Skip to main content Accessibility help
×
Home

Neuroimaging Findings and Repeat Neuroimaging Value in Pediatric Chronic Ataxia

  • Michael S Salman (a1) (a2), Bernard N Chodirker (a3) (a2) (a4) and Martin Bunge (a5) (a6)

Abstract

Background: Chronic ataxia, greater than two months in duration, is encountered relatively commonly in clinical pediatric neurology practise and presents with diagnostic challenges. It is caused by multiple and diverse disorders. Our aims were to describe the neuroimaging features and the value of repeat neuroimaging in pediatric chronic ataxia to ascertain their contribution to the diagnosis and management. Materials and Methods: A retrospective charts and neuroimaging reports review was undertaken in 177 children with chronic ataxia. Neuroimaging in 130 of 177 patients was also reviewed. Results: Nineteen patients had head computed tomography only, 103 brain magnetic resonance imaging only, and 55 had both. Abnormalities in the cerebellum or other brain regions were associated with ataxia. Neuroimaging was helpful in 73 patients with 30 disorders: It was diagnostic in 9 disorders, narrowed down the diagnostic possibilities in 14 disorders, and revealed important but non-diagnostic abnormalities, e.g. cerebellar atrophy in 7 disorders. Having a normal magnetic resonance imaging scan was mostly seen in genetic diseases or in the early course of ataxia telangiectasia. Repeat neuroimaging, performed in 108 patients, was generally helpful in monitoring disease evolution and in making a diagnosis. Neuroimaging was not directly helpful in 36 patients with 10 disorders or by definition the 55 patients with unknown disease etiology. Conclusions: Normal or abnormal neuroimaging findings and repeat neuroimaging are very valuable in the diagnosis and management of disorders associated with pediatric chronic ataxia.

Résultats en neuro-imagerie et utilité d’examens répétés en neuro-imagerie dans les cas d’ataxie chronique chez l’enfant. Contexte: L’ataxie chronique, dont la durée excède deux mois, est un phénomène relativement fréquent dans la pratique clinique en neurologie pédiatrique et représente un défi en matière de diagnostic. Elle est causée par plusieurs différents types de désordres. Notre objectif est ici de décrire les divers aspects de la neuro-imagerie et l’utilité d’examens répétés en neuro-imagerie dans les cas d’ataxie chronique chez l’enfant, et ce, afin de déterminer leur apport en ce qui concerne le diagnostic et le suivi de cette pathologie. Matériel et méthodes: Nous avons passé en revue des récapitulatifs graphiques ainsi que des rapports en neuro-imagerie concernant 177 enfants atteints d’ataxie chronique. Les examens en neuro-imagerie de 130 patients sur 177 ont également été analysés. Résultats: Dix-neuf patients n’ont passé qu’un examen de tomographie assistée par ordinateur; 103 d’entre eux n’ont passé qu’un examen d’imagerie par résonnance magnétique (IRM); enfin, 55 ont été soumis à ces deux examens. Des anomalies dans le cervelet et d’autres régions du cerveau ont été associées à l’ataxie. La neuro-imagerie s’est révélée utile dans le cas de 73 patients atteints de 30 désordres: ainsi, elle a permis le diagnostic formel de 9 désordres; elle a permis d’exclure des possibilités de diagnostic dans le cas de 14 désordres; elle a enfin permis de révéler d’importantes anomalies hors diagnostic, par exemple l’atrophie cérébelleuse dans le cas de 7 désordres. Le fait de passer des examens habituels d’IRM a été noté la plupart du temps dans le cas de maladies génétiques ou en ce qui regarde des cas d’ataxie télangiectasie encore à un stade précoce. Répéter des examens en neuro-imagerie chez 108 patients s’est avéré dans l’ensemble utile pour surveiller l’évolution des désordres et établir un diagnostic. La neuro-imagerie n’est pas apparue directement utile dans le cas de 36 patients atteints de 10 désordres ou, par définition, de 55 patients dont l’étiologie demeurait inconnue. Conclusions: Que les résultats en neuro-imagerie soient normaux ou anormaux, ils demeurent très utiles, à l’instar des examens qu’on répète, dans le diagnostic et le suivi des désordres associés à l’ataxie chronique chez l’enfant.

  • View HTML
    • Send article to Kindle

      To send this article to your Kindle, first ensure no-reply@cambridge.org is added to your Approved Personal Document E-mail List under your Personal Document Settings on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part of your Kindle email address below. Find out more about sending to your Kindle. Find out more about sending to your Kindle.

