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of Child Neurology

Topical Review: Acute Ataxia in Childhood

Monique M. Ryan and Elizabeth C. Engle
J Child Neurol 2003 18: 309
DOI: 10.1177/08830738030180050901

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Topical Review

Acute Ataxia in Childhood

Monique M. Ryan, MBBS, MMed; Elizabeth C. Engle, MD

ABSTRACT

Acute childhood ataxia is a common cause of presentation to the pediatric emergency room or child neurologist. The pri-
mary concern on initial assessment is to exclude serious causes of this clinical syndrome, including central nervous system
infections and mass lesions, while recognizing the essentially benign nature of acute ataxia in most children. Childhood
ataxia can be diagnostically approached by consideration of the temporal course and presence or absence of associated
neurologic abnormalities. In all forms of childhood ataxia, outcome is largely determined by etiology. In this review, the
various causes and syndromes of acute ataxia in childhood are described, with discussion of diagnostic considerations
and an approach to investigation, treatment, and prognosis. (J Child Neurol 2003;18:309–316).

The word “ataxia” (Greek: ataktos, lacking order) refers to
a pathologic abnormality of organization or modulation of
movement. Although ataxia is most commonly attributable
to cerebellar dysfunction, lesions at almost all levels of the
nervous system can result in motor incoordination.
Acute ataxia is a common cause of presentation to the
pediatric emergency room or child neurologist. Childhood
ataxia is best approached diagnostically by determination of
the temporal course and the presence or absence of asso-
ciated neurologic abnormalities. Ataxia may be congenital
or acquired. Congenital ataxia is usually associated with
central nervous system malformations. Acquired ataxia can
be classified as acute, episodic, or chronic. Episodic and
chronically progressive ataxias are uncommon in childhood
and are usually caused by inherited metabolic or genetic dis-
orders. In all forms of childhood ataxia, outcome is largely
determined by etiology. This review is confined to a discus-
sion of the causes, investigation, and outcome of acute child-
hood ataxia, which may be defined as unsteadiness of walking
or of fine motor movement, of less than 72 hours’ duration,
in a previously well child. The symptoms and signs of the var-
Received Dec 26, 2002. Received revised Jan 21, 2003. Accepted for publi-
cation Jan 21, 2003.
From the Department of Neurology (Drs Ryan and Engle), Children’s Hospital
Boston, Boston, MA.
Presented in part at the Michael J. Bresnan Child Neurology Course,
Children’s Hospital, Harvard Medical School, Boston, September 26, 2002.
Address correspondence to Dr Monique M. Ryan, Department of Neurology,
Fegan 11, Children’s Hospital Boston, 300 Longwood Avenue, Boston MA
02115. Tel: 617-355-2067; fax: 617-264-9691; e-mail: monique.ryan@tch.har-
vard.edu.
ious causative disorders are discussed, followed by an
overview of investigation, treatment, and prognosis in the
acute ataxic syndromes of childhood.
CLINICAL EVALUATION OF ACUTE ATAXIA IN
CHILDHOOD
Most children with acute ataxia are seen within several days
of symptom onset, usually because of refusal to walk or the
sudden development of a wide-based, “drunken” gait. Care-
givers less commonly remark on unsteadiness of arm move-
ments, truncal titubation, and dysarthria. At presentation, the
primary concern is to exclude serious causes of acute ataxia,
including central nervous system infections and mass lesions
(Table 1), while recognizing the essentially benign nature of
this clinical syndrome in most children. The history should
therefore include inquiry as to antecedent or current symp-
toms of systemic infection, including fever, rash, and gas-
trointestinal upset. Recent immunizations should be noted,
as should the child’s general state of health in the weeks and
months prior to presentation. Recurrent or persistent
headache and vomiting or diplopia suggests an intracranial
mass lesion and possible elevation of intracranial pressure.
A common cause of acute ataxia is inadvertent or deliber-
ate drug ingestion. The child’s activities should be reviewed
with respect to possible exposure to medications, alcohol,
and household chemicals. Recent head or neck trauma
should prompt consideration of vertebral artery dissection.
A history of previous similar episodes in the patient or other
family members should also be identified if present.
Specific details regarding physical findings in the var-
ious forms of acute ataxia are discussed in detail below. In

