Approach to the child with acute ataxia

1. Approach to the
child with acute
ataxia
Dr Mohammad AL Ktifan
AWH- Pediatric Emergency

2. The Clinical Case
• Two worried parents present to PEC with their 4-years old boy, Stephen. Until today, Stephen had been doing very well and was
otherwise healthy.
• Yesterday, Stephen’s parents brought him to a local playground to play with some friends.
• This morning, after Stephen got out of bed, his parents noticed that he was having trouble walking; he was much slower than
normal and seemed to be unsteady on his feet.
• Otherwise, he has been interacting with them normally and hasn’t complained of any pain
Physical examination: Stephen is afebrile with all vital signs within normal, he is alert with a GCS of 15.
Neurological examination :he has normal strength and reflexes, but his muscle tone seems a bit low and he has some mild
dysmetria on cerebellar testing , some mild nystagmus
- he does not endorse any visual troubles, and he sways a little bit while trying to sit still for visual testing. After doing the visual
testing, he says he has a headache, but no meningitis signs.
Examination of his cranial nerves is unremarkable, and no other focal neurological deficits.
When he try to walk, he has a very wide based gait and stays close to the wall, reaching at it for support .there is no change to his
balance when closed his eyes .

3. BACKGROUND
• Ataxia is the inability to make smooth, accurate coordination of movements.
• It is most commonly manifested as an unsteady gait, and in children, refusal to walk .
• Usually because of a dysfunction of
• 1- the cerebellum , its inputs or outputs.
• 2- sensory pathways in posterior columns of the spinal cord.
• 3- or a combination of these.
• Acute ataxia, defined as presence of ataxia for <7 days,
• is an uncommon and accounted for 0.02 percent of all emergency department visits by children 1 to 18 years of
age

4. Types of Ataxia by Affected Area
• Ataxia is caused by damage to different areas of the central nervous system.
including:
• Cerebellar (brain)
• Sensory (nerves)
• Vestibular (ears)

5. Cerebellar Ataxia
• Symptoms of cerebellar ataxia include:
• . Changes in voice
• Muscle tremors
• Slurred speech
• Trouble walking
• Wide gait
• Dizziness
• Fatigue
• Headaches

6. Sensory Ataxia
• Sensory ataxia is the result of damage to nerves in spinal cord or peripheral nervous system.
• Symptoms of sensory ataxia include:
• Difficulty touching the finger to the nose with closed eyes
• Inability to sense vibrations
• Trouble walking in dim light
• Walking with a “heavy step,” or stomping when walking.

7. Vestibular Ataxia
• Vestibular ataxia affects vestibular system. This system is made up of inner ear and ear canals, which
contain fluid. They sense the movements of head and help with the balance and spatial orientation.
• When the nerves in vestibular system are affected, the child can have the following problems:
• Blurred vision and other eye issues
• Nausea and vomiting
• Problems standing and sitting
• Staggering when walking
• Trouble walking in a straight line
• Vertigo, or dizziness

8. Causes of acute ataxia
• causes of acute ataxia include life-threatening conditions such as mass lesions and central nervous system
(CNS) infection
• the majority of children have a benign, self-limited process.
• 80 % of children with acute ataxia are :
• ●Acute cerebellar ataxia( is most common)
• ●Drug intoxication
• ●Guillain-Barré syndrome (GBS)

9. Life-threatening conditions
• 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
Tumors :
- Posterior fossa tumors may present with slowly progressive ataxia and symptoms of increased
intracranial pressure.
- Acute ataxia in association with opsoclonus-myoclonus (rapid, dancing eye movements and
rhythmic jerking) may be the presenting manifestation of an occult neuroblastoma.
Intracranial hemorrhage
- Hemorrhage into the cerebellum or posterior fossa as the result of trauma (severe ) or a
vascular malformation can cause ataxia with dramatic, rapid deterioration and life-threatening
elevation of intracranial pressure