      Note you can select to send to either the @free.kindle.com or @kindle.com variations. ‘@free.kindle.com’ emails are free but can only be sent to your device when it is connected to wi-fi. ‘@kindle.com’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.

      Find out more about the Kindle Personal Document Service.

      Neuroimaging Findings and Repeat Neuroimaging Value in Pediatric Chronic Ataxia
      Available formats
      ×

      Send article to Dropbox

      To send this article to your Dropbox account, please select one or more formats and confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your <service> account. Find out more about sending content to Dropbox.

      Neuroimaging Findings and Repeat Neuroimaging Value in Pediatric Chronic Ataxia
      Available formats
      ×

      Send article to Google Drive

      To send this article to your Google Drive account, please select one or more formats and confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your <service> account. Find out more about sending content to Google Drive.

      Neuroimaging Findings and Repeat Neuroimaging Value in Pediatric Chronic Ataxia
      Available formats
      ×

Copyright

Corresponding author

Correspondence to: Michael S. Salman, Section of Pediatric Neurology, Children’s Hospital AE 308, 820 Sherbrook Street, Winnipeg, MB, R3A 1R9, Canada. Email: msalman@hsc.mb.ca

References

Hide All
1. Poretti, A, Benson, JE, Huisman, TA, Boltshauser, E. Acute ataxia in children: approach to clinical presentation and role of additional investigations. Neuropediatrics. 2013;44:127-141.
2. Salman, MS, Lee, EJ, Tjahjadi, A, Chodirker, BN. The epidemiology of intermittent and chronic ataxia in children in Manitoba, Canada. Dev Med Child Neurol. 2013;55:341-347.
3. Brunberg, JA. Expert Panel on Neurologic Imaging. Ataxia. AJNR Am J Neuroradiol. 2008;29:1420-1422.
4. Mascalchi, M, Vella, A. Magnetic resonance and nuclear medicine imaging in ataxias. Handb Clin Neurol. 2012;103:85-110.
5. Vedolin, L, Gonzalez, G, Souza, CF, Lourenço, C, Barkovich, AJ. Inherited cerebellar ataxia in childhood: a pattern-recognition approach using brain MRI. AJNR Am J Neuroradiol. 2013;34:925-934; S1-2.
6. Boddaert, N, Desguerre, I, Bahi-Buisson, N, et al. Posterior fossa imaging in 158 children with ataxia. J Neuroradiol. 2010;37:220-230.
7. Salman, MS, Masood, S, Azad, M, Chodirker, BN. Ethnicity and geographic distribution of pediatric chronic ataxia in Manitoba. Can J Neurol Sci. 2014;41:29-36.
8. Poretti, A, Wolf, NI, Boltshauser, E. Differential diagnosis of cerebellar atrophy in childhood. Eur J Paediatr Neurol. 2008;12:155-167.
9. Harting, I, Seitz, A, Rating, D, et al. Abnormal myelination in Angelman syndrome. Eur J Paediatr Neurol. 2009;13:271-276.
10. Dunn, HG, MacLeod, PM. Rett syndrome: review of biological abnormalities. Can J Neurol Sci. 2001;28:16-29.
11. Esscher, E, Flodmark, O, Hagberg, G, Hagberg, B. Non-progressive ataxia: origins, brain pathology and impairments in 78 Swedish children. Dev Med Child Neurol. 1996;38:285-296.
12. Gleeson, JG, Keeler, LC, Parisi, MA, et al. Molar tooth sign of the midbrain-hindbrain junction: occurrence in multiple distinct syndromes. Am J Med Genet A. 2004;125A:125-134.
13. Shekdar, K. Posterior fossa malformations. Semin Ultrasound CT MR. 2011;32:228-241.
14. Al-Maawali, A, Blaser, S, Yoon, G. Diagnostic approach to childhood-onset cerebellar atrophy: a 10-year retrospective study of 300 patients. J Child Neurol. 2012;27:1121-1132.
15. Kanekar, S, Gent, M. Malformations of cortical development. Semin Ultrasound CT MR. 2011;32:211-227.
16. Anheim, M, Fleury, M, Monga, B, et al. Epidemiological, clinical, paraclinical and molecular study of a cohort of 102 patients affected with autosomal recessive progressive cerebellar ataxia from Alsace, Eastern France: implications for clinical management. Neurogenetics. 2010;11:1-12.
17. Rasalkar, DD, Chu, WC. Imaging in children presenting with acute neurological deficit: stroke. Postgrad Med J. 2012;88:649-660.
18. Mackay, MT, Prabhu, SP, Coleman, L. Childhood posterior circulation arterial ischemic stroke. Stroke. 2010;41:2201-2209.
19. Yock-Corrales, A, Mackay, MT, Mosley, I, Maixner, W, Babl, FE. Acute childhood arterial ischemic and hemorrhagic stroke in the emergency department. Ann Emerg Med. 2011;58:156-163.
20. Steenweg, ME, Vanderver, A, Blaser, S, et al. Magnetic resonance imaging pattern recognition in hypomyelinating disorders. Brain. 2010;133:2971-2982.
21. Limperopoulos, C, du Plessis, AJ. Injury to the Developing Cerebellum: Mechanisms and Consequences. Neoreviews. 2007;8:e409-e417.
22. Boycott, KM, Flavelle, S, Bureau, A, et al. Homozygous deletion of the very low density lipoprotein receptor gene causes autosomal recessive cerebellar hypoplasia with cerebral gyral simplification. Am J Hum Genet. 2005;77:477-483.
23. Rajab, A, Mochida, GH, Hill, A, et al. A novel form of pontocerebellar hypoplasia maps to chromosome 7q11-21. Neurology. 2003;60:1664-1667.
24. Cianfoni, A, Caulo, M, Cerase, A, et al. Seizure-induced brain lesions: A wide spectrum of variably reversible MRI abnormalities. Eur J Radiol. 2013;82:1964-1972.
25. Cartagena, AM, Young, GB, Lee, DH, Mirsattari, SM. Reversible and irreversible cranial MRI findings associated with status epilepticus. Epilepsy Behav. 2014;33:24-30.
26. Johnston, JM, Smyth, MD, McKinstry, RC. Basics of neuroimaging in pediatric epilepsy. In: Pellock JM, Bourgeois BFD, Dodson WE, editors. Pediatric epilepsy: Diagnosis and therapy. 3rd ed. New York: Demos Medical Publishing; 2008. pg. 213-226.
27. Cerase, A, Rubenni, E, Rufa, A, et al. CT and MRI of Wernicke’s encephalopathy. Radiol Med. 2011;116:319-333.
28. Baldarçara, L, Currie, S, Hadjivassiliou, M, et al. Consensus paper: radiological biomarkers of cerebellar diseases. Cerebellum. 2015;14:175-196.
29. Chokshi, FH, Poretti, A, Meoded, A, Huisman, TA. Normal and abnormal development of the cerebellum and brainstem as depicted by diffusion tensor imaging. Semin Ultrasound CT MR. 2011;32:539-554.

