Unilateral absence of swallow-tail sign in Parkinson disease

Case contributed by Dr Bárbara Trapp

Presentation

Progressive bradykinesia, tremor at rest, rigidity and postural instability since 2011, with more severe clinical manifestations on the right side.

Patient Data

Age: 75
Gender: Male

Enlarged high resolution multi-echo SWI/3D 3T MRI series of a Parkinson's disease patient showing unilateral absence of the hyperintense area on the dorso-lateral substantia nigra (nigrosome-1) on the left (absence of the swallow-tail sign on the left). Normal swallow-tail sign on the right. 

 

Absent swallow tail sign on the left - high resolution multi-echo SWI/3D 3T MRI of a Parkinson's disease patient showing unilateral absence of the normal high SWI signal within the nigrosome-1 on the left. Normal Swallow-tail sign on the right. 

The nigrosome is identified on magnetic resonance imaging as a SWI (susceptibility-weighted imaging) hyperintense area within the dorso-lateral substantia nigra. Enlarged high-resolution multi-echo SWI/3D 3T MRI through midbrain showing unilateral left swallow-tail sign absence. Normal swallow-tail sign on the right.

Illustrated diagrams of cross-sectional midbrain.

Case Discussion

Parkinson's disease (PD) is a progressive neurodegenerative disorder characterized by motor and non-motor impairment. Its diagnosis is quite challenging, based on the clinical evaluation of bradykinesia, tremor at rest, rigidity or postural instability. As often the clinical diagnosis can not be fully defined, there is a constant search for biomarkers capable of assisting in the identification of these patients.
Recently, it has been proposed the detection of nigrosome-1 as a biomarker in the diagnosis of the disease.

The nigrosome-1 constitutes the area with the highest concentration of dopaminergic neurons in the dorso-lateral mesencephalic substantia nigra. In normal individuals, the nigrosome is identified on magnetic resonance imaging as a SWI (susceptibility-weighted imaging) hyperintense area within the dorso-lateral substantia nigra, called swallow-tail sign.

As demonstrated by histopathological studies, patients with PD present depletion of nigrosome-1 dopaminergic neurons of substance nigra, which manifests as absence of the typical hypersignal of nigrosome-1 in dorsolateral substance nigra, in SWI-weighted sequences (loss of the swallow -tail sign). This finding has shown to be very promising in the diagnosis of PD, with high sensitivity, specificity and high positive and negative predictive values.

Depletion of nigrosome-1 dopaminergic neurons in PD may occur bilaterally or involve only one side. Some studies have shown that asymmetric depletion of nigrosome-1 neurons is also associated with more severe clinical manifestations on the contralateral side, showing high concordance of the laterality of the nigrosome-1 at 3T MRI and the clinical laterality.

In this way, the loss of swallow-tail sign seems not only to present good diagnostic properties, but also seems to determine clinical manifestations characteristics of PD.

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Case information

rID: 58513
Published: 26th Feb 2018
Last edited: 10th May 2018
System: Head & Neck
Inclusion in quiz mode: Included
Institution: Clínica Villas Boas

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