Chapter 183  u  Ataxia and ­Movement ­Disorders  631
CSVT can also be caused by infections or dehydration. Hem-
orrhagic strokes (HS) may be intraparenchymal (primary,
or secondary bleeding after AIS) or associated with intra-
ventricular, subarachnoid, subdural, or epidural bleeds. The
most common causes of HS are vascular malformations, head
trauma, and vasculitis.
Clinical Manifestations
The acute onset of focal neurologic deficits in a child is stroke
until proven otherwise. Symptoms may be subtle and non-
specific, which can lead to a delay in diagnosis. AIS typically
presents with acute, focal neurologic deficits. Hemiparesis is
most common, but visual, speech, sensory, or balance defi-
cits may be present. Unlike AIS in adults, focal seizures are
quite common in childhood presentation of AIS. Similarly,
neonatal AIS most often presents with acute symptom-
atic focal seizures (typically in the first day of life) or with
encephalopathy.
Symptoms of CSVT may progress more gradually and be
more variable and nonspecific than AIS. In CSVT, acute focal
deficits may be present, or the child may have progressive signs
of elevated intracranial pressure, including headache, papill-
edema, diplopia (most often from cranial nerve VI palsy), sei-
zures, lethargy, or confusion.
HS tends to present acutely, with a sudden “thunderclap”
headache. HS may also present with loss of consciousness,
nuchal rigidity, focal deficits, or seizures and can be rapidly
fatal.
Congenital hemiplegia becomes apparent as infants develop,
with decreased use of one side of the body, early handedness,
or ignoring one side. Neuroimaging reveals an area of enceph-
alomalacia in the contralateral cerebral hemisphere. The
details of the child’s intrauterine, labor, delivery, and postnatal
history often are unremarkable. The area of encephalomalacia
may predispose the child to epilepsy.
Differential Diagnosis
There are many conditions that mimic pediatric stroke, most
of which are more common than AIS, HS, or CSVT. Some of
these mimics are benign (migraine, psychogenic weakness,
musculoskeletal abnormalities), but others require specific,
prompt diagnosis and/or treatment (e.g., transient postictal
weakness, intracranial infection, inflammatory disease of the
central nervous system, tumor, or posterior reversible leuko-
encephalopathy syndrome).
Diagnostic Tests and Imaging
On initial presentation, acute neuroimaging is necessary. A
noncontrast head computed tomography (CT) scan is highly
sensitive to acute HS and can reveal larger, mature AIS; acute,
nonhemorrhagic stroke may not be seen on routine CT.
Therefore, MRI with diffusion-weighted imaging is required
for most presentations. Angiography by magnetic resonance
angiography (MRA) or CT angiography is used to confirm
arterial occlusion in AIS and can identify underlying arteri-
opathy, vascular malformations, and aneurysms. Diagnosis
of CSVT requires a high clinical suspicion and purposeful
imaging of the cerebral venous system with venography by
MRI or CT. If clinical assessment does not reveal the cause
of the stroke, a complete laboratory investigation should be
undertaken promptly based on suspected etiologies (see Table
182-5). Many children with AIS have more than one predis-
posing factor, so identifying one risk factor does not obviate
the need for a complete evaluation.
Treatment
Treatment must focus on limiting secondary neuronal injury
and prevention of future strokes. Neuroprotection by main-
taining control of temperature, blood pressure, glucose, and
seizures is essential. Emergency thrombolysis with medica-
tions or catheterization is not yet established for children,
but this is an area of active clinical research. Anticoagulants
(IV heparin, subcutaneous low-molecular-weight heparin,
oral warfarin) and platelet antiaggregants (aspirin) are used
for secondary stroke prevention in some instances. For those
with progressive CSVT, anticoagulation is the mainstay of
therapy. Long-term rehabilitation programs are required for
most survivors.
Chapter 183
ATAXIA AND
M
­ OVEMENT
D­ ISORDERS
ATAXIA
Ataxia is the inability to make accurate, smooth and coordi-
nated movements, usually due to a dysfunction of the cere-
bellar pathways. If either the afferent (joint position senses)
or efferent cerebellar connections (cerebellum through thal-
amus to cerebral cortex) are disturbed, the patient has ataxia.
Truncal ataxia reflects disturbances of the midline cerebellar
vermis (e.g., medulloblastoma, acute postinfectious cerebellar
ataxia, or ethanol intoxication). Appendicular ataxia reflects
disturbances of the ipsilateral cerebellar hemisphere (e.g., cys-
tic cerebellar astrocytoma). The most common causes of acute
ataxia in childhood are postinfectious acute cerebellar ataxia
and drug intoxications. Discrete lesions within the posterior
fossa, tumors (e.g., medulloblastoma, ependymoma, cerebel-
lar astrocytoma), multiple sclerosis, strokes, and hemorrhages
may cause ataxia. Other causes include benign paroxysmal
vertigo, head trauma, seizures, postictal states, migraine,
paraneoplastic opsoclonus-myoclonus syndrome associated
with neuroblastoma, and inborn errors of metabolism. Con-
genital disorders also may produce chronic, nonprogressive
ataxia. There are a number of inherited ataxia syndromes
(Table 183-1).
Clinical Manifestations
Decision-Making Algorithm
Available @ StudentConsult.com
Ataxia
632  Section 24  u Neurology

