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Case 178

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Female patient, 61 years of age, was sent to the Neurology service by an Ophthalmologist. She complains of difficulty in reading and in differentiating the steps of a stairs, which progressively worsened in the last 3 years, as well as difficulties in memorizing recent information for the past 5 months. She is currently dependent of others for instrumental activities of daily life. Denies comorbidities, family history of dementia or use medication. No other neurological signs. The work up was done with laboratory exams and a brain MRI.

Based on the clinical picture and the images, which one of the following is the most likely diagnosis?

a) Creutzfeldt-Jakob Disease

25%

b) Dementia with Lewy Bodies

25%

c) Alzheimer\\\\\\\\\\\\\\\'s Disease

25%

d) Cortico-basal degeneration

25%
   

Image analysis

Image 1 - Magnetic resonance imaging of the brain without venous contrast, axial view, weighed in T1. The area highlighted in red outlines important cortical atrophy in the parietal-occipital region, with loss of volume of the gyri posterior to the central sulcus.

 

Image 2 - Magnetic resonance imaging of the brain without venous contrast, axial view, weighted in T2. The area highlighted in pink defines important cortical atrophy in the parietal-occipital region, with loss of volume of the gyri posteiror to the central sulcus.

Highlights

- Posterior cortical atrophy (PCA) is considered a variant of Alzheimer Disease (AD), but may be found in other dementia;

- Predominates in pre-senile patients (before 65 years);

- Visuospatial symptoms precede the memory decline and often result in finding ophthalmologists as a first resort;

- Characteristics of the syndromes of Balint and / or Gerstmann may be present;

- There are no specific tests for the diagnosis that is, it is based on clinical manifestations, supported by complementary exams and made by exclusion of other diagnosis;

- It is an insidious, progressive disease and treatment is symptomatic.

References

- McKhann GM, Knopman DS, Chertkow H, Hyman BT, Jack CR Jr, Kawas CH, Klunk WE, Koroshetz WJ, Manly JJ, Mayeux R, Mohs RC, Morris JC, Rossor MN, Scheltens P, Carrillo MC, Thies B, Weintraub S, Phelps CH. The diagnosis of dementia due to Alzheimer's disease: recommendations from the National Institute on Aging -  Alzheimer's Association workgroups on diagnostic guidelines for Alzheimer's disease. Alzheimers Dement. 2011;7(3):263-269.

- Park KW, Kim HS, Cheon SM, Cha JK, Kim SH, Kim JW. Dementia with Lewy Bodies versus Alzheimer's disease and Parkinson's disease Dementia: A Comparison of Cognitive Profiles. J Clin Neurol.2011;7:19-24.

- Caramelli P, Barbosa MT. Como diagnosticar as quatro causas mais frequentes de demência? How to diagnoses the four most frequent causes of dementia? Rev Bras Psiquiatria.2002;24:7-10.

- Ferreira LK, Busatto GF. Neuroimaging in Alzheimer’s disease: current role in clinical practice and potential future applications. Clinics.2011;66:19-24.

- Rocha AJ, Vedolin L, Mendonça RA. Colégio Brasileiro de Radiologia e Diagnóstico por Imagem: Encéfalo. Rio de Janeiro: Elsevier; 2010.

- Barkhof F, Fox NC, Bastos-Leite AJ, Scheltens P. Neuroimaging in Dementia. Berlim: Springer; 2011.

- Caramelli P, Teixeira AL, Buchpiguel CA, Lee HW, Livramento JA, Fernandez LL, Anghinah R. Diagnóstico de doença de Alzheimer no Brasil - Exames complementares. Dement Neuropsychol. 2011;5(Suppl 1):11-20.

Authors

Carla Maria Fraga Faraco, 4th year medical student at UFMG. E-mail: cmffaraco[at]gmail.com

André Aguiar Souza Furtado de Toledo, 6th year medical student at UFMG. E-mail: asftoledo[at]gmail.com

Supervisors

David Márcio, Neurology residente at UFMG’s Hospital das Clínicas. E-mail: davidmarcio.med[at]gmail.com

Fernanda Moura Teatini, neuroradiologist at UFMG’s Hospital das Clínicas. E-mail: fernandateatini[at]live.com

Reviewers

Fábio Satake, André Guimarães, Marina Leão, Professor José Nelson Mendes Vieira,  Professor Viviane Parisotto

Translated by

Bárbara de Queiroz e Bragaglia, 5th year medical student at UFMG. E-mail: barbara.bragaglia[at]gmail.com

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