ATAXIA IN CHILDREN -CAUSES, MANAGEMENT, INVESTIGATIONS, TYPES, COMMONEST ATAXIA IN CHILDREN IN DETAIL, HOW WILL YOU FIND OUT THE CAUSE FOR ATAXIA IN CHILDREN FLOWCHART, DEFINITION, TREATMENT

2.  Ataxia : disturbance in the smooth, accurate
coordination of movements.
 It is most commonly manifested as an unsteady
gait.
 Ataxia is usually the result of cerebellar
dysfunction.
 Acute ataxia is an uncommon presenting
complaint in children.
 the majority of children have a benign, self-
limited process .

3.  Ataxia may be acute or chronic.
 Conditions that cause acute ataxia include:
 acute infections.
 post-infectious inflammatory conditions.
 Toxins.
 Tumors.
 Trauma
 80 % of children with acute ataxia: acute cerebellar
ataxia, a toxic ingestion, or Guillain-Barré syndrome

4.  Brain tumor
 Conversion reaction
 Drug ingestion
 Encephalitis (brainstem)
 Migraine
 Postinfectious/immune
 Acute postinfectious
cerebellitis
 Miller Fisher syndrome
 Multiple sclerosis
 Myoclonic
encephalopathy/neuroblast
oma
 Pseudoataxia (epileptic)
 Trauma
 Hematoma
 Postconcussion
 Vasculardisorders
 Cerebellarhemorrhage
 Kawasaki disease

5. EPISODIC (RECURRENT ATAXIA)
Toxin ingestion
CHRONIC
Brain tumours
Hydrocephalus
Metabolic
Nutritional
Congenital malformations
Hereditary ataxias
Basilar artery migraine
Seizure disorder

6. Chronic or progressive ataxia :
Degenerative diseases
Autosomal dominant inheritance
Autosomal recessive inheritance
Ataxia-telangiectasia
Ataxia without oculomotor
apraxia
Ataxia with episodic dystonia
Friedreich ataxia
Congenital anomalies
Cerebellar aplasias
Cerebellar hemisphere aplasia
Dandy-Walker malformation
Vermal aplasia
Brain tumors
 Cerebellar astrocytoma
 Cerebellar hemangioblastoma
 Ependymoma
 Medulloblastoma
 Supratentorial tumors

8. ATAXIA IN CHILDREN BEFORE 1 YEAR OF AGE:
ATAXIA IN CHILDREN BETWEEN 1-5 YEARS OF AGE:
- Drug Ingestion
- Acute cerebellar ataxia
- Myoclonic Encephalopathy and Neuroblastoma
- Inborn errors of Metabolism
- Brain tumors
- Ataxia Telangiectasia
- Refsum's disease
- Congenital malformations

9. Ataxia in Children between 5-10 years
Ataxia in children above 10 years of age:
- Friedreich's Ataxia
- Miller Fisher Syndrome
- Cerebellar hemorrhage
- Multiple Sclerosis
- Olivopontocerebellar degeneration
- Hereditary Ataxias
 - Drug Ingestion
 - Acute Cerebellar Ataxia
 - Brain tumors
 - Wilson's disease

10.  At least 12 forms have been described and
labeled sequentially from SCA1 to SCA12.
 Clinical:
◦ A great degree of overlap in phenotype is present
◦ Mainly symptoms of cerebellar and spinocerebellar
pathway dysfunction.
 Neuroimaging studies are nonspecific.

11.  Life-threatening causes of acute ataxia in children are
fortunately uncommon.
 For those conditions that create a mass effect, signs and
symptoms of increased intracranial pressure are typically
evident.
 Etiologies include
 Tumors,
 Trauma,
 Stroke, and
 Infection.

12.  Of all childhood brain tumors, 60 % arise in the brainstem or
cerebellum.
 Acute decompensation can occur as the result of obstructive
hydrocephalus, hemorrhage into the lesion, or edema.
 Primary brain tumors ordinarily cause chronic progressive
ataxia .
 Early clumsiness may not become apparent until it becomes
severe enough to cause an obvious gait disturbance.
 Brain imaging is a recommendation for most children with
acute cerebellar ataxia.

