Detection of corticospinal tract compromise in amyotrophic lateral sclerosis with brain MR imaging: relevance of the T1-weighted spin-echo magnetization transfer contrast sequence.

BACKGROUND AND PURPOSE: Hyperintensity in the posterior limb of the internal capsule at T2-weighted MR imaging, consistent with corticospinal tract (CST) degeneration, is described in amyotrophic lateral sclerosis (ALS). However, the lack of specific tests or biological markers hinders confirmation of the diagnosis, especially in the early stages. We investigated the CST in ALS with MR imaging. METHODS: We examined 25 patients (14 men, 11 women; mean age, 49.1 years; range, 29-68 years) and 21 age- and sex-matched control subjects without upper motor neuron signs. According to the revised El Escorial criteria, 22 patients had definite ALS; two, probable ALS; and one, suspected ALS. Fluid-attenuated inversion recovery (FLAIR; TR/TE/TI, 11,000/140/2600) and T1-weighted spin-echo (SE)/magnetization transfer contrast-enhanced (MTC; TR/TE, 510/12) imaging was performed at 1 T. Two experienced neuroradiologists blinded to the patients' history independently evaluated the CST. RESULTS: T1-weighted SE MTC imaging allowed visualization of the CST in both patients and control subjects. T1-weighted SE MTC images showed hypointensity along the CST and bilateral subcortical regions of the precentral gyri in all control subjects and hyperintensity in 80% of patients with ALS (P < .05). FLAIR images showed hyperintensity in these areas in both groups, with no significant difference. CONCLUSION: T1-weighted SE MTC imaging is sensitive and accurate in depicting CST lesions in ALS, whereas FLAIR imaging is not. T1-weighted SE MTC imaging is useful in diagnosing ALS by showing hyperintense areas along the CST, which separates patients from control subjects. This sequence should be included in the workup of patients with weakness and pyramidal signs.

Validation of the Amyotrophic Lateral Sclerosis Assessment Questionnaire (ALSAQ-40) scale in the Portuguese language.

The amyotrophic lateral sclerosis (ALS) is a degenerative neurological disorder that has a great impact in the quality of life of the patients. This study had the objective of validating the ALS Assessment Questionnaire in the Portuguese Language (ALSAQ-40/BR). The version of ALSAQ-40/BR, was adapted into the Portuguese language after the evaluation and re-evaluation of 20 patients with a defined ALS diagnosis. The demonstration of its reproducibility and reliability makes this instrument an additional and useful parameter which can be used in the evaluation of ALS for research or assistance.

Validation of the Amyotrophic Lateral Sclerosis Assessment Questionnaire (ALSAQ-40) scale in the portuguese language / Validação da escala ALSAQ-40 em pacientes com esclerose lateral amiotrófica para a língua portuguesa

The amyotrophic lateral sclerosis (ALS) is a degenerative neurological disorder that has a great impact in the quality of life of the patients. This study had the objective of validating the ALS Assessment Questionnaire in the Portuguese Language (ALSAQ-40/BR). The version of ALSAQ-40/BR, was adapted into the Portuguese language after the evaluation and re-evaluation of 20 patients with a defined ALS diagnosis. The demonstration of its reproducibility and reliability makes this instrument an additional and useful parameter which can be used in the evaluation of ALS for research or assistance.

Esclerose lateral amiotrófica, doença neurológica degenerativa, apresenta grande impacto na qualidade de vida dos pacientes. Este estudo teve como objetivo realizar a adaptação transcultural e validação da escala Amyotrophic Lateral Sclerosis Assessment Questionnaire (ALSAQ-40) nestes pacientes. Foi aplicada em 20 pacientes com reteste após 30 dias. A tradução para o português e sua adequação às condições socioeconômicas e culturais da nossa população, bem como a demonstração de sua reprodutibilidade e validade, tornam este instrumento um parâmetro adicional útil que pode ser utilizado na avaliação da ELA seja em nível de pesquisa ou assistencial.

Adaptação transcultural do questionário de avaliação da esclerose lateral amiotrófica (ALSAQ-40) na cultura e linguagem brasileira / Cross-cultural of the amyothrophic lateral sclerosis assessment questionnaire (ALSAQ-40) scale in Brazilian culture and language

A esclerose lateral amiotrófica (ELA) é uma doença neurológica degenerativa que apresenta grande impacto na qualidade de vida dos pacientes. Este estudo teve como objetivo realizar a adaptação transcultural do Questionário de Avaliação da ELA para língua portuguesa (ALSQ-40/BR). Foi realizada tradução para o português e sua adequação às condições socioeconômicas e culturais da nossa população. O ALSAQ-40/BR foi aplicado em 20 pacientes com reteste após 30 dias. A adaptação do ALSAQ-40/BR torna este instrumento um parâmetro adicional, útil para diferentes pesquisas e/ou assistência de pacientes com ELA.