t t p

t ss:
p t t p
t ss:
p
hht hht
rss rs s
b
eebooook92 
kee r Acute Cerebellar Ataxia
e bbooookkee r
e b o
b o
m ee/ e m ee/ / e
Harry T. Whelan, Gregory S. Aaen, Kumar Sannagowdara, and Megan B. DeMara-Hoth
/
: / /
/ t
/ .
t . m : / /
/ t
/ .
t . m
An expanded version of this chapter is available on www.expertconsult.com. See inside cover for registration details.

t t p
t ss
p : t t p
t ss
p :
hht
The term “cerebellum” is from Latin, meaning “the little
brain.” It is a part of the hindbrain situated in the posterior
cranial fossa. The cerebellum is organized into different
hht Children with postinfectious cerebellar ataxia are normally
alert and interactive. Abruptly altered responsiveness suggests
toxic ingestion or a severe central nervous system (CNS) dis-
regions with specialized functions. The cerebellum integrates turbance, such as stroke or an acute disseminated encephalo-
massive sensory information from many regions of the brain myelitis (ADEM). Therefore it is essential to differentiate poor

k eers
rs k e
to smoothly coordinate ongoing movements and to partici-
r
ers
and spinal cord. This information is used by the cerebellum
s coordination from weakness. Physical examination should
focus on identifying life-threatening conditions that may

b ooook b ooook
pate in motor planning. Detailed anatomy and physiology of
the cerebellum and its pathways are discussed in Chapter 91.
require immediate intervention. Symptoms such as confusion,
o o
hallucinations, or somnolence may indicate toxin ingestion,
b
eeb ee/ e
/ e b
Acute cerebellar ataxia refers to a group of acquired disor-
ders that result in acute dysfunction of the cerebellum. These
ee/ e
/ e b
demyelinating diseases, stroke, or meningoencephalitis
(Sivaswamy, 2014). Signs of elevated intracranial pressure

: // /.tm m
conditions are usually associated with either intoxication or
t .
inflammation of the cerebellum from infectious, postinfec-
: / /t/.tm m
such as papilledema should be excluded. Motor examination
.
should focus on eliciting weakness as younger children with

t p ss
p : /
tious, or paraneoplastic processes. The cerebellum regulates
tp pss : / hemiparesis or paraparesis may present with ataxia (i.e.,

t
hht t
the coordination of movement, muscle tone, and motor
control. The word “ataxia” is derived from the Greek word
ataktos, which means “lack of order.” Acute ataxia is defined
t
hht t “paretic ataxia”). A detailed cerebellar examination should
evaluate different components of cerebellar dysfunction.
Ataxia caused by pathology of the cerebellum is present even
as an unsteadiness of walking, coordination, or fine motor with eyes open and is not or only minimally exacerbated by
movement with duration greater than 72 hours. Acute cerebel- eye closure; therefore, a Romberg test will be negative. Sensory
lar ataxia is the most common cause of childhood ataxia, examination and an examination of deep tendon reflexes

k ee r ss
than 6 years of age.
keerrss
accounting for about 30% to 50% of all cases, in children less
r
should be performed to evaluate peripheral nerves or roots,
dysfunction of which may result in a “sensory” ataxia.

b ooookCLINICAL EVALUATION OF ACUTE ATAXIA booook b

Acute cerebellitis presents with a wide range of symptoms
oo
varying from subtle gait abnormality to more fulminant forms
eeb ee/e/e b
Acute cerebellar ataxia typically presents with rapidly progres-
ee/e e b
involving truncal instability, head titubation, intention tremor,
/
dysmetria, and nystagmus. Physical examination may possibly

: / / t
/ m
.t.m
sive symptoms evolving over a few hours but at most over 1

: / / t
/ m
.t.m reveal fever or meningismus, which can be indicative of a CNS

p ss : /
to 2 days. In cases associated with a prodromal illness, the

p ss
onset of ataxia is usually within 1 to 3 weeks after the onset
t p t p : / infection. Pharyngitis, lymphadenopathy, and splenomegaly
occur in Epstein–Barr virus infection. Otoscopic and skin
t
hhtt t
hhtt
of the prodromal illness. Although a gait disturbance is the
primary or only symptom in most patients, in other patients,
a wide range of clinical signs of cerebellar dysfunction could
(viral exanthema) examination may be useful. In addition,
ataxia is a common feature of acute postinfectious demyelinat-
ing encephalopathy. Brainstem encephalitis can involve the
be observed (Box 92-1). inflow and outflow tracts of the cerebellum, resulting in ataxia
Evaluating a pediatric patient presenting with acute ataxia (Ryan and Engle, 2003).
begins with obtaining a detailed history of the presenting

k e rrss
illness, including the onset, timing, and progression of symp-

e
toms. This process could be a key component for excluding
k ee rrss CAUSES OF ACUTE CEREBELLAR ATAXIA
o o
o o k o oo
serious etiologies (Ryan and Engle, 2003). The most com-
o k o o
eebb b b
The most common causes of acute cerebellar ataxia are inflam-

e / / e b
monly reported causes of childhood ataxia include postinfec-
e
tious, toxin ingestion, and Guillain–Barré syndrome (GBS). A
e / e e b
matory cerebellitis and toxin ingestion. Together they consti-
ee /
tute nearly 80% of the cases of childhood ataxia (Ryan and

