Paeds · neurology-neurodisability-and-neuromuscular
Ataxia in children
Also known as Acute cerebellar ataxia · Childhood ataxia · Post-infectious cerebellar ataxia · Friedreich ataxia · Opsoclonus-myoclonus syndrome · Sensory ataxia · Cerebellar ataxia in children
Fellowship guide to ataxia in children: separating the common, benign post-infectious acute cerebellar ataxia from the dangerous posterior fossa tumour and the progressive hereditary causes such as Friedreich ataxia, the gait and bedside examination that localises cerebellar from sensory ataxia, the red-flag screen that drives urgent neuroimaging, and the genetic and multidisciplinary management of chronic progressive ataxia including omaveloxolone for Friedreich ataxia.
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Red flags
- A child with acute ataxia and headache, vomiting, or a depressed conscious state has a posterior fossa tumour or cerebellitis with obstructive hydrocephalus until proven otherwise and needs urgent neuroimaging
- Opsoclonus, the chaotic multidirectional involuntary saccades of dancing eyes, with ataxia and extreme irritability is opsoclonus-myoclonus syndrome and warrants a search for occult neuroblastoma
- A progressive ataxia over weeks to months, especially with absent lower limb reflexes, an extensor plantar response, pes cavus, scoliosis, or cardiomyopathy, points to Friedreich ataxia or another hereditary degeneration
- Acute ataxia with encephalopathy, fever, or meningism demands exclusion of meningitis, encephalitis, or stroke rather than reassurance as a post-infectious cerebellar problem
- A head tilt, new squint, cranial nerve palsy, or papilloedema in a child with ataxia signals a posterior fossa or brainstem lesion and mandates magnetic resonance imaging
- Drug ingestion, particularly antiepileptics, benzodiazepines, or alcohol, is a leading cause of acute ataxia in a toddler and must be sought in the history and on toxicology screen
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- Recognises the common, benign post-infectious acute cerebellar ataxia and the red flags that demand urgent neuroimaging
- Distinguishes cerebellar gait ataxia from sensory ataxia and from weakness or vertigo at the bedside
- Identifies opsoclonus-myoclonus syndrome and initiates the search for neuroblastoma
- Diagnoses Friedreich ataxia on the constellation of early-onset ataxia, absent reflexes, extensor plantars, pes cavus, and cardiomyopathy, and confirms with GAA repeat testing of the FXN gene
- Applies the MOXIe trial evidence for omaveloxolone in Friedreich ataxia and coordinates multidisciplinary care
- Coordinates the workup of chronic progressive ataxia including magnetic resonance imaging and a targeted genetic and metabolic panel
- Acute versus chronic causes of ataxia in children and the red-flag screen for a posterior fossa tumour
- The clinical features of Friedreich ataxia, ataxia telangiectasia, and opsoclonus-myoclonus syndrome
- The bedside examination that localises the lesion: cerebellar versus sensory versus vestibular
- Examines the gait, performs the finger-to-nose and heel-to-shin tests, and checks for dysmetria, dysdiadochokinesia, nystagmus, and Romberg sign
- Communicates the reassuring prognosis of post-infectious acute cerebellar ataxia to an anxious family
- Frames the urgent imaging decision for a child with ataxia and a red flag
- Level 1: Recognition of acute and chronic ataxia and the red flags for a posterior fossa tumour
- Level 2: Bedside localisation and the investigation pathway for progressive ataxia
- Level 3: Multidisciplinary management of Friedreich ataxia and the hereditary ataxias, including genetic counselling
- Post-infectious acute cerebellar ataxia as the commonest cause of acute ataxia in a preschool child and its benign course
- The clinical and genetic features of Friedreich ataxia, ataxia telangiectasia, and opsoclonus-myoclonus syndrome
- Red flags for a posterior fossa tumour and the indications for urgent neuroimaging in a child with ataxia
- Examination of the ataxic gait and the cerebellar and sensory signs at the bedside
- Recognition of opsoclonus and its distinction from nystagmus
- Counselling on the benign prognosis of acute cerebellar ataxia and the implications of a hereditary diagnosis
- Recognition of acute cerebellar ataxia and exclusion of ingestion, tumour, and central nervous system infection
- Clinical features of Friedreich ataxia and the GAA repeat expansion in the FXN gene
- Opsoclonus-myoclonus syndrome and the association with neuroblastoma
- Structured assessment of the child with acute ataxia, separating benign from dangerous causes
