s

u

ZUSAMMENFASSUNG
Familiare Dysauionomie bei einem nicht judischen Madchen mit dem histologischen Nachweis eines
fortschreitenden Prozesses im N. suralis
Es wird uber eine typische familiare Dysautonomie (HSN, Typ 111) bei einem nicht jiidischen Madchen
berichtet. Die Anzahl der unmyelinisierten Fasern war vermindert und die Suralisbiopsie ergab den Anhalt
fur eine postaxonale Degeneration. AuPerdem fand man eine ausgepragte endoneurale Fibrose und einen
Mange1 an groDen myelinisierten Fasern. Der histologische Nachweis eines fortschreitenden Prozesses, der fur
den N. suralis bisher noch nicht beschrieben worden ist, und andere klinische und morphologische Merkmale
konnten durch unterschiedliche Penetranzgrade der Erkrankung erklart werden.
RESUMEN
Disauionomfa familiar en una niAa no judfa con evidencia histol6gica de progresidn a nivel del nervio sural
Se aporta un caso tipico de disautonomia familiar del tip0 I11 en una nifia no judia. Se ha116 que el nlimero
de fibras no mielinizadas estaba reducido y la biopsia del nervio sural mostr6 evidencia de una degeneraci6n
postaxonal. Tambitn existfa una marcada fibrosis endoneural y una falta de fibras largas mielinizadas. Los
signos de progresi6n histolbgica, todavia no descrita en el nervio sural, asi como otras caracteristicas clinicas
y morfologicas podrian explicarse por diversos grados de penetrancia de la enfermedad.
References
Aguayo, A. J., Nair, C. P. V., Bray, G. M. (1971)
‘Peripheral nerve abnormalities in the Riley-Day
syndrome.’ Archives of Neurology, 24, 106-1 16.
Axelrod, F. B., Nachtigall, R., Dancis, J. (1974)
‘Familial dysautonomia: diagnosis, pathogenesis
and management.’ Advances in Pediatrics, 21,
75-96.
- Iyer, K., Fish, I., Pearson, J., Sein, M. E.,
Spielholz, N. (1981) ‘Progressive sensory loss in
familial dysautonomia.’ Pediatrics, 67, 517-522.
Dyck, 0.J., Kawamura,Y., Low, P. A., Shimono, N.,
Solovy, J. S. (1978) ‘The number and sizes of
reconstructed peripheral autonomic sensory and
motor neurons in a case of dysautonomia.’Journal
of Neuropaihology and Experimental Neurology,
31, 741-755.
Ferritre, G., Denef, J.-F., Rodriguez, J., Guzzetta, F.
(1985) ‘Morphometric studies of normal sural
nerve in children.’ Muscle and Nerve, 8, 697-704.
Klebanoff, M. A., Neff, J. M. (1980) ‘Familial
dysautonomia associated with recurrent osteomyelitis in a non-Jewish girl,’ Jolournal of Pediatrics,
96,75-77.
Levine, S. L.. Manniello, R. L., Farrell, P. M. (1977)

Features of
I ntracrania I
Aneurysms in
Infants and Report
of a Case
Edgardo A . Crisostomo
Edward Leaton
Edwin L. Rosenblum

68

Familial dysautonomia: unusual presentation in
an infant of non-Jewish ancestry.’ Journal of
Pediatrics, 90, 79-8 1.
Metha, K. (1978) ‘Familial dysautonomia in a
Hindu boy.’ American Journal of Diseases of
Children. 132, 7 19-723.
Nordborg, C., Conradi, N., Sourander, P.,
Westerberg, B. (1981) ‘A new type of nonprogressive sensory neuropathy in children with
atypical dysautonomia.‘ Acta Neuropaihologica,
55, 135-141.
Pearson, J., Dancis, J., Axelrod, F., Grover, N.
(1975) ‘The sural nerve in familial dysautonomia.’
Journal of Neuropathology and Experimenial
Neurology, 34, 413-424.
- Pytel, B. A., Grover-Johnson, N., Axelrod, F.,
Dancis, J. (1978) ‘Quantitative studies of dorsal
root ganglia and neuropathologic observations on
spinal cords in familial dysautonomia.’ Journal of
ihe Neurological Sciences, 35, 77-92.
Riley, C. M., Moore, R. H. (1966) ‘Familial
dysautonomia differentiated from related disorder;
case reports and discussion of current concepts.
Pediairics, 37, 435-446.

