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Auditory and emotion-sensitive jerks in a patient with subacute sclerosing panencephalitis Volume 23, issue 6, December 2021

TEST YOURSELF

(1) What are the common causes of stimulus-sensitive myoclonus?

 

(2) Can SSPE present with stimulus-sensitive jerks?

 

(3) When can SSPE present with stimulus-sensitive myoclonus?

 

 

 

 

 

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Answers

(1) The common causes of stimulus-sensitive myoclonus are progressive myoclonic epilepsy, which includes Lafora body disease, neuronal ceroid lipofuscinoses (NCL), Unverricht-Lundborg disease (ULD), myoclonic epilepsy with ragged-red fiber syndrome and other mitochondrial disorders, sialidoses, dentate-rubro-pallidal atrophy (DRPLA), Tay-Sachs disease (TSD) and inborn errors of metabolism.

 

(2)Yes, it is a rare cause ofstimulus-sensitive jerks.

 

(3) Sensory stimuli delivered to the mantle area of the cerebral cortex may evoke SSPE complexes, especially when the rhythmic pattern is unstable (or in the early stages of disease), and after establishment of the periodic pattern, the EEG complexes and motor accompaniments remain unaffected by external stimuli.

 

 

 

 

 

 

 

 

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