: / t m
history of fever, antecedent respiratory and gastrointestinal
.t.m
infections, and varicella and Epstein–Barr virus infections
/ / : / / t
/ m
.t.m
Engle, 2003). Other common causes of childhood ataxia are

t p ss:
p /
are commonly reported in postinfection-mediated cerebellar

Inflammatory Cerebellitis ttp

ataxia. A history of recent vaccinations or an exposure to ss:
p /
listed in Box 92-2 and discussed in this section.

t
hht t Infectious/Postinfectious h
toxins, alcohol, drugs, or household medications and chemi-
cals commonly provides information suggesting a specific ht t
diagnosis. A family or personal history of recurrent ataxic
episodes, seizures, or migraine headaches may propose a met- Acute cerebellar ataxia most commonly results either from
abolic or genetic disorder. Recent head or neck trauma can postinfectious autoimmune-mediated cerebellar inflamma-

k e r s
present with ataxia and may be because of vertebrobasilar

rs
dissection or intracranial bleeding.
e k eers
rs
tion or as a result of direct infection of the cerebellum.
This accounts for about 30% to 40% of all cases. A history

o o
o o k o o
o k
Signs of cerebellar dysfunction (see Video 92-1) could
o
be categorized into the following groups: (1) speech deficits,
of antecedent illness 1 to 3 weeks before presentation is

oo
obtained in about 70% of patients (Ryan and Engle, 2003).

eebb e e
/ b
e b
(2) oculomotor disturbances, (3) limb ataxia, (4) deficits of
/
posture and gait, and (5) cognitive disturbances (Pandolfo
e e /e/ebb
Numerous infectious agents have been implicated (Table
92-1), with varicella being the most common organism associ-
e
and Manto, 2013).

: / / t
/ .
tm.m
Physical examination may be challenging in significantly
: / / /.tm m
ated with cerebellar inflammation (Bozzola et al., 2014). Cer-
t .
ebellar dysfunction can occur before the eruption of the

t p ss
p : /
ataxic children, especially in cases of altered responsiveness.

t p ss
p : /
exanthem. When direct infection of cerebellar parenchyma

t
hht t t
hht t 701
 t t p
t ss:
p t t p
t ss:
p
702
hht
PART X  Disorders of Balance and Movement
hht

k e r
e s
BOX 92-2  Causes of Acute Cerebellar Ataxia
rs k eers
r s
TABLE 92-1  Reported Causes of Infectious/Postinfectious
Cerebellitis in Childhood

o o
o o k
INFLAMMATORY

oooo k o
Infections

o
eebb b b
• Infectious cerebellitis Associated with
• Postinfectious cerebellitis
• Brainstem encephalitis
ee/ e
/e b ee/ e
/ e b Post- or
Parainfectious Systemic

: / / t .
• Acute disseminated encephalomyelitis

/ t m
. m : / / / .
t m
Direct Infection

t . m Cerebellitis Infections

• Multiple sclerosis
• Vasculitis
t p ss
p : / t p ss
p : /
• Bacterial meningitis
(pneumococcal,
• Coxsackie A
• Echovirus type 6
• Diphtheria
• Leptospirosis

t
hht t
• Paraneoplastic disorders
• Miller Fisher variant of Guillain–Barré syndrome t
hht t meningococcal)
• Coxsackie B*
• Echovirus type 9
• Enterovirus type 71
• Epstein–Barr virus*
• Hepatitis A
• Mycoplasma
pneumoniae
• Scarlet fever
INTOXICATION • Varicellar zoster • Herpes simplex • Typhoid fever
• Alcohol virus I
• Influenza A and B
• Drugs

k e rs
rs
BRAIN SPACE OCCUPYING/MASS LESIONS

e k er
erss
• Japanese B
encephalitis
• Legionella

ook ook
• Tumors

b
eeboo
• Abscesses
VASCULAR
/ e b o
bo / e b o
b o
pneumophila
• Malaria

• Vertebrobasilar dissection
m ee / e m ee / e • Measles
• Mumps
• Thromboembolism

: ///t/.t. m : / /
/t/.t . m • Mycoplasma
pneumoniae*
CNS TRAUMA
• Contusion
t t p
t ss
p : t tptpss : • Parvovirus B19
• Poliovirus type 1
• Hemorrhage
hht
• Postconcussion syndrome
MISCELLANEOUS
hht *Most commonly reported.
• Varicellar zoster*

