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Propriospinal myoclonus in a child.

Omer Faruk Aydin¹, Cagri Mesut Temuçin, Ozlem Eroglu Kayacik

¹Department of Pediatric Neurology, Ondokuz Mayis University, Faculty of Medicine, Samsun, Turkey. ofaydin@yahoo.com

Journal of Child Neurology

|March 4, 2010

Summary

A pediatric patient experienced severe, involuntary jerks starting in infancy, diagnosed as propriospinal myoclonus. This condition involves massive jerks affecting limbs and trunk, often leading to falls.

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Area of Science:

Neurology
Pediatric Neurology
Movement Disorders

Background:

Propriospinal myoclonus is a rare disorder characterized by involuntary, massive jerks.
Onset in childhood can be associated with specific triggers or trauma.

Observation:

A 6-year-old girl presented with repetitive, involuntary jerks involving limbs and trunk, beginning around age 1.
Motor events included upper limb abduction, lower limb abduction, and head-body hyperextension.
Jerks progressively increased in frequency and severity, causing falls.

Findings:

Neurological examination was otherwise normal, with unremarkable laboratory tests and neuroimaging (EEG, MRI).
Electromyography revealed myoclonic jerk durations of 100-300 ms.
The characteristic spreading pattern from the thoracolumbar paraspinal muscles to the limbs suggested propriospinal myoclonus.

Implications:

This case highlights the diagnostic considerations for childhood-onset myoclonus.
Propriospinal myoclonus should be considered in the differential diagnosis of pediatric movement disorders with axial and limb involvement.
Early and accurate diagnosis is crucial for appropriate management and understanding of the condition's impact.