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Poster 274 Botulinum Toxin Therapy of Cervical Dystonia (CD): Duration of Therapeutic Effects
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normal life expectancy, but it remains a progressive disorder that destroys motor neurons. Mutations in the senataxin gene causes ALS4. This case report describes the use of ITB to treat the spasticity in a person with ALS4. Oral agents may be ineffective or poorly tolerated. ITB can provide excellent control of spasticity with less side effects than oral agents. Conclusions: ITB to treat spasiticty in ALS4, or Juvenile Onset ALS, has not been reported in the literature. ALS4 is a life-long disease and is slowly progressive (unlike classical ALS) and can be associated with signficant spasticity. People with ALS4 and spasticity should have ITB as an option; a test doses of ITB, or a ⬙trial⬙, will determine if it will be effective. After implantation, careful titration based on effect may improve function and quality of life. Poster 273 Transient Quadriparesis and Cervical Instability in a College Football Player-A Case Report. Diane Vroenen (Eastern Virginia Medical School, Norfolk, VA, United States); Peter Gonzalez, MD. Disclosures: D. Vroenen, No Disclosures. Case Description: A 22-year-old man presented with his team’s athletic trainer for a suspected concussion. His history revealed transient quadriparesis after a helmet to helmet collision during a football game. He was found to have stenosis at C3-4 and C4-5 and ultimately atlantoaxial subluxation and instability. Program Description: Eastern Virginia Medical School, Physical Medicine and Rehabilitation, Norfolk, Virginia. Setting: Sports medicine clinic. Results or Clinical Course: The patient had bilateral hand and leg numbness and weakness immediately post-collision for 10 minutes, posterior headache and insomnia for 2 weeks and balance disturbance after a helmet to helmet collision during a football game. He received spinal precautions and emergency department evaluation. Cervical computed tomography (CT) showed early degenerative changes at C3-4, C4-5, C5-6 with mild congenital stenosis and kyphosis, and borderline Chiari 1 malformation, the head CT was negative. He was discharged with a diagnosis of cervical sprain and a concussion. He presented to a sports medicine clinic for concussion evaluation. His neurologic examination was intact except for balance disturbance. Due to clinical history of transient bilateral upper and lower extremity paresthesias and weakness further radiologic evaluation was initiated. Plain films showed instability at C1-C2 with 7-8mm of translation into flexion. MRI showed a disruption of the C1 transverse ligament but no cervical cord abnormality. The patient was referred to an orthopedic spine surgeon and underwent a C1/2 posterior fusion. Discussion: This injury is seen most commonly in football players with axial loading injuries of the neck. Proper diagnosis is critical prior to returning an athlete with a possible cervical spine injury to play. Conclusions: Athletes who present after a head on collision may appear to only have concussive-type symptoms however any history of bilateral sensory and/or motor symptoms, no matter how transient, should be evaluated thoroughly for transient quadriparesis and cervical spine instability. Work up should include neurologic examination, dynamic flexion extension radiographs, and MRI to rule out cord injury and/or ligamentous instability.