• Labyrinthitis
• Basilar migraine

keerrss
• Benign paroxysmal vertigo
• Epilepsy (nonconvulsive status, minor motor status)
k e e rrss
b ooook b o ook
Paraneoplastic
o b oo
eeb ee/e/e b ee/e/e b
The presence of malignancy frequently triggers a complex
immunologic response in the affected individual, including

/ / t
/ m
.t.m
occurs, postinfectious cerebellitis is felt to represent the major-
: : / / t
/ m
.t.m
activation of cell-mediated and humoral immune systems. As
ity of cases.

t ppss : / p ss : /
part of the normal humoral response to malignancy, antibod-
ies that cross-react with normal CNS antigens can be pro-
t p
Demyelinating t
hhtt
ADEM and multiple sclerosis (MS) are acquired inflammatory
t
hhtt
duced. Opsoclonus-myoclonus ataxia syndrome (OMAS) is a
rare paraneoplastic condition that is associated with neuro-
blastoma. The condition typically affects toddlers between
demyelinating diseases of the CNS white matter that can age 1 and 3 years. It presents with acute onset of ataxia, behav-
involve the cerebellum. ADEM is typically a monophasic con- ioral disturbance, rapid chaotic movements of the eyes (ops-
dition that usually occurs after a preceding infection. Patients oclonus), and myoclonic jerking of the limbs, leading to

k e rrss
e
including encephalopathy. The cerebellum is involved in
k e rrss
present acutely with multifocal neurologic deficits invariably

e
common description of the condition as “dancing eye, dancing
feet syndrome.” This was initially described as “Kinsbourne

o o
o o k o o o
about half of all cases. Magnetic resonance imaging (MRI)
o k o
encephalitis.” Ataxia may be the only initial symptom leading
o
eebb of the cerebral and cerebellar hemispheres.
ee / b
e b
often reveals large, confluent multifocal white matter lesions
/ e e / / b
e b
to an initial diagnosis of an acute cerebellar ataxia. With time,
e
opsoclonus and myoclonus become prominent. Early behav-
e
/ t m
MS is a recurrent demyelinating disorder of the CNS that
.t.m
often involves the cerebellum and spinal cord. Approximately
: / / : / / t m
ioral disturbances in a child with acute ataxia should raise
.t.m
suspicion for OMAS. Although the condition is felt to be
/
t ss:
p /
5% of all patients with MS have their first attack before

p
age 18. Cerebellar involvement at MS disease onset is more
t p ss:
p /
immune-mediated, no specific antibody has been identified.
However, a number of studies have identified autoantibodies
t
hht t
common in children compared with adults.
Neuromyelitis optica (NMO) is a recurrent demyelinating
t
hht t
to CNS antigens in patients with OMAS. The most commonly
reported target is to the cerebellar Purkinje cells. However,
condition caused by antibodies to aquaporin 4 water channels anti-Hu antibodies, usually associated with small-cell lung
on astrocytic foot processes. These channels are concentrated in cancers in adults, have also been reported. Neuroblastoma
the lining of the cerebrospinal fluid aqueductal system. Given underlies more than half of all cases of OMAS, but only 2%

k e r s
rs
with aquaporin 4 autoantibodies often have episodic ataxia
e k eers
the proximity of the fourth ventricle to the cerebellum, children

rs
to 3% of patients with neuroblastoma develop OMAS. Detect-
ing the presence of neuroblastoma requires CT or MRI, bone

o o
o k
(EA). Unlike ADEM and MS, Neuromyelitis optica (NMO)
o o o
oo k
relapses are more likely to result in permanent disability.
and metaiodobenzylguanidine (MIBG) scans, bone marrow

oo
testing, urine catecholamine measurements, and other studies.

eebb e e
/ b
e b
The Miller Fisher variant of GBS is another inflammatory
/
cause of acute ataxia consisting of ataxia, areflexia, and oph-
e e /e/ebb
Studies report strong diagnostic yields of 131I MIBG scintigra-
phy ranging from 70% to 92% in detecting the primary tumor.
e
: / / / .
tm m
thalmoparesis. These may occur in up to 10% of all children
t .
with GBS. It is associated with antibodies to the gangliosides
/ / /.tm m
Ataxia has also been described as a paraneoplastic phenome-
t .
non in isolated pediatric cases of Hodgkin disease, Langerhans
:
t p ss
p : /
GQ1b, GD3, and GT1a (van Doorn et al., 2008).

t p ss : /
cell histiocytosis, and hepatoblastoma.

