Investigating ataxia in childhood

Petley, Emily; Prasad, Manish; Ojha, Shalini; Whitehouse, William

doi:10.1136/archdischild-2018-315893

Cited by 3 publications

(7 citation statements)

References 25 publications

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“…• forme infettive e parainfettive: sono le più frequenti, per lo più di origine virale o secondarie a vaccinazione e coinvolgono direttamente le strutture nervose. Gli anticorpi prodotti in seguito all'infezione cross-reagiscono contro epitopi cerebellari, determinando un'attivazione della risposta infiammatoria e conseguente demielinizzazione [2][3][4]. Il virus varicella zoster era (prima della vaccinazione) il principale patogeno coinvolto nelle APCA, mentre attualmente i virus più frequentemente responsabili sono coxsackie virus, EBV, echovirus, enterovirus, parvovirus B19, HHV6, morbillo e parotite.…”

Section: Eziologiaunclassified

“…Il virus varicella zoster era (prima della vaccinazione) il principale patogeno coinvolto nelle APCA, mentre attualmente i virus più frequentemente responsabili sono coxsackie virus, EBV, echovirus, enterovirus, parvovirus B19, HHV6, morbillo e parotite. Fra gli agenti batterici si segnalano legionella, micoplasma e borrelia [1,4,6]; • encefalomielite acuta disseminata (ADEM): rara patologia infiammatoria autoimmune demielinizzante che insorge, generalmente, a distanza di circa due settimane da un'infezione virale delle alte vie aeree [4]; • cerebellite acuta: spesso considerata nello spettro dell'APCA, è una condizione che ha caratteristiche cliniche e neuroradiologiche distintive. Questi bambini appaiono molto compromessi e all'atassia acuta si associano vomito, cefalea, alterato stato di coscienza.…”

Section: Eziologiaunclassified

“…Alcuni pazienti possono avere un decorso fulminante con edema cerebellare severo e, in questi casi, la risonanza magnetica nucleare (RMN) dimostrerà segni tipici di infiammazione cerebellare; • sindrome opsoclono-mioclono: è caratterizzata dalla triade atassia, opsoclono (movimenti coniugati oculari multidirezionali rapidi e caotici) e mioclono (rapida contrazione muscolare). È una AA con tendenza alla cronicizzazione e si riscontra prevalentemente in un contesto di sindromi paraneoplastiche, spesso in associazione a neuroblastoma (50% dei casi) o come forma parainfettiva [2,4,[8][9]; • sindrome di Guillain-Barré (GBS): è una condizione autoimmune, rapidamente progressiva, secondaria a un'infezione delle vie aeree o gastrointestinale da Campylobacter, H. influenzae, EBV o CMV. Può presentarsi con atassia (tipo sensoriale) o ipostenia, irritabilità e dolore, segni di compromissione del secondo motoneurone con riflessi tendinei profondi assenti o alterati.…”

Section: Eziologiaunclassified

“…L'atassia è una manifestazione neurologica relativamente rara in età pediatrica con una prevalenza di circa 26:100.000 [5]. La forma più comune, l'atassia cerebellare acuta post infettiva (APCA) rappresenta fino al 50% delle AA (atassia acuta) ed è tipica della fascia d'età 1-6 anni [1,4,[6][7]. Uno studio multicentrico italiano stima che l'AA possa essere la causa dello 0,02% degli accessi in pronto soccorso pediatrico (PSP) [7].…”

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Il bambino con atassia acuta

Cattazzo¹,

Accomando

Aricò

2023

QACP

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Pediatric acute ataxia generally represents an infrequent occurrence of pediatric emergency department access, but the broad differential diagnosis concerning it requires, starting with anamnesis and objective examination, to mainly exclude life-threatening causes in the child. For these reasons, the diagnosis of acute post infectious cerebellar ataxia, a benign and self-limiting form, which is the underlying cause in most cases of acute ataxia must always be a diagnosis of exclusion. The broad differential diagnosis also requires a correct approach that must consider the setting about the use of imaging techniques proportional to the urgency, in order to reduce the risk of unnecessary radio exposure. Starting from this, we have attempted to summarize the literature regarding the clinical history, physical examination, and diagnostic tests necessary to arrive at a correct diagnosis.

