Poster 113 Improvement of speech following stereotactic thalmotomy in a multiple sclerosis patient: A case report

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ACADEMY ANNUAL ASSEMBLY ABSTRACTS

Poster 109 "An Uncommon Cause of Compressive Peroneal Nerve Palsy." F. Marlene Perez, MD (The New York HospitaI-Cornell Medical Center, New York, NY); Elisabeth A. Lachmann, MD; Richard S. Tunkel, MD. One of several unusual etiologies of peroneal nerve palsy is compression by a ganglion cyst. Although synovial cysts are relatively common entities, they rarely involve peripheral nerves. Two cases are presented in which the common peroneal nerve is compressed by a post-traumatic ganglion cyst in the proximal tibiofibular joint. In the first case, compression occurred several months after blunt trauma to this region, and although the symptoms resolved following surgical excision of the cyst and rehabilitation treatment, the cyst reoccurred. In the second case, compression of the common peroneal nerve by a synovial cyst occurred after years of repetitive trauma to this patient's feet from ballet. Magnetic resonance imaging and electrodiagnostic studies confirmed compression of the common peroneal nerve by a ganglion cyst. The pathogenesis of ganglion cyst formation and the mechanism of compression are delineated.

Poster 110 "Clinical Outcomes of Patients after Conservative Management for Cervical Radiculopathy." Banwari Ladha, MD (Sinai Hospital, Detroit, MI); Parmod K. Mukhi, MD; Maury Ellenberg, MD; Amy Indenbaum, MD; Joseph C. Honet, MD. We retrospectively studied 46 patients with cervical radiculopathy to determine the outcome with nonsurgical treatment. We reviewed 200 patient charts seen in an outpatient physiatric referral practice between June 1991 and September 1994. The ICD-9 code (723.4) for cervical radiculopathy was used to separate out the initial patient group. To assure definite cervical radiculopathy was present, patients included in the study had to have at least 2 major criteria; 1 major and 2 minor criteria; or 3 minor criteria. Major criteria were a positive Spurling maneuver and positive EMG; minor criteria included radicular pain, muscle weakness and absent or decreased reflexes. Patients who did not fulfill these criteria were excluded from the study. Outcomes were recorded as excellent, good, fair, or poor. Excellent consisted of the presence of no residual symptoms, return to baseline work and activities. Good consisted of greater than 80% improvement in pain with returnto-work, baseline activities, and minimal residual complaints. Fair consisted of the return to most baseline activities with the presence of pain complaints requiring pain medication. Poor consisted of either no change or worsening of symptoms or requiting surgical intervention. Forty-six patients met the inclusion criteria. There were 33 men and 13 women; mean age was 53 years with a range of 31 to 96. Patients were followed for a mean time frame of 29 weeks, with a range of 1 to 101 weeks. Two patients were lost to follow-up. Thirty-four patients (73.9%) had a C7 radiculopathy; 2 patients (4.3%) had a C8 radiculopathy; and 1 patient (2.2%) had a C6 radiculopathy. Nine patients (19.6%) had indeterminate levels of radiculopathy (ie, C5 or C6, C7, or C8). Twenty patients (43.5%) had an excellent outcome; 12 (26.1%) a good outcome; 7 (15.2%) a fair outcome; and 5 patients (10.9%) had a poor outcome. Of the 5 patients, 2 had epidural steroids followed by surgical intervention yielding an excellent outcome, whereas 1 had acupuncture resulting in a good outcome. Of the 46 patients examined, a total of 39 patients (84.8%) had a fair to excellent outcome using nonsurgical management. When this data is statistically applied to the general population, 74.4% to 95.2% of patients would fit these outcomes at the 95% confidence level. This suggests that the majority of patients with cervical radiculopathy seen in an outpatient referral practice would resolve their radiculopathy and pain, and return to normal activities with nonsurgical management.