Table 183-1    Causes of Ataxia

Brain tumors  Inhalants
 Medulloblastoma*   Drugs of abuse
 Ependymoma Vascular disorders
  Cerebellar astrocytoma*   Cerebellar hemorrhage or infarction
  Brainstem glioma   Vertebral artery dissection

Paraneoplastic disease Demyelinative disorders

  Neuroblastoma (opsoclonus-myoclonus-ataxia)   Multiple sclerosis

Infectious disorders   Acute disseminated encephalomyelitis

 Encephalitis* Structural or congenital disorders

  Brainstem encephalitis   Cerebellar hypoplasia

  Vermal aplasia
 Meningitis
  Dandy-Walker malformation
 Labrynthitis*
  Arnold-Chiari malformation
  Cerebellar abscess
 Hydrocephalus
Postinfectious disorders
Hereditary ataxic disorders
 Acute cerebellar ataxia (acute postinfectious
cerebellitis)*   Episodic ataxia
  Guillain-Barré syndrome   Friedreich ataxia
Migrainous disorders   Ramsay Hunt syndrome

  Basilar migraine*   Spinocerebellar ataxia 1 and 2

  Benign paroxysmal vertigo  Ataxia-telangiectasia
Trauma   Marinesco-Sjögren syndrome
  Cerebellar hemorrhage Genetic and metabolic disorders
  Cerebellar contusion   Metachromatic leukodystrophy
 Concussion  Adrenoleukodystrophy
  Postconcussive syndrome*   Maple syrup urine disease
  Vertebrobasilar occlusion   Hartnup disease
Toxic ingestions*  GM2 gangliosidosis (juvenile)
 Ethanol   Refsum disease
 Anticonvulsants   Vitamin E deficiency
 Antihistamines   Leigh disease

 Benzodiazepines   Wilson disease

  Carbon monoxide  Abetalipoproteinemia

*Common.

The usual symptoms of ataxia are a broad-based, unsteady
gait (truncal ataxia) and intention tremor or dysmetria (over-
or undershooting of the target due to abnormal distance
perception). An intention tremor worsens as the arm/hand
approaches the target. Classically, these symptoms stem from
disorders of the cerebellar pathways, but peripheral nerve
lesions causing loss of proprioceptive inputs to the cerebellum
(Guillain-Barré syndrome) may present with similar symp-
toms. In addition, weakness may intensify or mimic ataxia, so
strength must be assessed, along with coordination.
Etiologies
Decision-Making Algorithm
Available @ StudentConsult.com
Ataxia
Drug intoxication is the most common cause of acute ataxia
among children. Overdosage with any sedative-­hypnotic agent
can produce acute ataxia and lethargy, but ataxia without lethargy
usually results from intoxication with ethanol or anticonvulsant
drugs. It is important to ask about any medications or drugs of
abuse the patient may have access to. Treatment is supportive.
Postinfectious acute cerebellar ataxia may occur 1 to
3 weeks following varicella, infectious mononucleosis, mild
respiratory or gastrointestinal viral illnesses, or other infec-
tions. The pathogenesis is uncertain and may represent either
a direct viral infection of the cerebellum or, more likely, an
autoimmune response precipitated by the viral infection
and directed at the cerebellar white matter. Symptoms begin
abruptly, causing truncal ataxia, staggering, and frequent
falling. Dysmetria of the arms, dysarthria, nystagmus, vom-
iting, irritability, and lethargy may be present. Symptoms,
which may be severe enough to prevent standing or sitting,
usually peak within 2 days, then stabilize and resolve over
 Chapter 183  u  Ataxia and ­Movement ­Disorders  633

