Neuroradinlngg

Neuroradiology 18, 281-284 (1979)

© by Springer-Verlag 1979

Occlusion of the Cerebral Arteries in Recklinghausen's Disease
D. Taboada, A. Alonso, J. Moreno, D. Muro and F. Mulas
Section of Pediatric Neuroradiologyand Pediatric Neurology,Hospital Infantil 'La Fe', Valencia, Spain
Summary. Two children, aged 18 months and 6
years, who had Recklinghausen's disease, had occlusion of cerebral arteries. One child had no motor
deficit but the other had right hemiparesis and partial
occlusion of the left posterior cerebral artery, a fact
not found in the literature.

Key words: Arterial occlusive disease - Neurofibromatosis - Reckl~ghausen's disease

Introduction
Occlusion of the cerebral arteries in Recklinghausen's disease was first described by Hilal et al. in 3
patients in 1971 [4]. Subsequently, Tomsick et al. [8]
compiled all the published cases and found a total of
14 cases, including two patients of their own. From
1972 to 1978 among our patients with Recklinghausen's disease we found two children who had
occlusion of the cerebral arteries and basal telangiectasias. In one of the cases the posterior cerebral
artery was affected, a fact which has not been
reported in any of the published cases.

Case Reports

Case 1
Female, admitted to hospital at the age of 18 months
(21.4.72) because of gastroenterocolitis.

Personal history: Normal pregnancy and delivery.
Weight 3.7 kg. Numerous cafe-au-lait spots from
birth. No convulsions. No motor deficit.

Family history: Mother of 35 years affected by Recklinghausen's disease. This was the fourth pregnancy;
had two miscarriages. A younger brother with Recklinghausen's disease was studied angiographically
and will be referred to in the discussion. No other
record of neurofibromatosis in the family.
Examination: Macrocephaly p.98. Size p.3. Weight
p.3. Normal arterial pressure. Strabismus in the left
eye. Ophthalmoscopy normal. Slight psychomotor
retardation. Did not begin to walk until the age of 21
months. This retardation continues at present,
although not very marked. Multiple cafe-au-lait spots
distributed mainly on the trunk and abdomen, some
of them large, about 14 in number. At present the
patient continues without convulsions or motor
deficit.
Brain scan normal. Echoencephalography (A-scan)
normal. During these years there has been a slowing
tendency in the EEG.
Radiological study. Plain skull film: macrocephaly
with Cronqvist index of 62; enlarged chiasmatic
groove. These findings have not been modified over
the years.
Pneumoencephalography. (25.4.72) Moderate dilatation of the lateral ventricles, particularly the left,
with an Evans index of 0.30. Dilatation of interpenduncular and optochiasmatic cisterns, assuming
the form of a cyst. Large cisterna magna.
Left carotid angiography (25.4.72). Partial stenosis of the internal carotid artery 5 cm above the
posterior communicating artery. Complete occlusion
of the middle cerebral artery with marked hypertrophy of all basal branches which have a telangiectasic,
pseudoangiomatous formation (Fig. 1).
Hypertrophy of all branches of the posterior cerebral artery, mainly the temporal branches. Occlusion
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D. Taboada et al.: Arterial Occlusive Disease

Fig. 1. Case 1. Left carotid angiography. Complete
occlusion of middle cerebral artery with marked
hypertrophy of all basal branches which have a
telangiectasic, pseudoangiomatous appearance

Fig. 2. Case 2. Left carotid angiography. Occlusion
of internal carotid artery with multiple telangiectasic, pseudoangiomatous branches

Fig. 3. Case 2. Vertebral angiography. Telangiectasic branches coming from left posterior cerebral
artery. Complete occlusion of left posterior cerebral
artery

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D. Taboada et al.: Arterial Occlusive Disease

of the pericallosal artery, which is replaced by the
posterior pericallosal. Numerous collateral connections at all levels with retrograde filling of the
branches of the middle cerebral artery, principally
from the territory of the posterior cerebral.

