: l1 Short Communication
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P. Pavonel
P. G. smilari2
R. Trifiletti3
E. par an^',^-*

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Late Primary Unilateral Thalamic Hemorrhage
in Infancy: Report of Two Cases
Division of Pediatric Neurology, University of Catania. ltaly
Pediatric Clinic. University of Catania, Italy
Department of Neurology and Neuroscience. Cornell University, New York, USA
IBFSNC. CNR. Catania, Italy

Abstract

Introduction

hemorrhage can occur In seemingly well infants outside the
neonatal period.
.

Key words: Thalamus

- Hemorrhage - Infant - Seizures

erythrocyte sedimentation rate were normal, suggesting absence of a local or severe generalized infection. Echocardiography. electrocardiography and abdominal ultrasound were
all normal.

Thalamic hemorrhage is usually observed in premature infants
(3,9)or in term newborns (5) after a severe pathological neonatal event (8). Less commonly, thalamic hemorrhage has
been reported in full-term infants who were born by norrnal The child was initially critically ill. and developed signs of indelivery (7) and initially appeared well; these infants then de- creased intracranial pressure. Clinical status improved with
velop abnormal neurological signs during the first or second ventricular drainage. He was discharged home after three
week of life. This condition is usually unilateral (2) and is a l ~ weeks following ventriculoperitoneal shunt placement. Phemost always associated with intraventricular hemorrhage (6). nobarbital was started prophylacticaIly and no seizures were
initially observed.
We now report on two patients who manifested acute neurological symptoms at two months of age and who were found to The patient was then seen at the Division of Pediatric Neurolohave unilateral thalamic hemorrhage with intraventricular in- gy at the University of Catania, Italy, at three months of age.
volvement. Both of these children later developed seizures.
Physical examination showed a weight and height at the 50th
percentile, with head circumference 45.5 cm (75 percentile).
The head was asymmetric being flattened along the right occiput. Lateral gaze palsy was present bilaterally and head control
Case Reports
was poor. Deep tendon reflexes were symmetrically increased
in all extremities. Startle reflex in response to either auditory
Case I
or somatosensory stimuli was markedly increased. Episodes
The patient was born at term (38 weeks) to unrelated healthy
of opisthotonus were observed during periods of agitation.
parents. The mother had two previous miscarriages. Birth Routine electroencephalogram (EEG)was markedly asymmetweight was 3.45 kg. height 50 cm and head circumference ric with spike waves on left side, 'while the right side showed
3 5 cm, all parameters being near the 50th percentile for age. near-normal activity. Ophthalmological examination again
The child appeared well until the age of two months. While showed no evidence of retinal hemorrhage or other abnormalbeing breastfed, the child suddenly developed hypertonia fol- ities. Brainstem and visual evoked responses were normal.
lowed by right head deviation and right gaze preference, then
by stupor which progressed to coma within a few hours' time. At the age of 5 months, a repeat MRI showed signs of previous
Non-contrast head (Tscan on admission showed massive left hemorrhage in the left tbalamic region and marked ventriculomegaly (Fig. 1C). At the age of 6.5 months, the patient was notthalamic hemorrhage with intraventricular extension and
modest hydrocephalus (Fig.I A). MRI on admission indicated
ed to have episodes of flexion and rotation of the head and
a thalamic origin of the bleed (Fig.1 B). MRl-angiography failed flexion of arms many times a day followed by cry. EEG showed
to disclose an underlying vascular malformation (Fig.1 D) modified hypsarrythmia with right side being slightly more afOphthalmological examination showed no evidence of retinal fected than left, and a diagnosis of infantile spasms was made.
hemorrhage. Extensive evaluation of coagulation status, in- Gamma-vinyl gamma-aminobutyric acid (Vigabatrin) was
cluding prothrombin and partial thromboplastin times, fibri- added and resulted in complete cessation of spasms in a few
nogen. protein C, protein S and factor V Leiden, revealed no ab- days.
normalities. Blood cultures were sterile. white cell count and
When last examined. the patient was 12 months old. Weight
and height continued on the 50th percentile, and head circumReceived: January 11.1999: revised, accepted: May 12.1999
ference on the 75th percentile. The child showed progressive
acquisition of motor and language milestones, but was still deNeuropediatrics 30 (1999) 264- 267
layed in all fields of development-The clinical course showed
0 Hippohates Verlag Stungart