Intracranial aneurysms in infancy are rare.
A review of the English literature up to
1984 could turn up only 33 cases which
apparently were unrelated to trauma and
occurred in the first two years of life
(Alpers 1945; Matson 1965; Devadiga
et al. 1969; Pickering et al. 1970; Arai et al.
1972, 1973; Becker et al. 1978; Grode et al.
1978; Lipper et al. 1978; Orozco et al. 1978;
Keren et al. 1980; Ventureyra et al. 1980;
Kitano et al. 1981). Some of these reports
have been challenged-Stehbens
(1982)
proposed that a number of the cases could
actually have been ‘arteriovenous malformations’ and not true berry aneurysms.
We now report a case of a saccular
aneurysm in an 1I-month-old infant who
was followed for a year after surgery. We
highlight certain clinical and diagnostic

features which may help to distinguish
aneurysms in infants from those in adults.

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Case report
An 11-month-old girl was admitted to the Albany
Medical Center Hospital in June 1982 after
developing stiffness in the left arm and leg, followed
by a generalized tonic-clonic seizure. Additional
seizures had been noted in the ambulance and at a
local hospital where she was seen initially. A lumbar
puncture showed 116,000mm’ red blood-cells and
22,000mm’ white blood-cells, with a protein of
150mg/dl. Several more seizures occurred after
transfer to our hospital but were subsequently
controlled with anticonvulsants.
Her birth and developmental histories were
unremarkable, and she was able to walk without
support at 10 months of age. She had just begun to
utter occasional words. Amoxicillin had been
prescribed the day before she entered hospital with
possible otitis media. No trauma or any past medical
problems were reported. The family history was noncontributory.
The vital signs and head circumference were
normal. She cried at times but did not spontaneously
open her eyes or respond t o verbal stimuli. There were
no signs of trauma. The tympanic membranes were
normal. The neck was supple and the rest of the
general examination was normal, including her
peripheral pulses. On neurological examination, the
pupils measured 3mm in diameter and reacted
sluggishly to light; marked papilledema with multiple
retinal hemorrhages were found. Roving conjugate
eye-movements were seen occasionally, while
oculocephalic and corneal responses were normal.
There was a noticeable decrease in spontaneous
movements of the left extremities. Bilateral plantar
responses were extensor.
The initial hemogram showed a white-cell count of
15,600, with 21 bands, but the other routine studies,
including coagulation profile and arterial blood
gases, were within normal limits. An emergency
cranial C T scan showed a large collection of
subarachnoid blood and a temporal-region
hematoma, producing a shift of the mid-line
structures with compression of the right lateral
ventricle (Fig. 1). An angiogram performed the
following day demonstrated a saccular aneurysm in
the right middle cerebral artery, originating about
lcm distal to the double bifurcation and measuring
approximately 0 ~ 8 x 1 ~ 5 x 0 ~ 7(Fig.
c m 2).
The patient remained obtunded during the
following week. An EEG showed marked amplitude
suppression over the right hemisphere. Clipping of
the aneurysm was performed on the 16th day after
admission. When discharged five weeks after
admission she had a mild left hemiparesis. At a
follow-up visit one year later she had achieved the
normal developmental milestones for a two-year-old
and no residual neurological deficit could be detected.
Her EEG was normal. A CT scan disclosed cortical
hemispherical and focal opercular atrophy of the
right cerebrum (Fig. 3).

Discussion
It is the general consensus that aneurysms
result from an interday of structural

a

Fig. 1. CT scan shows high-density lesion in right
temporal region, with gyral staining and compression
of right lateral ventricle.

