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Cerebrospinal fluid free choline in movement disorders of paediatric onset
M R Pranzatelli1, I Hanin, E Tate
1National Pediatric Myoclonus Center, Washington, DC 20004, USA.
Insights
Cerebrospinal fluid (CSF) choline levels were similar in most movement disorders but lowest in children with seizure disorders, including progressive myoclonus epilepsy. Reduced CSF choline may suggest altered brain phospholipid metabolism.
Area of Science:
- Neuroscience
- Biochemistry
- Metabolic Disorders
Background:
- Choline is essential for phospholipid synthesis and neurotransmission.
- Alterations in choline metabolism are implicated in various neurological conditions.
- Cerebrospinal fluid (CSF) choline serves as a potential biomarker for central nervous system metabolism.
Purpose of the Study:
- To investigate free choline concentrations in CSF of pediatric-onset movement disorder patients.
- To assess CSF choline as an index of central phospholipid metabolism in various neurological conditions.
- To explore relationships between CSF choline and specific movement and seizure disorders.
Main Methods:
- Measured free choline in CSF samples from 78 patients and controls.
- Included patients with myoclonic disorders, other movement disorders, seizure disorders, and controls.
- Analyzed CSF choline levels in relation to patient age, gender, and sample storage conditions.
Main Results:
- No significant differences in CSF choline were found in myoclonic or other movement disorders compared to controls.
- CSF choline levels were lowest in children with seizure disorders, including progressive myoclonus epilepsy.
- In progressive myoclonus epilepsy, CSF choline resembled levels in other epileptic disorders, not other myoclonic disorders.
Conclusions:
- CSF choline levels do not significantly differ in most movement disorders compared to controls.
- Reduced CSF choline in seizure disorders may indicate altered choline metabolism or incorporation into brain phospholipids.
- Further developmental studies are warranted to explore the biphasic relationship between age and CSF choline.
Abstract:
We measured free choline in cerebrospinal fluid (CSF) of 78 patients with movement disorders of paediatric onset and various controls as a putative index of central phospholipid metabolism. Most of the disorders studied were myoclonic disorders, such as progressive myoclonus epilepsy, the opsoclonus-myoclonus syndrome, and essential myoclonus, but other movement disorders, interictal seizure disorders, and different neurological and nonneurological disorders were also included. There were no significant differences in CSF choline concentrations in myoclonic disorders or other movement disorders compared with controls. The CSF choline levels were lowest in children with seizure disorders including progressive myoclonus epilepsy. In progressive myoclonus epilepsy, the CSF choline values resembled other epileptic disorders rather than other myoclonic disorders. When all the data were analysed collectively, no significant relation of CSF choline was found to patient age, gender, aliquot of CSF measured, or the length of time the sample was stored at -70 degrees C. Separate analyses of data from children and adults showed a trend toward a biphasic relation between patient age and CSF choline which could be pursued in developmental studies of normal subjects. Reduced CSF choline may indicate increased choline incorporation into brain phospholipids, disturbances of choline metabolism, decreased choline release, or non-neural factors.
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