Amyotrophic lateral sclerosis

Case contributed by Yuliia Solodovnikova
Diagnosis certain

Presentation

The patient presented with a 2-year history of progression of combined tetraparesis and severe pseudobulbar palsy.

Patient Data

Age: 50 years
Gender: Male
mri
This study is a stack
Sagittal
FLAIR
This study is a stack
Axial
FLAIR
This study is a stack
Coronal
FLAIR
This study is a stack
Sagittal
DTI
This study is a stack
Coronal
DTI
This study is a stack
Axial
DTI
This study is a stack
DST-3D
DTI
Sagittal
FiberTractography DTI
Coronal
FiberTractography DTI
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Info

FLAIR sequences reveal hyperintensities of the corticospinal tracts (CST) bilaterally ("wine glass" sign).

Fractional anisotropy (FA) along the right CST is reduced.

There is an apparent diffusion coefficient (ADC) elevation at the brainstem level (cerebral peduncles and medulla) within the right CST and at the level of the centrum semiovale and medulla within the left one.

Case Discussion

Atypical values of FA and ADC indicate microstructural changes resulting from a reduction in neurone count and myelin volume within the corticospinal tracts.

The presence of the 'wine glass' sign is a common occurrence among patients with CST involvement.

NCS/EMG results revealed indications of motor neurone disease.

Based on these factors and the patient's clinical presentation, the diagnosis of amyotrophic lateral sclerosis was confirmed.

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