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We report on two infants with primary unilateral thalarnic
hemorrhage which occurred a t two months of age. Both infants were normal prior to the onset of hemorrhage. Both
children presented with seizures and subsequently developed
epilepsy. These cases suggest that primary unilateral thalamic

Thalomic Hemorrhage in Infancy

Neuropediatrics 30 (1999)
Fig. I A-E Case 1.
A Non-contrast

ference 34 cm. The first two months of life were uneventful.
At two months, the patient suddenly developed generalized
hypotonia. pallor and tonic clonic seizures upon awakening
from sleep. A 0 scan, performed on admission, showed left
thalamic hemorrhage (Fig.2 A). Extensive evaluation of coagulation status. including prothrombin and partial thromboplastin times, fibrinogen, protein C and protein S, revealed no abnormalities. Blood cultures were sterile, white cell count and
erythrocyte sedimentation rate were normal, suggesting absence of a local or severe generalized infection. Echocardiography and electrocardiography were normal.
progressive improvement. He was able to sit unsupported and
showed good head control. He could grasp the objects and
could perfom bimanual transfer. Mild paralytic strabismus
was present on right side. He had just begun to say.his first
words. Follow-up MRI showed marked resolution of ventricular dilatation and signs of old left thalamic hemorrhage
(Fig.1E).
Case 2

The child was the second born at term from unrelated parents.
Her birth weight was 3.65 kg, height 49 cm and head circum-

Initial EEG showed generalized high-voltage slow waves. After
a few days, a gradual improvement was noticed, and one
month after the patient was discharged on phenobarbital. Unlike. Case 1, progressive hydrocephalus did not develop. Head
MRI four months later (patient's age 6 months) displayed significant resolution of the thalamic hemorrhage (Fig. 2 B).
The patient remained seizure-freeon phenobarbital until age4
years when, during a febrile episode, she had a long generalized convulsion. When last examined at age 6 years, she was
attending regular school. She had no convulsions, no mental
retardation or language disturbances. Formal IQtesting was

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I3

head CTscan on
admission (patient's
age 2 months):
showing massive
hemorrhage on left
side.
B Brain MRI on
admission (patient's
age 2 months)
showing thalamic
origin of hemorrhage.
C Brain MRI three
months later
(patient's age
5 months): signs
of previous hemorrhaqe in left thala-

Neuropediotrics 30 (1999)

G.Incorpora eta/.
ported in the context of significant hypoxic-ischemic injury.
where it may be unilateral (2) or bilateral (8).

Fig.2A-C Case 2.
A Non-contrast

head CTscan on
admission (patient's
age 2 months)
showing ventricular
hemorrhage and
left thalamic hemorrhage.
B Brain MRI four
months later
(patient's age
6 months): signs
of previous hemorrhage with resolution of thalamic
hemorrhage.
C MRl angiography
performed at age
6 years: no signs of
underlying vascular
malformation.

