
-

+ not found, sometimes

+
-

+ + + + and sometimes not

typically

found,

typically

found

cerebral atrophy instead of hypertnophy, and no giant neuron is present (Fig. 5). Giant neurons are found in two other diseases, tuberous sclerosis and focal cortical dysplasia. Tuberous sclerosis (15) can easily be ruled out by its specific clinical and radiobogic signs. Focal cortical dysplasia (16, 17) exhibits some clinical and radiobogic similarities with hemimegabencephaby. More experience is needed to confirm or exclude the possibility that focal dysplasia is a minor expression of the same disorder. In both focal cortical dysplasia and hemimegalencephaby the cause of the condition memains unknown.

CONCLUSION
imaging features have been observed in our experience in lesions accompanied by gbiosis. MR imaging proved to be very efficient in aiding the diagnosis of the different lesions, giving more precise details than CT did. For all these reasons, the diagnosis of hemimegalencephaly seems likely in our patients, even without histologic proof. cunrence of seizures before 1 month of age carries a poor prognosis, but the severity also seems related to the extent of the lesions. For instance, our two patients with localized mabformations (patients 4 and 5) expenienced a less-severe evolution than the early symptoms would have suggested. The child who presented with the most severe encephabopathy (patient 3) had the most important cortical thickening. However the beast mental retardation was seen in a patient (patient 2) with a moderately thickened cortex. No other cases have been reported in the families of patients with hemimegabencephaly.
Hemimegalencephaly is a rare

brain
by by

malformation
early onset of

revealed
seizures.

clinicalThe di-

agnosis now MR imaging, one hemisphere

Presentation

and

Outcome

In our cases, as in the literature, the clinical presentation is fairly homogeneous. The patients present with early epilepsy, severe encephabopathy, and focal neunobogic signs. However, the prognosis may be diffenent from one patient to another: In some patients this disorder is lethal in the newborn period, whereas othen patients survive lessen intellectual
32

can be made easily with which demonstrates in a largely diffuse hypentrophy, lateral ventricular dilatation, abnormal gynab pattern, thickened cortex, and high-intensity signal in the white matter. The extent of the lesion seen on MR imaging examination seems to correlate with the severity of the prognosis. The cause of hemimegalencephaly
remains unknown. There is no famil-

jab incidence, but relation cortical dysplasia may be in the future. U References

to focal confirmed

Differential
In the 2) an ruled differential

Diagnosis
diagnosis (Table can be easily the absence of

1.

with greater or deficiency. The oc-

infiltrative tumor out, considering

Ward J, Lerner HH. Review of subject congenital hemihypertrophy and comp!ete case report. J Pediatr 1947; 31:403414.

of

Radiology

October

1987

2.

3.

4.

5.

6.

7.

Laurence KM. A case of unilateral megalencephaly. Dev Med Child Neurol 1964; 6:585-590. Bignami A, Palladini G, Zappella M. Unilateral mega!encephaly with cell hypertrophy: an anatomical and quantitative histochemical study. Brain Res 1968; 9: 103-114. Townsend JJ, Nielsen SL, Malamud N. Unilateral megalencephaly: hamartoma or neop!asm? Neurology 1975; 25:448-453. King M, Stephenson JBP, Ziervoge! M, Doyle D, Ga!braith S. Hemimegalencephaly: a case for hemispherectomy? Neuropediatrics 1985; 16:46-55. Dom R, Brucher JM. Hamartob!astome (gangliocytome diffus) unilateral de l#{233}corce c#{233}r#{233}brale. Neurol Rev 1969; 120:307318. Manz HJ, Phillips TM, Rowden C, McCullough DC. Unilateral megalencepha!y, cerebra! cortical dysplasia, neuronal hypertrophy, and heterotopia: cytomorphometric, fluorometric cytochemical, and biochemical analyses. Acta Neuropathol (Berl) 1979; 45:97-103.

8.

9.

10.

1 1.

12.

13.

14.

15.

Hallervorden J. Angeborene hemihypertrophie der linken korperh#{228}!fte einschliesslich des gehirns. Zentrabl ges Neurol Psychiat 1923; 33:518-519. Cross H, Viberrak B. Klinish-anatomishe befunde bei hemimegalencephalie: Virchow. Arch Pathol Anat 1955; 327:577589. Tjiam AT, Stefanko S, Schenk VWD, de Vlieger M. Infantile spasms associated with hemihypsarrhythmia and hemimegalencephaly. Dcv Med Child Neurol 1978; 20:779-798. Crome L. Infantile cerebral gliosis with giant nerve cells. J Neurol Neurosurg Psychiatr 1957; 20:117-124. Dambska M, Wisniewski K, SherJ. An autopsy case of hemimegalencephaly. Brain Dev 1984; 6:60-64. Ohno K. Lissencephaly in computed tomography. J Comput Assist Tomogr 1979; 2:92-95. Zimmerman RA, Bilaniuk L, Crossman RJ. Computed tomography in migratory disorders of human brain development. Neuroradiology 1983; 25:257-263. Cravioto H, Feigin I. Localized cerebral g!iosis with giant neurons histologically resembling tuberous sclerosis. J Neuropathol Exp Neurol 1960; 19:572-579.

16.

17.

Sourander P. Nordborg C, Silfvenius H, Blom S, Zetterlund B. Mild cortical dysplasia with intractable seizures: histological studies on 10 cases. Presented at the 16th Epilepsy International Symposium, Hamburg, Federal Republic of Cermany, September 6-9, 1985. Andermann F, Olivier A, Melanson D, Robitaille Y. Focal cortical dysplasia, macrogyria, and epilepsy: a study of 15 patients. Presented at the 16th Epilepsy International Symposium, Hamburg, Federal Republic of Cermany, September 6-9, 1985.

Volume

165

Number

Radiology

#{149} 33