Mark V. Ragucci

Rehabilitation essential in the recovery of multifactorial subacute combined degeneration.

Subacute combined degeneration (SCD) is a rapidly progressive myelopathy with a constellation of neurologic deficits, including progressive sensory abnormalities in the vibratory and proprioceptive domains, ascending paresthesias, gait ataxia, hyper- or hypo-reflexia, and, less commonly, motor weakness and loss of bowel and bladder control [1,2]. SCD is a result of dorsal and lateral spinal cord demyelination [3]. This form of myelopathy is secondary to a cobalamin or vitamin B12 deficiency, dietary insufficiency, malabsorption and/or, less commonly, excretion. Pernicious anemia, also known as autoimmune gastritis, is a common cause of vitamin B12 deficiency. Nitrous oxide (N2O) exposure during recreational use or surgical intervention has also been reported to convert B12 to B12 analogues, which are then excreted in the urine, reducing B12 levels [3,4]. Up until now, much of the literature has presented the clinical manifestations of SCD primarily in the context of nitrous oxide (N2O) abuse and exposure. Conversely, we report on a patient with a triple-insult SCD of the spinal cord, one insult secondary to noncompliance with B12 replacement therapy for pernicious anemia, one insult secondary to alcohol abuse, and one insult secondary to prolonged recreational use of nitrous oxide.

Poster 330: Physiatric Coordinated Inpatient Rehabilitation Management and Ambulatory Follow-up of a Patient with Guillain-Barre Syndrome Secondary to Zika Virus Infection: A Case Report

Setting: Acute Inpatient Rehabilitation Hospital. Results: During inpatient rehabilitation, the patient developed progressively worsening dysphagia of unclear etiology. This was later followed by the onset of new myoclonus and he was transferred to an acute care hospital for further assessment. Video electroencephalography was negative for epileptiform activity and a swallow study demonstrated moderate-to-severe oral dysphagia. This was thought to be secondary to oropharyngeal myoclonus, and he was diagnosed with chronic post-hypoxic myoclonus (PHM). He required percutaneous endoscopic gastrostomy tube placement and was started on valproic acid for myoclonus management. He subsequently completed inpatient rehabilitation with notable functional gains demonstrated at discharge. Discussion: Chronic post-hypoxic myoclonus, also known as LanceAdams syndrome, is a rare complication following HIE. The myoclonus appears days to weeks after cardiopulmonary resuscitation in patients who have regained consciousness. It is triggered by movement, sensory stimulation, or anxiety and has no electroencephalographic correlate. Myoclonus most commonly affects the arms and legs but can also affect the palate, larynx, and pharynx. While myoclonus and dysphagia have previously been described in association with a number of neurologic diagnoses, to our knowledge, dysphagia has never been described as a presenting symptom of chronic PHM. Conclusions: New onset dysphagia following HIE in the acute inpatient rehabilitation setting should warrant further workup for chronic PHM. Prompt diagnosis leading to earlier treatment initiation may have significant implications on functional outcomes in the acute inpatient rehabilitation setting. Level of Evidence: Level V

Poster 190: Atypical Presentation of Inflammatory Myopathy Related to Dermatomyositis: A Case Report

Disclosures: Jason Roth: I Have No Relevant Financial Relationships To Disclose Case/Program Description: A 23-year-old woman with history of asthma who presented with gradual onset polyarticular pain and swelling causing difficulty walking, along with skin lesions, dysphagia, dyspnea and weight loss. Patient was noted to have hemolytic anemia, transaminitis, multifocal pneumonia, along with both proximal and distal muscle weakness. Workup included liver and muscle biopsies. Prior tobiopsy results being available, patientwas treatedwith steroids. Patient was transferred to acute inpatient rehab without a known etiology of her symptoms. Physical exam at rehab revealed dry skin and icteric sclerae. Active and passive range ofmotionwere decreased in all extremities due to weakness and pain, respectively. Motor testing revealed diffuse weakness, specifically 3/5 bilateral shoulder abduction, 3/5 bilateral elbow extension, 3/5 bilateral hip flexion; otherwise, 4/5 throughout. Elbows tender to palpation with moderate effusions. Right thigh hematoma at the site of muscle biopsy. MRI Femur revealed diffuse symmetric myositis. Muscle biopsy confirmed dermatomyositis. Setting: Inpatient. Results: Patient was admitted to acute inpatient rehabilitation requiring moderate assistance for transfers and ADLs, and minimal assistance with rolling walker for ambulation. Despite patient’s rehab course being limited by persistent severe pain, patient did make significant gains in functional mobility such as: sit-to-stand with minimal assistance and ambulation with supervision without assistive device. Discussion: Dermatomyositis is a rare, acquired inflammatorymyopathy that affects 4-10/1,000,000 annually. Symptoms include skin changes and proximal and pharyngoesophageal muscle weakness. Dermatomyositis maybeassociatedwith interstitial lungdiseaseandmalignancy.Diagnosis is confirmed with muscle enzyme levels, autoimmune antibodies, electromyography, and muscle biopsy. Treatment involves immunosuppression. In this case, we encountered an atypical presentation which included polyarticular pain and swelling, distal muscle weakness in addition to proximal, transaminitis and hemolytic anemia. Conclusions: While dermatomyositis is uncommon, the physiatrist should be conscious of the associated risks and impairments of ADLs and functional mobility it may cause. Level of Evidence: Level V