PRESENTATIONS
Poster 274 Botulinum Toxin Therapy of Cervical Dystonia (CD): Duration of Therapeutic Effects. Dirk Dressler (Hannover Medical School, Hannover, Germany); Fereshte Adib Saberi, MD Pawel Tacik, MD. Disclosures: D. Dressler, Consulting fees: Allergan, Ipsen, Elan/ Eisai/Solstice, Merz Pharmaceuticals. Objective: To explore the duration of the therapeutic effect of botulinum toxin (BT). Design: Open-label, non-interventional study. Setting: Single center in Germany. Participants: Patients with CD. Interventions: IncobotulinumtoxinA, onabotulinumtoxinA, or both treatments given in sequence. Doses, target muscles, and inter-injection intervals (II) were at the discretion of the physician based on guideline recommendations and patient needs. Main Outcome Measures: Treatment duration (TD; time between application of BT and patient-reported waning of therapeutic effect), II, and excess time (ET; ET⫽II-TD). Results: Fifty-nine patients were studied (male, n⫽22; mean age, 52.6 years; mean Tsui score, 9.0). Altogether, 1289 treatment cycles were evaluated, with a mean of 21.8 (standard deviation [SD], 14.0; range, 4-66) treatment cycles recorded for each patient. Mean (SD) TD per patient ranged from 7.8 (1.4) to 21.0 (3.9) weeks (overall mean, 11.8 [2.7]). Mean TD was ⱕ12 weeks in 83.1% of patients and ⱕ10 weeks in 35.6%. Mean (SD) II per patient ranged from 11.3 (1.3) to 27.8 (11.6) weeks (overall mean, 15.4 [3.4]). The overall mean (SD) ET was 3.5 (2.4) weeks (22.7% of the overall mean II). TD and II were generally stable throughout the treatment course. Treatment delays occurred in 16.9% of patients (due to appointment difficulties, test of treatment duration, test of CD severity, fear of adverse events or psychiatric comorbidities). Prolonged treatment effects, probably due to CD fluctuations, occurred in 18.6% of patients. Singular unexplained therapy failure (SUTF) occurred following 0.4% of injection series. Antibody-induced therapy failure did not occur. Conclusions: TD and II were generally stable with long-term BT application, but could be influenced by treatment delays, CD fluctuations, or SUTF. ET indicated that patients were treated suboptimally for ⬃23% of their treatment cycle, supporting a reduction of II. BT formulations associated with low antigenicity may be useful if II is reduced to ⱕ12 weeks. Poster 275 Rapid In-Game Progression of Primary Dystonia in a High School Football Player: A Case Report. Drahmane Kaba, MD (UVA, Charlottesville, VA, United States); Mary G. Bryant, MD; Daniel Herman, MD, PhD; Robert P. Wilder, MD. Disclosures: D. Kaba, No Disclosures. Case Description: A 17-year-old male high school football offensive linemen with a 6-month history of right hand focal dystonia presented to a sports medicine physician complaining of his ⬙arm going dead⬙ over the course of a game. On examination, he had right upper extremity non-dermatomal hypoesthesia with extreme rigidity at the fingers, wrist, elbow, and shoulder. He had intact distal pulses, normal cognition, and no neck pain. He shortly developed prominent facial and neck fasciculations, and was sent emergently to an academic tertiary care hospital (TCH).
normal life expectancy, but it remains a progressive disorder that destroys motor neurons. Mutations in the senataxin gene causes ALS4. This case report describes the use of ITB to treat the spasticity in a person with ALS4. Oral agents may be ineffective or poorly tolerated. ITB can provide excellent control of spasticity with less side effects than oral agents. Conclusions: ITB to treat spasiticty in ALS4, or Juvenile Onset ALS, has not been reported in the literature. ALS4 is a life-long disease and is slowly progressive (unlike classical ALS) and can be associated with signficant spasticity. People with ALS4 and spasticity should have ITB as an option; a test doses of ITB, or a ⬙trial⬙, will determine if it will be effective. After implantation, careful titration based on effect may improve function and quality of life. Poster 273 Transient Quadriparesis and Cervical Instability in a College Football Player-A Case Report. Diane Vroenen (Eastern Virginia Medical School, Norfolk, VA, United States); Peter Gonzalez, MD. Disclosures: D. Vroenen, No Disclosures. Case Description: A 22-year-old man presented with his team’s athletic trainer for a suspected concussion. His history revealed transient quadriparesis after a helmet to helmet collision during a football game. He was found to have stenosis at C3-4 and C4-5 and ultimately atlantoaxial subluxation and instability. Program Description: Eastern Virginia Medical School, Physical Medicine and Rehabilitation, Norfolk, Virginia. Setting: Sports medicine clinic. Results or Clinical Course: The patient had bilateral hand and leg numbness and weakness immediately post-collision for 10 minutes, posterior headache and insomnia for 2 weeks and balance disturbance after a helmet to helmet collision during a football game. He received spinal precautions and emergency department evaluation. Cervical computed tomography (CT) showed early degenerative changes at C3-4, C4-5, C5-6 with mild congenital stenosis and kyphosis, and borderline Chiari 1 malformation, the head CT was negative. He was discharged with a diagnosis of cervical sprain and a concussion. He presented to a sports medicine clinic for concussion evaluation. His neurologic examination was intact except for balance disturbance. Due to clinical history of transient bilateral upper and lower extremity paresthesias and weakness further radiologic evaluation was initiated. Plain films showed instability at C1-C2 with 7-8mm of translation into flexion. MRI showed a disruption of the C1 transverse ligament but no cervical cord abnormality. The patient was referred to an orthopedic spine surgeon and underwent a C1/2 posterior fusion. Discussion: This injury is seen most commonly in football players with axial loading injuries of the neck. Proper diagnosis is critical prior to returning an athlete with a possible cervical spine injury to play. Conclusions: Athletes who present after a head on collision may appear to only have concussive-type symptoms however any history of bilateral sensory and/or motor symptoms, no matter how transient, should be evaluated thoroughly for transient quadriparesis and cervical spine instability. Work up should include neurologic examination, dynamic flexion extension radiographs, and MRI to rule out cord injury and/or ligamentous instability.