Fig. 2. Carotid angiogram shows aneurysm just
beyond double bifurcation of right middle cerebral
artery, with ‘square’-typeshift of anterior cerebral
artery (arrows).

69

Fig. 3. CT scan reveals marked widening of right
cortical sulci, signifving appreciable brain atrophy.

70

deficits and the pulsatile influence of
hemodynamic stress (Sekhar and Heros
1981). The occasional association of
polycystic renal disease and coarctation of
the aorta with cerebral aneurysms is
believed to be due to hemodynamic stress
induced by hypertension (Stehbens 1962).
The pathogenesis remains unknown,
however, for aneurysms occurring in
infants without those associated congenital
defects.
There are several distinguishing features
of this case and other reports in the
literature which may lead to early
diagnosis of intracranial aneurysm. The
ma1e:female ratio of a little less than 2: 1 in
patients below 20 years of age (Sahs et al.
1969) does not hold for aneurysms in
infants, where 18 out of 34 are male. 23 of
the 34 aneurysms occupied the anterior or
internal carotid segments. The location of
the aneurysm in our case was distal to the
major branching of the middle cerebral
artery and other authors have noted a
similar tendency for intracranial aneurysms
in infants to occur in the periphery
(Matson 1965, Arai etal. 1972, Grode et al.
1978, Lipper et al. 1978, Amacher and
Drake 1979). Further, the aneurysms
reported in 19 cases were greater than lcm
in diameter, similar to Thompson's study
in children (Thompson et al. 1973).

Of the 29 infants in the literature with
courses long enough to warrant description,
14 had manifestations of seizure episodes.
It is tempting to speculate that the
relatively high incidence of seizures in
ruptured aneurysms of infants is somehow
related to their more peripheral location,
regardless of whether the events are
secondary to rebleeding or to vasospasm.
A previous study had shown that late
seizures in adults, defined as those
occurring at least 12 hours after the initial
bleed, are usually due to rebleeding and are
associated with a poor prognosis (Hart
et al. 1981). The data for infants seem to
suggest the same (Pickering et al. 1970,
Ferry et al. 1974, Grode et al. 1978, Lee
et al. 1978).
Since aneurysms in infants are often
located distally they may be more
accessible to surgical manipulation
(Almeida et al. 1977). Consequently,
despite their rarity, it is crucial to recognize
their distinctive features. Several authors
have argued for an aggressive diagnostic
approach in dealing with these lesions
(Almeida et al. 1977, Lipper et al. 1978,
Storrs et al. 1982). Although computerized
tomographic (CT) scans have proven
helpful in the evaluation of intracranial hemorrhages in this age-group
(Krishnamoorthy et al. 1977), Hungerford
et al. 1981, Inoue et al. 1981), a high
degree of clinical suspicion is indispensable.
Based on this report and a review of the
literature, we have found that intracranial
aneurysms in infants are: (1) usually
greater than lcm in diameter, (2) often
peripherally located, (3) frequently accompanied by seizures, and (4) relatively
amenable to direct surgical attack by virtue
of their distal location.
Accepted for publication 28th May 1985.
Acknowledgements
We thank Dr. Venkatesh and Ms. Heath for their
assistance in the preparation of this manuscript.
Authors' Appointments
'Edgardo A. Crisostomo, M.D., Fellow, Department
of Medicine (Neurology), Duke University Medical
Center, V.A. Hospital Bldg. 16, Durham, NC 27705;
Edward Leaton, M.D., Fellow, Department of
Neurology, University of Virginia, Charlottesville,
VA.
Edwin L. Rosenblum, M.D., Associate Professor,
Department of Neurology, Albany Medical College,
New Scotland Avenue, Albany, NY 12208.
*Correspondence to first author.

