Del Giudice et al.

Vol. 10, No. 2, 1979

| Neuropadiatrie

ATYPICAL ASPECTS OF HYPERTENSIVE ENCEPHALOPATHY
IN CHILDHOOD
E. Del Giudice and /. Aicardi

Giudice, E. Del and Aican&i, ].: Atypical aspects of hypertensive encephatopathy in childhood.
Neuropadiatrie 10: 150—157 (1979). Four patients with hypertensive encephalopathy and misleading focal symptomatology are reported in order to point out the problems of differential
diagnosis. The first patient, together with a classical syndrome of hypertensive encephalopathy,
had peculiar EEG features consistent with a possible diagnosis of herpes simplex encephalitis.
Case 2 presented with a complex clinical syndrome associating bizarre spells suggestive of a
psychiatric condition with ocular symptoms pointing to an upper brain stem involvement.
The third child was remarkable because of the presence of neuroradiological signs compatible
with a space-occupying lesion of the posterior fossa not eventually found after a surgical
exploration performed as an emergency procedure.
The last patient had central nervous system signs in the context of a Guillain-Barre syndrome:
in this case the central symptomatology would not fit the already described pattern of encephalomyelo-radiculo-neuropathy but had to be entirely ascribed to the ill controlled arterial hypertension.
Hypertensive encephalopathy
culo-neuropathy

periodic EEG

Introduction
Hypertensive encephalopathy can be
defined as an acute reversible syndrome
caused by a sudden increase in blood
pressure which exceeds the limits of
cerebral autoregulation (Finnerty 1972,
Gifford and Westbrook 1974, Van
Vught et al. 1976, Cuneo and Caronna
1977). Brain edema has been the most
common finding at autopsy in fatal
cases and has also been experimentally
studied (Auer 1977 a and b). Presenting
symptoms are usually severe generalized headache followed by progressive
Received: Oct. 9, 1978

posterior fossa mass

encephalo-myelo-radi-

clouding of the sensorium which may
ultimately culminate in coma. The fullblown picture may include vomiting,
generalized seizures and myoclonic
movements of the extremities. Focal
neurologic signs such as hemiplegia and
hemiparesis, hemianopia, aphasia, cortical disorders of vision and partial seizures have all been reported as belonging to the clinical picture of hypertensive encephalopathy. Their frequency
appears to be very low: in the 43
cases of Ziegler et al. focal neurologic signs were present in one pa-

Accepted: Nov. 21, 1978

Address: E. del G., Clinica Pediatrica delle 2da Faculta di Medicina. Via Pansini 5, 80131 Naples
Acknowledgements: We wish to thank Pr. Cloup, Dr. Lavaud for their kind permission to study their patients.

150

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Hopital Saint-Vincent de Paul, 74 Avenue Denfert-Rochereau, 75674 Paris Cedex 14, France

Hypertensive encephalopathy in childhood

encountered, to point out the diagnostic problems that hypertensive encephalopathy can bring about.
Case histories
Case 1

J. S., a five year-old girl suffered
from disseminated non-Hodgkin lymphoma diagnosed six months previously
and associated with hypercalcemia (18
mgldl), hypertension (1501100 mmHg)
and renal failure due both to hypercalcemia and tumoral infiltration. On the
morning of November 16, 1977 while
hypercalcemia, hypertension and renal
fonction were well controlled, vomiting, headache and visual disturbances
appeared, together with a sudden elevation of blood pressure (1601110
mmHg) : progressive stupor followed

LFC
-

-

-

\

Fig. 1 EEG profile showing regular periodic complexes

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tient only and in the extensive review
on acute hemiplegias and hemisyndromes in childhood by Isler (82 cases) there
were two instances of acute hemiplegia
and aphasia associated with severe hypertension. Blindness of cortical origin
was the presenting symptom in three of
the patients reported by Jellinek et al.
(1964) and in one of them extremely
disturbing and highly organized visual
and auditory hallucinations were present.
Prompt reversal of the syndrome
after hypotensive treatment has been
suggested as a definitive criterion for
the diagnosis of hypertensive encephalopathy (Gifford and Westbrook 1974,
Cuneo and Caronna 1977).
We report 4 cases in whom misleading focal symptoms and signs were

