Related Experiment Videos
Presynaptic and postsynaptic striatal dopaminergic function in neuroacanthocytosis: a positron emission tomographic
D J Brooks1, V Ibanez, E D Playford
1MRC Cyclotron Unit, Hammersmith Hospital, London, England.
Annals of Neurology
|August 1, 1991
Summary
Neuroacanthocytosis patients show reduced dopamine storage and D2-receptor loss in the posterior putamen, similar to Parkinson's disease. This explains chorea and rigidity seen in neuroacanthocytosis.
Area of Science:
- Neuroscience
- Radiology
- Neurology
Background:
- Neuroacanthocytosis is a rare genetic disorder characterized by neurological symptoms and red blood cell abnormalities.
- Patients often present with chorea, seizures, dementia, and axonal neuropathy.
Purpose of the Study:
- To assess striatal dopamine storage, dopamine D2-receptor integrity, and regional cerebral blood flow in neuroacanthocytosis patients.
- To compare these findings with normal controls and Parkinson's disease patients.
Main Methods:
- Positron emission tomography (PET) using [18F]dopa, [11C]raclopride, and C15O2.
- Assessment of striatal dopamine uptake, D2-receptor binding, and cerebral blood flow.
- Comparison with 30 healthy controls and 16 Parkinson's disease patients.
Main Results:
- Neuroacanthocytosis patients exhibited normal caudate and anterior putamen [18F]dopa uptake.
- Posterior putamen [18F]dopa uptake was reduced to 42% of normal, similar to Parkinson's disease.
- Significant reductions in striatal D2-receptor binding sites (caudate: 65%, putamen: 53%) and depressed striatal/frontal blood flow were observed.
Conclusions:
- Severe loss of D2-receptor-bearing striatal neurons and dopaminergic projections contribute to chorea and rigidity in neuroacanthocytosis.
- PET imaging reveals distinct patterns of dopaminergic dysfunction in neuroacanthocytosis, overlapping with Parkinson's disease in specific regions.