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Journal ArticleDOI

Frontal ataxia in childhood.

01 Dec 2004-Neuropediatrics (Neuropediatrics)-Vol. 35, Iss: 6, pp 368-370

TL;DR: Signs compatible with the term frontal disequilibrium, a clinical pattern of frontal gait disorder, are shown and frontal ataxia may mimic developmental delay and be the leading mild symptom in extensive frontal lobe damage.
Abstract: Frontal ataxia may be the result of a unilateral frontal lesion In this report three cases are presented with ataxia due to right frontal lesions One case concerns a boy presenting with an unsteady gait and titubation of the trunk, mimicking developmental disequilibrium and with complex partial seizures It proved to be caused by a small right-sided cavernoma in the middle frontal gyrus After surgical intervention the symptoms and the seizures disappeared Two subsequent cases concern teenage patients presenting with headache after an ENT infection and on physical examination mild dysmetric function of the upper limbs and slight disequilibrium, due to right-sided frontal lobe abscesses After neurosurgical and antibiotic therapy the symptoms were relieved The frontal origin of ataxia should be considered in children presenting with a "cerebellar syndrome" Frontal gait disorders consist of a clinical pattern of different gait disorders The syndrome has been mentioned in the literature under different names Our patients show signs compatible with the term frontal disequilibrium, a clinical pattern of frontal gait disorder This assumes walking problems characterized by loss of control of motor planning, leading to imbalance Remarkably, frontal ataxia may mimic developmental delay as demonstrated in the first case and may be the leading mild symptom in extensive frontal lobe damage as demonstrated by the two other cases We suppose that frontal ataxia is the result of a disturbance in the cerebellar-frontal circuitries and an impairment of executive and planning functions of the basal ganglia-frontal lobe circuitry
Topics: Middle frontal gyrus (69%), Ataxia (61%), Frontal lobe (61%), Neurological disorder (50%)

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Abstract
Frontal ataxia may be the result of a unilateral frontal lesion. In
this report three cases are presented with ataxia due to right
frontal lesions. One case concerns a boy presenting with an un-
steady gait and titubation of the trunk, mimicking developmen-
tal disequilibrium and with complex partial seizures. It proved to
be caused by a small right-sided cavernoma in the middle frontal
gyrus. After surgical intervention the symptoms and the seizures
disappeared. Two subsequent cases concern teenage patients
presenting with headache after an ENT infection and on physical
examination mild dysmetric function of the upper limbs and
slight disequilibrium, due to right-sided frontal lobe abscesses.
After neurosurgical and antibiotic therapy the symptoms were
relieved. The frontal origin of ataxia should be considered in chil-
dren presenting with a ªcerebellar syndromeº. Frontal gait disor-
ders consist of a clinical pattern of different gait disorders. The
syndrome has been mentioned in the literature under different
names. Our patients show signs compatible with the term frontal
disequilibrium, a clinical pattern of frontal gait disorder. This as-
sumes walking problems characterized by loss of control of mo-
tor planning, leading to imbalance. Remarkably, frontal ataxia
may mimic developmental delay as demonstrated in the first
case and may be the leading mild symptom in extensive frontal
lobe damage as demonstrated by the two other cases. We sup-
pose that frontal ataxia is the result of a disturbance in the cere-
bellar-frontal circuitries and an impairment of executive and
planning functions of the basal ganglia-frontal lobe circuitry.
Key words
Childhood ´ frontal lobe ´ ataxia
Introduction
Gait ataxia or frontal lobe ataxia is a well known clinical entity in
neurological practice. The stance in gait ataxia is wide based, the
trunk upright, gait is unsteady, and falls are common. There are
no sensory or other cerebellar symptoms. Often medial frontal
lobe dysfunction, especially medial bifrontal damage, is respon-
sible. Occasionally a unilateral frontal pathology may cause gait
disturbances in childhood.
In this report this is illustrated by the description of a 2-year-old
boy presenting with unsteady gait and titubation of the trunk
and head due to a small unilateral frontal cavernoma and the his-
tory of two teenage patients with a mild ataxic dysfunction
caused by a unilateral frontal lobe abscess.
