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:
- 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%)
- 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.]
- Commonly associated infections:
- 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]
- “Basic Labs” will be normal.
- Glucose is always worth checking!
- Electrolytes and urine catecholamines may be useful if concern for opsoclonus-myoclonus.
- 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.
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.