Polyminimyoclonus.pptx

Polyminimyoclonus
Minipolymyoclonus

1. Polyminimyoclonus Ade Wijaya , MD October 2022

2. Introduction Polyminimyoclonus = minipolymyoclonus Was first used by Dennis Giblin and then described by Alfred J. Spiro  at 1970 Hyperkinetic movement disorder Affect hands – several fingers Visible and palpable movements Mostly noticed while the individual is maintaining a posture (commonly outstretched hands) or during action (especially the initial phase of movement)  Spiro AJ. Minipolymyoclonus : a neglected sign in childhood spinal muscular atrophy.  Neurology.  1970;20:1124–1126

3. Semiology  Spiro AJ. Minipolymyoclonus : a neglected sign in childhood spinal muscular atrophy.  Neurology.  1970;20:1124–1126

4. Pathophysiology Motor cortex Anterior horn cells Possible role of brainstem, cerebellum, and basal ganglia Ganguly J, Chai JR, Jog M. Minipolymyoclonus : a critical appraisal. Journal of Movement Disorders. 2021 May;14(2):114.

5. Peripheral Variants Spinal muscular atrophy (SMA) Brachial monomelic amyotrophy Bulbospinal muscular atrophy Syringomyelia (secondary AHC involvement) Congenital nemaline myopathy anti-CASPR2-associated paraneoplastic Morvan syndrome Ganguly J, Chai JR, Jog M. Minipolymyoclonus : a critical appraisal. Journal of Movement Disorders. 2021 May;14(2):114.

6. Central Variants Multisystem atrophy parkinsonian type (MSA-P) Alzheimer’s disease Parkinson’s disease Corticobasal degeneration Epileptic disorders such as Lennox- Gastaut syndrome, progressive myoclonic epilepsy, absence seizure Down’s syndrome Cerebral palsy. Ganguly J, Chai JR, Jog M. Minipolymyoclonus : a critical appraisal. Journal of Movement Disorders. 2021 May;14(2):114.

7. Differentiate: central or peripheral ? Peripheral: denervation Central: parkinsonism or epilepsy symptoms EEG – EMG Ganguly J, Chai JR, Jog M. Minipolymyoclonus : a critical appraisal. Journal of Movement Disorders. 2021 May;14(2):114.

8. Summary Intriguing clinical phenomenology of central or peripheral origin Hyperkinetic movement disorder Intermittent; low amplitude, arrhytmic Affect hands or fingers ; visible/palpable On outstreched hands or during actions

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