Case Reports
Patient 1
This two-year-old boy started walking independently at 18
months. Birth and gestation were unremarkable. He was the
firstborn. The parents were non-consanguineous. His mother no-
ticed from the beginning an unsteadiness of gait and a titubation
of the head without a tendency of improvement. He often fell
down. His speech development was mildly delayed. Otherwise
the medical history was unrevealing. On paediatric physical ex-
amination no abnormalities were noted. On neurological exami-
nation an ataxic gait disturbance and brief periodic staring spells
with eyelid contraction without any oculomotor deficits were
noticed. Deep tendon reflexes were normal. Laboratory investi-
Short Communication
368
Affiliation
1
Department of Paediatric Neurology, University Medical Centre St. Radboud, Nijmegen, The Netherlands
2
Department of Neurosurgery, University Medical Centre St. Radboud, Nijmegen, The Netherlands
Correspondence
C. E. Erasmus MD ´ Department of Child Neurology ´ University Medical Centre St. Radboud ´ PO Box 9101 ´
6500 HB Nijmegen ´ The Netherlands ´ E-mail: c.erasmus@cukz.umcn.nl
Received: May 2, 2004 ´ Accepted after Revision: September 12, 2004
Bibliography
Neuropediatrics 2004; 35: 368 ± 370 Georg Thieme Verlag KG Stuttgart ´ New York ´
DOI 10.1055/s-2004-830370 ´ Published online November 15, 2004 ´
ISSN 0174-304X
C. E. Erasmus
1
T. Beems
2
J. J. Rotteveel
1
Frontal Ataxia in Childhood
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gations revealed no abnormalities on the blood and CSF exami-
nations. Metabolic screening was normal. A cerebral MRI exami-
nation revealed a small cavernoma in the medial frontal gyrus on
the right side (Fig.1a). An EEG showed right-sided frontal hypo-
functional activity with frequent prefrontal low-voltage spikes.
The patient underwent a minimally invasive, navigated frontal
craniotomy with extirpation of the right frontal cavernoma.
There were no complications. Immediately afterwards he was
able to walk independently without falling. The seizures disap-
peared.
Patient 2
This 13-year-old girl started complaining of headaches six weeks
before admission. An ENT surgeon diagnosed a nose septum ab-
scess requiring drainage and a 2-week-treatment with antibiot-
ics. Two days before admission the headaches worsened. She had
always been a healthy girl. There was no history of trauma, drug
abuse or seizures. The family history was unremarkable. She at-
tended high school. She used an analgesic (paracetamol) at that
time. Medical and physical examinations were normal. She was
not febrile. On neurological examination she showed no signs of
raised intracranial pressure. No abnormalities except for slight
disequilibrium and a discrete dysmetric function of the left
upper limb with mild titubation of the trunk were seen. A cere-
bral CT with contrast enhancement revealed a right-sided
3 5 cm large abscess in the upper frontal gyrus, surrounded by
edema (Fig. 1b). There was a shift of the midline. There were no
signs of sinusitis. Blood examination showed no signs of an ac-
tive infection. The neurosurgeon removed 25 mL of pus with
burr-hole drainage. Streptococcus milleri was cultured. Appropri-
ate high doses of antibiotics were administered intravenously for
6 weeks. The dysmetric dysfunction disappeared soon after sur-
gery. The balance normalized.
Patient 3
This healthy, 10-year-old boy experienced a subacute throbbing
headache accompanied by a brief period of difficulty with speak-
ing and an unsteady gait. One month prior to admission he expe-
rienced an inflammation of the middle ear. He remained tired,
had periodic headaches and episodes of nausea following the
ear inflammation. He frequently required analgesics (paraceta-
mol). On admission he was ill, but had no fever. No signs of shock
were present. On neurological examination he was restless and
impaired in verbal communication. No cranial nerves, motor or
sensory dysfunctions were noticed. A slight disequilibrium ex-
isted and reaching reactions showed mild dysmetric features.
Stretch reflexes were normal. However, at the left side he showed
a Babinski reflex. He refused to walk.
The cerebral CT after contrast administration showed a large,
right-sided frontal abscess surrounded by edema and accompa-
nied with midline shift (Fig.1c). The inflammatory parameters in
blood were normal. Fifty mL of pus were drained by burr-hole
drainage and Streptococcus pneumoniae was cultured. Postopera-
tively he went on a 6-week-regime of high dose intravenous anti-
biotic treatment. Recovery was uncomplicated and the symp-
toms of disequilibrium and dysmetria disappeared over several
days.
Discussion
Cerebellar and sensory ataxia are well delineated clinical phe-
nomena. Gait ataxia or frontal lobe ataxia is less well known.
The neuronal circuitry for motor planning and balance regulation
contains the projections between the frontal premotor regions
and the pontocerebellar areas and between the basal ganglia
and supplementary motor area (SMA) [4, 5,10].
Not only primary frontal damage, but also a lesion in the cortico-
cerebellar pathways, corticocortical fibers and basal ganglia/
frontal lobe circuitry may impair motor activation.