Vol. 10, No. 2, 1979

1 NeumpUiatrie

and after a few hours a brief general- mmHg), which was followed by repeaized seizure was observed. At that time ted generalized seizures rapidly turning
hypernatremia was present (150 mEq/l). into status epilepticus. CSF findings at
Fundoscopic examination was normal that time were within normal limits, as
as were CSF findings.
were full blood count, blood urea and
The EEG recorded on November 19, electrolytes. Neurological symptoms
1977 displayed generalized ~eriodic disappeared gradually over a two-daycomplexes (Fig. 1) similar to those ob- period and consciousness returned to
served in herpes simplex encephalitis. normal: a frank nuchal rigidity was preThe hypothesis of a viral encephalitis sent. A normal carotid angiogram was
was discussed. Neurological examina- then obtained. Selective renal arteriotion revealed no focal abnormalities: graphy showed at the same time a stenothe child seemed to be blind. The course sis of the superior polar artery of the
of the acute episode was marked by pro- transplanted kidney, roba ably due to
found disturbances of consciousness thrombosis.
On December 6 he became again stuwith periods of restlessness and delirium
and by difficult control of blood pres- porous and had episodes of apnea which
sure: no seizures were observed after the required tracheal intubation and assisted
first one at the beginning of symptoma- ventilation for two days. Recorded
tology. Gradually, over a five day- apneic spells lasted several seconds (max.
period, improvement of the clinical 18 sec.) and were not associated with
state ensued together with normaliza- electroencephalographic abnormalities.
Disturbances of consciousness consisted
tion of the EEG.
Summary: A five year-old child with in periods of uncontrolled agitation and
a classical picture of hypertensive ence- frank delirium: the symptoms were not
phalopathy. The EEG features, how- accompanied by hypertension and reever, were reminiscent of those descri- mitted spontaneously four days latei. A
bed in herpes simplex encephalitis, thus normal brain C T scan was obtained on
December 8.
raising a serious diagnostic problem.
The boy remained then in a normal
Case 2
clinical state until December 14. At that
P. L., a seventeen year-old boy under- time, repeated episodes of agitation with
went renal transplantation in Septem- disorientation and apparent hallucinaber 1977 because of chronic renal tions occurred in rapid succession over
failure, hypertension and renal osteody- 48 hours. These bouts started with a
strophy associated with bilateral renal complaint of dizziness, immediately
segmental hypoplasia. The acute episode followed by loss of consciousness lasting
began abruptly on the evening of De- approximately three minutes. They were
cember lst, 1977 when hecomplainedof associated with head turning alternaviolent bitemporal headache concomi- tively to the right and to the left side,
tant with a hypertensive bout (2101100 with mydriasis. After about two minu-

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Del Giudice et al.

tes, respiratory motion stopped and
cyanosis developped. Consciousness reappeared on resumption of respiratory
movements, but was initially blurred
while purposeless choreiform activity
was observed. These episodes were spontaneous, stereotyped in character and
completely amnesic. Vigilance was normal in-between.
Examination showed horizontal and
vertical nystagmus when gazing downwards. Total paralysis of upward gaze
was evident and downward gaze was
slightly impaired. Pupillary reactions
to light and accomodation were normal.
These bouts gradually disappeared
and no neurological abnormality was
detected on discharge.
Summary: This case was characterized by bizarre complex spells and by an
oculomotor symptomatology suggesting
an upper brain stem lesion. C T scan,
however, was normal and all clinical
abnormalities resolved within a few
days.

cluded unequal non-reactive pupils and
absent corneal reflexes. Painful stimulation elicited a decerebrate pattern with
inward turning movements of the extended upper limbs. Respiration was
shallow and irregular with frequent
pauses. At lumbar puncture the CSF
protein was 1.50 g/l with 50 cells per
cubic millimeter (2,600 red cells/mm3).
Electroencephalogram showed focal
slowing confined to the right hemisphere.
The next morning, the coma deepened
to grade IV: pupils were small and nonreactive. Neurological examination
showed hypotonia and areflexia. Painful stimuli evoked decerebrate rigidity.
Ocular fundi were normal. Echoencephalography and right carotid arterio-

Case 3

P. C., a seven year-old boy, had been
on hemodyalysis since August 1974 because of renal failure due to focal sclerosing glomerulopathy with segmental
hyalinosis, until he underwent renal
transplantation in May 1977.
After a period of good control of
renal function he experienced a hypertensive crisis (2001140 mmHg) with
headaches on November 29, 1977.
Thereafter, he suddenly developed
progressive stupor and coma. On admission the child was in coma grade 111
(Seshia et al. 1977): ocular findings in-

Fig. 2 CT scan of the brain showing multiple
areas of increased density in the posterior fossa

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Hypertensive encephalopathy in childhood

