A Case and Cranial MRI Characteristics of Progressive Supranuclear Palsy

Volume 2 - Issue 1

Asuman Ali¹, Cemile Haki², Oğuzhan Gümüştaş³*, Ersin Budak⁴ and Ramazan Yalçın⁵

Received: December 05, 2018;   Published: December 13, 2018

DOI: 10.32474/TOOAJ.2018.02.000128

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Abstract

Progressive supranuclear palsy (PSP) is a neurodegenerative disease described by vertical supranuclear gaze paralysis, axial rigidity and postural instability involving falls in the early stage of the disease. Imaging studies present brain atrophy in especially midbrain, premotor cortex, basal ganglia and thalamus. There is no bioindicator of the disease although it is clinically identified. PSP is also classified as tauopathy. However, no specific indicator for tau protein could be identified. Hence, imaging findings, especially midbrain atrophy, functions as a biomarker in clinical practice and determination of disease prognosis. It has been shown that midbrain atrophy has a diagnostic value for PSP accompanied with the finding of “hummingbird” or “penguin” encountered by magnetic resonance imaging (MRI).

In this case report, we have aimed to review the diagnostic features of the disease based on the results of clinical and radiological results and to demonstrate the “penguin” sign and midbrain atrophy as a biomarker for clinical follow-up process. We have also detected a correlation with the results of clinical and neuropsychological tests.

We have analyzed a 61-year-old male patient who was a retired teacher for the complaints of progressive difficulty in walking since the recent year, slowness in speech fluency, forgetfulness and easy falling. Our patient was performed 1.5 Tesla Cranial MRI study due to the signs of postural instability, easy falling backward while walking, cognitive impairment, apathia, conjugate vertical gaze palsy, disinhibition and anxiety. T1W, T2W, T2 flair and diffusion sections on the axial plane were obtained in this imaging study. In addition, Mini-Mental State Examination and neuropsychological test were also performed in our patient.

The patient had particularly diffuse cerebral atrophy in predominantly frontal and parietal lobes. Peripheral CSF spaces were enlarged due to atrophy. Brainstem also showed atrophy and particularly mesencephalon atrophy was more remarkable. The appearance of brainstem in the sagittal slices due to mesencephalon atrophy were termed as “penguin” sign. A mild atrophy was also encountered in the bilateral cerebellar hemispheres. As a result of neuropsychological evaluation, mild to moderate cognitive impairment were detected.

We have evaluated with this case that atrophy of midbrain and mesencephalon which presented a significant visuality with classic “penguin” sign has an important diagnostic value in the PSP cases. Additionally, we have reviewed the fact in the light of literature data that imaging of midbrain atrophy may play a role as a biomarker in performing clinical follow-up and determination of the prognostic features of the PSP cases.

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