PRESENTATIONS
Poster 274 Botulinum Toxin Therapy of Cervical Dystonia (CD): Duration of Therapeutic Effects. Dirk Dressler (Hannover Medical School, Hannover, Germany); Fereshte Adib Saberi, MD Pawel Tacik, MD. Disclosures: D. Dressler, Consulting fees: Allergan, Ipsen, Elan/ Eisai/Solstice, Merz Pharmaceuticals. Objective: To explore the duration of the therapeutic effect of botulinum toxin (BT). Design: Open-label, non-interventional study. Setting: Single center in Germany. Participants: Patients with CD. Interventions: IncobotulinumtoxinA, onabotulinumtoxinA, or both treatments given in sequence. Doses, target muscles, and inter-injection intervals (II) were at the discretion of the physician based on guideline recommendations and patient needs. Main Outcome Measures: Treatment duration (TD; time between application of BT and patient-reported waning of therapeutic effect), II, and excess time (ET; ET⫽II-TD). Results: Fifty-nine patients were studied (male, n⫽22; mean age, 52.6 years; mean Tsui score, 9.0). Altogether, 1289 treatment cycles were evaluated, with a mean of 21.8 (standard deviation [SD], 14.0; range, 4-66) treatment cycles recorded for each patient. Mean (SD) TD per patient ranged from 7.8 (1.4) to 21.0 (3.9) weeks (overall mean, 11.8 [2.7]). Mean TD was ⱕ12 weeks in 83.1% of patients and ⱕ10 weeks in 35.6%. Mean (SD) II per patient ranged from 11.3 (1.3) to 27.8 (11.6) weeks (overall mean, 15.4 [3.4]). The overall mean (SD) ET was 3.5 (2.4) weeks (22.7% of the overall mean II). TD and II were generally stable throughout the treatment course. Treatment delays occurred in 16.9% of patients (due to appointment difficulties, test of treatment duration, test of CD severity, fear of adverse events or psychiatric comorbidities). Prolonged treatment effects, probably due to CD fluctuations, occurred in 18.6% of patients. Singular unexplained therapy failure (SUTF) occurred following 0.4% of injection series. Antibody-induced therapy failure did not occur. Conclusions: TD and II were generally stable with long-term BT application, but could be influenced by treatment delays, CD fluctuations, or SUTF. ET indicated that patients were treated suboptimally for ⬃23% of their treatment cycle, supporting a reduction of II. BT formulations associated with low antigenicity may be useful if II is reduced to ⱕ12 weeks. Poster 275 Rapid In-Game Progression of Primary Dystonia in a High School Football Player: A Case Report. Drahmane Kaba, MD (UVA, Charlottesville, VA, United States); Mary G. Bryant, MD; Daniel Herman, MD, PhD; Robert P. Wilder, MD. Disclosures: D. Kaba, No Disclosures. Case Description: A 17-year-old male high school football offensive linemen with a 6-month history of right hand focal dystonia presented to a sports medicine physician complaining of his ⬙arm going dead⬙ over the course of a game. On examination, he had right upper extremity non-dermatomal hypoesthesia with extreme rigidity at the fingers, wrist, elbow, and shoulder. He had intact distal pulses, normal cognition, and no neck pain. He shortly developed prominent facial and neck fasciculations, and was sent emergently to an academic tertiary care hospital (TCH).