Vol. 10, No. 2, 1979

1 Neuropidiatrie

Fig. 3 Ventriculography with
the hydrosoluble medium meglumine iocarmate showing
anterior displacement of the
aqueduct and fourth ventricle,
suggestive of a cerebellar spaceoccupying lesion

graphy (ipsilateral to the previously and needed rehabilitation because of
noted m~driasis)were ~erformedand persistent spasticity. The electroencegave normal results. Hyponatremia was phalogram remained markedly dipresent (121 mEq/l). In the afternoon a sturbed and showed bilateral posterior
C T scan demonstrated a slight ventricu- slowing. The boy was seen again a few
lar dilatation and multiple areas of in- months later. No neurological abnormacreased density in the posterior fossa lity was noted and his mentation was
normal.
(Fig. 2).
Summary: This case was remarkable
Ventriculography with the hydrosoluble medium meglumine iocarmate by the presence of neuroradiological
(Dimer X) showed anterior displace- signs suggesting an expansive lesion of
ment of the aqueduct and fourth ven- the posterior fossa which lead to operatricle suggesting a cerebellar space- tion. No significant parenchymal heoccupying lesion (Fig. 3). A neurosurgi- morrhage was found, however, and it is
cal exploration was performed on the probable that the areas of increased
fourth day after admission. A small density showed by the C T scan repreamount of dark blood was found on sented foci of edema and microscopic
opening the dura mater in the occipital hemorrhage due to blood-brain barrier
region but no further hemorrhage was impairment.
encountered by needling the cerebellum
Case 4
which appeared of soft consistency.
F. A,, a nine-year-old boy began to
Thereafter, gradual improvement of the
level of consciousness took place until complain of diffuse myalgias and low
December 14th when the child reached a back pain, followed by impairment of
grade I coma: henceforth he stagnated swallowing and phonation. Two days

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Del Giudice et al.

later, weakness of the lower limbs developed, rapidly progressing to complete
~ a r a l ~ s iOn
s . admission, on March 22,
1978, he was normally conscious and
afebrile but could hardly speak. He
complained of lumbar pain and paresthesiae in the feet and fingers. Moderate weakness of the upper limbs and
profound weakness of the lower limbs
was present with absent tendon jerks:
cremasteric and abdominal reflexes
were also absent. Cranial nerves VII,
IX, X were affected. Nuchal rigidity
was evident together with positive Kernig and Brudzinski signs. Ocular fundi
were normal. At lumbar puncture the
CSF protein was 1.60 g/l with 2 cells
per cubic millimeter. Following an episode of acute respiratory distress he was
intubated and mechanically ventilated.
On March 25, at 12 o'clock, coincident
with a hypertensive paroxysm (170/110
mmHg), progressive stupor and coma
developed together with generalized
seizures. No abnormalities of optic
discs were found. The EEG was diffusely abnormal with an excess of slow
waves associated with bilateral spikes in
the frontal regions. Frequent abnormal
decerebrate movements of upper limbs
were noted. Blood pressure remained
difficult to control even with intravenous diazoxide. Until March 29th, he
remained in coma grade 111: pupils were
small, equal and reactive to light. No
decorticate or decerebrate pattern could
be obtained in response to stimulation.
Focal slowing in the left parieto-occipital region was present on the EEG. On
March 28, acute right ventricular failure
with tachycardia, hepatomegaly and

distended jugular veins developed coincident with a hypertensive bout (1501
100 mmHg).
After neurological symptomatology
was established, autonomic lability
manifested itself as paroxysmal blood
pressure rises, persistent tachycardia
associated with episodes of transitory
bradycardia, and persistent fever of
39 OC.
After March 29th, consciousness returned to normal and neurologic symptoms disappeared gradually. EEG
showed again diffuse slowing which was
progressively replaced by physiological
rhythms. Control of blood pressure,
however, remained difficult with periodic paroxysms superimposed on elevated basal levels.
Summary: In this case the classical
symptomatology of hypertensive encephalopathy was present. The association
of central abnormalities with the picture
of the Guillain-BarrC syndrome was
confusing and would have led to a
diagnosis of encephalo-myelo-radiculoneuropathy (Gamstorp 1974), had not
the arterial hypertension ben detected.
Discussion
Although focal symptoms and signs
are a well recognized feature of hypertensive encephalopathy (Cuneo and Cavonna 1977), they are generally limited
to partial convulsions and to localized
deficits such as hemiparesis, aphasia,
hemianopia. The present cases demonstrate that the spectrum of the neurological manifestations of acute hypertensive encephalopathy is broader than
generally thought and may include pa-