Poster 111 "Rare Rehabilitation Challenge in a Patient with Hypo-kalemic Periodic Paralysis and Charcot-Marie-Tooth Disease: A Case Report." Joseph P. Laluya, DO (Rush-Presbyterian-St Lukes Medical Center/Rush University, Chicago, IL). Hypokalemic periodic paralysis (HPP) is a rare illness characterized by episodes of low serum potassium followed by paralysis of limb muscles, proximal more than distal, and occasionally bulbar and respiratory involvement, which may prove fatal. Charcot-Marie-Tooth (CMT) is the most common hereditary peripheral neuropathy characterized by

Arch Phys Med Rehabil Vol 76, November 1995

symmetric weakness, distal greater than proximal, as well as significant ankle deformities and sensory deficits. We present a 28-year-old man who was diagnosed with CMT at age 7 and HPP at age 16. CMT resulted in severe pes cavus and equinus deformities requiring bilateral achilles tendon elongation at age 12. Eventually, bilateral foot-drop developed, requiring ankle-foot-orthoses. HPP episodes became more frequent during the patient's twenties, with paresis lasting from 4 to 18 hours. The most recent episode resulted in persistent lower extremity weakness. Other rehabilitation challenges involved medical treatment with potassium replacement, pain management, and psychosocial adjustment for this young man. Functional electrical stimulation, isokinetic exercises, and orthotics were utilized to upgrade strength and range of motion. This case illustrates the unique characteristics seen in patients with HPP and CMT. A detailed rehabilitation course and pertinent literature are discussed.

Poster 112 "Botulinum Toxin Injection Technique of the Lateral Cricoarytenoid in Spastic Dysphonia." Arthur Alfred Rodriquez, MD (University of Wisconsin Medical School, Madison, WI); Chales N. Ford, MD. Spastic dysphonia has been treated with botulinum toxin (botox) injection into the intrinsic laryngeal muscles. Recent studies suggest that unilateral injection of the thyroarytenoid (TA) may be more effective than bilateral injection, and that results are enhanced when botox is injected specifically into the lateral cricoarytenoid (LCA). This study demonstrates the technique for percutaneous injection or electromyography of the LCA. Injection techniques were developed by dissection of four human larynges and have been performed on approximately 20 patients. With the subject supine, the injection needle (covered with teflon, except for the tip which serves as a monopolar EMG electrode) is inserted through the unanesthetized skin just lateral to the midline just above the superior border of the cricoid cartilage. The needle is advanced through the cricothyroid and just over the cricothyroid membrane, and directed 45 ° from the sagittal plane (vs 15° for the TA), and 40 ° from the transverse plane (vs 65 ° for the TA). The subject is asked to phonate sustained/i/, and the electrode is advanced in a submucous location until motor units are observed. Placement into the adductor muscle is confirmed by requesting a gentle valsalva or vocal click maneuver.

Poster 113 "Improvement of Speech Following Stereotactic Thalmotomy in a Multiple Sclerosis Patient: A Case Report." William J. Hwang, MD (The University of Texas Southwestern Medical Center at Dallas, Dallas, TX); Stephanie A. Kesler, M.Ed, CCC/SLP; Denise Frankoff, MA, CCC/SLP; Geetha Pandian, MD; Kevin R. Krafft, MD. Stereotactic thalamotomy is known as an effective surgical treatment for intention tremor among Parkinsonian and multiple sclerosis patients; however, worsening of speech has also been described as a side effect in post-thalamotoy course. Contrary to this finding, we report a case in which the patient had demonstrated improvement in her speech after a thalamotomy procedure. The patient is a 22-year-old woman with multiple sclerosis who underwent stereotactic thalamotomy for her tremor in September, 1994. Preoperatively, the patient had severe mixed dysarthria with hyperkinetic dysarthric features such as exaggerated, involuntary movements of the head, neck and oral musculature; further, difficulty coordinating phonation, respiration, and articulation characterized by bursts of phonation, pitch breaks and lack of pitch control, excessive loudness, and imprecise articulation were also noted. The above symptoms were worsened by patient's involuntary movements of the head, neck, and oral musculature. Postoperatively, in addition to improvement in tremor, her speech pattern also improved to moderate mixed dysarthria. Speech intelligibility was increased by decreased bursts of phonation and less severe pitch breaks due to better control of inhalation and exhalation, decreased volume of speech, increased precision of articulation, and resolution of involuntary movements of the head, neck, and oral musculature. Overall, in our patient, thalamotomy was associated with improved speech. So far, few reports have been made regarding the effectiveness of thalamotomy and speech rehabilitation as means

ACADEMY ANNUAL ASSEMBLY ABSTRACTS

of managing speech impediment. It is recommended that speech rehabilitation should be conducted as part of prethalamotomy and post-thalamotomy management of MS patients to assess the outcome.