309
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310 Journal of Child Neurology / Volume 18, Number 5, May 2003

Table 1. Causes of Acute Ataxia in Childhood ETIOLOGY OF ACUTE ATAXIA IN CHILDHOOD

Infectious/immune-mediated cerebellar disorders
Acute cerebellar ataxia* Cerebellar Disorders
Acute demyelinating encephalomyelitis*
Systemic infections
Acute ataxia secondary to cerebellar dysfunction is char-
Brainstem encephalitis acterized by hypotonia, gait abnormalities (wide based,
Multiple sclerosis lurching, possibly staggering), poor maintenance of truncal
Toxic: alcohol and drug related* position (titubation), and speech abnormalities (fluctua-
Mass lesions tions in clarity, rhythm, tone, and volume). Poor coordina-
Tumors
Vascular lesions
tion of voluntary movements (dysmetria) results in
Abscesses nystagmus, under- or overshooting of limb movements (best
Hydrocephalus seen on finger-nose or toe-target testing), and difficulty
Trauma with rapid alternating movements (dysdiadochokinesia),
Cerebellar contusion or hemorrhage which may be associated with intention tremor (which is
Posterior fossa hematoma
Postconcussion syndrome
oscillatory, to and fro in a plane perpendicular to the object
Vertebrobasilar dissection being approached). The deep tendon reflexes can be pen-
Stroke dular, with slowed contraction and relaxation phases,
Vertebrobasilar dissection or thromboembolism although this is often difficult to appreciate.
Cerebellar hemorrhage
Some clue to localization of the lesion within the cere-
Paraneoplastic disorders
Opsoclonus-myoclonus syndrome
bellum can be apparent on examination: vermal (midline)
Sensory ataxia
lesions cause dysarthria, truncal titubation, and gait abnor-
Guillain-Barré syndrome malities, whereas lesions of the cerebellar hemispheres
Miller Fisher syndrome spare speech but result in ipsilateral limb hypotonia, dys-
Paretic ataxia metria, and tremor. When walking, patients tend to veer in
Upper motoneuron Lesions of frontal lobe,
corticospinal pathways
the direction of the affected cerebellar hemisphere. Lesions
Lower motoneuron Spinal cord: transverse of the deep cerebellar nuclei cause resting tremor, myoclonus
myelitis, vascular lesions, of the extremities and palate, and opsoclonus. Findings in
cord compression
Peripheral nerve:
cerebellar ataxia remain unchanged in severity whether
Guillain-Barré syndrome, the eyes are open or closed.
Miller Fisher syndrome,
tick paralysis
Infectious or Immune Mediated
Other neurologic disorders
Inborn errors of metabolism (see Table 3)*
Basilar migraine, benign paroxysmal vertigo Acute Cerebellar Ataxia
Nonconvulsive seizures The most common cause of childhood ataxia, accounting
Central pontine myelinolysis
Wernicke’s encephalopathy
for about 40% of all cases, is acute cerebellar ataxia.1 Acute
Functional ataxia
cerebellar ataxia usually results from postinfectious cere-
bellar demyelination, less commonly occurring as a result
*Most common.
of direct infection of the cerebellum. Postinfectious cere-
bellar demyelination is thought to be an autoimmune phe-
general, the physical examination can be difficult as sig- nomenon incited by infection or immunization, with
nificantly ataxic children are often hesitant and irritable, subsequent cross-reaction of antibodies against cerebellar
necessitating prolonged observation to differentiate poor epitopes, although specific autoantibodies have only rarely
coordination from weakness. The mental state is impor- been identified.2
tant. Children with postinfectious cerebellar ataxia are nor- Acute cerebellar ataxia is most common in younger
mally alert and interactive. Abruptly altered responsiveness children (2–4 years) but may be seen in older children
suggests an ingestion, acute disseminated encephalomyelitis, and adolescents.1,3 Boys are more commonly affected.3 A
or stroke. Extreme irritability and decreased speech output history of antecedent illness in the 5 to 21 days before pre-
can be seen in the opsoclonus-myoclonus syndrome. sentation is obtained in about 70% of patients. Numerous
Nystagmus is common to disorders affecting the cere- infectious agents have been implicated in the pathogene-
bellar hemispheres. Additional abnormalities of cranial sis of this condition (Table 2). As many as 26% of cases are
nerve examination, such as papilledema and cranial nerve preceded by varicella.3 Rarely, the development of ataxia
palsies, suggest an intracranial focal lesion or hydrocephalus. precedes the eruptive phase of varicella infection.4 The
Pupillary abnormalities can be seen with mass lesions, introduction of universal immunization against varicella is
raised intracranial pressure, stroke, or intoxication. Asym- likely to render varicella-related cerebellar ataxia uncom-
metries of limb tone, power, and the deep tendon reflexes mon. To date, only one case of acute cerebellar ataxia
are uncommon in acute cerebellar ataxia and should prompt temporally related to varicella vaccination has been
consideration of other conditions. Truncal ataxia is easier described.5 Although cerebellar ataxia has long been tem-
to appreciate when the child sits unsupported. porally linked to childhood immunizations (most com-