10. Life-threatening conditions
• Stroke :
• is rare in children and include sickle cell disease, hypercoagulable states (as can occur with systemic lupus
erythematous), and homocystinuria.
• Infection :
• rarely, ataxia may be an early symptom of meningitis
• Cerebellar abscesses, often caused by contiguous spread of infection from either otitis media or mastoiditis
• Brainstem encephalitis is a rare cause of acute ataxia that is associated with high morbidity, infectious agents include Listeria
monocytogenes, Lyme disease, EBV, and herpes virus
•
Acute cerebellitis, although often considered on the spectrum of postinfectious acute cerebellar ataxia, is a rare condition that has
distinctive clinical and imaging features. Children with acute cerebellitis typically are ill with acute ataxia accompanied by vomiting,
headache, altered consciousness, and increasing sedation. Some may present with severe cerebellar edema. (MRI) will demonstrate
cerebellar inflammation
• acute demyelinating encephalomyelitis (ADEM). ADEM typically occurs 1-4 weeks after a febrile illness and is characterized by
encephalopathy associated with wide range of possible neurological deficits from ataxia to optic neuritis to hemiplegia.

11. Common conditions
• Common conditions that cause acute ataxia in children are often treatable and/or self-limited.
• Acute cerebellar ataxia :
• is the most common cause of acute ataxia, accounting for about 30 - 75 percent of all cases
• It is a self-limited syndrome that is frequently postinfectious and typically seen in children between 2-5 years of
age
• Varicella is one of the primary infectious agents that affects the cerebellum. In this case, ataxia begins 2–6 days
after the onset of rash, but it may also occur during the incubation period or after resolution of the rash , it has
also been known to occur following receival of live inactivated varicella vaccines
• other infectious agents :including coxsackievirus, echovirus, enteroviruses, EBV , hepatitis A, herpes simplex
virus 1, measles, mumps, (Lyme disease), malaria, Mycoplasma pneumoniae, and typhoid fever.

12. Acute
cerebellar
ataxia
The onset is explosive. A previously healthy child awakens from a
nap and cannot stand.
ataxia reaches its worst within the first hours after onset of the
condition.
It varies from mild unsteadiness while walking to complete
inability to stand or walk.
Dysmetria will be mild; there may also be nystagmus, but also
quite mild if present
The diagnosis can only be made after exclusion of other more
serious illnesses

13. Acute
cerebellar
ataxia
• 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.
• Symptoms begin to remit after a few days, but
recovery of normal gait takes 3 weeks to 5
months.
• Treatment is not required.

14. Toxic exposure
• is responsible for up to 30 percent of cases of acute childhood ataxia.
• include anticonvulsants (phenytoin, carbamazepine, phenobarbital, and Lamotrigine), lead, carbon monoxide,
alcohol, benzodiazepines.
• 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

15. Guillain-Barré syndrome
• It affects motor nerves, although as many as 15 percent of children with GBS also lose sensory input to the
cerebellum and develop sensory ataxia.
• The Miller Fisher syndrome is a form of GBS classically characterized by the triad of ataxia, areflexia, and
ophthalmoplegia.
• Ataxia associated with GBS usually progresses over several days. The prognosis for children with GBS is
generally better than that for adults. As many as 85 percent of children can be expected to have an excellent
recovery.

16. Other conditions
• Migraine syndromes
• Labyrinthitis — Inflammation of the vestibular apparatus, often from bacterial or viral infections
such as otitis media
• Degenerative/genetic conditions(associated with chronic and/or progressive ataxia include ataxia-
telangiectasia, spinocerebellar ataxia)
• Trauma
• Hypoglycemia
• Seizure disorder
• ●Opsoclonus myoclonus syndrome
• Inborn errors of metabolism
• Tick paralysis
• Congenital anomalies(Dandy-Walker syndrome, Chiari malformation)

17. Gluten ataxia
• is a rare immune-mediated disease in which the body's immune system attacks the nervous system as a
reaction to ingestion of gluten. It can be related to celiac disease as well as non-celiac gluten sensitivity
• Children present with gait ataxia, nystagmus, peripheral neuropathy and brain involvement upon MRI as a
consequence of a gluten trigger.
• A gluten-free diet has been reported to improve the clinical signs in the affected patients
• the presence of cerebellar gluten-ataxia was documented in two large studies and reported in 2.7 and 5.4% of
children patients.