several weeks. Cerebrospinal fluid (CSF) examination some- or explosive movements of voluntary muscles. They are gener-
times shows a mild lymphocytic pleocytosis or mild elevation ally the result of abnormalities of the extrapyramidal system or
of protein content. Brain magnetic resonance imaging may the basal ganglia. Movement disorders in children are typically
reveal cerebellar enhancement. No specific therapy is available hyperkinetic (increased movement) patterns. The abnormal
except to prevent injury during the ataxic phase. movements are activated by stress and fatigue and often disap-
Brain tumors are the second most common neoplasm in pear in sleep. They are typically diffuse and migratory (chorea)
children. About 50% arise from within the posterior fossa. but may be isolated to specific muscle groups (segmental myoc-
Tumors that arise in the posterior fossa or brainstem produce lonus, palatal myoclonus) and may not disappear in sleep.
progressive ataxia with headache that may be acute or gradual Chorea is a hyperkinetic, rapid, unsustained, irregular, pur-
in onset. There is a progressive worsening over days, weeks, poseless movement that seems to flow from one body part
or months, typically with associated signs and symptoms of to another. Affected patients demonstrate difficulty keeping
elevated intracranial pressure. The ataxia and dysmetria may the tongue protruded or maintaining grip (milkmaid grip).
result from primary cerebellar invasion or from obstruction Patients often attempt to incorporate the involuntary move-
of the CSF pathways (aqueduct of Sylvius or fourth ventricle) ments into more purposeful movements, making them appear
with resultant hydrocephalus. The most common tumors in fidgety. Choreiform movement abnormalities may be autoim-
this region include medulloblastoma, ependymoma, cerebel- mune/parainfectious, infectious, genetic, structural, metabolic
lar astrocytoma, and brainstem glioma. or toxic in origin (Table 183-2). The movements may occur
Rarely, a neuroblastoma located in the adrenal medulla or
anywhere along the paraspinal sympathetic chain in the tho-
rax or abdomen is associated with degeneration of Purkinje Table 183-2    Causes of Movement Disorders
cells and the development of severe ataxia, dysmetria, irrita-
bility, myoclonus, and opsoclonus. An immunologic reac- CHOREA
tion directed toward the tumor may be misdirected to attack Autoimmune/parainfectious (acute rheumatic fever,* systemic lupus
Purkinje cells and other neuronal elements. The myoclonic erythematosus)
movements are irregular, lightning-like movements of a limb Infectious (human immunodeficiency virus, neurosyphilis, scarlet
or the head. Opsoclonus is a rapid, multidirectional, conju- fever, encephalitis)
Genetic (Huntington disease, ataxia-telangiectasia)
gate movement of the eyes, which suddenly dart in random Structural (stroke, neoplasm)
directions. The presence of opsoclonus-myoclonus in a child Metabolic/toxic (hepatic/renal failure, hyperthyroidism)
should prompt a vigorous search for an occult neuroblastoma. Drug induced
Several rare inborn errors of metabolism can present with ATHETOSIS
intermittent episodes of ataxia and somnolence. These include
Cerebral palsy*
Hartnup disorder, maple syrup urine disease, mitochondrial Kernicterus
disorders, abetalipoproteinemia, and vitamin E deficiency. Hypoxic-ischemic encephalopathy
Difficulty walking with a severe staggering gait is one man- Drug induced
ifestation of acute labrynthitis, but the diagnosis usually is Pantothenate kinase-associated neurodegeneration
Pelizaeus-Merzbacher disease
clarified by the associated symptoms of a severe sense of spin-
ning dizziness (vertigo), nausea and vomiting, and associated DYSTONIA
signs of pallor, sweating, and nystagmus. Inherited primary dystonias
Ataxia-telangiectasia, an autosomal recessive genetic disor- Acute dystonic reaction*
der, is the most common of the degenerative ataxias. Affected Tardive dyskinesia
Cerebral palsy*
patients present with ataxia around age 2 years, progressing to Metabolic disorders (Wilson disease)
loss of ambulation by adolescence. In mid-childhood, telangi-
