Acute Ataxia – From Pediatric EM Morsels And PedsCases

This post contains links to and excerpts from:

  • Acute Cerebellar Ataxia
    BY SEAN M. FOX · PUBLISHED DECEMBER 22, 2017 · UPDATED JANUARY 17, 2018 from Pediatric EM Morsels
  • Approach to Acute Ataxia                                                                                  By  Drs. Aran Yukseloglu and Francois Jacobs. October 5, 2019 from PedsCases  

We’ll start with excerpts from Dr. Fox, Acute Cerebellar Ataxia:

Basics

  • Acute cerebellar ataxia is a common pediatric neurologic problem.
    • Incidence of 1 in 100,000 – 500,000.
  • Some causes of ataxia in children: [Thakkar, 2016]
    • Post-infectious Cerebellar Ataxia – (~30 – 60%)
    • Drug Intoxication (~8%)
      • ex, Alcohol, Benzos, Heavy Metals, CO poisoning, Anticonvulsants
    • Opsoclonus Myoclonus Ataxia (~8%)
      • Rare, but a true medical emergency!
      • May be misdiagnosed as benign post-infectious cause at first.
      • Has severe ataxia, opsoclonus (chaotic ocular movements), and myoclonus.
      • Is a Paraneoplastic disorder (often neuroblastoma)! [Tate, 2005]     
    • Acute Cerebellitis (~2%)
      • Most severe end of the spectrum of cerebellar inflammation/infection. [Rossi, 2016]
      • Previously, “Acute Cerebellitis” was used interchangeably with Post-infectious, But:
        • Acute Cerebellitis has a distinctly worse disease course.
        • Has abnormalities on brain MRI.
        • Can lead to rapid posterior fossa edema and lead to morbidity and mortality.
    • Cerebellar Stroke (~2%)
    • Acute Disseminated Encephalomyelitis (ADEM) (~2%)
      • Immunologically mediated inflammatory disease
      • Polyfocal neurological signs (multiple sites involved in CNS)
      • Rapid onset of encephalopathy (altered mental status)
    • Meningitis (<1%)
    • Cerebral Venous Thrombosis (<1%)
    • Miller Fisher Syndrome (<1%)
    • Hereditary conditions (ex, Ataxia-telangiectasia)

Acute Cerebellar Ataxia: Post-infectious

The most common cause of acute cerebellar ataxia in children is post-infectious cerebellar ataxia. [Thakkar, 2016; Rossi, 2016]

  • Generally seen in kids younger than 6 years.
  • Most common among 2 – 4 year olds.
  • Often, symptoms begin suddenly.
  • NOT associated with fever, seizures, change in mental status, or other systemic signs. [Doan, 2016]
  • Is a diagnosis of exclusion, because other ominous conditions can present similarly. [In other words, you can make this diagnosis ONLY AFTER a thorough clinical and neurologic exam, labortory studies, and imaging studies.] 
  • Work-up is generally negative!
    • Cerebrospinal fluid analysis has low diagnostic yield. [Thakkar, 2016]
      • Certainly CSF analysis is helpful if you are more concerned for meningitis or encephalitis.
      • LP, if performed, should wait until after imaging to rule-out posterior fossa mass or edema. [Doan, 2016]
    • Imaging is typically normal. [Thakkar, 2016; Doan, 2016]
      • MRI is preferred given higher resolution and superior imaging of posterior fossa. [Rossi, 2016]
      • CT should be obtained for patients with altered mental statusatypical disease courseasymmetric focal neurologic deficits, or when hemorrhage or mass is higher on the Ddx list.
    • “Basic Labs” will be normal.
    • Urine Tox screens should be considered, particularly in the toddlers who like to eat random items in the house. [Doan, 2016]
  • Patient recover without lasting sequelae. [Thakkar, 2016]
    • Usually has resolution of symptoms in 2-8 weeks.
    • Complete resolution by 2-3 months.

Next I reviewed the PedsCases’ podcast on Acute Ataxia. The podcast and transcript is outstanding and worth reviewing completely. But, since these posts are my study notes I’ve made excerpts for quick review. 

First, here is the complete list of the Table Of Contents of the PedsCases website. On this page the site has organized all of the contents into two groups: Clinical Presentation and Specialty Area.

This post contains links to Approach To Acute Ataxia [Link To The Podcast] [Link To The Transcript] from PedsCases. Published: Oct 05, 2019:

This podcast will cover an approach to acute ataxia. It was developed by Dr. Aran Yukseloglu, a pediatric resident at the University of Alberta, in collaboration with Dr. Francois Jacob, a pediatric neurologist at the Stollery Children’s Hospital in Edmonton, AB

Here are excerpts: 

Life-Threatening Causes Of Acute Ataxia:

In some cases, acute ataxia may in fact be an early presentation of a life-threatening illness. It is critical to rule-out these serious causes before considering other less dangerous possibilities.

One of a family’s initial fears is “Does my child have a brain tumor?” since in children, 45-60% of brain tumor occur in the posterior fossa and they may present with ataxia. One would expect, depending on the grade of the tumor, that the ataxia would have been progressively getting worse. However, there may be a more abrupt onset of ataxia such as; obstructive hydrocephalus, hemorrhage within the tumor, or edema that begins to impact nearby structures.

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