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Table 2. Reported Causes of Infectious/Postinfectious Cerebellitis in Childhood
Direct Infection
Echovirus type 9
Coxsackie B
Varicella zoster
Bacterial meningitis (pneumococcal, meningococcal)
monly the initial measles vaccination), a causal relation-
ship has not been proven.3,6
Postinfectious cerebellitis presents with the explosive
onset of gait abnormalities, ranging in severity from unsteadi-
ness and a wide-based stance to complete inability to walk.
Symptoms are maximal at onset and may be more severe in
cases following varicella infection.3 The extremities are less
affected than the trunk. Associated findings can include trun-
cal instability, head titubation, intention tremor, dysmetria,
and nystagmus. Abnormalities of ocular movement, includ-
ing ocular flutter (horizontal) and opsoclonus (multidirec-
tional), are occasionally seen. A small minority of patients has
associated cranial nerve palsies or long-tract signs.3 Mental
status is normal. “Pure” acute cerebellar ataxia is not asso-
ciated with fever, seizures, or other systemic upset.
Acute Postinfectious Demyelinating Encephalomyelitis
Ataxia is a common feature of acute postinfectious demyeli-
nating encephalopathy, a multifocal immune-mediated
encephalomyelopathy. Acute postinfectious demyelinating
encephalopathy also develops during the recovery phase
from viral illness or vaccination7,8 but is distinguished from
acute cerebellar ataxia by alteration of consciousness or the
fulminant onset of multifocal neurologic deficits. Seizures
are common, as are cranial neuropathies, hemiparesis, and
transverse myelitis.7–10 Systemic symptoms such as fever,
headache, and meningism can be seen.8,9 Repeated episodes
of demyelination raise concern as to multiple sclerosis,
which occasionally presents in childhood with ataxia aris-
ing from lesions within the cerebellum or its major con-
nections. Associated findings include optic neuritis and
long-tract signs.11
Brainstem Encephalitis
Brainstem encephalitis can involve the inflow and outflow
tracts of the cerebellum, resulting in ataxia in association
with cranial neuropathies, hemiparesis, and respiratory
irregularities. Causative agents include Epstein-Barr virus,12
Listeria monocytogenes, 13 and, in recent epidemics,
enterovirus type 71.14 Differentiation between brainstem
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Acute Ataxia in Childhood / Ryan and Engle 311
Infections Associated With
Post- or Parainfectious Cerebellitis Systemic Infections
Varicella zoster Typhoid fever
Epstein-Barr virus Scarlet fever
Mumps Mycoplasma pneumoniae
Legionella pneumophilia Diphtheria
Hepatitis A Leptospirosis
Influenza A and B
Herpes simplex virus I
Coxsackie A
Echovirus type 6
Enterovirus type 71
Malaria
Poliovirus type 1
Japanese B encephalitis
Parvovirus B19
Measles
Mycoplasma pneumoniae
encephalitis and Miller Fisher syndrome (see below) can be
difficult. Mental state changes, cerebrospinal fluid pleocy-
tosis, and abnormal electroencephalography (EEG) are
more common in brainstem encephalitis, whereas areflexia
is usually present in Miller Fisher syndrome.
Systemic infections not directly involving the cerebel-
lum or other parts of the central nervous system can also
cause ataxia, the pathophysiology of which is poorly under-
stood (see Table 2).
Toxic Cerebellar Syndromes
In as many as 32.5% of cases, acute childhood ataxia is attrib-
utable to drug ingestion.1 Cerebellar symptomatology com-
monly follows ingestion of anticonvulsants, benzodiazepines,
alcohol, and antihistamines. Less often, ataxia develops
