Spinal myoclonus with dermal and retinal changes affected by myelitis

Area of Science:

• Neurology
• Neurophysiology

Background:

• Chronic intermittent myoclonus present for over 50 years in a 68-year-old male.
• Patient presented with acute transverse myelitis and subsequent paraparesis.

Observation:

• Myoclonus frequency significantly increased during acute transverse myelitis.
• Myoclonic movements were readily induced by urinary bladder irrigation during paraparesis.

Findings:

• Hypothesized mechanism involves removal of inhibitory influences on a spinal cord myoclonic focus, leading to heightened excitability.
• Direct irritation of the myoclonic focus by the inflammatory process is also considered.

Implications:

• Findings suggest a link between spinal cord inflammation and exacerbation of pre-existing myoclonus.
• The patient's history of keratosis palmoplantaris hereditaria and retinal pigment degeneration suggests a potential underlying congenital neuroectodermal dysplasia influencing neurological conditions.