- Bedside localisation of the lesion through gait and coordination examination
- Coordination of genetic and multidisciplinary care for chronic progressive ataxia
- Canadian approach to acute ataxia in children and the restrained, selective use of neuroimaging
- Diagnosis and multidisciplinary management of Friedreich ataxia including omaveloxolone
- Recognition of ataxia telangiectasia and the hereditary ataxias in a diverse population
Overview & Definition
Picture the four-year-old who is brought to the emergency department walking as though drunk, feet wide apart and reeling, two weeks after a bout of chickenpox, and whose parents are terrified. That child carries the whole teaching point of paediatric ataxia: the unsteady gait is alarming to the family but is usually benign, the diagnosis is clinical, and the clinician's job is to confirm the safe, common pattern and to exclude the small minority that are dangerous. The decisive first question is not which drug to give but whether this is acute or chronic, and whether red flags are present, because that single distinction separates reassurance and observation from urgent imaging and oncology. [1] [2]
Ataxia is impaired coordination of voluntary movement that cannot be explained by weakness, sensory loss, or extrapyramidal rigidity alone. The lesion usually lies in the cerebellum or its connections, producing a wide-based, unsteady gait, dysmetria (past-pointing), intention tremor, dysdiadochokinesia (impaired rapid alternating movement), slurred speech, and nystagmus. A second, distinct mechanism is sensory ataxia, in which loss of proprioception from the dorsal columns deprives the brain of position sense, so the child walks unsteadily and falls in the dark and stamps the feet, with a positive Romberg sign. The clinician's first bedside task is to localise the ataxia to the cerebellum, to the sensory pathway, or to a vestibular cause, because the differential and the workup diverge from there. [1]
The framework that organises the whole topic is the acute-versus-chronic split combined with the red-flag screen. Acute ataxia, evolving over hours to days, is dominated by post-infectious acute cerebellar ataxia, drug ingestion, and the dangerous causes, posterior fossa tumour, cerebellitis, stroke, and central nervous system infection. Chronic or progressive ataxia, evolving over weeks to months, points to the hereditary degenerations, of which Friedreich ataxia is the prototype, and to the metabolic and congenital disorders. The red-flag screen, for headache, vomiting, encephalopathy, focal signs, and a progressive course, is the bridge between the bedside pattern and the imaging decision. [1] [3]
References12ShowHide
- [1]Desai J, Mitchell WG Acute cerebellar ataxia, acute cerebellitis, and opsoclonus-myoclonus syndrome. J Child Neurol, 2012.PMID 22805251
- [2]Connolly AM, Dodson WE, Prensky AL, Rust RS Course and outcome of acute cerebellar ataxia. Ann Neurol, 1994.PMID 8210223
- [3]Wilne S, Collier J, Kennedy C, Koller K, Grundy R, Walker D Presentation of childhood CNS tumours: a systematic review and meta-analysis. Lancet Oncol, 2007.PMID 17644483
- [4]Brandão LA, Young Poussaint T Posterior Fossa Tumors. Neuroimaging Clin N Am, 2017.PMID 27889018
- [5]Cook A, Giunti P Friedreich's ataxia: clinical features, pathogenesis and management. Br Med Bull, 2017.PMID 29053830
- [6]Rummey C, Corben LA, Delatycki M, Wilmot G, Mathews K, Subramony SH, et al Natural History of Friedreich Ataxia: Heterogeneity of Neurologic Progression and Consequences for Clinical Trial Design. Neurology, 2022.PMID 35817567
- [7]Lynch DR, Chin MP, Delatycki MB, Subramony SH, Corti M, Hoyle JC, et al Safety and Efficacy of Omaveloxolone in Friedreich Ataxia (MOXIe Study). Ann Neurol, 2021.PMID 33068037
- [8]Rossor T, Yeh EA, Khakoo Y, et al. Diagnosis and Management of Opsoclonus-Myoclonus-Ataxia Syndrome in Children: An International Perspective. Neurol Neuroimmunol Neuroinflamm, 2022.PMID 35260471
- [9]Rothblum-Oviatt C, Wright J, Lefton-Greif MA, McGrath-Morrow SA, Crawford TO, Lederman HM Ataxia telangiectasia: a review. Orphanet J Rare Dis, 2016.PMID 27884168
- [10]Corben LA, Collins V, Milne S, et al. Clinical management guidelines for Friedreich ataxia: best practice in rare diseases. Orphanet J Rare Dis, 2022.PMID 36371255
- [11]Reetz K, Lischewski SA, Dogan I, et al. Friedreich's ataxia-a rare multisystem disease. Lancet Neurol, 2025.PMID 40541211
- [12]Lynch DR, Chin MP, Boesch S, Delatycki MB, Giunti P, Goldsberry A, et al Efficacy of Omaveloxolone in Friedreich's Ataxia: Delayed-Start Analysis of the MOXIe Extension. Mov Disord, 2023.PMID 36444905