The precise localization within the frontal lobe of the dysfunc-
tional generator resulting in the described symptoms is un-
known [2]. Bilateral lesions in the medial part of the frontal lobes
are most often reported to be responsible for gait ataxia, in
Fig.1a to caCerebral MRI, T
2
-weighted image of Patient 1. Before
craniotomy. A small lesion compatible with a cavernoma is seen in
the right middle frontal gyrus. b Preoperative cerebral CT of Patient 2
after contrast administration showing an contrast-enhancing lesion in
the right upper frontal gyrus, very suspect for an intracerebral abscess.
c Preoperative cerebral CT of Patient 3 showing an intracerebral ab-
scess in the right upper frontal gyrus.
Erasmus CE. Frontal Ataxia in ¼ Neuropediatrics 2004; 35: 368 ± 370
Short Communication
369
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particular, substantial lesions in the SMA [2, 3]. On the other
hand, Sunohara et al described an adult patient with the phe-
nomenon of gait ataxia with only unilateral vascular damage in
the inferior and middle frontal gyri [9]. Bennett et al described
three pediatric patients presenting with episodic or progressive
gait ataxia due to ªsilentº irritative activity. The EEG showed
slow spike and wave discharges in the bifronto-temporal regions
in the presence of normal occipital activity. The ataxia disap-
peared after administration of antiepileptic drugs [1].
Takakusaki et al described a two-year-old boy with a unilateral
demyelinating lesion in the white matter adjacent to the right
frontal horn resulting in subacute episodes of ataxic gait [11]. Si-
bon et al reported in a review that in adults focal lesions in the
basal ganglia and frontal subcortical white matter can produce a
frontal gait disorder mimicking parkinsonism [7, 8].
Gait ataxia has been described in association with different etio-
logical entities, especially with structural lesions such as cere-
bral tumors (gliomas), abscesses, traumas and strokes [2]. The
syndrome of gait ataxia has been named differently by different
authors [2]. Our first patient presented with a developmental
gait disorder and titubation with abrupt falls. Patient 3 had ob-
vious trunk imbalance, moving from one side to the other, on
the other hand patient 2 had no walking problems, but a minimal
dysmetric function of the left arm and mild titubation of the
trunk. All three patients had a unilateral frontal lesion and
showed clinical signs compatible with the term frontal disequi-
librium. There is impairment in equilibrium and postural re-
flexes and the presence of mild signs of upper limb ataxia. Fron-
tal gait disorders consist of a clinical pattern of various gait dis-
orders. Frontal disequilibrium is one clinical pattern. Others
mimic parkinsonism, a particular form of gait disorder is the syn-
drome of gait ignition failure (previous called ªlower half parkin-
sonismº, Marche à petits pas) [6 ±8].
The frontal abscesses caused visible extensive frontal lobe dam-
age and frontal ataxia seems likely. A unilateral, small frontal le-
sion in a young child can cause a frontal lobe dysfunction, espe-
cially when the lesion gives rise to epileptic activity.
Focal cortical lesions can cause irritative dysfunction which may
spread to the contralateral frontal region and can therefore cause
frontal disequilibrium and bilateral ataxic signs, especially in
childhood. The substantial frontal dysfunction can be demon-
strated by EEG registrations.
In conclusion, frontal disequilibrium has its own specificity. In a
toddler it may mimic a developmental motor disorder. It should
be included in the differential diagnosis of ataxia in childhood. In
the assessment of ataxia frontal lobe lesions must be excluded.
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Della Sala S, Francescani A, Spinnler H. Gait apraxia after bilateral
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Erasmus CE. Frontal Ataxia in ¼ Neuropediatrics 2004; 35: 368 ± 370
Short Communication
370
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Abstract: The present retrospective study aimed to investigate the presence of truncal instability or titubation after the first seizure and second phase in patients with acute encephalopathy with reduced subcortical diffusion (AED). Of the 15 patients with AED who were admitted to our hospital for 3 years and 2 months and had reached developmental milestones for sitting before disease onset, six experienced moderate-to-severe truncal instability while sitting after the first seizure. These patients had a significantly longer first seizure duration and significantly lower GCS scores 12-24 h after the first seizure, as well as significantly higher Tada score and Creatinine and blood glucose levels than those with mild or no truncal instability while in a seated position after the first seizure. Three 1-year-old children with bilateral frontal lobe lesions, particularly in the bilateral prefrontal lobe regions, demonstrated truncal titubation, which has not previously been reported as a clinical feature of AED. Tada score reported to be a predictor of AED prognosis and truncal instability in the sitting position after the first seizure may represent disease severity, but not the specific lesions. Conversely, truncal titubation might be suggestive of bilateral frontal lobe lesions, particularly in patients without severe instability. Further studies on the role of bilateral prefrontal lobe lesions to truncal titubation in patients with AED using more objective evaluation methods, such as stabilometry, are necessary.