p
t
hht t t
hht t
 t t p
t ss:
p t t p
t ss:
p

hht hht Acute Cerebellar Ataxia 703

include glucose transporter-1 deficiency syndrome, pyruvate

k e r
e s
BOX 92-3  Reported Toxicology Screening for
rs Acute Ataxia
k eers
r s dehydrogenase deficiency, and biotinidase deficiency. Further- 92

o o
o o k oooo k more, disorders affecting mitochondrial function are also well

o
known to have ataxia as an early and prominent symptom. A
o
eebb b b
• Alprazolam
• Triazolam and temazepam
• Diazepam and lorazepam
ee/ e
/e b e e
/ b
recent study found that specific risk factors were helpful in
/ e
differentiating acquired (i.e., noninherited) from inherited
e
• Benzodiazepine

: / / t
/ .
t m
. m : / / t t m
etiologies of subacute or chronic childhood ataxia (Benini
. . m
et al., 2012). These risk factors included (1) duration of symp-
/
• Phenytoin
• Phenobarbitone
t p ss
p : / ss : /
toms greater than 2 weeks; (2) consanguinity; (3) a first-degree

t p p
• Carbamazepine
• Phenothiazine t
hht t t
hht
relative with similar presentation; (4) presenting symptoms of
t
abnormal gait, rash, ichthyosis, or multiorgan abnormalities;
and (5) an abnormal examination finding of motor function
(gait, tone, strength), deep tendon reflexes, and clonus, dys-
metria, pes cavus, or sensory deficits. The presence of these risk
factors suggested a greater likelihood of a genetic or metabolic
Intoxication

k e rs
rs err
Accidental poisoning in children aged less than 6 years is
e k e ss
disorder that frequently could be determined by specific
testing, including mitochondrial DNA testing, chromosomal

b ooookthe most common form of toxin ingestion. Ataxia may

ooook
be seen with ingestion of lead, alcohol, anticonvulsants,
b
microarray, serum lactate, pyruvate, carnitine, acylcarnitine,

b o o
vitamin E and amino acid determinations, urine organic acid

eeb ee/ e
/ e b
benzodiazepines, antihistamines, organic chemicals, or heavy
metals. A retrospective study reports that 32.5% of acute ataxia
ee/ e
/ e b
screening, and testing for fragile X syndrome. For more infor-
mation on hereditary ataxias, see Chapter 91.

: // /.tm m
in children is caused by ingestion regardless of whether a
t .
history of toxin exposure is elicited (Gieron-Korthals et al.,
: / / /.tm m
Recurrent genetic ataxias (EA type 1 [EA1] to EA7) may
t .
mimic acute cerebellar ataxia at first presentation. However,

ss : /
1994). Agents responsible for positive screening are listed in
t p p tp pss : /
recurrent episodes, a favorable response to acetazolamide, and

t
hht t
Box 92-3 (Whelan et al., 2013). Several studies have reported
benzodiazepines as a common cause of ataxia. Isopropanol
(found in rubbing alcohol) ingestion has been reported to
t
hht ttypical clinical features (see Chapter 91) distinguish these dis-
orders from acute cerebellar ataxia. EA1 (KCN1A) and EA2
(CACNA1A) account for the majority of cases.
cause ataxia in 5% of 91 children younger than 6 years of age.
Clinical evidence of toxicity typically develops between 0.5
and 2 hours postingestion. Other Neurologic Disorders

ke rrss
Mass Lesions
e keerrss Ataxia is common in basilar migraine, in which it can be

b ooook o ook
Posterior fossa tumors, including medulloblastoma, pilocytic
b o
associated with vertigo and focal neurologic deficits. Focal

oo
causes of cerebellar dysfunction have to be ruled out, although
b
eeb e e/e b
astrocytoma, and ependymoma, account for approximately
/
50% of all childhood brain tumors. These usually present with
e e /e/e
nature of the episode.
e b
positive visual phenomena strongly suggest the migrainous

/ / m
.t.m
a slowly progressive ataxia and symptoms of increased intra-

/ t
cranial pressure such as headache, vision disturbances, and
: : / / / m
.t.m
Benign paroxysmal vertigo is thought to be a migraine
t
equivalent and is more common than benign paroxysmal

t ppss : /
focal deficits on neurologic examination. Cerebellar abscess

t ppss : /
positional vertigo, which can also present as acute ataxia.