after exposure to organic chemicals and heavy metals.
Accidental poisoning in children aged less than 6 years
is the most common form of toxic ingestion. There is a sec-
ond peak in adolescence, when intoxication occurs as a
result of substance abuse.1 A high index of suspicion should
always be maintained as a history of ingestion or exposure
might not be forthcoming. After ingestion, ataxia is often
accompanied by mental status changes such as lethargy, con-
fusion, inappropriate speech, or behavior. Nystagmus can
be present.
Mass Lesions
Forty-five to sixty percent of all childhood brain tumors arise
in the brain stem or cerebellum.15 Posterior fossa tumors
usually present with slowly progressive ataxia and symp-
toms of increased intracranial pressure. Acute decompen-
sation can be related to the development of hydrocephalus
or, less commonly, hemorrhage into the lesion. Other clin-
ical features can include headache, personality change,
and focal abnormalities on neurologic examination (eg,
papilledema, cranial nerve palsies, and hemiparesis). A
minority of supratentorial tumors produce ataxia, usually
those in the midline. Parenchymal lesions of the frontal
lobes can cause ataxia owing to involvement of fronto-
cerebellar associative fibers.16
312 Journal of Child Neurology / Volume 18, Number 5, May 2003
Hydrocephalus
Ataxia arising from acute hydrocephalus owing to mass
lesions or hemorrhage from vascular lesions (such as arte-
riovenous malformations) is usually accompanied by other
symptoms such as headache and vomiting. Clinical signs can
include papilledema and paresis of lateral gaze.
Trauma
Head injuries sometimes cause ataxia owing to cerebellar
contusion or hemorrhage or because of extraparenchymal
posterior fossa hematoma. Occasionally, ataxia accompa-
nies concussion. In younger children with postconcussive
syndromes, gait unsteadiness is more marked than nystag-
mus or appendicular dysmetria; older children also complain
of headache and dizziness.17 Magnetic resonance imaging
scans can be normal or demonstrate foci of high signal on
T2-weighted sequences, lesions that are felt to demonstrate
areas of diffuse axonal injury.18 After neck injuries, ataxia
can be a pointer to vertebral artery dissection.
Stroke
Posterior circulation strokes are rare in young children19
but should be considered after neck trauma (causing ver-
tebral artery dissection) or in those predisposed to
thromboembolic disease.20 In such cases, cerebellar deficits
are often asymmetric and evolve in association with deficits
attributable to brainstem ischemia. Cerebellar hemorrhage
is rare in childhood and, in the absence of a bleeding
diathesis, is most commonly associated with arteriovenous
malformations.21
Paraneoplastic Cerebellar Syndromes
Acute ataxia is occasionally associated with rapid chaotic
multidirectional conjugate eye movements (opsoclonus);
myoclonic jerks of the extremities, head, trunk, and face;
and encephalopathy in opsoclonus-myoclonus syndrome.
This develops as a postviral phenomenon in a minority of
cases.22–24 In at least 50% of children, however, opsoclonus-
myoclonus syndrome is the presenting manifestation of an
occult neuroblastoma or ganglioneuroblastoma, usually
sited in the mediastinum or abdomen, in which case ataxia
is thought to arise as a paraneoplastic autoimmune phe-
nomenon involving cross-reactivity of tumor and cerebel-
lar antigens.25,26
Opsoclonus-myoclonus syndrome is seen in young chil-
dren (6 months to 3 years), with a trend to earlier onset in
infants with occult neoplasms.25 There can be associated
extreme irritability and recurrent vomiting.22,25 Opsoclonus
