A case study about the treatment of hereditary spastic paraplegia.
Spasticity is a debilitating condition that frequently manifests following the occurance of upper-motor-neuron lesions, which are often seen in poststroke, multiple sclerosis, traumatic brain injury, and other upper-motor-neuron disorders. Individuals usually develop spasticity following a stroke. A common presentation of spasticity is loss of normal control of one’s muscles. A commonly accepted medical definition of spasticity is simply, "A velocity-dependent resistance to passive movement or increased muscle tone."
Spasticity can have a tremendous impact on function. Left untreated, spasticity can often lead to muscle contractures, bone deformities, and mobility problems. It can also interfere with the fit and usefulness of orthoses or seating and mobility devices.
Patient Background
The following case study highlights one common manifestation of spasticity—ambulating on the "supinated" foot—and presents a possible treatment protocol for this condition. The supinated foot is a product of the dominant lower-extremity extensor synergy that preferentially places the lower extremity in hip flexion with internal rotation, knee extension, and ankle inversion with plantarflexion.
We have found that the supinated foot in weight-bearing is a fairly common presentation and causes much difficulty, including poor fit of braces, decreased balance, callus buildup and/or hetertrophic bone along the lateral border of the fifth metatarsal, and stress fractures of the fourth and fifth metatarsals.
Our case study involves a 54-year-old male named Bill, who was diagnosed with hereditary spastic paraplegia. He has taken the maximum dosage of oral antispasticity medications for years. He initially reported to physical therapy due to increasing instability with his gait. At the time of his initial evaluation, a callus and a hetertrophic bone was found along his fifth metatarsal, including a grade–2 area of skin breakdown (figure 1, page 34). A further evaluation from his physician showed fractures in his fourth and fifth metatarsal. His objective examination showed fairly strong ankle clonus on the left side, approximately 14 beats. His Modified Ashworth Tone rating was 2 for the gastrocnemius and 2 for the invertors of the foot. Functionally, he was weight-bearing on a supinated foot in midstance (figure 2, page 34). His single-limb balance was obviously impaired, largely because he was unable to bear weight on a stable base of support.
Bill was unable to comfortably stay in an ankle-foot orthotic (AFO) because his spasticity caused his foot to pull out of the brace and he was experiencing skin breakdown from the brace itself. He and his neurologist formulated a treatment plan, which included botulinum toxin Type A injections to the left tibialis posterior and to his gatroc in a lesser dosage of units, followed by aggressive stretching of plantar flexors and invertors. Bill also performed assisted active range of motion using electrical stimulation to primarily the peronues tertius and the extensor digitorium longus and brevis to promote neutral dorsiflexion or dorsiflexion with a eversion moment. Bill’s exercise program was designed to strengthen the antagonistic muscle groups involved in his lower-extremity extensor synergy pattern.
Over a 2-month treatment period, Bill regained motor control in his left ankle and was able to control his supination in stance well enough to tolerate wearing an AFO, which ensured a neutral ankle/foot in midstance for safer weight-bearing. His balance subsequently improved greatly, and his activity level increased. He now receives botulinum toxin Type A injections every 4–6 months.
Interventional Options
Not everyone who has spasticity requires medical treatment. However, in many individuals (like in Bill’s case), spasticity can interfere with function, mobility, self-care, or the caregiver’s ability to care for an individual. Appropriate management and treatment can help lessen the severity of spasticity that interferes with day-to-day functioning, and can allow for greater independence and functioning. If an individual has good selective motor control underlying his or her spasticity, reducing spasticity may significantly improve mobility and function, such as the ability to walk or to functionally use one’s arm.
The variation of spasticity from one individual to another is great and is partially dependent on the location and size of the lesion. The symptoms can be focal or global. The patient may present with localized spasticity in one joint, or all joints in the same limb could be involved. Mild spasticity can often be treated successfully with a combination of range–of–motion exercises, neuromuscular re-education/strengthening, splinting, orthotics, and oral medication and/or focal treatments, such as botulinum toxin Type A injections. In severe widespread spasticity, focal treatment of chemodenervation, such as botulinum toxin Type A, may not improve function unless it is used in conjunction with global tone reduction, such as what is achieved with inthrathecal drug therapy. The benefit of intrathecal drug therapy over oral administration is that a constant dose can be delivered continuously to more effectively manage severe spasticity without the typical side effects seen with oral medications.
There is an array of management options for spasticity. The decision regarding whether, when, and how to manage spasticity is influenced by many factors and may not simply follow the strategy of conservative to aggressive interventions. Factors to consider include the distribution, chronicity, severity, and cost of spasticity. The goals of intervention must be clearly established prior to choosing the intervention. A multidisciplinary team approach to spasticity management should be used to maximize the outcomes. In most cases, the medical intervention without the therapy component, or therapy solely without the medical management of the spasticity, is a flawed approach without good outcomes. The coordination of treatment with the physiatrist, neurologist, and therapist allows for the best functional outcomes.
Kevin Lockette, PT, is the president and owner of Ohana Pacific Rehab Services LLC, which operates clinics in Honolulu and Kailua, Hawaii.