t
hhtt
is an uncommon cause of acute ataxia as symptoms usually
develop slowly over a period of days and weeks. Nevertheless,
the sudden hemorrhage into a posterior fossa tumor with
t
hhtt
Detailed event description, family history, and the Dix–
Hallpike maneuver could be revealing.
Ataxia can be prominent during the ictal or postictal phases
resultant acute ataxia may be the first evidence of malignancy. of seizures and in nonconvulsive seizures. Glutamic acid
decarboxylase (GAD) antibody (GAD-ab) has been implicated
Trauma in a spectrum of neurologic syndromes. However, there is

k errss
Isolated acute ataxia is uncommon from blunt force trauma.
e k
Cerebellar contusion or posterior fossa bleeds can cause
e rrss
e
insufficient presently data regarding the role of GAD-ab in
childhood acute ataxia. The role of antimyelin-associated gly-

oooo k o o o k
ataxia, which can be associated with headache. This may be
o
coprotein, glutamate receptor delta-2 autoantibody, and post-
o o
eebb Vascular b b
varicella anticentrosomal antibodies in acute ataxia remains

ee/ / e b
caused by direct axonal injury or because of mass effect.
e ee/ e
/ e
to be clearly delineated.
b
Sensory ataxia results from loss of sensory input to the

: / / t
/ m
.t.m : / / t
/ m
.t.m
cerebellum owing to lesions in the posterior column of the

/
Posterior circulation strokes, although rare in children, can

p ss:
present with acute ataxia. This possibility should be consid-
t p t p ss: /
spinal cord or the peripheral nervous system. It is character-
ized by a positive Romberg sign (ataxia worse on closing eyes)
p
t
hht t
ered especially in children predisposed to thromboembolic
disease. Arteriovenous malformations with hemorrhage and
traumatic vertebral artery dissection can present with acute
t
hht t
and diminished deep tendon reflexes. Sensory ataxia is present
in as many as 15% of pediatric cases of GBS, usually in asso-
ciation with neuropathic weakness and other sensory symp-
ataxia. Cerebral angiography identifies an abnormality mostly toms (Gieron-Korthals et al., 1994).
in the V2 segment (C1 to C6) of the vertebral artery in Optic ataxia is caused by impaired visual control of the
dissection. direction of limb-reaching for a visual target. It may be associ-

k e r s
rs
Metabolic/Genetic
e k ee rs
rs ated with lesions in the superior parietal lobule, which also
affects visual-guided saccades and other forms of eye–hand

o o
o o k o ooo
Many inborn errors of metabolism can present with ataxia,k coordination.
oo
eebb e e
/ b
e b
which can develop acutely or intermittently because of trig-
/ Psychogenic
gered decompensation (see Table 92-2). The first presentation
e ee/e/ebb
/ / / .
tm m
of such disorders in childhood can mimic an acute cerebellar
t .
ataxia. Several inborn errors of metabolism can present with
: : / / /.tm m
A psychogenic or nonorganic cause of ataxia must be consid-
t .
ered when the examination findings are bizarre, inconsistent,

ss : /
ataxia as an early, prominent symptom. Some examples

t p p t p ss
p : /
or incongruent. Further evaluation should address possible

t
hht t t
hht t
 t t p
t ss:
p t t p
t ss:
p
704
hht
PART X  Disorders of Balance and Movement
hht
previous psychiatric illness, secondary gain, or a precipitating there is a history of epilepsy or evidence of an altered mental

k e rrss
event. Psychogenic ataxia is a diagnosis of exclusion.
e k e r
e s
r s status or the clinical suspicion of nonconvulsive status epilep-

o o
o o k oo o o k ticus. Nonconvulsive seizures are usually seen with preexisting

o
seizure disorders and cognitive impairment. This diagnosis is
o
eebb INVESTIGATIONS IN ACUTE ATAXIA
ee/ e
/ebb
Acute postinfectious cerebellar ataxia is a diagnosis of exclu-
e / e
/ b
e b
associated with underlying EEG abnormalities with dramatic
clinical and electrographic improvement when treated appro-
e
: / / t . m
. m
sion. A thorough history and physical examination are far
/ t : / / t
/ t m
priately (Whelan et al., 2013).
. . m
t p : /
more likely to identify the etiology rather than an extensive
ss Toxicology
workup. The selection of primary laboratory and radiologic
p t p ss
p : /
hhtt t
investigations should be based on clinical scenario. Serious
conditions that may present with ataxia should be excluded.
Of all investigations performed in the diagnostic workup of
t
hht t Ataxia after ingestion is most frequently caused by medica-
tions but less often from exposure to organic chemicals, sol-
vents, and heavy metals. Accidental poisoning in children less
acute cerebellar ataxia, the urine and/or serum drug screen than 6 years of age is the most common form of toxin inges-
are most likely to prove diagnostic, even where a source tion, with a second peak in adolescence, where intoxication
of ingestion is not immediately apparent (Gieron-Korthals

k eer s
rs k e er s
r s
et al., 1994). Neuroimaging should be obtained if there is
occurs as a result of substance abuse (Gieron-Korthals et al.,
1994). A high index of suspicion should always be main-

ook ook
concern of a mass lesion. CSF analysis should be done if tained, because a history of ingestion or exposure might not

b oo
eeb Computed Tomography and Magnetic   ee/e
uncertain.
b o
there is concern for acute CNS infection or the diagnosis is

