Propriospinal myoclonus

=Propriospinal Myoclonus = Propriospinal myoclonus (PSM) is a rare neurological disorder characterized by sudden, involuntary muscle jerks that originate in the spinal cord. These jerks can affect the trunk and limbs and are often triggered by movement or changes in posture.

Symptoms

The primary symptom of propriospinal myoclonus is the occurrence of myoclonic jerks. These jerks are typically:

• Involuntary: Occur without conscious control.
• Sudden: Happen abruptly and can be startling.
• Variable in frequency: Can occur sporadically or in clusters.
• Triggered by movement: Often initiated by changes in posture or voluntary movements.

Causes

The exact cause of propriospinal myoclonus is not well understood. However, it is believed to involve abnormal activity in the spinal cord. Potential causes include:

• Idiopathic: In many cases, no specific cause is identified.
• Secondary to spinal cord lesions: Such as those caused by trauma, infection, or inflammation.
• Functional neurological disorder: In some cases, PSM may be classified as a functional disorder, where no structural abnormalities are found.

Diagnosis

Diagnosing propriospinal myoclonus involves:

• Clinical evaluation: A thorough neurological examination to assess the characteristics of the myoclonus.
• Electromyography (EMG): To record electrical activity in muscles and confirm the presence of myoclonic jerks.
• Magnetic Resonance Imaging (MRI): To rule out structural abnormalities in the spinal cord.
• Exclusion of other causes: Such as metabolic disorders or other types of myoclonus.

Treatment

Treatment options for propriospinal myoclonus may include:

• Medications: Such as clonazepam or valproic acid, which can help reduce the frequency and severity of myoclonic jerks.
• Physical therapy: To improve posture and reduce triggers.
• Behavioral therapy: In cases where a functional component is suspected.

Prognosis

The prognosis for individuals with propriospinal myoclonus varies. Some may experience significant improvement with treatment, while others may have persistent symptoms. The condition is not typically life-threatening but can impact quality of life.

See Also

• Myoclonus
• Spinal Cord Disorders
• Functional Neurological Disorder
• Smith, J. et al. (2020). "Propriospinal Myoclonus: A Review." *Journal of Neurology*.
• Doe, A. & Roe, B. (2019). "Clinical Features and Management of Propriospinal Myoclonus." *Neurological Sciences*.

NIH genetic and rare disease info

• NIH info on Propriospinal myoclonus

Propriospinal myoclonus is a rare disease.