SUMMARY
An 11-month-old girl presented with seizures, papilledema and a left hemiparesis. Radiographic examination
revealed a saccular right middle cerebral-artery aneurysm. Clipping was performed 16 days after admission.
At follow-up a year later there was no residual neurological deficit. Aneurysms occurring in infants are
relatively large and more peripheral in location compared with adults, and frequently are accompanied by
seizures. Infants’ aneurysms also tend to occupy the anterior circulation distribution, and their peripheral
location may make them more amenable to surgical intervention.

I.
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G
m

9

R~SUMB
Caractiristiques des anivrysmes intracraniens du nourrisson: rapport dun cas
Une fillette de 11 mois pr6sentait des crises, un oedtme papillaire et une htmipartsie gauche. Un examen
radiographique rtvtla un antvrysme sacculaire de l’arttre sylvienne droite. Une ligature fut rtaliste 16 jours
aprbs l’admission. Au suivi de l’observation, une annte plus tard, il n’y avait pas de dtficit neurologique
rtsiduel. Les antvrysmes du nourrisson sont relativement larges et de localisation plus ptriphtrique comparts
ii ceux de l’adulte; ils sont frtquemment accompagnts des crises. Les antvrysmes du nourrisson tendent
tgalement B occuper la distribution circulatoire anttrieure et leur localisation pkiphbrique peut les rendre
plus accessibles B I’intervention chirurgicale.

d

ZUSAMMENFASSUNG
Symptome intracranieller Aneurysmen bei Siiuglingen: ein Fallbericht
Der Fall eines 11 Monate alten Madchens mit Anfallen, Papilknodem und einer linksseitigen Hemiparese
wird geschildert. Rontgenologisch wurde ein sackformiges Aneurysma der A. cerebri media nachgewiesen. 16
Tage nach Aufnahme des Kindes wurde das Clipping durchgefiihrt. Bei der Kontrolle nach einem Jahr
fanden sich keine neurologischen Ausfalle. Aneurysmen bei Kindern sind relativ groR und im Vergleich zu
Erwachsenen mehr peripher gelegen und sie sind haufig mit einer Anqallen verbunden. Aneurysmen bei
Sauglingen neigen dazu, sich im Berich der vorderen Hirnzirkulation auszubreiten und ihre periphere
Lokalisation macht den chirurgischen Eingriff einfacher.
RESUMEN
Caracteristicas de los aneurismas iniracraneales en lactanies: aportacion‘ de un caso
S e trata de una niAa de 1 1 meses de edad con convulsiones, edema de papila y hemiparesia izquierda. El
examen radiogrifico revel6 un aneurisma sacular de la arteria cerebral media izquierda. Se colocb un clip a
10s 16 dias de su ingreso. Vista un aAo miis tarde no habia rastro de dtficit neurol6gico. Los aneurismas en
el lactante son relativamente grandes y de localizacibn m i s periferica que en el adulto y se acompaiian con
frecuencia de convulsiones. Tienden a ocupar la distribucibn de la circulacibn anterior y su localizaci6n periftrica
10s puede hacer mis asequibles a1 tratamiento quinlrgico.
References
Almeida, G. M., Pindaro, J., Plese, P., Bianco, E.,
Shibata, M. K. (1977) ‘Intracranial arterial
aneurysms in infancy and childhood.’ Child‘s
Brain, 3, 193-199.
Amacher, A. L., Drake, C. G. (1979) ‘The results of
operating upon cerebral aneurysms and angiomas
in children and adolescents. I: Cerebral aneurysms.
Child’s Brain, 5, 151-165.
Arai, H., Sugiyama, Y., Kawakami, S. (1972)
‘Multiple intracranial aneurysms and vascular
malformations in an infant.’ Journal of
Neurosurgery, 31, 357-360.
- Ueki, K., Takahara, Y. (1973) ‘Intracranial
aneurysm in an infant.’ Surgical Neurology, 1,
94-96.
Becker, D. H., Silverberg, G. D., Nelson, D. H.,
Hanbery, J. W. (1978) ‘Saccular aneurysm of
infancy and early childhood.’ Neurosurgery,
2, 1-7.
Devadiga, K. V., Mathai, K. V., Chandy, J. (1969)
‘Spontaneous cure of intracavernous aneurysm of
the internal carotid artery in a 14-month-old child.’
Journal of Neurosurgery, 30, 165-168.
Ferry, P. G., Kerber, C., Peterson, D., Gallo, A. A.
(1974) ‘Arteriectasis, subarachnoid hemorrhage
in a 3-month-old infant.’ Neurology, 24,494-500.
Forster, F. M., Alpers, B. J. (1945) ‘Anatomical
defects and pathological changes in congenital
cerebral aneurysms.’ Journal of Neuropaihology
and Experimental Neurology, 4, 146-1 54.
Grode, M. L., Saunders, M., Carton, C. A. (1978)
‘Subarachnoid hemorrhage secondary to ruptured
aneurysms in infants.’ Journal of Neurosurgery,
49. 898-902.

