KEYWORDS:
Glioma; Behaviour; Behavioural symptoms; Brain stem neoplasms
PALAVRAS CHAVE:
Glioma; Comportamento; Sintomas comportamentais; Neoplasias do tronco encefálico
This five-year-old girl presented with a history of two weeks of behavioural changes (irritability, aggressiveness and school problems). No underlying cause was found on psychiatric assessment. In the following 5 days, she presented a left sixth nerve palsy and ataxic gait and then she was referred to the neurosurgery team. Magnetic resonance imaging showed a diffuse intrinsic pontine glioma – DIPG – (Figures A and B). Radiotherapy was then started.
Diffuse intrinsic pontine glioma characterized by a hyperintensity on T2 (A, axial view) and iso-hypointensity on T1 weighted MRI (B, sagittal view). In this case, the lesion did not have significant contrast enhancement.
DIPG accounts for 10% of childhood brain tumours. Most common signs at presentation include corticospinal tract deficits, cranial nerve palsies and ataxia. The brainstem was not regarded as a structure involved in complex affective behaviour, but today it is well established that cerebrocerebellar and cerebellum-cerebral connections play a role in affective and cognitive behavioural.11. Riva D, Giorgi C. The cerebellum contributes to higher functions during development: evidence from a series of children surgically treated for posterior fossa tumours. Brain. 2000;123 (Pt5):1051-61. Actually, behavioural changes are present in only 5% of the cases.22. 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;8(8):685-95.,33. Hargrave DR, Mabbott DJ, Bouffet E. Pathological laughter and behavioural change in childhood pontine glioma. J Neurooncol. 2006;77(3):267-71. However, in children with unexplainable behavioural changes, DIPG should be considered as a differential diagnosis.
REFERENCES
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1Riva D, Giorgi C. The cerebellum contributes to higher functions during development: evidence from a series of children surgically treated for posterior fossa tumours. Brain. 2000;123 (Pt5):1051-61.
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2Wilne 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;8(8):685-95.
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3Hargrave DR, Mabbott DJ, Bouffet E. Pathological laughter and behavioural change in childhood pontine glioma. J Neurooncol. 2006;77(3):267-71.
Publication Dates
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Publication in this collection
July 2018
History
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Received
18 Oct 2017 -
Accepted
23 Oct 2017
