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Cerebral Subcortical Neurodegeneration
A 66-year-old woman presenting with left-sided motor deficits, including increased tone and loss of dexterity. PET, positron emission tomography.
A 59-year-old woman presenting with asymmetrically increased tone and bradykinesia on the left side. MRI, magnetic resonance imaging; PET, positron emission tomography.
A 62-year-old woman presenting with ataxia, dysarthria, saccadic breakdown of smooth visual pursuit, appendicular ataxia, and orthostasis. MRI, magnetic resonance imaging; PET, positron emission tomography.
A 48-year-old woman presenting with ataxia, dysarthria, and labile emotions, including crying disproportionate to perception of sadness. MRI, magnetic resonance imaging.
DESCRIPTION OF FINDINGS
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Case A: A brain FDG-PET examination shows cortical hypometabolism in the right frontal lobe that is most pronounced in the perirolandic region (arrow). Additionally, hypometabolism is present in the right thalamus and, to a lesser extent, in the right striatum. The relative hypometabolism in the left cerebellum represents crossed cerebellar diaschisis related to the right frontal lobe abnormality.
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Case B: A brain FDG-PET examination shows marked hypometabolism involving the right striatum (arrow), corresponding to atrophy in these nuclei seen on the axial T2 MRI (arrow). Note that the right and left thalami are approximately normal and symmetric in size and activity.
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Case C: A sagittal T2 MRI shows marked atrophy involving the midbrain, pons, and cerebellum. Brain FDG-PET shows corresponding marked and diffuse hypometabolism in the cerebellum.
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Case D: Sagittal T1 and axial T2 MRI shows marked cerebellar atrophy and a normal appearance of the brainstem and cervical spinal cord.
Diagnosis
CaseA
Corticobasal degeneration
CaseB
Multiple systems atrophy—Parkinson type (MSA-P, striatonigral degeneration)
CaseC
Multiple systems atrophy—cerebellar type (MSA-C, olivopontocerebellar degeneration)
CaseD
Spinocerebellar ataxia (SCA)
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