Hart, R. G., Byer, J . A., Slaughter, J. R., Hewett,
J. E., Easton, J. D. (1981) ‘Occurrence and
implications of seizures in subarachnoid hemorrhage due to ruptured intracranial aneurysms.’
Journal of Neurosurgery, 8, 417-421.
Hungerford, G. D., Marzluff, J. M., Kempe, L. G.,
Powers, J. M. (1981) ‘Cerebral arterial aneurysm
in a neonate.’ Neuroradiology, 21, 107-1 10.
Inoue, Y., Saiwai, S., Miyamoto, T., Ban, S.,
Yamamoto, T., Takemoto, K., Taniguchi, S.,
Sato, S., Namba, K., Ogata, M. (1981) ‘Postcontrast CT in subarachnoid hemorrhage from
ruptured aneurysm.’ Journal of Computer Assisted
Tomography, 5, 341-344.
Ke,ren, G., Barzilay, Z., Cohen, B. E. (1980)
Ruptured intracranial arterial aneurysm in the first
year of life.’ Archives of Neurology, 31, 392-393.
Kitano, I., Fukui, M., Kinoshita, K. (1981) ‘Infantile
cerebral aneurysm.’ Surgical Neurology, 16,
386-390.
Krishnamoorthy, K. S., Fernandez, R. A., Momose,
K. J., DeLong, G. R., Moylan, F. M. B., Todres,
I. D., Shannon, D. C. (1977) ‘Evaluation of
neonatal intracranial hemorrhage by CT.’
Pediatrics, 59, 165-172.
Lee, Y. J., Kandall, S. R., Ghali, V. S. (1978) ‘Intracranial arterial aneurysm in a newborn.’ Archives
of Neurology, 35, 171-172.
Lipper, S., Morgan, D., Krigman, M. R., Staab,
E. V. (1978) ‘Congenital saccular aneurysm in a
19-day-old neonate: case report and review of
the literature.’ Surgical Neurology, 10, 161-165.
Matson, D. D. (lq65) ‘Intracranial arterial aneurysms
in childhood. Journal of Neurosurgery, 23,
578-583.

71

Orozco, M., Trigueros, F.,. Quintana, F. (1978j
‘Intracranial aneurysm In early childhood.
Surgical Neurology, 9, 247-252.
Pickering, L. K.,Hogan, G. R.,Gilbert, E. F. (1970)
‘Aneurysm of the posterior inferior cerebellar
artery: rupture in a newborn.’ American Journal
of Diseases of Children, 119, 155-158.
Sahs, A. L., Perret, C. T., Locksley, H. B. (1969)
Intracranial Aneurysms and Subarachnoid Hemorrhage. Philadelphia: Lippincott.
Sekhar, L. N., Heros, R. C. (1981) ’Origin, growth
and rupture of saccular aneurysms: a review.’
Journal of Neurosurgery, 8, 248-260.
Stehbens, W. E. (1962) ‘Cerebral aneurysms and
congenital abnormalities.’ Australasian Annals of