18. History
• Onset of symptoms
• A history of head trauma ,neck trauma
• Patients with a recent infection or vaccination
• Access to medications.
• Associated symptoms :Otalgia, vertigo, and vomiting suggest acute labyrinthitis
• Recurrent night-time or early-morning headaches with or without vomiting
• Personality and behavioral changes.
• Abnormal mental status
• Previous similar episodes of acute ataxia.
• Children with family members with ataxia

19. Physical examination
• A systematic, flexible, approach to the physical examination is necessary to localize the source of
the child's symptoms.
• Red flags on physical examination include:
• • Abnormal vital signs ( as Elevated BP and/or tachycardia can also occur with peripheral processes such as
GBS).
• Any altered level of consciousness or encephalopathy
• Any new focal neurological deficits, such as weakness, sensory changes, brisk reflexes or absent reflexes
• Visual loss
• Papilledema
• Head tilt can be associated with posterior fossa tumors or CN deficits (VI)
• Abnormal eye movements such as nystagmus or opsoclonus
• Rash
• Meningismus: neck stiffness, headache, photophobia

20. Neurologic examination
• The neurologic examination includes specific examination techniques as well as observations made
while taking the history and throughout the general physical examination
• Mental status: abnormal mental status suggests ingestion , encephalitis , meningitis , ADEM, or stroke .
. Lethargy: may be present in many inborn error of metabolism.
• Cranial nerves .:abnormalities of cranial nerve function suggest post fossa lesion, brainstem encephalitis,
or the Miller Fisher syndrome
• Motor examination :Children with the acute onset of weakness may stagger as an attempt to
compensate. This gait abnormality may be mistaken for true ataxia and has been called "paretic" ataxia.
Paretic ataxia is proportional to the degree of weakness. It may be due to GBS, botulism,, or tick
paralysis
• Sensory and cerebellar examination .

21. Cerebellar signs
• Cerebellar signs include abnormalities in gait, speech, and coordination of voluntary movement.
• Gait is typically wide-based, unsteady, lurching, or staggering.
• Speech abnormalities include fluctuations in clarity, rhythm, tone, and volume.
• Patients may have difficulty maintaining truncal position (titubation).
• Coordination of voluntary movement, as demonstrated with over- or under-shooting (best seen on finger-nose
testing)
• difficulty with rapid alternating movements is called (dysdiadochokinesia).
• Hypotonia and tremor may also occur

22. Laboratory
• Blood gas (electrolytes, 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.
• 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.
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

23. Neuroimaging
Should be obtained before a lumbar puncture is performed when there is concern for increased
intracranial pressure.
(MRI) is the imaging modality of choice for patients with acute ataxia, although emergency
MRI may be difficult to obtain.
It is superior to computed tomography (CT) for detection of posterior fossa lesions such as
tumors, strokes, and abscesses.

24. Continuing the Clinical Case
• His parents state that Stephen tripped and fell once at the playground, but he got up quickly and seemed to be
okay.
• They also explain that Stephen was sick about a week ago, with a runny nose and red spots on his palms and
around his mouth , his parents gave him some paracetamol .
• He is fully immunized, and doesn’t take any medications regularly.
• Regarding medications, Stephen’s mother states that she does have some benzodiazepines she uses for
anxiety attacks, but she keeps them locked away safely and out-of-reach.
• As mentioned before, his physical examination was remarkable for mild dysmetria and nystagmus, slightly low
muscle tone, and a wide-based gait.
• Bloodwork ordered CBC, electrolytes, and glucose all come back normal.
• to rule out any life-threatening causes of ataxia, such as an intracranial bleed, so he was sent for an CT(normal)
• LP, was not needed as Stephen looked well, was afebrile and only had a mild headache, with no meningitic
signs.
• acute cerebellar ataxia.

25. Conclusion
• Time-course/progression of symptoms helps narrow a differential .
• Acute ataxia in a toxic ingestion until proven otherwise.
• Always ask about drugs that are at home.
• Concussion and mild head trauma, as well as migraine variants are often overlooked as a cause of
ataxia in children.
• The presence of encephalopathy or new focal neurological deficits is always concerning and
should prompt further investigations
• Brain tumors can present acutely – progressive symptoms always warrant investigations