can be temporally distinct from myoclonus and can be fleet-
ing. It is accentuated by agitation and visual saccades and
persists during sleep. Ataxia in this disorder can be of sub-
acute onset and can fluctuate. Isolated ataxia, without
abnormal eye movements or myoclonus, has also been
reported as the presenting sign of neuroblastoma.25, 27
Ataxia has also been described as a paraneoplastic
phenomenon in isolated pediatric cases of Hodgkin’s disease,
Langerhans’ cell histiocytosis, and hepatoblastoma.28–30
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Sensory Ataxia
Ataxia can result from loss of sensory input to the cerebellum
owing to lesions in the posterior column of the spinal cord,
spinal nerve roots, or peripheral nerves. Sensory ataxias are
characterized by a positive Romberg’s sign and decreased
deep tendon reflexes. Loss of posterior column sensory
functions (proprioception and vibration sense) causes
pseudoathetosis of the hands and a wide-based, “steppage”
gait. These findings worsen with the eyes closed.
Sensory ataxia is present in as many as 15% of pediatric
cases of Guillain-Barré syndrome, usually in association with
neuropathic weakness (which can initially be relatively sub-
tle) and pain.1,31 Pure sensory postinfectious neuropathies
have also been reported in childhood.32 The Miller Fisher
variant of Guillain-Barré syndrome commonly follows 5 to 10
days after an infectious illness (often Campylobacter gas-
troenteritis) and is defined by the triad of ataxia, areflexia, and
ophthalmoplegia.33 Vertical gaze is most commonly affected,
horizontal gaze usually being preserved, although nystagmus
of the abducting eye is often present. Ptosis can develop,
pupillary abnormalities and facial weakness are not uncom-
mon, and there can be mild proximal limb weakness.33 Ataxia
is often more marked in the extremities. This postinfectious
immune-mediated phenomenon is related to antibodies to
the GQ1b myelin ganglioside in more than 90% of cases.34
Sensory ataxia can also be seen with acute postinfec-
tious demyelinating encephalopathy or multiple sclerosis,
other forms of sensory ataxia usually presenting less acutely.
Motor (Paretic) Ataxia
Weakness from focal cerebral (commonly frontal or pari-
etal)16 or corticospinal tract lesions can present with a
“paretic” ataxia, possibly associated with sensory loss,
altered deep tendon reflexes, and impaired sphincter con-
trol. Ataxia is proportional to the degree of weakness. More
peripherally, weakness, presenting as ataxia, can develop in
Guillain-Barré syndrome and with tick paralysis.35
Other Neurologic Disorders
Ataxia is common in basilar migraine, in which it can be asso-
ciated with vertigo, hemiparesis and cranial nerve dys-
function, nausea, vomiting, and headache.36 The initial
episode should cause concern as to a focal (vascular or
other) lesion, although description of positive visual phe-
nomena—commonly blobs or flashes of light rather than
well-defined fortification spectra—suggest the migrainous
nature of the episode. Subsequent episodes are often stereo-
typed and respond to standard migraine therapies.36
Ataxia can be prominent during the ictal or postictal
phases of seizures. Nonconvulsive seizures can also mani-
fest with ataxia with or without alteration of consciousness
in the absence of clonic movements but possibly with minor
myoclonus and atonic episodes.37 Nonconvulsive seizures
are more common in children with cognitive impairment and
preexisting mixed seizure disorders.
Many inborn errors of metabolism can present with
ataxia (Table 3), which could develop acutely or intermittently
 Acute Ataxia in Childhood / Ryan and Engle 313