CongenitaI
Polycyst ic Disease
of the Brain: Report
of an Unusual

Case
Naim-ur-Rahman
Khalifa A. Rahim Adam

72

Intracerebellar epithelial cysts are rarefewer than 20 cases of supratentorial
ependymal cysts have been reported to
date (Fried and Yasargil1977). We present
a case with multiple congenitalintracerebral
cysts which are sufficiently characteristic
to distinguish them from the other more
common fluid-filled cystic lesions such as
intraventricular colloid cysts or extracerebral arachnoid cysts (Moore and Book
1966, Bouch et al. 1973). Our case differs
from previously reported cases in the
following ways: (1) multiple glial/
ependymal cysts almost totally occupied
the white central matter of the frontal and
temporoparietal lobes bilaterally, leaving
no recognisable ventricular system; and (2)
all 17 cases reported in the literature
presented in adult life, the average age
being 47 years (Bouch et al. 1973, Fried
and Yasargil 1977), whereas our patient
developed progressive hydrocephalus and
neurological deficit in early infancy.

Medicine, 11, 102-112.

- (1982) ‘Intracranial berry aneurysms in infancy.’
Surgical Neurology, 18, 58-60.
Storrs, B. B., Humphreys, R. P., Hendrick, E. B.,
Hoffman, H. J. (1982) ‘1nt:acranial aneurysms in
the pediatric age-group. Child‘s Brain, 9,
358-361.
Thompson, J. R., Harwood-Nash, D. C., Fitz, C. R.
(1973) ‘Cerebral aneurysms in children.’ American
Journal of Roentgetzofogy, Radium Therapy and
Nuclear Medicine, 118, 163-175.
Ventureyra, E. C. G., Choo, S. H., Benoit, B. G.
(1980) ‘Super giant globoid intracranial aneurysm
in an infant.’ Journal of Neurosurgery, 53,411-416.

Case report
This two-month-old boy was brought to us in August
1983, his mother having noticed a big head and
occasional seizures since birth. Delivery had been
normal and there was no history of trauma. Head
circumference at two months was 42.5cm and the
fontanelles were full. A period of observation
confirmed the progressive nature of the hydrocephalus. At the age of five months his head started
enlarging more rapidly and he developed clear-cut
evidence of raised intracranial pressure, including a
bulging fontanelle and vomiting. Slowness in passing
normal milestones and spasticity of all four limbs was
also noticed at this stage.
A CT scan performed on 5th November 1983
revealed multiple cystic cavities of varying sizes
almost totally occupying both cerebral hemispheres
and leaving no trace of a recognisable ventricular
system (Fig. 1).
Initial management consisted of serial cyst
punctures and aspiration of their contents to relieve
the raised intracranial pressure. Different cysts were
punctured at different times and revealed three types
of contents under pressure: two contained watery
clear fluid, one contained milky viscous and mucoid
liquid and one contained greenish-brown motor oillike fluid. The protein content of the cyst fluids
ranged from lOOmg to 3.5g per 100ml. Air was
introduced into different cysts following aspiration of
their contents. Figure 2 shows CT-air-cystography
carried out after multiple cyst aspirations. Reduction
in the size of the cysts as well as air delineating a
shrunken cyst can be seen.
The regime of repeated cyst drainage through the
fontanelle resulted in temporary improvement, with
satisfactory control of intracranial pressure, arrest of
hydrocephalus and a slack fontanelle. However, this
improvement lasted for only two months, when the
child was readmitted with rapid progression of
hydrocephalus and signs of raised intracranial
pressure. CT scan showed an enlargement of all the
cysts. An extensive right temporoparieto-occipital
craniotomy was carried out at this stage, revealing an
area of temporoparietal cerebral cortex which was
normal-looking but tense. At a depth of about Icm
the brain canula met the resistance of the cyst wall and
its puncture revealed a large quantity of clear watery
fluid. The cyst cavity was opened, its wall partially
resected and partitions between this and adjacent