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Case 319

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19 year-old male patient refers 2 days with difficulty to walk and slightly ataxic gait. History of neuropsychomotor developmental delay, with difficulty to learn in early childhood. Denies comorbidities. Attending doctor requested a brain CT (Image 1) which revealed lesion in the right internal capsule area. Patient also brought previous MRI results (Image 2 and 3).

Considering the information provided and the images shown, the probable aetiology for this case is:

a) Developmental venous anomalies

25%

b) Arteriovenous malformation

25%

c) Hemangioblastoma

25%

d) Cavernoma

25%
   

Image analysis

Image 1 - Analysis: Computerised tomography (CT) of the brain, axial view, lateral ventricles level, without injection of intravenous iodine-based contrast. Presence of hyperdense area (yellow highlight) in the posterior arm of the right internal capsule.

 

Image 2 - Analysis: Magnetic resonance imaging (MRI) of the brain, T2-weighted, axial view, lateral ventricles level. Presence of nodular lesion in the posterior arm of the right internal capsule, heterogenous, with areas of hyper and hyposignal, giving the appearance of  “popcorn" (marked in green), surrounded by areas of hyposignal (highlighted in yellow), suggesting hemoglobin residues in different stages of degradation, without anomalous impregnation of paramagnetic contrast (gadolinium).

 

Image 3 - Analysis: Magnetic resonance imaging (MRI) of the brain, gradient-echo, axial view, lateral ventricles level. Presence of nodular lesion in the posterior arm of the right internal capsule, heterogenous, with areas of hyper and hyposignal, giving the appearance of  “popcorn" (marked in green), surrounded by areas of hyposignal (highlighted in red), without anomalous impregnation of paramagnetic contrast (gadolinium).  

Highlights

-   Cavernoma is a brain venous malformation of sporadic or familiar occurrence, usually autosomal dominant;

-   It is constituted of thin walled capillaries, predisposing to ruptures and local haemorrhages;

-   MRI is the first line exam for diagnosis. Angiography is of no application in those cases;

-   It is generally asymptomatic or manifests by local haemorrhages, seizures or neurological deficits; 

-   Surgical treatment is indicates in cases of progressive neurologic deficit, untreatable seizures and/or recurrent haemorrhage.

References

- Robert J Singer, Christopher S Ogilvy, Guy Rordorf. Vascular malformations of the central nervous system. In: UpToDate, Post TW (Ed), UpToDate, Waltham, MA. [Acesso em 24/04/2018]. Disponível em: https://www.uptodate.com/contents/vascular-malformations-of-the-central-nervous-system

- Rocha, A.J; Vedolin, L; Mendonça, R.A. Malformações Vaculares e Aneurismas Intracranianos. Rio de Janeiro: Elsevier; 2010 In : Colégio Brasileiro de Radiologia e Diagnóstico por Imagem – Encéfalo. p. 472-484. 

- Rocha, A.J; Vedolin, L; Mendonça, R.A. Neoplasias Parenquimatosas Infratentoriais. Rio de Janeiro: Elsevier; 2012In: Colégio Brasileiro de Radiologia e Diagnóstico por Imagem – Encéfalo. p. 343-346.

- Osborn, A ; Jhaveri, M ; Salzman, K. Pathology Based Diagnoses : Vascular Malformations- Carvernous Malformation. Philadelphia: Elsevier; 2016 In: Diagnostic imaging. Brain. 3rd ed. p. 420-423

- McDonald, L; Rennie, A; Tolmie, J; Galloway, P; & McWilliam, R. (2006). Investigation of global developmental delay. Archives of Disease in Childhood, 91(8), 701-705.

- Meschino, W.S. (2003). The child with developmental delay: An approach to etiology. Paediatrics & child health, 8(1), 16-19.

Author

André Dias Nassar Naback, 6thyear medical student at Universidade Federal de Minas Gerais.

E-mail: andrenaback[at]gmail.com

Supervisor

Marcos Antônio Dellaretti Filho, MD. Neurosurgeon at the Universidade Federal de Minas Gerais.

E-mail: mdellaretti[at]mac.com

 

Carlos Magno da SilvaMD. Neuroradiologist at the Universidade Federal de Minas Gerais.

E-mail: carlosbresil[at]hotmail.com

Reviewers

Lucas Cantaruti, Guilherme Carvalho, Bernardo Finotti, Eduardo Paolinelli, Ariádna Andrade, Prof. José Nelson M. Vieira,M.D., Ph.D.,Prof. Viviane Santuari Parisotto Marino, M.D., Ph.D.

Translated by

Bruno Campos Santos, 5thyear medical student at Universidade Federal de Minas Gerais.

E-mail: bruno_campos[at]outlook.com

Test question

(Rio de Janeiro Military Police, Neurosurgery – 2010) The lesion most frequently associated to cavernoma is:

a) Basilar aneurysm

25%

b) Capillary telangiectasia

25%

c) Venous angioma

25%

d) Dural arteriovenous malformation

25%

e)

25%
   

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