There have been prior reports of infants as old as 3 months
with intraventricular hemorrhage developing spontaneously
or in the context of re-existinn brain iniury (5).although it is
unclear if there wasAathalamicsource olf hemorrhage in these
cases. We view these cases as a late-onset variant of the "new
clinical entity" of primary thalamic hemorrhage proposed by
Trounce et al. (7), and we suggest that primary thalamic hemorrhage may have a wider age spectrum than previously believed. As Trounce et al. also observed (7) ventriculornegaly is
common, but not invariable in this condition. As our Case 1 illustrates, short-term morbidity and mortality may be closely
related to increased intracrania1 pressure, and prompt ventricular drainage may result in a reasonably good neurodevelopmental outcome, as others have found (2.7).
The long-term outcome of children with primary thalamic
hemorrhage is unknown. Both of our patients eventually developed seizures. In our Case 1, infantile spasms were diagnosed at 6.5 months of age. Intraventricular hemorrhage is
one of the many forms of brain injury that have been previously reported to be associated with infantile spasms (1). Infantile
spasms were easily controlled with Viabatrin, but it is unclear
if epileptic encephalopathy plays some role in the moderate
developmental delay evident in this patient. In contrast, in
our Case 2, signs of increased intracranial pressure did not
dominate the early clinical picture and seizures, once controlled, did not recur until age four. This patient now has an EEG
with interictal spike and slow-wave abnormalities during slow
wave sleep (4):neither pattern has previously reported in patients with primary thalamic hemorrhage.
In summary, our cases illustrate that primary unilateral thalami~
hemorrhage can occur in apparently well infants outside
the neonatal period, and may be associated with seizures several months to years after the bleed.

in the normal range. The awake EEC showed occasional spike
wave of 2 - 2.5 Hz: EEC during sleep showed a pattern of continous spike waves during slow sleep. MRI-angiography excluded an underlying vascular malformation (Fig.2C).

Discussion
Within the pediatric population, thalamic hemorrhage has
been most frequently reported in the neonatal period, with
most reported cases occurring in infants under two weeks of
age (2.7,8).In several cases, thalamic hemorrhage has been re-

References
Adams C. Hochhauser L. Logan WJ. Primary thalamic and caudate
hemorrhage in term neonates presenting with seizures. Pediatr
Neurol1988; 4: 175 - 177
De Vries LS, Smet M, Goemans N, Wilms G,Devlieger H, Casaer P.
Unilateral thalamic haemorrhage in the pre-term and full-term
newborn. Neuropediatrics 1992; 23: 153-156
Donat JF, Okazaki H, Kleinberg F. Reagan TJ. lntraventricular
hemorrhages in full-term and premature infants. Mayo Clin
Proc1978; 53: 437 -441
Jayakac PB, Seshia SS. Electrical status epilepticus during slowwave sleep: a review. j Clin Neurophysiol1991; 8: 299-311

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There have also been reports of apparently healthy children
presenting with thalamic hemorrhage under the age of two
weeks (7). The two patients reported here were apparently
well until two months of age, and were not ill up until the day
of presentation. Extensive evaluation failed to reveal evidence
of coagulopathy and/or local or severe generalized infections.
MRI-angiography in both patients (Figs. 1D and 2C) failed to
provide evidence of an underlying vascular malformation.
Thus, we believe that these patients have had spontaneous
late-onset primary thalamic hemorrhage.

Tholamic Hemorrhage in lnfoncy

Neuropediotrics 30 (7999)

Mitchell W, OqTuama L Cerebral intraventricular hemorrhages
in infants: a widening age spectrum. Pediatrics 1980: 65: 35-39
Roland EH, Flodrnark 0,Hill A. Thalamic hemorrhage with intraventricular hemorrhage in the full-term newborn. Pediatrics
1990: 85: 737 - 742
Trounce JQDodd KL, Fawer CL, Fielder AR. Punt J, Levene MI. Primary thalarnic haemorrhage in the newborn: a new clinical entity. Lancet 1985; 1: 190-192
Voit T,Lemburg P. Neuen E,Lumenta C. Stork W. Damage of thalamus and basal ganglia in asphyxiated full-term neonates. Neuropediatrics 1987; 18: 176- 181
VolpeJJ. Neurology of the newborn. Philadelphia, W.B. Saunders.
1995

Dr. Enrico Pamno

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Divisione di Neurologia Pediatrica
Clinica Pediatrica, Universiti di Catania
Viale Andrea Doria 6
1-95125 Catania
Italy