Poster 246 Unusual Cause of Myelopathy Related to Neurofibromatosis Type 1: A Case Report

Diagnosis is usually confirmed with molecular genetic testing. Treatment consists of eliminating ammonia either with medications or dialysis. Conclusions: A mild urea cycle enzyme deficiency may not present until adulthood. While morbidity and mortality are high in UCDs, prompt diagnosis and treatment is essential for reversal of symptoms, improvement of survival rates and functional outcomes. Level of Evidence: Level V

Autoimmune Cerebellitis with Limited Improvement despite Aggressive Medical and Rehabilitation Treatment: A Case Report

Main Outcome Measures: MAS or AS ankle score, MAS/AS responder analyses, Physician Global Assessment of Response (PGA), and Goal Attainment Scale (GAS). Results or Clinical Course: Greater AS ankle score improvements and higher response rates for patients receiving onabotulinumtoxinA versus placebo were reported in all 3 studies. In Study 1, reductions in MAS ankle score were greater with onabotulinumtoxinA versus placebo, with significant differences at weeks 4, 6, and 8 (p<.001). Proportion of responders ( 1 point reduction from baseline muscle tone ankle score) was greater with onabotulinumtoxinA than placebo at weeks 4, 6, and 8 (p<.001). Study 2 demonstrated a significant difference between onabotulinumtoxinA 300U and placebo in AS at week 8 (p1⁄4.011); the 200U dose did not differentiate from placebo. PGA improved with onabotulinumtoxinA versus placebo, with significant differences at weeks 4, 6, and 8 (p<.05) in Study 1 (results similar in studies 2 and 3). Study 3 demonstrated clinically and statistically significant improvements with onabotulinumtoxinA relative to placebo in functioning measured by GAS. In all 3 studies, significant correlations between measures of muscle tone and study-specific global measure by physician were observed at relevant time-points (p .008). OnabotulinumtoxinA was well-tolerated in all 3 studies. Most frequently-reported AEs were myalgia, injection site pain, fall and urinary tract infection, with no differences in frequency of AEs between onabotulinumtoxinA and placebo. Conclusions: OnabotulinumtoxinA (300U) significantly decreased ankle flexor tone in adults with PS lower limb spasticity. Decrease in muscle tone was considered clinically meaningful, as shown by significant correlations with study-specific investigator global assessments.

Poster 283 Autoimmune Cerebellar Ataxia Associated with Glutamic Acid Decarboxylase Antibodies Minimally Responsive to Immunotherapy and Acute Rehabilitation: A Case Report

Disclosures: J. Liu, No Disclosures. Case Description: A 45-year-old woman with no past medical history initially presented to the emergency ward with complaints of vertigo, double vision, and gait instability for 2 months. Physical examination showed no deficits in strength, sensation, or reflexes, but was notable for nystagmus, dysarthria, mild bilateral dysmetria, and severe truncal ataxia. She was functionally bed-bound and unable to ambulate with maximum assistance due to prominent ataxia. Broad medical work-up ruled out infectious, toxic, and metabolic etiologies. Magnetic resonance imaging of the brain revealed no ischemic insults or masses. An autoimmune cerebellar etiology was suspected. The patient was given empiric intravenous immunoglobulin (IVIG) therapy and transferred to the acute inpatient rehab service. Setting: Acute inpatient rehab. Results or Clinical Course: Further serologic work-up revealed that the patient had antibodies to glutamic acid decarboxylase-65 (GAD-Ab). She received plasma exchange (PLEX) therapy, was given oral steroids, and continued to participate in the acute rehab therapy program. Despite these interventions, she had minimal improvement in her functional deficits. A family meeting was held to discuss her poor prognosis. She was eventually discharged home requiring supervision for wheelchair propulsion, moderate assistance for transfers, and variable assistance for activities of daily living. Discussion: Cerebellar ataxia associated with the GAD-Ab is a rarely reported disorder with limited evidence-based treatment options like immunotherapy. To our knowledge, this is the first case that describes its response to both immunotherapy and acute rehab. In this patient, IVIG, PLEX, oral steroids, and acute rehab provided minimal return of function. GAD is a major enzyme of the nervous system that catalyzes the conversion of glutamate to -aminobutyric acid (GABA). It has only recently been implicated in movement disorders. The role of the GAD-Ab and the underlying pathophysiology is unknown. Conclusions: This case highlights the need for further research to define the pathophysiology and therapeutic management of a rarely reported autoimmune cerebellar disorder. Testing for GAD-Ab in patients with sporadic cerebellar ataxia is important as this subset of patients may potentially benefit from immunotherapy.

