Important to establish the timeline and evolution symptoms. Hyperacute onset could indicate a life-threatening vascular event.

Red flags: ^(1,6)

• Altered mental status
• Papilledema
• Nuchal rigidity
• Headache
• Opsoclonus (“dancing eyes”) myoclonus (“dancing legs”) syndrome
• Seizures
• Severe irritability
• Focal neurological deficits (motor, sensation, or reflexes)
• History or signs of trauma

• Definition and DDx ^(1,2,5)
  • Trouble performing smooth, coordinated movements.
  • Wide-based “drunken” gait; unsteady upper limb movements; or, often, refusal to walk.
  • Cerebellar or motor ataxia → cerebellar deficits.
  • Sensory ataxia → proprioceptive deficits (i.e., extra-cerebellar deficits).
  • Most cases are self-limited but significant proportion (up to one third) can be
    life-threatening. ⁽⁵⁾
• Common causes
  • Acute cerebellar ataxia (most common).
  • Toxic ingestions, medication induced.
  • Guillain-Barré syndrome (GBS).
  • Migraine or vertigo related.
• Can’t miss causes
  • Tumors.
  • Intracranial bleed.
  • Vascular (stroke, vertebral artery dissection.)
  • CNS infections.
  • Autoimmune processes (e.g., acute demyelinating encephalomyelities, ADEM).
  • Trauma.
• Other causes
  • Metabolic.
  • Congenital.

• History ^(1,2,5,7,8)
  • What’s the symptom timeline and evolution of symptoms (acute <7d vs. subacute vs.
    chronic/episodic?)
  • Has this happened before?
  • Possibility of toxic ingestion?
  • Recent infections (e.g., varicella, adenovirus, herpes)?
  • Change in mental status? 🚩
  • Recent irritability? 🚩
  • History of seizures? 🚩
  • History of trauma? 🚩
  • Has this occurred in siblings, parents?
  • Associated symptoms
    • Any pain or headache?
    • Any changes in vision, hearing, gait, coordination, balance?
  • Any symptoms of elevated ICP symptoms (headache, n/v, limb weakness or sensory
    deficits). 🚩
  • Complete medication, OTC, supplement history.
  • Past and current medical history.
• Physical exam ^(1,2,4,5,6)
  • Ensure physical exam is age-appropriate. Pay special attention to coordinated movements.
  • General Exam Findings and Potential Cause ⁽¹⁾
    • Fever →  infection
    • Abnormal respirations or pulse 🚩 →  increased ICP
    • Head tilt 🚩→  posterior fossa tumor
    • Bulging anterior fontanelle 🚩→  increased ICP
    • Opsoclonus and/or myoclonus 🚩→ opsoclonus-myoclonus-ataxia syndrome
      (neuroblastoma?)
    • Otitis media with vomiting, vertigo 🚩→  labyrinthitis
    • Meningismus (headache, nuchal rigidity, photophobia) 🚩
    • Tick bites
    • Rash from prior infection → acute cerebellar ataxia
• Neurological Exam (^*essential)
  • Gait^* – Wide-based? Unsteady? Refusal to walk
  • Speech changes
  • Eye movements – Nystagmus?
  • Coordination^*
    • Finger nose test
    • Rapid alternating hand movements
    • Heel tapping
    • Heel/shin trace
    • Hold full glass of water without spilling
  • Proprioceptive testing, e.g., Romberg test (non-cerebellar ataxia)
  • Mental status
    • Normal MSE → post infectious acute cerebellar ataxia
    • Abnormal MSE 🚩→  ingestion, autoimmune (e.g., ADEM), stroke
• Labs ^(1,2,4,5,7)

• Imaging ^(1-3,5,7)
  • Not all children with acute ataxia need imaging in ED. Many etiologies are imaging
    negative (e.g., acute cerebellar ataxia).
  • MR ideal (and better for posterior fossa tumors) but CT allowable to rule out surgical
    emergencies.
  • Refer to pediatric neurology for guidance.

Management ^(1,2)

• Treatment depends on etiology.
• Symptomatic and supportive care for most patients.
• Refer for treatment of non-acute causes.

• Admit with patients with any red flag symptoms 🚩:
  • Altered mental status
  • Papilledema
  • Nuchal rigidity
  • Headache
  • Opsoclonus (“dancing eyes”) myoclonus (“dancing legs”) syndrome
  • Seizures
  • Severe irritability
  • Focal neurological deficits (motor, sensation, or reflexes)
  • History or Signs of trauma
• Admit and order EEG for any patient with seizures, altered mental status, or
  fluctuating symptoms. ⁽⁷⁾
• Admit and consult infectious disease and/or neurology on persistent. ⁽⁷⁾

Base transport decisions on resources available at current center, acuity of presenting illness, and need for intensive interventions (e.g., pediatric neurosurgery).

• Outpatients with acute ataxia should be monitored by primary care. Any new focal neurological changes or development of red flag symptoms (see above) may warrant neuroimaging. ^(1,7)
• Episodic or chronically progressing ataxia warrant referral to pediatric neurology. Conditions of this nature include ⁽¹⁾
  • Episodic
    • Migraine
    • Seizure
    • Benign paroxysmal vertigo
    • Inborn errors of metabolism (e.g., amino acid, organic acids, mitochondrial irregularities)
  • Chronic
    • Degenerative disorders
    • Inborn errors of metabolism
    • Genetic disorders (spinocerebellar ataxias, SCA)
    • Multiple Sclerosis

• Considerations for discharge
  • Child is stable, not deteriorating.
  • Ruled out dangerous etiology with history, physical, labs, neuroimaging.
  • Diagnosis identified and/or patient has minimal risk of acutely worsening.
• For post-infectious acute cerebellar ataxia (most common cause) most children completely
  recover in 2-4 w. ⁽⁷⁾

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2. Gilbert D. Acute cerebellar ataxia in children [Internet]. Patterson M, Teach S, editors. UpToDate; 2022 [cited 2024 Jan 5]. Available from:

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4. Rare Disease Video – Opsoclonus Myoclonus Syndrome – National Organization for Rare Disorders [Internet]. rarediseases.org. [cited 2024 Jan 8]. Available from:

   https://rarediseases.org/videos/opsoclonus-myoclonus-syndrome/

   
   

5. Marx J, Hockberger R, Walls R. Rosen’s Emergency Medicine – Concepts and Clinical Practice. London: Elsevier Health Sciences; 2013.

   
   

6. Garone G, Reale A, Vanacore N, Parisi P, Bondone C, Suppiej A, Brisca G, Calistri L, Cordelli DM, Savasta S, Grosso S. Acute ataxia in paediatric emergency departments: a multicentre Italian study. Archives of Disease in Childhood. 2019 Aug 1;104(8):768-74.

   
   

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8. Shakkottai V. Assessment of Ataxia [Internet]. Klockgether T, Perlman S, editors. BMJ Best Practice. BMJ; 2022 [cited 2024 Jan 4]. Available from:

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