Table 3. Causes of Episodic or Intermittent Ataxia in Childhood aim of laboratory and radiologic investigations in children
Recurrence of acute cerebellar ataxia* with acute ataxia is identification of serious conditions
Migraine or migraine equivalents mimicking postviral cerebellitis. In practice, a thorough
Basilar migraine* history and physical examination are far more likely to
Benign paroxysmal vertigo*
Benign paroxysmal torticollis of infancy
identify the etiology of acute ataxia than is a battery of
Alternating hemiplegia of childhood “screening” investigations.
Metabolic disorders Of all investigations performed in the diagnostic work-
Mitochondrial disorders up of acute cerebellar ataxia, the urine and/or serum drug
Pyruvate decarboxylase deficiency
Pyruvate dehydrogenase deficiency
screen are most likely to prove diagnostic, even where a
Leigh disease source of ingestion is not immediately apparent.1 Drug lev-
Amino acidopathies els should be determined where a specific intoxicant is
Maple syrup urine disease, intermittent form
Hartnup disease
identified.

-Glutamylcysteine synthetase deficiency Neuroimaging is often obtained in the emergency room
Urea cycle disorders to exclude a mass lesion. In the absence of altered con-
Carbamoyl phosphate synthetase type 1 deficiency
Ornithine transcarbamylase deficiency
sciousness, focal neurologic signs, or marked asymmetry of
Citrullinemia ataxia, the yield of such scans is low. Computed tomogra-
Arginosuccinic aciduria phy (CT) and magnetic resonance imaging (MRI) of the
Organic acidopathies
Holocarboxylase deficiency
brain are normal in most children with postinfectious
Biotinidase deficiency ataxia.1,3 Lesions suggestive of focal cerebellar or cerebel-
Isovaleric acidemia lopontine demyelination are occasionally identified.5,38,39
Carnitine acetyltransferase deficiency
Very rarely, cerebellitis is acutely complicated by obstruc-
Recurrent genetic ataxias
Episodic ataxia type 1 (paroxysmal ataxia with
tive hydrocephalus, necessitating urgent posterior fossa
myokymia) decompression.40,41
Episodic ataxia type 2 (acetazolamide responsive) CT scans are often normal in acute postinfectious
Episodic ataxia types 3 and 4
Episodic ataxia with paroxysmal dystonia
demyelinating encephalopathy,9 although acutely useful to
CAPOS syndrome (cerebellar ataxia, areflexia, exclude mass effect, which can contraindicate lumbar punc-
pes cavus, optic atrophy, sensorineural ture. MRI in acute postinfectious demyelinating encephalo-
hearing loss)
pathy demonstrates multiple asymmetrically located foci of
Nonconvulsive seizures
demyelination within the cerebral white matter and deep
Drug ingestion*
gray nuclei.7–10 Acute lesions often enhance after adminis-
Paroxysmal tonic upgaze of childhood
tration of gadolinium contrast, reflecting local breakdown
Cogan’s syndrome
of the blood–brain barrier.8–10 Brainstem encephalitis is
*Most common.
associated with areas of increased signal intensity within the
brain stem and cerebral peduncles on T2-weighted MRIs.14
because of decompensation triggered by dietary intake or Neuroimaging is usually normal at presentation of opso-
intercurrent illness. The first presentation of such disorders clonus-myoclonus syndrome.25,27, 42 Variable degrees of cere-
in childhood can mimic acute cerebellar ataxia. Detailed dis- bellar atrophy can be seen on follow-up imaging, independent
cussion of these conditions is beyond the scope of this of corticosteroid therapy and of neurobehavioral outcome.43
review, but they should be suspected where there is coex- Posterior fossa tumors and strokes can be difficult to visu-
isting developmental delay, a family history of similarly alize on CT scans, where artefacts sometimes obscure sub-
affected relatives or of consanguinity, or where ataxia is tle hypodensities within the brain stem and cerebellum.
accompanied by significant lethargy, overt encephalopa- MRI (with diffusion-weighted imaging where acute ischemia
thy, excessive vomiting and diarrhea, or unusual body odor. is suspected) is the imaging modality of choice for such
lesions.
Functional Ataxia Cerebrospinal fluid examination is commonly normal
Hysterical gait disorders are not uncommon, especially in in postinfectious acute cerebellar ataxia and acute postin-
female adolescents. In such cases, although the patient fectious demyelinating encephalopathy, with mild pleocy-
lurches and appears to stagger, the gait is often not wide tosis and elevation of cerebrospinal fluid protein being
based, and falls are rare. Clinical signs are inconsistent present in 26 to 50% of cases.1,3,8,25 In postinfectious ataxia,
and often fail to conform to recognized neuroanatomic identification of mild cerebrospinal fluid lymphocytosis
distributions. provides evidence of an inflammatory process. Significant
pleocytosis and elevation of cerebrospinal fluid protein,
INVESTIGATION OF ACUTE ATAXIA IN with or without hypoglycorrachia, suggest meningitis or
CHILDHOOD encephalitis and should prompt further investigation. A
mild lymphocytic pleocytosis is identified in fewer than
Acute postinfectious cerebellar ataxia, an essentially benign 20% of cases of opsoclonus-myoclonus syndrome and does
clinical syndrome, is a diagnosis of exclusion. The primary not predict etiology.24,27,42,43 Cerebrospinal fluid examina-

