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Arquivos de Neuro-Psiquiatria

Print version ISSN 0004-282X

Arq. Neuro-Psiquiatr. vol.72 no.11 São Paulo Nov. 2014 

Images in Neurology

Anterior temporal white matter lesions in adult-form myotonic dystrophy type 1

Lesões temporais anteriores da substância branca na forma adulta da distrofia miotônica tipo 1

Leonardo Ferreira Caixeta1 

Giane Souza Reis2 

Ana Caroline Marques Vilela3 

1Departamento de Neurologia, Faculdade de Medicina, Universidade Federal de Goiás, Goiânia GO, Brazil

2Departamento de Neurologia, Universidade Federal de Goiás, Goiânia GO, Brazil

3Universidade Federal de Goiás, Goiânia GO, Brazil

A 57-year-old woman presented with a 4-years history of progressive weakness, distal muscular atrophy and myotonia in left hand (Figure 1). Her electromyography had a myotonic pattern. Patient was first diagnosed as paraneoplasic limbic encephalitis based on her brain MRI (Figure 2). Diagnosis was genetically confirmed for myotonic dystrophy type 1 (DM1).

Figure 1  Dystrophy and sequence of myotonia in left hand. 

Figure 2  MRI (T2 weight coronal and axial slices) showing bilateral anterior temporal hypersignal in our case. Notice that mesial temporo-limbic structures are not affected. 

DM1 or Steinert's disease is an autosomal-dominant disorder characterized by muscle weakness and unusual features, compared with other dystrophies, including myotonia, anticipation, and multiple organ involvement1,2. Anterior temporal lobe subcortical white matter lesions are described in DM1, but not in DM2 patients3. Limbic encephalitis and CADASIL are the most important imaging differential diagnosis.


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. Kornblum C, Reul J, Kress W, Grothe C, Amanatidis N, Klockgether T et al. Cranial magnetic resonance imaging in genetically proven myotonic dystrophy type 1 and 2. J Neurol. 2004;251(6):710-4. http://10.1007/s00415-004-0408-1 [ Links ]

Received: April 02, 2014; Revised: July 22, 2014; Accepted: August 01, 2014

Correspondence: Leonardo Ferreira Caixeta; Avenida Cristo Rei 626, Setor Jaó; 74674-290 Goiânia GO, Brasil; E-mail:

Conflict of interest: There is no conflict of interest to declare.

Creative Commons License This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.