Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2015 Apr-Jun;18(2):249-51.
doi: 10.4103/0972-2327.150624.

Fasciculations masquerading as minipolymyoclonus in bulbospinal muscular atrophy

Sushanth Bhat  1 , Wei Ma  1 , Elena Kozochonok  1 , Sudhansu Chokroverty  1
Affiliations
Case Reports

Fasciculations masquerading as minipolymyoclonus in bulbospinal muscular atrophy

Sushanth Bhat et al. Ann Indian Acad Neurol. 2015 Apr-Jun.

Abstract

Minipolymyoclonus has been described in both anterior horn cell disorders and central nervous system degenerative conditions. While its etiology remains unclear and speculative, a central generator has been previously proposed. We describe a case of bulbospinal muscular atrophy (Kennedy's disease), where minipolymyoclonus-like movements corresponded to fasciculations in neurophysiological studies. Our novel finding suggests that the etiologies of minipolymyoclonus in central and peripheral nervous system disorders are distinct, despite outward clinical similarity. The term "minipolyfasciculations" may be more reflective of the underlying process causing minipolymyoclonus-like movements in lower motor neuron disorders.

Keywords: Bulbospinal muscular atrophy; EMG-EEG back averaging; Kennedy's disease; minipolymyoclonus; polyminimyoclonus.

PubMed Disclaimer

Conflict of interest statement

Conflict of Interest: None declared.

Figures

Figure 1
Figure 1
One-second polymyographic recording from our patient. Note simultaneous occurrence of fasciculations in needle electromyography (EMG) channels (1, 4) and muscle bursts in the surface EMG channels (2, 5) in muscles where both were performed concomitantly. Other channels have irregular, asynchronous discharges, the right extensor digitorum brevis surface EMG channel (R EDB-S) shows relative quiescence. An “S” suffix represents a surface channel, and an “ N” suffix represents a needle EMG channel. FDI, first dorsal interosseous APB = Abductor pollicis brevis, EDC = Extensor digitorum communis
See this image and copyright information in PMC

References

    1. Spiro A. Minipolymyoclonus. A neglected sign in childhood spinal muscular atrophy. Neurology. 1970;20:1124–6.
    1. Dubowitz V. Nonprogressive neurogenic muscular atrophy with ‘voluntary fasciculation’. Proc R Soc Med. 1964;57:117–8. - PMC - PubMed
    1. Misra UK, Kalita J, Mishra VN, Kesari A, Mittal B. A clinical, magnetic resonance imaging, and survival motor neuron gene deletion study of Hirayama disease. Arch Neurol. 2005;62:120–3. - PubMed
    1. Colamaria V, Zanetti R, Simeone M, Tomelleri G, Orrico D, Dordi B, et al. Minipolymyoclonus in congenital nemaline myopathy: A nonspecific clinical marker of neurogenic dysfunction. Brain Dev. 1991;13:358–62. - PubMed
    1. Nogués MA, Leiguarda RC, Rivero AD, Salvat F, Manes F. Involuntary movements and abnormal spontaneous EMG activity in syringomyelia and syringobulbia. Neurology. 1999;52:823–34. - PubMed

Publication types