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314 Journal of Child Neurology / Volume 18, Number 5, May 2003
tion can demonstrate cytoalbuminologic dissociation in
Guillain-Barré and Miller Fisher syndromes, but the cere-
brospinal fluid protein is normal in as many as 20% of chil-
dren within a week of onset of Guillain-Barré syndrome.44
Assays for the anti-GQ1b antibody should be obtained if
Miller Fisher syndrome is suspected.34 Oligoclonal bands and
elevation of the serum:cerebrospinal fluid immunoglobulin
index and myelin basic protein level can be present in acute
cerebellar ataxia, acute postinfectious demyelinating
encephalopathy, and multiple sclerosis7 and do not differ-
entiate between these disorders. These investigations are
therefore not generally indicated at the initial presentation
of acute ataxia.
EEG is indicated in those with altered consciousness and
fluctuating clinical signs. Epileptiform discharges or slow-
ing is seen on EEG in as many as two thirds of children with
acute cerebellar ataxia45 and is even more frequently abnor-
mal in acute postinfectious demyelinating encephalopa-
thy.8,10 EEG is normal in opsoclonus-myoclonus syndrome.42
With nonconvulsive seizures, EEG commonly reveals gen-
eralized spike-wave complexes (spike-wave stupor).37
Electromyography is indicated where sensory ataxia is
suspected. In Guillain-Barré syndrome, diagnostic abnor-
malities can be identified on neurophysiologic examina-
tion in over 90% of children within a week of symptom
onset.31 In Miller Fisher syndrome, electromyography is
less frequently diagnostic but might demonstrate abnor-
mal sensory potentials and delayed late responses.46
A number of further investigations should be under-
taken on suspicion of neuroblastoma. Urinary excretion of
catecholamine metabolites is increased in only 47 to 60% of
patients with paraneoplastic opsoclonus-myoclonus syn-
drome,47,48 in comparison with children with uncomplicated
neuroblastoma (> 95%).48 Identification of small tumors
could require CT or MRI of the chest and abdomen or scintig-
raphy with I123 metaiodobenzylguanidine scanning. Whole-
body metaiodobenzylguanidine scintigraphy has been shown
to have a sensitivity of 95% and a specificity of 100% for the
identification of neuroblastomas overall, but its ability to iden-
tify tumors associated with the opsoclonus-myoclonus syn-
drome has not specifically been studied.49 Newer imaging
techniques could increase the proportion of cases of opso-
clonus-myoclonus syndrome, which can be demonstrated to
be paraneoplastic in origin, but a proportion of neuroblas-
tomas undergo spontaneous involution and might not be
identifiable even after extensive investigation.50,51
Investigations indicated for work-up of a possible inborn
error of metabolism include a complete blood count and liver
function tests; determination of blood ammonia, lactate,
pyruvate, and ketone levels; and assessment of acid-base sta-
tus by blood gas determination. Additional screening tests
possibly indicated include plasma and urinary amino acid
assays, urine organic acids, serum biotinidase level, and cere-
brospinal fluid lactate and pyruvate assays. With the first pre-
sentation of acute ataxia and in the absence of developmental
delay, similar previous episodes, or a positive family history,
such investigations are unlikely to be contributory.1
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TREATMENT AND PROGNOSIS OF ACUTE ATAXIA
IN CHILDHOOD
Most children with acute cerebellar ataxia recover com-
pletely. Improvement usually begins within a week of symp-
tom onset. Recovery is full within 3 months of onset in at
least 50% of children45 but remains incomplete in a minor-
ity of cases.
In the first major review of outcome in acute cerebel-
lar ataxia, 6 of 18 children were felt to have permanent
neurologic sequelae in the form of persistent gait distur-
bance, ataxia, and delayed speech development.45 More
recently, Connolly and associates reported a series of 73 chil-
dren treated in St. Louis between 1967 and 1989. Fifty-five
of 60 children (91%) for whom 4-month outcome data were
available recovered completely, including all 16 children
with postvaricella cerebellitis. Recurrences occurred in
four patients, usually after a second viral infection. The
only clinical feature distinguishing children with recur-
rences from those with a monophasic illness was a longer
latency between the prodrome and development of the ini-
tial attack of ataxia. Behavioral or learning difficulties were
present in 20% of children during the recovery phase from
acute cerebellar ataxia but resolved in most within 6 months.3
There is no evidence that immunosuppressive therapies
improve outcome in acute cerebellar ataxia.
Foci of demyelination seen on acute MRI in childhood
acute cerebellar ataxia can resolve completely,52 can persist,53
or can evolve into cerebellar atrophy.54 Most patients make
full recoveries even in the face of persisting neuroradiologic
abnormalities.53
Recovery from acute postinfectious demyelinating
encephalopathy is typically somewhat slower than that from
acute cerebellar ataxia and can be hastened by treatment with
corticosteroids.8 Although most patients recover completely,
a minority have significant sequelae.7–10 Single relapses can
occur in as many as 10% of affected children,8 multiple
relapses raise the possibility of multiple sclerosis.
Brainstem encephalitis should be treated with broad-
spectrum antibiotics (including coverage for Listeria mono-
cytogenes) and acyclovir, pending identification of a
causative organism, but is commonly complicated by sig-
nificant neurologic sequelae in surviving patients.12–14
Treatment of ingestions depends on the nature and
amount of the ingested substance. Often no specific treat-
ment is available. In some cases, administration of an anti-
dote, chelation, dialysis, or other therapies are required.
Outcome in most other conditions associated with
acute ataxia in childhood is dependent on the underlying dis-
ease process. Tumors, stroke, and traumatic brain injury are
commonly complicated by significant sequelae.
Neuroblastoma, if found, should be removed surgically.
The underlying tumor is small and well differentiated in most
cases of paraneoplastic opsoclonus-myoclonus syndrome.25,27
Systemic chemotherapy is not usually required. The neurologic
syndrome of opsoclonus-myoclonus syndrome can remit par-
tially or completely over time but does not generally respond