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Section: Eziologiaunclassified

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Il bambino con atassia acuta

Cattazzo¹,

Accomando

Aricò

2023

QACP

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“…43 Ataxia may be observed in children with recent viral infections (e.g., varicella) or vaccination but also as side effect of several drugs that are frequently used in children (e.g., antiepileptics, benzodiazepines, and antihistamines). 44 Acute dystonia and status dystonicus may be drug-induced (antipsychotics and antiemetics such as metoclopramide) or the expression of a clinical worsening in patients with dystonic cerebral palsy or genetic disorders (e.g., GNAO 1 encephalopathy). 45 Status dystonicus is a potentially lifethreatening condition presenting with continuous severe episodes of generalized dystonic contractions and requiring an adequate support of vital functions and the activation of therapeutic protocols to prevent metabolic derangement, rhabdomyolisis, and respiratory arrest.…”

Section: Acute Movement Disordersmentioning

confidence: 99%

Management of Neurological Emergencies in Children: An Updated Overview

Mastrangelo

Baglioni

2021

Neuropediatrics

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Neurological emergencies account for about one-third of the highest severity codes attributed in emergency pediatric departments. About 75% of children with acute neurological symptoms presents with seizures, headache, or other paroxysmal events. Life-threatening conditions involve a minor proportion of patients (e.g., less than 15% of children with headache and less than 5% of children with febrile seizures). This review highlights updated insights about clinical features, diagnostic workup, and therapeutic management of pediatric neurological emergencies. Particularly, details will be provided about the most recent insights about headache, febrile seizures, status epilepticus, altered levels of consciousness, acute motor impairment, acute movement disorders, and functional disorders, as well as the role of diagnostic tools (e.g., neuroimaging, lumbar puncture, and electroencephalography), in the emergency setting. Moreover, the impact of the current novel coronavirus disease2019 (COVID-19) pandemic on the evaluation of pediatric neurologic emergencies will also be analyzed.

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Clinical, Cognitive and Behavioural Assessment in Children with Cerebellar Disorder

D’Arrigo

Ciaccio

et al. 2021

Applied Sciences

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Cerebellar disorders are characterised clinically by specific signs and symptoms, often associated with neurodevelopmental disorder. While the clinical signs of cerebellar disorders are clearly recognisable in adults and have a precise anatomo-functional correlation, in children the semiotics are less clear and vary with age because of the particular nature of the cerebellum’s maturation. Unlike other structures of the central nervous system, this begins at a later stage of foetal development and extends over a longer period of time, even after birth. As a result, the typical signs of cerebellar dysfunction will only become evident when the cerebellar functions have become integrated into the complex circuits of the central nervous system. This means that poor motor coordination in the very early years of life may not necessarily correlate with cerebellar dysfunction, and this may also be encountered in healthy children. The cerebellum’s role in cognitive and emotional functions relies on its structure and the complexity of its connections. Cognitive and behavioral impairment in cerebellar disorders can be the results of acquired lesions or the action of genetic and environmental risk factors, to which the cerebellum is particularly vulnerable considering its pattern of development. In the pathological setting, early evidence of cerebellar damage may be very vague, due, partly, to spontaneous compensation phenomena and the vicarious role of the connecting structures (an expression of the brain’s plasticity). Careful clinical assessment will nonetheless enable appropriate instrumental procedures to be arranged. It is common knowledge that the contribution of neuroimaging is crucial for diagnosis of cerebellar conditions, and neurophysiological investigations can also have a significant role. The ultimate goal of clinicians is to combine clinical data and instrumental findings to formulate a precise diagnostic hypothesis, and thus request a specific genetic test in order to confirm their findings, wherever possible.

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Investigating ataxia in childhood

Cited by 3 publications

References 25 publications

Il bambino con atassia acuta

Il bambino con atassia acuta

Management of Neurological Emergencies in Children: An Updated Overview

Clinical, Cognitive and Behavioural Assessment in Children with Cerebellar Disorder

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