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Case 226

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32 years old, male patient presents a change in the walking patterns, motor coordination, vision and the speech, of slow progression for 14 years. At the time of the first symptoms, he lost control of the anal sphincters and he is in a wheel chair for 3 years. He denies acute symptoms or spontaneous improvement. At the examination, he has loss of the visual acuity, change in the postural kinetic sensitivity, presence of the Babinsky sign and bilateral hyperreflexia of the Achilles tendon, and global muscular strength reduced in the lower limbs.

Based on the clinical history and in the imaging studies presented, what is the most likely diagnosis?

a) Multiple sclerosis

25%

b) Wilson’s disease

25%

c) Lateral Amyotrophic Sclerosis

25%

d) Machado–Joseph disease

25%
   

Image analysis

Image 1: Flair-Weighted Magnetic Resonance imaging of the cranium.  It is similar to the T2 weighted, the free water signal, like the liquor is erased and it appears as a hypointense signal/black. The water linked to proteins or the result of an inflammatory process remains as a hyperintense signal/white. The sagittal plane image shows a hyperintense (white) lesion in the periventricular white matter, with the major axis perpendicular to the corpus callosum, with an ovoid shape. These lesions, called “Dawson’s fingers”, are plaques of demyelination, related to inflammatory changes around the medullary veins. It is also possible to visualize the hyperintense lesion on the cerebellum.

Image 2: T1-weighted MR imaging of the cranium. In this case, the water and liquor are hypointense (black) and the grey matter is darker than the white matter.  The image in axial plane reveals small hyperintense ovoid lesions (white) in the periventricular region, correspondents to demyelination (circled areas).

Image 3: T2-weighted MR imaging of the cranium, in this case water and liquor are shown hyperintense/white. The grey matter is lighter and the white matter is darker. The axial plane reveals hyperintense ovoid lesions (white) that corresponds to demyelination and discreet brain atrophy.

Highlights

- Multiple Sclerosis is a degenerative and immune mediated inflammatory disease of the central nervous system;

- It has several clinical forms: relapsing-remitting, secondarily progressive, primarily progressive or primarily progressive with relapses;

- The most common symptoms are fatigue, visual disturbances, neuritis optic, spasticity, weakness, imbalance, sensory changes, bladder dysfunction and/or bowel, among others;

- The main finding on the MR is the sign of “Dawson’s fingers” that are plaques of demyelination, usually on the periventricular region;

- The MS does not have a cure, but it can be managed. The treatment is focus on controlling the crisis and the symptoms.

References

- Polman CH, et al. Diagnostic Criteria for Multiple Sclerosis: 2010 Revisions to the McDonald Criteria. Ann Neurol. 2011 Feb;69(2):292-302.

- Miller DH, et al. Differential diagnosis of suspected multiple sclerosis: a consensus approach. MultScler. 2008 Nov;14(9):1157-74.

- Das SK, Ray K. Wilson’s disease: an update. NatClinPractNeurol. 2006 Sep;2(9):482-93.

- da Rocha AJ, Maia AC Jr, Nogueira RG, Lederman HM.Magnetic resonance findings in amyotrophic lateral sclerosis using a spin echo magnetization transfer sequence. Preliminary report. ArqNeuropsiquiatr. 1999 Dec;57(4):912-5

- Bürk K, et al. Autosomal dominant cerebellar ataxia type I clinical features and MRI in families with SCA1, SCA2 and SCA3. Brain. 1996 Oct;119 ( Pt 5):1497-505.

- Centro de Investigação em Esclerose Múltipla de Minas Gerais. [Accessed 30/09/2015]. Available at: http://www.ciem.com.br/

Authors

Vinícius de Moraes Palma, 4th year medical student at Faculdade de Medicina da UFMG.

E-mail: viniciusmpalma[at]gmail.com

 

Lívia Pires Calastri, 4th year medical student at da Faculdade de Medicina da UFMG.

E-mail:livia.calastri[at]gmail.com

 

Laura Defensor Ribeiro, 4th year medical student at da Faculdade de Medicina da UFMG.

E-mail: lauradefensorribeiro[at]hotmail.com

 

Raíra Cezar, 5th year medical student at da Faculdade de Medicina da UFMG.

E-mail: rairacezar[at]gmail.com

Supervisor

Dr. Ravi Félix de Melo Gajo, médico neurologista pela Santa Casa.

Email: ravifelix[at]outlook.com

Reviewers

Fabio M. Satake, Débora Faria Nogueira, Daniela Braga, Cairo Mendes, Profa. Viviane Parisotto.

Translated by

Ana Carina Breunig Nunes, 5th year studentent at Faculdade de Medicina da UFMG.

E-mail: anacbreunig[at]gmail.com

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