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[Propriospinal myoclonus--a case report]

K Nishiyama¹, Y Ugawa, K Takeda

¹Department of Neurology, Japanese Red Cross Medical Center.

Rinsho Shinkeigaku = Clinical Neurology

|July 1, 1993

Summary

This study details a case of propriospinal myoclonus, a spinal cord disorder causing involuntary trunk jerks. Electrophysiological findings confirmed axial muscle involvement without central nervous system abnormalities.

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Area of Science:

Neurology
Neurophysiology

Background:

Propriospinal myoclonus is a rare movement disorder characterized by involuntary jerks originating in the spinal cord.
Detailed electrophysiological findings are crucial for understanding its pathophysiology.

Observation:

A 23-year-old woman presented with involuntary, irregular flexion jerks of the trunk affecting bilateral axial muscles (sternocleidomastoid, paravertebral, abdominal, intercostal).
Jerks were exacerbated by mental stress, absent during sleep, and not associated with pain or hiccups.
Routine examinations, including MRI, showed no abnormalities.

Findings:

Polymyography confirmed synchronous activation of bilateral axial muscles, with EMG burst durations of 50-250 ms.
Jerk-locked averaging (JLA) and movement-related cerebral potential (MRCP) studies did not reveal preceding EEG activity, suggesting a non-cortical origin.
Absence of MRCP indicated the jerks were not self-initiated voluntary movements.

Implications:

This case highlights the utility of comprehensive electrophysiological studies in diagnosing propriospinal myoclonus.
Findings support the hypothesis of a spinal cord origin for this type of myoclonus.
Further research into spinal cord circuitry is warranted to elucidate the mechanisms of propriospinal myoclonus.

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