Poster 114 cancelled.

Poster 115 "An Atypical Presentation of Syringomyelia: A Case Report." Angela L. Guccione, MD (Saint Francis Medical Center, Pittsburgh, PA); Mary Ann Miknevich, MD; Mary E. Langer-Schnepp, PTC. We present a case of syringomyelia with symptoms consistent with C8 radiculopathy in a previously asymptomatic patient. A 29-year-old woman noted acute onset of right axillary pain and right hand numbness precipitated by a sneeze. Neck flexion produced shocklike paresthesias down her right arm. Physical exam revealed hypoesthesia of the fourth and fifth digits of her right hand, with mild decreased strength of the right hand interossei and thumb opposition. Other motor, sensory and deep tendon reflex abnormalities were absent. Cervical magnetic resonance imaging (MRi) was performed in view of her neurological findings. A multiseptated cervicothoracic syrinx was identified with an associated Arnold-Chiari type 1 malformation. The patient elected for surgical correction and underwent suboccipital craniectomy, upper cervical laminectomy, and ventriculostomy. She noted complete relief of pain and weakness, without improvement of the hypoesthesia. No symptom progression has occurred 2 years after surgery, and a small residual syrinx remains on MRI. The advent of MRI has allowed for greater detection of the disease, often incidentally, with milder and occasionally asymptomatic cases being recognized. This presents new challenges for management and will alter the perception of the natural history and clinical spectrum of the disease.

Poster 116 "Acute Gastric Dilatation in Duchenne Muscular Dystrophy: A Case Report." Elizabeth S. Benson, MD (University of Washington/ Children's Hospital and Medical Center, Seattle, WA); Kenneth M. Jaffe, MD. Duchenne muscular dystrophy (DMD) is the most common neuromuscular disorder of childhood. Its clinical characteristics that derive from skeletal muscle involvement have been well described. Less well known is that visceral smooth muscle is affected by dystrophin deficiency. We report a case of a 19-year-old man with DMD who presented with severe nonradiating epigastric pain. He was initially sent home from the emergency department with a diagnosis of costochondritis. Acute gastric dilatation was not considered in the differential diagnosis despite the presence of a markedly enlarged gastric silhouette on chest radiograph. On repeat presentation two hours later, his examination was remarkable for a mildly distended abdomen with diminished bowel tones and diffuse tenderness. An abdominal radiograph demonstrated marked gastric dilatation without perforation. Treatment included rehydration, gastric decompression by nasogastric tube, and administration of prokinetic agents. This case illustrates the lack of familiarity by clinicians of the gastrointestinal (GI) manifestations of DMD, including acute gastric dilatation and intestinal pseudo-obstruction. A review of the literature on GI dysfunction in DMD will be presented, emphasizing the clinical manifestations, pathophysiology, pathology, and clinical management. In addition, the discussion will address directions for future research to improve our understanding and clinical management of these problems.

Poster 117 "Botulinum Toxin A Effectiveness Against Spasticity and Muscle Spasms." Bahman Jabbari, MD (Walter Reed Army Medical Center, Uniformed Services University of the Health Sciences, Washington, DC); Marybeth Grazko, MD; Kathleen Polo, MD; Gwendolyn Ford, MD. The purpose of this study was to determine the effectiveness of botulinum toxin A against spasticity and painful muscle spasms. We studied 24 patients, aged 4 to 82 years, 12 according to an open and 12 according to a double-blind crossover protocol. The cause of spasticity was diverse and included stroke, trauma, and multiple sclerosis. Using the Ashworth scale, we rated the muscle tone and frequency of muscle spasms and videotaped the patients. In the double-blind group, the patients were rerated 2 weeks following administration of the first agent (Saline or