b o / e b o
b o
be forthcoming. After ingestion, ataxia is often accompanied
by mental status changes such as lethargy, confusion, inap-

m / e ee / e
propriate speech, or unconsciousness. Toxicology screens are
m
Resonance Imaging
: ///t/.t. m : / /t/.t . m
therefore of value in the initial evaluation of acute ataxia in
children. Other agents associated with ataxia in children
/
t t p
t ss
p :
The diagnostic screening yield of CT and MRI is low; only 5%
t tptpss :
include phencyclidine and other recreational drugs, antiepi-
leptic drugs, anticholinergic agents, muscle relaxants, “magic”
hht
of children initially being evaluated for ataxia showed any
telling abnormalities (Figure 92-1). CT and MRI are more hht mushrooms, and cold remedies.

Urinary Catecholamines/Metaiodobenzylguanidine
commonly used to rule out a specific diagnosis such as tumor,
stroke, ADEM, or the risk of herniation before lumbar punc-
ture (Whelan et al., 2013).
Scintigraphy

keerrss keerr
and pelvis, with thin cuts through the adrenals, is recom-s
In children with OMAS, MRI or CT of the chest, abdomen,
s Opsoclonus-myoclonus syndrome is a rare autoimmune
disorder in which a neuroblastoma is found in at least 50%

b ooook tional imaging is nondiagnostic.

b ooook
mended. Nuclear scintigraphy is also recommended if conven- of affected individuals. Evaluation for an occult neuroblas-

b oo
toma should occur in all children with opsoclonus or myoc-
eeb Cerebrospinal Fluid ee/e/e b ee/e/e b
lonus accompanying the ataxia as well as in children with
isolated ataxia whose symptoms do not begin to resolve

: / / t
/ m
.t.m : / / t
/ m
.t.m
within 2 weeks (Figure 92-2). On the use of MIBG scintigraphy

t p ss : /
CSF examination is frequently performed in the evaluation of
children with acute ataxia. Data from reported studies of chil-
p t ppss : /
in patients suspected of having neuroblastoma, studies have
reported a sensitivity for tumor detection in the range of
t
hhtt
dren with acute ataxia found abnormal CSF findings in 43%
of children undergoing lumbar puncture (Whelan et al.,
2013). CSF studies showed mild pleocytosis and variable ele-
t
hhtt 70% to 92% (Brunklaus et al., 2012). One study detected
residual, recurrent, or metastatic neuroblastoma in 16 of
20 patients (80%). Another study showed MIBG sensitivity
vations of protein. In one study, CSF was analyzed according of 70% in detecting abdominal or pelvic neuroblastoma
to the suspected etiology (e.g., viral, vaccine-related, and idio- and 83% in detecting thoracic neuroblastoma (Brunklaus
pathic) and no significant differences were noted (Connolly et al., 2012). In a third study, sensitivity differences between

k e e rrss
et al., 1994).

k e errss 131
I MIBG and 123I MIBG were not observed. Neuroblastomas
frequently spontaneously involute, which may account for the

o o
o o k
Electromyography and Electroencephalography
o o oo k o
observation that no tumor is found in up to half of all cases
o
eebb e / e
/ b
e b
Electromyography (EMG) and nerve conduction velocity
e
of OMAS.

e / e
/ b
e b
Neuroblastoma may be associated with elevated levels of
e
/ t m
studies should be considered in evaluation of acute-onset
.t.m
ataxia in cases in which clinical diagnosis of the Miller Fisher
: / / : / / t m
one or more urinary catecholamine metabolites; vanillylman-
.t.m
delic acid (VMA) and homovanillic acid (HVA) are the most
/
t ss:
p /
variant of GBS is suspected (Whelan et al., 2013). Very early

p
(less than 4 days) electromyography assessment in GBS may be
t p ss:
p /
common catecholamines examined. In a reported study, only
37 of 408 (9%) patients had elevated urinary HVA or VMA

hhtt t
nondiagnostic; however, testing multiple motor and sensory
nerves (greater than or equal to 3) may detect abnormal find- hht t t
levels, and neuroblastoma was subsequently diagnosed.