to removal of the causative tumor. A majority of children with
opsoclonus-myoclonus syndrome have long-term neurologic
deficits related to persistence of their movement disorder or
to cognitive and behavioral problems.27,43,55 Some response
might be seen to treatment with high-dose corticosteroids or
intravenous immunoglobulin.27,43,55 There is inconclusive evi-
dence that neurologic outcome can be improved in children
receiving chemotherapy for their neuroblastoma.43
Children with Guillain-Barré and Miller Fisher syn-
dromes should be admitted to hospital, with careful moni-
toring of their respiratory and autonomic function, until
their clinical nadir is reached.56 Pain is common in childhood
Guillain-Barré syndrome and should be treated aggres-
sively.31,44 Specific treatments for Guillain-Barré and Miller
Fisher syndromes include intravenous immunoglobulin and
plasmapheresis, which are presumed to act by reducing
levels of circulating antibodies or by down-regulating
cytokine and complement-mediated activity. Neither form
of therapy has been subjected to randomized controlled
pediatric trials, but the consensus of opinion, based on a
number of uncontrolled series, is that both treatments has-
ten recovery in both conditions.56–58 Active treatment is usu-
ally withheld unless children lose ambulation or develop
significant respiratory or bulbar dysfunction.58 With or with-
out specific treatment, more than 90% of children with Guil-
lain-Barré and Miller Fisher syndromes recover completely
within 6 to 12 months of disease onset.44
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