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Cortical myoclonus and cerebellar pathology
M A Tijssen1, M Thom, D W Ellison
1Institute of Neurology, London, UK.
Neurology
|April 4, 2000
Summary
Cortical myoclonus shows brain cortex dysfunction electrophysiologically, yet pathology reveals cerebellar degeneration. This suggests cerebellar issues may trigger cortical excitability in myoclonic ataxic syndrome.
Area of Science:
- Neuroscience
- Neurology
- Pathology
Background:
- Cortical myoclonus is often linked to cerebellar pathology, but antemortem physiological characterization with postmortem cerebellar findings is rare.
- Celiac disease is associated with myoclonic ataxic syndrome in some cases.
Observation:
- Electrophysiology revealed sensorimotor cortex dysfunction with enlarged somatosensory evoked potentials (SEPs) and a cortical potential preceding myoclonus.
- Neuropathology showed cerebellar degeneration, including Purkinje cell loss and Bergmann gliosis, particularly in the outer folial aspects.
- Cerebral cortices were largely unremarkable, with unilateral motor cortex gliosis in one patient.
Findings:
- Electrophysiological evidence points to sensorimotor cortex dysfunction in cortical myoclonus.
- Pathological examination revealed significant cerebellar degeneration, contrasting with minimal cerebral cortical abnormalities.
- Abnormal motor cortical inhibition was noted in one patient.
Implications:
- Cerebellar pathology may be the primary driver of sensorimotor cortex hyperexcitability in certain cortical myoclonus cases.
- Understanding this link could lead to new therapeutic targets for myoclonic ataxic syndrome, potentially involving the cerebellum.