Spasticity / Movement Disorders
Akhil Golla, MD
PMR resident
Moss Rehab
Bridgewater, New Jersey, United States
Kimberly Heckert, MD
Physician
Jefferson
Philadelphia, Pennsylvania, United States
Akhil Golla, MD
Moss Rehab
Bridgewater, New Jersey, United States
A 19-year-old male with history of congenital hydrocephalus (status post ventriculoperitoneal shunt) and Chiari I malformation (status post decompression) presented for evaluation of progressive spasticity and clonus affecting the right limbs. Symptoms began at age 13, initially in the right leg, later involving the arm. Brain MRI revealed cerebellar tonsillar herniation with hydrocephalus. He was referred to Neurosurgery, who adjusted his shunt.
He continued to have movement-triggered clonus that caused frequent falls. He previously trialed oral baclofen and an ankle foot orthosis, which were not well tolerated. Botulinum toxin injections were initiated to the right gastrocnemius and soleus, with moderate lower limb improvement but excellent response in the right upper limb. Despite this, he remained dissatisfied with his persistent right ankle clonus.
Following Vulpius plantar flexor lengthening surgery, ankle clonus significantly improved, but additionally, unexpected reduction in upper limb clonus was noted. With physical therapy he progressed to ambulating independently.
Discussions:
This case highlights a complex interplay between the afferent sensory input and efferent hyperexcitability of the pathophysiology of spastic reflex arc. Reducing the afferent input into the spinal cord, achieved by surgically lengthening the plantar flexors to alter Achilles tendon length and tension, consequently diminished the muscle spindle input. This action decreased the activation of the propriospinal interneuron system, which can otherwise produce hyperactive nociceptive reflexes. The result was a reduction in clonus observed in both the lower and upper limbs.
Conclusions:
This case demonstrates how modulating afferent drive within the spinal reflex pathway can significantly reduce efferent hyperexcitability, underscoring the importance of multimodal therapy including orthopedic intervention and focal chemodenervation in addressing spasticity.