Right carotid: Narrowing of primitive carotid artery
at cervical level, measuring 2 cm in length. Spasm
cannot be ruled out.

Righ~ carotid angiography. Stenosis of the anterior
cerebral artery at its very beginning with small telangiectasic branches filling the left anterior cerebral.
Vertebral angiography. Almost complete occlusion of
the left posterior cerebral artery with multiple telangiectasic branches coming out at the level of the
stenosis (Fig. 3).

Left carotid. (June 1974) The same findings persist.

Discussion

Vertebral angiography. Fine telangiectasic branches
coming from the first portions of the posterior cerebral artery, with hypertrophy of the posterior pericallosal, posteromedial and lateral choroid and the
thalamoperforanting arteries.

The involvement of the posterior cerebral artery has
not been described in any of the 14 cases found in the
literature [1-6, 8-12]. Case 2 had incomplete occlusion of the left posterior cerebral artery with projection of multiple telangiectasic branches.
The occlusion of the cerebral arteries in Recklinghausen's disease does not appear to be progressive. We checked our second case at the age of 2
years and did not find any angiographic changes.
Neither does Recklinghausen's disease appear to
affect all members of one family since we studied the
brother of our first case at the age of i year and at the
age of 6 years and did not find occlusion of any cerebral artery. The first case did not have any motor
deficit. This fact has been found in three other cases
published [4, 8]. Our two patients did not have any
accompanying intracranial tumor, nor had they
received radiation therapy. Thus, our two patients,
together with the other cases in literature, support
the theory that the occlusion of the cerebral arteries
in Recklinghausen's disease is primary.

Urography. Normal. Skeleton normal (6.6.78).
Computed tomography. No zones of cerebral infarct.
The slight increase in size of the ventricular system
persists, particularly the left and of the interpenduncular and optochiasmatic cislLerns.
Case 2
Male, 6 years old, admitted to hospital because of
acute right hemiplegia 1 week old.
. . . .
Normal pregnancy and delivery.
Weight 3.3 kg. Multiple cafe-au-lait spots from birth.

Family history. Father aged 31 years. Mother aged 30
years has Recklinghausen's disease, also a brother
aged 2. No other family record.
Examination. Head circumference normal (P50).
Normal weight and size. Normal psychomotor
development except for slight speech retardation.
Numerous cafe-au-lait spots all over the body, particularly on the trunk, about 25 in number. Right
hemiparesis. Ophthalmoscopy and blood pressure
normal. EEG: Spikes in the fight parietal region.
Echoeneephalography, normal.
Radiological study. Plain skull film normal, with
index of Cronqvist of 55. Normal sella and optic
foramens.
Urography. Normal. Skeleton normal.
Computed tomography normal. ?No infarct.
Left carotid angiography. Complete occlusion of the
internal carotid 4 m m from !Ihe posterior communicating artery (Fig. 2). Multiple telangiectasic,
pseudoangiomatous branches of fine caliber in entire
basal area.

References
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children. Neuropediatrie 4, 187-206 (1973)
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(1971)
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neurologia infantil. Barcelona: Cientifico Media 1971
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Neurofibromatosis and intracranial arterial occlusive disease.
Neuroradiology 11, 229-234 (1976)

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9. Umezu, R., Fujiu, M., Abe, T.: Two cases of yon Recklinghausen's disease with juxta-basal telagiectasia. Clin. Neurol.
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12. Voigt, K., Beck, U.: Arterial developmental anomalies of one
hemisphere with inter- and intrahemispheric steal effects in

D. Taboada et al.: Arterial Occlusive Disease
neurofibromatosis (Recklinghausen's disease). Radiol. Clin.
Biol. 43, 483-493 (1974)
Received: 8 June 1979
Dr. D. Taboada
Secci6n de Neuroradiologfa Pediatrica
Hospital Infantil "La Fe"
C/Alferez Provisional, 21
Valencia 9, Spain