ectasia is evident over the sclerae, nose, ears, and extremities. TREMOR
Abnormalities in immune function and greatly increased risk Physiologic*
of lymphoreticular tumors result in early death. Essential tremor*
Hereditary, degenerative (Huntington disease, Wilson disease)
Friedreich ataxia is a relentlessly progressive, autosomal Stroke
recessive disorder. Children present in the late elementary years Metabolic (hyperthyroidism, hepatic encephalopathy, electrolyte
with ataxia, dysmetria, dysarthria, diminished proprioception disturbances)
and vibration, absent deep tendon reflexes, and nystagmus, Drugs/toxins* (caffeine, bronchodilators, amphetamines, tricyclic
and many develop hypertrophic cardiomyopathy and skeletal antidepressants)
Psychogenic tremor
abnormalities (high-arched feet, hammer toes, kyphoscoliosis).
MYOCLONUS
Epilepsy
MOVEMENT DISORDERS Benign*
Infection
Toxin
Decision-Making Algorithm Metabolic encephalopathies
Available @ StudentConsult.com
TICS*
Involuntary Movements Transient
Chronic motor tic disorder
Tourette syndrome
Movement disorders or dyskinesias are a diverse group of enti-
ties associated with abnormal excessive, exaggerated, chaotic, *Common
VII. Approach to Unsteady Gait
A. Definition. Ataxia is a disturbance in smooth coordination of movements and often manifests
as unsteady gait. Ataxia can be the result of cerebellar or proprioceptive dysfunction (sensory
loss ataxia).
B. Differential diagnosis. A variety of neurologic problems can give the appearance of an
unsteady gait.
1. Cerebellar dysfunction. Children with a cerebellar gait have an unsteady, wide-based
stance with irregular steps, also known as a “drunken gait.” See Table 12-4 for the
causes of cerebellar ataxia.
2. Weakness. Any cause of muscle weakness or sensory loss, such as spinal cord lesions or
acute disorders of the motor unit [e.g., Guillain–Barré syndrome, see section VII.D], can
lead to an unsteady gait.
3. Encephalopathy as a result of infection, drug overdose, or recent head trauma may
cause decreased levels of consciousness, which may affect gait.
4. Seizures. During a seizure, or while in the postictal period, the patient’s gait may be
irregular and unsteady.
5. Vision problems can mimic the appearance of an unsteady gait.
6. Vertigo from migraines, acute labyrinthitis, and brainstem tumors may lead to unsteady
walking.
C. Acute cerebellar ataxia of childhood
1. Definition. Acute cerebellar ataxia is an unsteady gait secondary to a presumed
autoimmune or postinfectious cause.
2. Epidemiology
a. Acute cerebellar ataxia is the most common cause of ataxia in children.
b. Age of onset is between 18 months and 7 years. Acute cerebellar ataxia rarely
occurs in children older than 10 years.
3. Etiology
a. Common preceding infections include varicella, coxsackievirus, Epstein–Barr
virus (EBV), and mycoplasma. The ataxia usually follows a viral illness by 2–
3 weeks.
b. The postulated cause is thought to be molecular mimicry, where molecular
similarity between antigens from the infectious entity and the patient (e.g.,
cerebellar structures) triggers an autoimmune response.
4. Clinical features
a. Truncal ataxia with deterioration of gait is characteristic. Young children may
refuse to walk for fear of falling.
b. Slurred speech and nystagmus are often present, and hypotonia and tremors are
less common.
c. Fever is absent.
5. Diagnosis. Diagnosis is by history and physical examination and by exclusion of other
causes of ataxia. For patients with clear acute cerebellar ataxia, neuroimaging is generally
not necessary. If there are atypical features or concern for other causes of ataxia,
however, neuroimaging is necessary (e.g., to rule out acute life-threatening causes such
as tumors or hemorrhage in the posterior fossa). If head CT is obtained to rule out acute
life-threatening causes, it is normal in acute cerebellar ataxia. MRI provides better detail
to visualize the posterior fossa for evaluation of other causes of ataxia.
6. Management. Treatment is supportive. Complete resolution of symptoms generally
occurs in 2–3 weeks. Physical therapy is often needed.

