Volume 14, Issue 9 pp. 971-975

Motor neuron disease associated with non-fluent rapidly progressive aphasia: case report and review of the literature

A. J. Da Rocha

A. J. Da Rocha

Division of Neuroradiology, Santa Casa de Misericórdia de São Paulo, São Paulo, Brazil

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B. C. O. Valério

B. C. O. Valério

Division of Neurology, Santa Casa de Misericórdia de São Paulo, São Paulo, Brazil

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R. P. Buainain

R. P. Buainain

Division of Neurology, Santa Casa de Misericórdia de São Paulo, São Paulo, Brazil

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M. E. Ferraz

M. E. Ferraz

Division of Neurology, Santa Casa de Misericórdia de São Paulo, São Paulo, Brazil

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C. J. Da Silva

C. J. Da Silva

Division of Neuroradiology, Santa Casa de Misericórdia de São Paulo, São Paulo, Brazil

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A. C. M. Maia Jr.

A. C. M. Maia Jr.

Division of Neuroradiology, Santa Casa de Misericórdia de São Paulo, São Paulo, Brazil

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V. P. Andrade

V. P. Andrade

Division of Pathology, Fleury Centro de Medicina Diagnóstica, São Paulo, Brazil

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C. L. P. Lancellotti

C. L. P. Lancellotti

Division of Pathology, Santa Casa de Misericórdia de São Paulo, São Paulo, Brazil

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First published: 20 August 2007
Citations: 7
Dr Antonio J. da Rocha, Rua Cincinato Braga 232 CEP 01333-010, Paraiso, São Paulo Brazil (tel.: +55 11 50146824;
fax: +55 11 50146805; e-mail: [email protected]).

Abstract

The superimposed clinical features of motor neuron disease (MND) and frontotemporal lobar degeneration (FTLD) comprise a rare neurological overlap syndrome that represents a diagnostic challenge to neurologists. Currently, FTLD-MND is considered a distinct entity and its clinicopathological basis has recently been reviewed. Our aim is to present a patient with MND and non-fluent rapidly progressive aphasia with clinical, imaging and histopathological correlation, as well as a brief review of the literature. We demonstrated the selective corticospinal tract (CST) and temporal lobe involvement using T1 spin-echo with an additional magnetization transfer contrast pulse on resonance (T1 SE/MTC) and FLAIR MR sequences in our patient, with further clinical and histopathological correlation. To the best of our knowledge, there is no description about the use of these particular MR sequences in the evaluation of FTLD-MND patients.

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