ings suggestive of disease. EMG is also useful in confirming the

Other Tests
diagnosis of EA1, by showing continuous motor unit activity,
most often in the hands. Autoimmune disorders are believed to play an important role

k e r
e s
r
Studies reported electroencephalographic (EEG) findings
s k e r
in 24 of 57 children with acute ataxia. Data on 24 children
e s
rs
in acute ataxia. Testing performed to screen for autoimmune
disorders includes examination for the presence of antinuclear

o o
o o k with acute ataxia showed nonspecific EEG abnormities
in 42%.
o o
oo k antibodies—anti-SS-A/Ro and anti-SSB/ladanticardiolipin

oo
antibody, and antiphospholipid antibodies. There are insuf-

eebb e e
/ b
e b
Acute ataxia may be present during the ictal or postictal
/
phase of seizures in children (Ryan and Engle, 2003). Non-
e e /e/ebb
ficient data regarding the role of GAD-ab in childhood acute
ataxia (Whelan et al., 2013). There are insufficient data to
e
: / / / .
tm m
convulsive epileptic states have also been referred to as pseu-
t .
doataxia and may present as ataxia with or without alteration
/ / /.tm m
draw conclusions regarding the diagnostic yield of screening
t .
testing of autoimmune disorders in children presenting with
:
t p ss
p : /
of consciousness. Clinicians can consider obtaining an EEG if

t p ss : /
acute ataxia.

p
t
hht t t
hht t
 t t p
t ss:
p t t p
t ss:
p

hht hht Acute Cerebellar Ataxia 705

Treatment and Prognosis 100 mg daily). Acetazolamide has been shown to abort acute

k e r
e s
rs k eers
r s
Acute postinfectious cerebellitis is usually a self-limited condi-
attacks of ataxia in pyruvate dehydrogenase deficiency. 92

o o
o o k o oo
tion with most children showing a spontaneous recovery
o k Basilar artery migraine can be managed the same as other

o
forms of migraine. Migraine prophylaxis is useful when attacks
o
eebb bb b b
within the first week. Full recovery is expected within 3 months

e/ e
/e
of onset in at least 50% of children, but recovery remains
e e / e
are frequent, as in benign paroxysmal vertigo.

/ e
Nonmetastatic neuroblastoma should be removed surgi-
e
: / t
/ .
t m
incomplete in a minority of cases. The typical approach to a

. m
child with inflammatory cerebellitis is to exclude a primary
/ : / / t t m
cally. If no obvious tumor is detectable at first presentation,
. . m
periodic surveillance is essential in suspected cases. Adreno-
/
t p ss
p : /
infectious etiology. After an active infection is ruled out, a trial
of immunosuppression is usually warranted. Corticosteroids,
t p ss
p : /
corticotrophic hormone, corticosteroids, and IVIg will improve

t
hht t
such as solumedrol in a daily dose of 20 to 30 mg/kg up to
1000 mg a day, are usually pulsed for 3 to 5 days. If there is
no improvement in symptoms, then plasmapheresis or intra-
t
hht t symptoms in 80% of patients with neuroblastoma. Relapses
may occur during course of treatment or after discontinuing
therapy. Epileptic ataxia (pseudoataxia) responds to anticon-
vulsant drugs.
venous immunoglobulin is the second-line alternative. Intra- Behavioral or learning difficulties were present in 20% of
venous immunoglobulin (IVIg) is frequently given at 2 g/kg/ children during the recovery phase from acute cerebellar ataxia

k eers
rs k er
ers
day. Plasmapherersis is typically done with five exchanges per-
s
formed every other day. In patients who do not have the reso-
but resolved in most within 6 months (Connolly et al, 1994).
Some data suggest that immunosuppression improves devel-

b ooookfor 4 weeks has been reported to show benefit.

b ooook
lution of symptoms, the use of rituximab 375 mg/m2 weekly opmental outcome in children with OMAS.

b o o
eeb e e
/ e b
Children with OMAS frequently require prolonged treat-
/
ment with IVIg and corticosteroids to prevent relapses. Typical
e
REFERENCES

ee/ e
/ e b
The complete list of references for this chapter is available in the

: // t/.tm
IVIg dosing is 1 to 2 g/kg/day every month or every other
. m
month. Pranzatelli and colleagues (Tate et al., 2012) have
: / /t/.tm. m
e-book at www.expertconsult.com.
See inside cover for registration details.

ss : /
described symptom improvement using a multimodal

t p p tp pss : /
t
hht t
approach of adrenocorticotropic hormone in conjunction
with IVIg or rituximab for over 1 year.
Children with the Miller Fisher variant of GBS should be
t
hht t SELECTED REFERENCES
Benini, R., Ben Amor, I.M., Shevell, M.I., 2012. Clinical clues to dif-
ferentiating inherited and noninherited etiologies of childhood
admitted to the hospital for careful monitoring of respiratory ataxias. J. Pediatr. 160 (1), 152–157.
and autonomic function. Corticosteroids are felt to be contra- Bozzola, E., Bozzola, M., Tozzi, A.E., et al., 2014. Acute cerebellitis in
indicated for GBS, with IVIg or plasmapheresis as the first-line varicella: a ten year case series and systematic review of the litera-

ke rrs
therapy. The recommended dose of immunoglobulin is 2 g/
s e rrss
kg over 2 to 5 days. The decision for active treatment depends
e k e
ture. Ital. J. Pediatr. 40, 57.
Brunklaus, A., Pohl, K., Zuberi, S.M., et al., 2012. Investigating neuro-

ook ook
blastoma in childhood opsoclonus-myoclonus syndrome. Arch.
on status of ambulation and respiratory and bulbar involve-

b
eeboo b oo
ment. With or without specific treatment, more than 90% of

/e b bboo
Dis. Child. 97 (5), 461–463.
Connolly, A.M., Dodson, W.E., Prensky, A.L., et al., 1994. Course