475
 D. Guillain–Barré Syndrome (acute inflammatory demyelinating polyneuropathy)
1. Definition. Guillain–Barré syndrome is a demyelinating polyneuritis characterized by
ascending weakness, areflexia, and paresthesias.
2. Etiology. The most commonly associated infectious agent is Campylobacter jejuni,
which causes a prodromal gastroenteritis. Many other infectious agents have been
associated with Guillain–Barré syndrome, such as Mycoplasma pneumoniae,
cytomegalovirus, EBV, herpes zoster virus, influenza, varicella, and coxsackievirus.
3. Pathophysiology
a. The principal sites of demyelination are the ventral spinal roots and peripheral
myelinated nerves.
b. Injury is triggered by a cell-mediated immune response to an infectious agent that
cross-reacts to antigens on the Schwann cell membrane.
4. Clinical features
a. Ascending, symmetric paralysis may progress to respiratory arrest.
b. No sensory loss occurs, although low-back or leg pain may be present in 50% of
patients.
c. Cranial nerve involvement. Facial weakness occurs in 40–50% of patients and is
often bilateral.
d. Dysautonomia, arrhythmia, orthostatic hypotension, and urinary retention may
occur.
e. Miller Fisher syndrome, a variant of Guillain–Barré syndrome, is characterized by
ophthalmoplegia, ataxia, and areflexia.
5. Diagnosis
a. LP shows albuminocytologic dissociation (i.e., increased CSF protein in the
absence of an elevated cell count), which is usually evident 1 week after symptom
onset.
b. EMG demonstrates decreased nerve conduction velocity or conduction block.
c. Spinal MRI may be necessary in children younger than 3 years to rule out
compressive lesions of the spinal cord, because the sensory examination in children
of this age is often difficult to evaluate.
6. Management. Treatment should be initiated as soon as the diagnosis is established
because of the risk of respiratory muscle paralysis.
a. Intravenous immune globulin (IVIG), given for 2–4 days, is the preferred
treatment for children because of its relative safety and ease of use.
b. Plasmapheresis removes the patient’s plasma along with the presumed antimyelin
antibodies and is performed over a 4- to 5-day period.
7. Prognosis. Complete recovery is the rule in children but depends on the severity and
extent of the weakness. Physical therapy may be necessary for several weeks or longer to
aid recovery.

Table 12-4
Differential Diagnosis of Cerebellar Ataxia

Brain tumors Pilocytic astrocytoma (occurring in the cerebellum)

Medulloblastoma (occurring in posterior fossa)
Neuroblastoma (opsoclonus myoclonus ataxia syndrome)
Trauma Cerebellar contusion
Subdural hematoma
Toxins Ethanol
Antiepileptic medications
Vascular Cerebellar infarction or hemorrhage
Infections Meningitis

476
 Encephalitis
Inflammatory Acute cerebellar ataxia of childhood
Demyelination Acute disseminated encephalomyelitis (ADEM)
Multiple sclerosis
Migraine syndromes Basilar migraines and familial hemiplegic migraines
Benign paroxysmal vertigo (may have history of migraine)

477