Keywords

Type Description Title
UNKNOWN
Supplementary materials

Salman supplementary material
Image 1

 Unknown (5.6 MB)
5.6 MB
UNKNOWN
Supplementary materials

Salman supplementary material
Image 2

 Unknown (4.1 MB)
4.1 MB
UNKNOWN
Supplementary materials

Salman supplementary material
Image 3

 Unknown (5.7 MB)
5.7 MB
UNKNOWN
Supplementary materials

Salman supplementary material
Image 4

 Unknown (4.9 MB)
4.9 MB
UNKNOWN
Supplementary materials

Salman supplementary material
Image 5

 Unknown (4.6 MB)
4.6 MB
UNKNOWN
Supplementary materials

Salman supplementary material
Image 6

 Unknown (5.5 MB)
5.5 MB
UNKNOWN
Supplementary materials

Salman supplementary material
Image 7

 Unknown (4.2 MB)
4.2 MB
UNKNOWN
Supplementary materials

Salman supplementary material
Image 8

 Unknown (5.7 MB)
5.7 MB
UNKNOWN
Supplementary materials

Salman supplementary material
Image 9

 Unknown (4.6 MB)
4.6 MB
WORD
Supplementary materials

Salman supplementary material
Tables S1-S5

 Word (56 KB)
56 KB

Neuroimaging Findings and Repeat Neuroimaging Value in Pediatric Chronic Ataxia

  • Michael S Salman (a1) (a2), Bernard N Chodirker (a3) (a2) (a4) and Martin Bunge (a5) (a6)

Metrics

Full text views

Total number of HTML views: 0
Total number of PDF views: 0 *
Loading metrics...

Abstract views

Total abstract views: 0 *
Loading metrics...

* Views captured on Cambridge Core between <date>. This data will be updated every 24 hours.

Usage data cannot currently be displayed