13.  Mild head injuries are common in children and are an almost
daily occurrence in toddlers.
 Recovery is always complete despite considerable parental
concern.
 More serious head injuries, associated with loss of
consciousness, seizures, and cerebral contusion, are less
common but account for several thousand deaths in children
annually.
 Ataxia may follow even mild head injuries.
 In most cases, ataxia is part of the so-called postconcussion
syndrome, in which imaging studies do not show any structural
derangement of the nervous system. In others, a cerebellar
contusion or posterior fossa hematoma may be present.

14.  In infants and small children, the most prominent postconcussive
symptom is ataxia.
 In older children headache and dizziness are as common as ataxia.
The headache description is usually low grade and constant.
 Gait is less disturbed, possibly because an older child compensates
better for dizziness, but the sensation of unsteadiness is still present.
 The clinical syndrome is the basis for diagnosis.
 (CT) at the time of injury to exclude intracranial hemorrhage is
normal.
 Ataxia usually clears completely within 1 month and always within 6
months.
 Decreased activity during the time of ataxia is the only treatment
needed.

15.  Hemorrhage into the cerebellum or posterior fossa as
the result of trauma or a vascular malformation can
cause ataxia with dramatic, rapid deterioration and life-
threatening elevation of intracranial pressure

16.  Acute ataxia may develop as the result of vertebral or
basilar artery disease.
 Conditions that cause cerebrovascular disease are rare
in children and include sickle cell disease,
hypercoagulable states, and homocystinuria

17.  Life-threatening infectious processes that cause acute
ataxia generally produce other symptoms as well.
 Rarely, ataxia may be an early symptom of meningitis
 Patients with inflammatory post-infectious conditions,
such as (ADEM), may also develop ataxia.

18. Ataxia may be the initial feature of viral encephalitis
affecting primarily the structures of the posterior fossa.
Potential etiological agents include
Echoviruses,
Coxsackieviruses,
Adenoviruses, and
Coxiella burnetti.

19.  Cranial nerve dysfunction often associated with the ataxia.
 Generalized encephalitis characterized by declining
consciousness and seizures may develop later.
 Meningismus is sometimes present. The course is variable,
and although most children recover completely, some have
considerable neurological impairment.
 Diagnosis requires showing a cellular response, primarily
mononuclear leukocytes, in the (CSF), with or without some
elevation of the protein content.
 No specific treatment is available for the viral infection.

20. 1) Acute cerebellar ataxia
 is a self limited syndrome that is frequently post infectious
 usually affects children between 2 and 7 years old, but it may
occur in 16-year-olds.
 The disorder affects both genders equally, and the incidence
among family members is not increased.
 following vaccination for varicella, hepatitis B and rabies,
without evidence of systemic infection .

21.  The onset is explosive. A previously healthy child
awakens from a nap and cannot stand.
 Ataxia is maximal at onset. Some worsening may
occur during the first hours
 Ataxia varies from mild unsteadiness while
walking to complete inability to stand or walk.
 The diagnosis can only be made after exclusion of
other more serious illnesses

22.  Even when ataxia is severe, sensorium is clear, and
the child is otherwise normal.
 Tendon reflexes may be present or absent; their
absence suggests Miller Fisher syndrome.
 Nystagmus, when present, is usually mild.
 Symptoms begin to remit after a few days, but
recovery of normal gait takes 3 weeks to 5 months.
 Treatment is not required.

23.  The Miller-Fisher syndrome is a form of GBS classically
characterized by the triad of ataxia, areflexia, and ophthalmoplegia
 result from a post-infectious immune-mediated process
 A viral illness precedes the neurological symptoms by 5 to 10 days in
50% of cases.
 Either ophthalmoparesis or ataxia may be the initial feature. Both are
present early in the course.
 Recovery generally begins within 2 to 4 weeks after symptoms
become maximal and is complete within 6 months.
 Corticosteroids, adrenocorticotropic hormone, and plasmapheresis
are not beneficial. The outcome in untreated children is excellent.

