51 y/o male with ataxia,bradykinesia,scanning speech,rigidity&spasticity

 

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Doctor's Information

Name : Farzad
Family : Ashrafi
Affiliation :Neurology Department,Shohada Tajrish Hospital,SBMU
Academic Degree : Associate Professor of Neurology
Email : This email address is being protected from spambots. You need JavaScript enabled to view it.
Resident : rahebe jafarinia

 

Case Section

Neuroradiology

 

Patient's Information

Gender : Male
Age : 51

 

Clinical Summary

a 51 y/o male with ataxia,bradykinesia,scanning speech,rigidity,spasticity.

 

Imaging Findings

there is cerebellar,midbrain,pons atrophy.hot cross bun sign is present in axial T2 image as a cross on atrophied pons.mickymouse sign is seen in axial T2 image in midbrain.humming bird sign is seen in sagital T2 image related to flattening of superior aspect of midbrain.

 

Differential Diagnosis

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Final Diagnosis

PSP+olivopontocerebellar atrophy

 

Discussion (Related Text)

Olivopontocerebellar degeneration (OPCD) is a neurodegenerative disease, and one of the clinical manifestations of multiple systemic atrophy (MSA).
Olivopontocerebellar degeneration presents predominantly with cerebellar and brainstem symptoms and signs. Typically ataxia and bulbar dysfunction are pronounced.
In MRI There is disproportionate atrophy of the cerebellum and brainstem (especially olivary nuclei and middle cerebellar peduncle)
The hot cross bun sign refers to the MRI appearance of the pons in a variety of neurodegenerative diseases.
T2 hyperintensity forms a cross on axial images through the pons, representing selective degeneration of pontocerebellar tracts.
Progressive supranuclear palsy (PSP), also known as the Steele-Richardson-Olszewski syndrome, is a neurodegenerative disease with no efficacious treatment.
Progressive supranuclear palsy is characterised by decreased cognition, abnormal eye movements (supranuclear vertical gaze palsy), postural instability and falls, as well as parkinsonian features and speech disturbances In MRI , feature can usually at most be suggestive of the diagnosis include:
•midbrain atrophy
◦reduction of anteroposterior midline midbrain diameter, at the level of the superior colliculi on axial imaging (from interpeduncular fossa, to the intercolicular groove: < 12mm 8) : which can give a mickey mouse appearance
◦reduced area of the midbrain on midline sagittal and reduced midbrain to pons area ratio: approx 0.12 (normal approx 0.24) on midline sagittal
◦Loss of the lateral convex margin of the tegmentum of midbrain has been described as the morning glory sign
◦hummingbird sign also known as the penguin sign. The key is a flattening or concave outline to the superior aspect of the midbrain which should be upwardly convex
•T2 : diffuse high-signal lesions in :
◦pontine tegmentum
◦tectum of the midbrain
◦inferior olivary nucleus

 

References

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