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toxin). If there was no significant improvement (2 grades or higher) we then injected the second agent. In case of improvement, the second agent was not injected until the tone returned to the baseline. The dose varied from 10 to 200 units per muscle. Twenty-three of 24 subjects displayed a significant tone improvement. Seven of 8 patients with painful muscle spasms reported more than 50% decrease in the number of spasms. Improvement of tone and spasms resulted in better nursing care and physical therapy in half of the patients. We conclude that botulinum toxin A effectively improves spasticity and associated muscle spasms and facilitates physical therapy.

Poster 118 "Cervical Spondylotic Myelopathy: Functional Outcome Over a Period of Time." Aerie Rim, MD (Bronx Veterans Administration Medical Center, Bronx, NY). Cervical spondylotic myelopathy (CSM) is known as a generally benign disorder caused by the compression of the cord in which disability develops in an initial phase of variable duration then remains static thereafter. The purpose of this study was to evaluate patients' functional status over a period of time. Twenty-two male patients with GSM were retrospectively followed from 3 years to 10 years. Their ages varied from between 47 and 84 years. Sixteen patients had been treated by cervical laminectomy and 6 patients had been treated conservatively. Fifteen patients had low back pain of variable causes during the followup. Among them, 4 underwent lumbar laminectomy for a narrow canal. Eleven patients became wheelchair confined. All but one patient had cervical laminectomy. The interval from the onset of symptoms to wheelchair confinement varied from 1 year to 10 years. Five patients used an assistive device such as crutches, walker, or cane. From this study, CSM was found to be a chronic and slowly but progressively deteriorating disease that affects functional ability.

Poster 119 "Polyneuropathy as a Manifestation of Sarcoidosis: A Case Study and Literature Review." Stacy A. Harris, MD (Rehabilitation Institute of Chicago/Northwestern University Medical School, Chicago, IL). Sarcoidosis is an idiopathic granulomatous disease that may involve any organ system. The frequency of neurological involvement has been reported between 5% and 10%. This case study describes a 29-year-old woman with 5-day history of numbness and tingling of fingers, toes, and perioral region with evidence of facial diplegia, areflexia, and symmetric upper and lower extremity weakness. Initial lumbar puncture, CT of the head, and EMG/NCV studies were normal, and presumptive diagnosis of acute Guillain-Barre syndrome was made. She underwent plasmaphoresis but without improvement of symptoms. Due to lack of neurological recovery and evidence of hilar adenopathy on chest x-ray, a diagnosis of sarcoidosis was investigated. Repeat lumbar puncture 5 days after presentation revealed acellular fluid with elevated protein. Repeat EMG/NCV 9 days after first study showed predominantly motor demyelinating and axonal polyneuropathy. A lower extremity skin lesion previously attributed to insect bite was identified as probable erythema nodosum. Axillary node biopsy was positive for granulomatous lymphadenitis consistent with sarcoidosis. High-dose steroids resulted in neurological improvement. After transfer to rehabilitation program she continued to gain marked upper and lower extremity motor recovery but facial diplegia persisted. Literature review finds rare association between sarcoidosis and polyneuropathy. This study reviews the literature on neurological sarcoidosis and discusses its diagnosis and treatment. The long-term functional outcome and unique rehabilitation aspects of this case are described.

Poster 120 "Opsoclonus-Myoclonus Syndrome Secondary to Coxsackie B Virus: A Case Study in the Rehabilitation Setting." William J. Sullivan, MD (Rehabilitation Institute of Chicago, Chicago, IL); Joseph P. Zuhosky, MD. The clinical syndrome of opsoclonus and myoclonus in combination is rare. The distinguishing features of this syndrome include cerebellar ataxia, myoclonic jerks of striated muscles affecting the trunk and extremities, and opsoclonus (abnormal eye movement, flutter, or dyskinesia). The majority of reports in the literature attribute this syndrome to manifestations of neuroblastoma, especially in children. There are,

Arch Phys Med Rehabil Vo| 76, November 1995