24.  Inflammation of the vestibular apparatus
 Symptoms include hearing loss, vomiting,
and intense vertigo exacerbated by head
movements
 Labyrinthitis may be difficult to distinguish
from acute cerebellar ataxia in a toddler

25.  30 percent of cases of acute childhood ataxia .
 The incidence of accidental drug ingestion is highest between
ages 1 and 4 years.
 Associated symptoms.
 anticonvulsants ,lead, carbon monoxide, inhalants, alcohol,
benzodiazepines, and other drugs of abuse.
 Toxic doses of anticonvulsant drugs, especially phenytoin,
may cause marked nystagmus and ataxia without an
equivalent alteration in sensorium.
 Excessive use of antihistamines in the treatment of an infant
with allergy or an URTI may cause ataxia. This is especially
true in children with otitis media, who may have underlying
unsteadiness because of middle ear infection.
 urine toxicology screens, specific blood tests.

26.  Diagnosis.
 Carefully question the parents or care providers of every child with acute
ataxia concerning drugs intentionally administered to the child and other
drugs accessible in the home.
 Specific inquiry concerning the use of anticonvulsant or psychoactive
drugs by family members is mandatory.
 Screen urine for drug metabolites, and send blood for analysis when a
specific drug is suspected.
 Management.
 Treatment depends on the specific drug ingested and its blood
concentration.
 In most cases of ataxia caused by drug ingestion, the drug can be safely
eliminated spontaneously if vital function is not compromised, if acid-
base balance is not disturbed, and if liver and kidney function is normal.
 In life-threatening situations, dialysis may be necessary while supporting
vital function in an intensive care unit.

27.  Acute ataxia is a clinical feature of several
migraine syndromes.
 Associated symptoms such as headache and
vomiting, episodic course.

28. Clinical Features :
Hysterical gait disturbances are relatively common in
children, especially girls between 10 and 15 years old.
Hysteria is involuntary and usually provides a secondary
gain.
In contrast, malingering is a voluntary act.
Hysterical gait disturbances are often extreme.
The child appears to sit without difficulty, but when
brought to standing immediately begins to sway from the
waist.
Strength, tone, sensation, and tendon reflexes are
normal.

29.  Diagnosis:
 The diagnosis of hysterical gait disturbances is by observation;
 laboratory tests are not ordinarily required to exclude other
possibilities.
 Management:
 Determination of the precipitating stress is important.
 Conversion may represent a true call for help in a desperate situation,
such as sexual abuse.
 Such cases require referral to a multispecialty team able to deal with
the whole family.
 Most children with hysterical gait disturbances are responding to an
immediate and less serious life stress.

30.  Autosomal recessive disorder localised to long arm of
chromosome 11
 characterised by progressive cerebellar ataxia starting
at 1-3yrs
 Telangiectasia over the conjunctiva and skin can be
observed
 child have IgA deficiency or impaired cell mediated
immunity
 increased predisposition to lymphoreticular
malignancies

31.  They have elevated alpha feto protein levels &
defects in DNA repair
 increased chromosomal breakage or cell death of
white blood cells after exposure to X-rays,
 absence of ATM protein in white blood cells, or
mutations in each of the person’s ATM genes
 Immunodeficiency with low levels of
immunoglobulins

32.  Friedreich's ataxia is the most common type of
hereditary ataxia (caused by genes you've
inherited).
 It's thought to affect at least 1 in every 50,000
people.
 Signs and symptoms of Friedreich's ataxia can
include: problems with balance and co-ordination,
often causing , clumsiness and frequent falls
 increasingly slurred, slow and unclear speech
(dysarthria)

33.  increasing weakness in the legs
 scoliosis
 total or partial vision loss and hearing loss
 thickening of the heart muscles (hypertrophic
cardiomyopathy), which can cause chest pain,
breathlessness and an irregular heartbeat
 dysphagia

34.  peripheral neuropathy
 The symptoms of Friedreich's ataxia usually get
gradually worse over many years. People with the
condition tend to have a shorter life expectancy
than normal.