/e
ee /e
children with GBS and Miller Fisher syndrome recover com-
pletely within 6 to 12 months of disease onset.
m
673–679.
m ee /e
and outcome of acute cerebellar ataxia. Ann. Neurol. 35 (6),

: / t
/ .t.m
Recovery from acute postinfectious demyelinating enceph-

/ /
alopathy is typically somewhat slower than that from acute
: / / t
/ .t.m
Gieron-Korthals, M.A., Westberry, K.R., Emmanuel, P.J., 1994. Acute
/
childhood ataxia: 10-year experience. J. Child Neurol. 9 (4),

p ss :
cerebellar ataxia and can be hastened by treatment with cor-

t t p t t ppss : 381–384.

hhtt hhtt
ticosteroids. However, a minority of patients were left with
significant sequelae. Single relapses can occur in as many as
10% of affected children. Multiple relapses raise the possibility
of an underlying diagnosis of MS.
Systemic infections and brainstem encephalitis should be
treated with standard antibiotic/antiviral protocols, although
Pandolfo, M., Manto, M., 2013. Cerebellar and afferent ataxias.
Continuum. (Minneap. Minn.) 19 (5 Movement Disorders),
1312–1343.
Ryan, M.M., Engle, E.C., 2003. Acute ataxia in childhood. J. Child
Neurol. 18 (5), 309–316.
Sivaswamy, L., 2014. Approach to acute ataxia in childhood: diagnosis
and evaluation. Pediatr. Ann. 43 (4), 153–159.

k e rrss
outcome in brainstem encephalitis is uncertain.

e k e
Treatment of toxic ingestion depends on the nature and
e rrss Tate, E.D., Pranzatelli, M.R., Verhulst, S.J., et al., 2012. Active
comparator-controlled, rater-blinded study of corticotropin-

o o
o o k oo k
amount of the ingested substance. Monitoring the blood con-
o o
based immunotherapies for opsoclonus-myoclonus syndrome. J.
o o
eebb b b
centration of the drug, the liver and kidney functions, and Child Neurol. 27, 875–884.

e/ e
/ e b
stabilizing vitals while allowing spontaneous elimination of
e
the ingested agent is important. Often times, no specific treat-
ee/ e
/ e b
van Doorn, P.A., Ruts, L., Jacobs, B.C., 2008. Clinical features, patho-
genesis, and treatment of Guillain-Barré syndrome. Lancet Neurol.

/ t m
.t.m
ment is available. In some cases, administration of an anti-

: / /
7 (10), 939–950.

/ / t
/ m
.t.m
Whelan, H.T., Sumit, V., Guo, Y., et al., 2013. Evaluation of the child
:
and Engle, 2003).
t ss:
p /
dote, chelation, dialysis, or other therapies is required (Ryan

p t p ss:
p
15–24. /
with acute ataxia: a systematic review. Pediatr. Neurol. 49 (1),

t
hht t
Ataxia related to postconcussion syndrome usually clears
by 6 months. Tumors, stroke, and traumatic brain injury
are likely to have significant sequelae with incomplete re­­
t
hht t
E-BOOK FIGURES AND TABLES
covery. Vertebrobasilar dissection may need anticoagulation
in the short-term followed by a period of antiplatelet therapy.
Significant cerebellar bleeds need posterior fossa decom­

k e r
e s
rs k eers
rs
pression. Determination of the precipitating stress is impor-
The following figures and tables are available in the e-book
at www.expertconsult.com. See inside cover for registration

o o
o o k o oo k
tant in managing psychogenic ataxia. EA1 may respond to
antiepileptic medication. Both EA1 and EA2 respond to daily
o
details.

oo
eebb b b
Fig. 92-1 Cerebellitis.
acetazolamide.

e / e
/ e b
Daily nicotinamide administration (50–300 mg) may
e /e e b
Fig. 92-2 Metaiodobenzylguanidine scintigraphy.

ee /
Box 92-1 Signs of cerebellar dysfunction.

/ / t tm
reverse neurologic complication in Hartnup disease. Acute
. .m
ataxia in some cases of maple syrup urine disease may respond
: / : / / t
/.tm
. m
Table 92-2 Causes of Acute Episodic Cerebellar Ataxia

ss : /
to thiamine 1 g and ongoing supplementation (maintenance

t p p t p ss
p : /
Video 92-1 Signs of Ataxia

t
hht t t
hht t