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Myoclonus and Startle Syndromes

  • Roongroj BhidayasiriEmail author
  • Onanong Jitkritsadakul
Chapter

Abstract

The term “myoclonus” is used to identify brief and jerky involuntary movements, originating from brief active contractions of muscles (positive myoclonus) or brief interruptions of ongoing electromyographic activities (negative myoclonus). Myoclonic movements can have variable clinical manifestations, which make it difficult for practicing neurologists to develop an organized framework for the diagnosis and treatment of the underlying disorder. This chapter is structured as if physicians encounter patients who primarily present with myoclonus. A step-by-step approach is described starting from the phenomenology identification, followed by a classification, a diagnostic approach, examples of selected myoclonic syndromes, investigations, and concludes with a concise review of therapeutic options for myoclonus. Video clips are provided to illustrate salient clinical features associated with common myoclonic disorders. Tables are included as a summary of each section, which emphasize important useful clinical points for physicians. A recommended reading list is intended for readers who would like to expand their knowledge on certain topics.

Keywords

Myoclonus Jerks Asterixis Postural lapses Reticular reflex myoclonus Myoclonus-dystonia Propriospinal myoclonus Psychogenic myoclonus 

Supplementary material

Video 30.1

A 50-year-old man with hepatic encephalopathy exhibited flapping tremor of both hands, predominantly observed when both arms are outstretched (MOV 1963 kb)

Video 30.2

A 55-year-old man developed bouncing gait 1 month after a hypoxic event following cardiorespiratory arrest. He needed assistance with standing. After standing for a few seconds, the patient exhibited jerky movements of the trunk and both legs. This phenomenon has been termed bouncing gait, which indicates the presence of negative myoclonus of the axial muscles (MOV 3867 kb)

309600_1_En_30_MOESM3_ESM.mov (2.5 mb)
Video 30.3 A 46-year-old woman developed a 6-month history of intermittent twitching of the right facial muscles. Although it is called spasm, the movements are indeed myoclonus, characterized by unilateral (right in this case), involuntary, irregular, clonic, or tonic movements of muscles innervated by the right facial nerve. In this patient, the spasms started as twitching of the lower eyelid, followed by involvement of other periorbital, facial, and platysma muscles (MOV 2591 kb)
Video 30.4

The patient exhibited regular clonic muscular twitching of the right arm for the past three days. The movements were persistent throughout the observed duration. Mild right hemiparesis was also observed (MOV 15010 kb)

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Recommended Reading List

  1. Bhidayasiri R, Truong DD. Startle syndromes. Handb Clin Neurol. 2011;100:421–30.CrossRefPubMedGoogle Scholar
  2. Caviness JN. Treatment of myoclonus. Neurotherapeutics. 2014;11(1):188–200. Pubmed Central PMCID: 3899494.CrossRefPubMedGoogle Scholar
  3. Caviness JN, Brown P. Myoclonus: current concepts and recent advances. Lancet Neurol. 2004;3(10):598–607.CrossRefPubMedGoogle Scholar
  4. Cockerell OC, Rothwell J, Thompson PD, Marsden CD, Shorvon SD. Clinical and physiological features of epilepsia partialis continua. Cases ascertained in the UK. Brain. 1996;119(Pt 2):393–407.CrossRefPubMedGoogle Scholar
  5. Shibasaki H. Neurophysiological classification of myoclonus. Neurophysiol Clin. 2006;36(5–6):267–9.CrossRefPubMedGoogle Scholar
  6. van der Salm SM, Erro R, Cordivari C, Edwards MJ, Koelman JH, Ende TV, et al. Propriospinal myoclonus: clinical reappraisal and review of literature. Neurology. 2014;83(20):1862–70.Google Scholar

Copyright information

© Springer-Verlag Wien 2017

Authors and Affiliations

  1. 1.Chulalongkorn Centre of Excellence for Parkinson’s Disease and Related Disorders, Chulalongkorn University and King Chulalongkorn Memorial Hospital, Thai Red Cross SocietyBangkokThailand
  2. 2.Department of Rehabilitation Medicine, Faculty of MedicineJuntendo UniversityTokyoJapan
  3. 3.Department of Medicine, Faculty of MedicineChulalongkorn Center of Excellence for Parkinson’s Disease and Related Disorders, Chulalongkorn University and King Chulalongkorn Memorial Hospital, Thai Red Cross SocietyBangkokThailand

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