35.  A child with acute cerebellar ataxia
 hyperkinetic spontaneous movement of eyes in
many directions( opsoclonus)
 myoclonic jerks of face and body should always
be investigated for occult malignancy especially
neuroblastoma

36.  It is due to disturbances in phytanic acid
metabolism
 C/F Ataxia, atypical retinitis pigmentosa with
night blindness, deafness, ichthyosis, and
conduction defects in the heart
 Protein level in CSF is high
 These patients should be treated by excluding
green vegetables(rich in phytanic acid) from the
diet

37.  Cerebellar ataxia may also occur due to
rudimentary development of cerebellar folia
 It is also associated with diplegia both spastic and
flaccid, congenital chorea, and mental defect
 These are non progressive and may appear to
improve with physiotherapy

38.  Ataxia is an important component of disorders of
demyelination
 visual involvement, pyramidal tract involvement,
waxing and waning course may be observed
 Lipidoses can also present with cerebellar
features

39.  Hypoglycemia
 Seizure disorder
 Inborn errors of metabolism
 Congenital anomalies
 Degenerative/genetic conditions

40.  History:
 refusal to walk or with a wide-based, "drunken" gait.
 Onset of symptoms
 Associated symptoms
 Otalgia, vertigo, and vomiting
 An older child with inner ear disease may complain of dizziness.
Most children also have nystagmus.
 Recurrent night-time or early-morning headaches with or without
vomiting
 Personality and behavioral changes.
 Abnormal mental status

41.  Access to medications.
 A history of head trauma ,neck trauma
 Patients with a recent infection or vaccination
 Previous similar episodes of acute ataxia.
 Children with family members with ataxia

42.  A systematic, flexible, approach to the physical
examination is necessary to localize the source of
the child's symptoms.
 careful observation.
 Abnormal vital signs must be recognized
immediately.
 Bulging of the anterior fontanel

43.  An ipsilateral head tilt
 fundoscopic examination
 Nystagmus
 Opsoclonus.
 Otitis media and hearing loss in association with
vomiting and intense vertigo
 Meningismus with fever and a toxic appearance.
 healing rash or viral exanthem

44.  The neurologic examination includes specific
examination techniques as well as observations made
while taking the history and throughout the general
physical examination
 Mental status, Lethargy.
 Cranial nerves .
 Motor examination :"paretic" ataxia.
 Sensory examination .
 Cerebellar examination .

45.  Gait: typically wide-based, unsteady, lurching, or
staggering.
 speech : fluctuations in clarity, rhythm, tone, and
volume.
 coordination of voluntary movements :over- or under-
shooting (best seen on finger-nose testing) and
difficulty with rapid alternating movements
(dysdiadochokinesia)
 Hypotonia and tremor may also occur.
 Patients may have difficulty maintaining truncal
position (titubation).

46.  Toxicologic screen - A urine screen for drugs of abuse or
blood for specific drug levels (as suggested by the
history) may be the most useful diagnostic test for
children with acute ataxia.
 Blood glucose.
 Metabolic evaluation - liver function tests, blood pH,
CBC, quantitative amino acid determinations of blood
and urine, serum lactate, pyruvate and ammonia levels,
and urine organic acids.

47.  Cerebrospinal fluid (CSF) examination - CSF should be
obtained whenever there is concern for CNS infection, such
as meningitis or encephalitis.
 Moderate CSF protein elevation can occur in acute cerebellar
ataxia, ADEM, and multiple sclerosis , GBS
 Neuroimaging should be obtained before a lumbar puncture
is performed when there is concern for increased intracranial
pressure.

49.  There's no treatment specifically for ataxia. In some
cases, treating the underlying cause resolves the ataxia,
such as stopping medications that cause it. In other
cases, such as ataxia that results from chickenpox or
other viral infection, it's likely to resolve on its own.
Adaptive devices
 Ataxia caused by conditions such as multiple sclerosis
or cerebral palsy might not be treatable. In that case,
your doctor may be able to recommend adaptive
devices. They include:
 Hiking sticks or walkers for walking
 Modified utensils for eating
 Communication aids for speaking

50. Therapies
 You might benefit from certain therapies,
including:
 Physical therapy to help your coordination and
enhance your mobility
 Occupational therapy to help you with daily living
tasks, such as feeding yourself
 Speech therapy to improve speech and aid
swallowing

51. THANKS