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Hypertensive encephalopathy in childhood

roxysmal EEG complexes (case I), brain
stem involvement (case 2) and posterior
fossa syndromes (case 3) with impressive neuroradiological abnormalities.
The relation of the unusual signs we
observed to hypertensive encephalopathy is confirmed by their prompt disappearance with control of the blood
pressure in every case, as well as by
their temporal association with other,
more conventional, manifestations,
Moreover, periodic EEG paroxysmal
activity has already been mentioned by
Jellinek et al. (1964). In their patients,
however, the EEG paroxysms as shown
by their Figures 1 and 3 were not as
reminiscent of those of herpes simplex
encephalitis as were the periodic complexes observed in our patient 1.
The misleading character of the
neurological features we are reporting is
illustrated by the diagnostic difficulties
raised by our patients. The third patient
was submitted to unnecessary operation
on account of the C T findings which
suggested an intracerebellar hemorrhage
(Muller et al. 1975, Blank et al. 1978).
The bizarre seizures of our second patient were initially regarded as possibly
psychogenic in origin because of the lack
of any abnormal EEG activity, until a
more thorough neurological examination disclosed the presence of a Parinaud
syndrome. In case 4, the occurrence of
central nervous system dysfunction in
the course of a severe Guillain-Barrk
syndrome, raised the issue of the possible
involvement of the brain by the primitive disorder. Central nervous system
has been
in PO'Yradiculoneuritis and the term "ence-

Vol. 10, NO. 2, 1979

I Neuropidiatrie

phalo-myelo-radiculo-neuropathy" has
been coined by Gamstorp to designate
such cases. We would like to suggest
that such a diagnosis should not be
accepted so long as any possibility of
hypertensive encephalopathy has not
been ruled out, since arterial hypertension is so commonly observed in
Guillain-Barrd patients (Davidson and
Jellinek 1977) and since hypertensive
encephalopathy calls for an immediate
and effective therapy.
No satisfactory explanation is available for the localized character of some
of the CNS lesions due to hypertension.
The disruption of the blood brain barrier which is described in hypertensive
encephalopathy (Auer 1977) is not
homogeneously distributed. Circumscribed areas of edema and hemorrhage
could account for the C T scan picture
in our third patient. A small lesion in
the upper brain stem interfering with
the activating reticular system would
explain the curious clinical picture
shown by the second patient.
Computed tomography has been
little utilized so far in hypertensive
encephalopathy and it is likely that
more information about the nature and
the extent of the process will result
from its more liberal use.
References
1. Auer, L.: Brain edema in acute arterial
hypertension. Acta Neuropathol. 38:67:
(1977 a).
2. Auer, L.: The role of cerebral perfusion
pressure as origin of brain edema in acute
arterial hypertension.
Eur. Neurol. 15: 153
~.
(1977 b).
3. Blank, N. K. et al.: Posterior fossa subdural hematomas in neonates, Arch. Neurol. 35: 108 (1978).

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Del Giudice et al.

4. Cuneo, R. A., Caronna, J. J.: The neurologic complications of hypertension. Med.
Clin. North Amer. 61: 565 (1977).
5. Davidson, D. L. W., Jellinek, E. H.: H y pertension and papilledema in the Guillain-BarrC syndrome. J. Neurol. Neurosurg.
Psych. 40: 144 (1977).
6. Finnerty, F. A.: Hypertensive encephalopathy. Am. J. Med. 52: 672 (1972).
7. Gamstorp, I.: Encephalo-myelo-radiculoneuropathy. Involvement of the C N S in
children with Guillain-BarrC-Strohl syndrome. Develop. Med. Child Neurol. 16:
654 (1974).
8. Gifford, R. W., Westbrook, E.: Hypertensive encephalopathy: mechanisms, clinical
features and treatment. Progr. Cardiovasc.
dis. 42: 115 (1974).
9. Isler, W.: Acute hemiplegias and hemi-

syndromes in childhood. Clinics in developmental medicine Nos. 41 and 42. Spastics International Medical Publications
(1971).
10. Jellinek, E. H. et al.: Hypertensive encephalopathy with cortical disorders of vision. Quart. J. Med. 33: 239 (1964).
11. Muller, H. R. et al.: The contribution of
computerized axial tomography to the
diagnosis of cerebellar and pontine hematomas. Stroke 6: 467 (1975).
12. Seshia, S. S. et al.: Coma in childhood.
Develop. Med. Child Neurol. 19: 614
(1977).
13. Van Vught et al.: Hypertensive encephalopathy in childhood. Neuropadiatrie 7:
92 (1976).
14. Ziegler, D. K. et al.: Hypertensive encephalopathy. Arch. Neurol. 12: 472 (1965).

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Hypertensive encephalopathy in childhood