Acute Ataxia in Children: Approach to Clinical Presentation and Role of Additional Investigations

Poretti, Andrea; Benson, Jane E.; Huisman, Thierry A. G. M.; Boltshauser, Eugen

doi:10.1055/s-0032-1329909

Cited by 16 publications

(8 citation statements)

References 129 publications

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“…1 , 6–10 Incoordination may result from nonconvulsive status epilepticus, which is often associated with decreased level of awareness, confusion, myoclonic jerks, and interrupted smooth movements of the limbs, leading to pseudoataxia. 6 , 10 In addition, motor deficits, confusion, sleepiness, and lethargy occurring in the postictal phase of an epileptic seizure may also be mistaken for ataxia, especially if the seizure was not witnessed. 8 …”

Section: Discussionmentioning

confidence: 99%

“… 8 , 13 Rarely, acute ischemic stroke, particularly involving the posterior cerebral circulation, may result in acute ataxia. 10 The ingestion of toxic substances is also a common cause of ataxia, as outlined above. 6 , 7 Traumatic injury may also cause ataxia.…”

Section: Differential Diagnosis Of Poor Coordinationmentioning

confidence: 99%

“…Gait disturbance is a common feature of psychogenic or functional movement disorder. 10 With this disorder, the gait disturbance can be differentiated from true ataxia by two main observations. First, the patient will lack any adaptation, such as a wide-based gait, to improve balance.…”

Section: Differential Diagnosis Of Poor Coordinationmentioning

confidence: 99%

“…In addition, the tremor associated with psychogenic disorder will worsen during clinical examination and improve when the patient is distracted. 10 …”

Section: Differential Diagnosis Of Poor Coordinationmentioning

confidence: 99%

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Assessing Children with Poor Coordination Can Be Tricky – A Review on Ataxia and Ataxia Mimickers and a Study of Three Children with Severe Epilepsy

Martin¹,

Salman²

2021

JMDH

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While ataxia is a relatively common presenting feature in pediatric patients, it represents only one possible cause of uncoordinated movements. Other possible causes of uncoordinated movements include ingestion of toxic substances, musculoskeletal diseases, psychogenic disorders, extrapyramidal movement disorders, peripheral neuropathies, spasticity from any cause, and epilepsy. Therefore, primary health care providers must recognize and exclude other etiologies of uncoordinated movements before attaching the label “ataxia” to any patient presenting with poor coordination. Once the presence of ataxia is confirmed, the cause should be investigated. As ataxia may be vestibular, sensory, or cerebellar in origin, medical practitioners must evaluate the diverse symptoms and signs to effectively differentiate the various types of ataxia. Three case studies are presented to illustrate the complexity associated with the assessment of ataxia. Each case will discuss a pediatric patient who displays cerebellar ataxia as a concurrent feature of a gene-specific developmental and epileptic encephalopathy. These cases will provide an example of how ataxia may be differentiated from other causes of uncoordinated movements related to epilepsy and anti-seizure medications, namely: nonconvulsive seizures, postictal state, and medication side effects or toxicity. The assessment of poor balance can be challenging at times; however, with knowledge of the differential diagnosis of poor balance, medical practitioners will be able to confidently determine the presence of true ataxia from various ataxia mimickers, thereby allowing for timely and accurate diagnosis, and appropriate management.

show abstract

Section: Discussionmentioning

confidence: 99%

Section: Differential Diagnosis Of Poor Coordinationmentioning

confidence: 99%

Section: Differential Diagnosis Of Poor Coordinationmentioning

confidence: 99%

“…In addition, the tremor associated with psychogenic disorder will worsen during clinical examination and improve when the patient is distracted. 10 …”

Section: Differential Diagnosis Of Poor Coordinationmentioning

confidence: 99%

See 2 more Smart Citations

Assessing Children with Poor Coordination Can Be Tricky – A Review on Ataxia and Ataxia Mimickers and a Study of Three Children with Severe Epilepsy

Martin¹,

Salman²

2021

JMDH

View full text Add to dashboard Cite

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“…The most severe manifestation of this pathological spectrum is represented by cerebellitis, characterized by acute neurological and systemic symptoms associated with neuroimaging cerebellar abnormalities [ 9 , 10 ] and meningeal involvement [ 11 ]. The differential diagnosis of acute forms of cerebellar ataxia in children is quite wide; a complete medical history and physical examination can easily suggest a correct diagnosis, although some symptoms, such as major pain, should suggest further tests.…”

Section: Discussionmentioning

confidence: 99%

Acute post-infectious cerebellar ataxia due to co-infection of human herpesvirus-6 and adenovirus mimicking myositis

et al. 2014

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Acute cerebellar ataxia (ACA) is a relatively common neurological disease in children. Most common types of ACA are acute post-infectious (APCA) and acute disseminated encephalomyelitis (ADEM). Less common but important causes include opsoclonus-myoclonus syndrome (OMS) and acute cerebellitis. Cerebellar neoplasms and acute hydrocephalus are additional causes of paediatric ataxia. APCA is the most common cause of ACA in children, comprising about 30-50% of total cases. This is a report about an immunocompetent 4-yrs-old male affected by APCA, due to co-infection by human herpesvirus-6 (HHV-6) and adenovirus, with symptoms mimicking myositis.

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Myoclonus‐Ataxia Syndromes: A Diagnostic Approach

Rossi

Veen

Merello

et al. 2020

Movement Disord Clin Pract

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Background Background: A myriad of disorders combine myoclonus and ataxia. Most causes are genetic and an increasing number of genes are being associated with myoclonus-ataxia syndromes (MAS), due to recent advances in genetic techniques. A proper etiologic diagnosis of MAS is clinically relevant, given the consequences for genetic counseling, treatment, and prognosis. Objectives Objectives: To review the causes of MAS and to propose a diagnostic algorithm. Methods Methods: A comprehensive and structured literature search following PRISMA criteria was conducted to identify those disorders that may combine myoclonus with ataxia. Results Results: A total of 135 causes of combined myoclonus and ataxia were identified, of which 30 were charted as the main causes of MAS. These include four acquired entities: opsoclonus-myoclonus-ataxia syndrome, celiac disease, multiple system atrophy, and sporadic prion diseases. The distinction between progressive myoclonus epilepsy and progressive myoclonus ataxia poses one of the main diagnostic dilemmas. Conclusions Conclusions: Diagnostic algorithms for pediatric and adult patients, based on clinical manifestations including epilepsy, are proposed to guide the differential diagnosis and corresponding work-up of the most important and frequent causes of MAS. A list of genes associated with MAS to guide genetic testing strategies is provided. Priority should be given to diagnose or exclude acquired or treatable disorders.

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Acute Ataxia in Children: Approach to Clinical Presentation and Role of Additional Investigations

Cited by 16 publications

References 129 publications

Assessing Children with Poor Coordination Can Be Tricky – A Review on Ataxia and Ataxia Mimickers and a Study of Three Children with Severe Epilepsy

Assessing Children with Poor Coordination Can Be Tricky – A Review on Ataxia and Ataxia Mimickers and a Study of Three Children with Severe Epilepsy

Acute post-infectious cerebellar ataxia due to co-infection of human herpesvirus-6 and adenovirus mimicking myositis

Myoclonus‐Ataxia Syndromes: A Diagnostic Approach

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