Poster 79: Idiopathic Hypereosinophilic Syndrome in a Man With Mononeuritis Multiplex: A Case Report

tion in number of voluntary motor unit action potentials (MUAP) in the affected myotomal distribution and different degrees of positive sharp waves and fibrillation potentials. The sensory nerve action potentials (SNAP) distal sensory latencies and conduction velocities are mildly affected. The key EMG finding can be shown during paraspinal muscle needling. EMG needling of paraspinal region corresponding to the myotomal weakness demonstrates significant membrane instability. The electromyographer will find decreased recruitment of motor unit action potentials, increased insertional activity, fibrillation potentials, and positive sharp waves. This finding verifies that the lesion is as proximal as the spinal nerve bifurcation. The pathogenesis behind motor axonal involvement is during re-activation in the dorsal root ganglia, the HVZ virus causes a local neuritis in the spinal nerve and subsequently gains access to the motor nerves. Setting: Physiatry outpatient clinic. Results: Three patients were found to have documented HVZ viral infection, presenting with characteristic rash in a dermatomal distribution along with myotomal weakness and abnormal EMG and NCS of the lower extremity significant for lumbosacral radiculopathy. Conclusions: EMG and NCS evidence of lumbosacral radiculopathy in 3 patients with documented HVZ viral infection.

Poster 414: Neuromyelitis Optica (Devic Syndrome) in Setting of Lupus in an Acute Inpatient Rehabilitation Patient: A Case Report

osteoarthropathy. Setting: Urban rehabilitation hospital. Results: The rehabilitation plan included (1) modifications to his KAFOs to compensate for altered leg length and gait dynamics, (2) prescription of an ultralight manual wheelchair, (3) functional reassessment of mobility and activities of daily living (ADL) with an emphasis on positioning, and (4) manual therapy and shoulder exercises to provide muscular balance and reduce tendinopathy. He was able to continue selected ambulation activities safely, including golf. Discussion: We describe a rare case of delayed neuropathic osteoarthropathy of the hip several months after a fall in an individual with long-term SCI. The goal of rehabilitation was a comprehensive approach, consisting of therapeutic exercise, manual therapy, orthotic modification, procurement of a customized wheelchair and reassessment of ADLs and mobility. The priority was to maintain quality of life and function, balancing safety with recreational pursuits. Conclusions: This case demonstrates the importance of ongoing rehabilitation and emphasis on function and quality of life in the management of secondary conditions in people with SCI.

Poster 117: Bilateral Subtrochanteric Femoral Insufficiency Fractures in a 75-Year-Old Woman Rceiving Long-Term Alendronate Therapy: A Case Report

Patients or Programs: A 65-year-old woman complaining of pain in her bilateral inguinal areas, lower back and buttocks. Program Description: The patient states the pain has been getting worse over the past 10 years. Medical history revealed that when the patient was a freshman in high school she was diagnosed with cerebellar encephalitis. Her symptoms at that time were increasing lethargy, ataxic gait, double vision and loss of sensation on her right side. Years later, around her late 20s, she started experiencing the same symptoms and then had a third relapse at 30 years old. Since then she has not had any similar symptoms. Palpation of her lower extremities revealed some tenderness over her bilateral groin areas as well as lumbar paraspinals and gluteus maximus muscles. She had decreased lumbar spine flexion greater than extension and increased muscle tone in her bilateral hip flexors and knee extensors. The suspicion of multiple sclerosis was high according to her current findings and history. MRI of the brain without contrast showed pathognomonic findings of multiple sclerosis. Setting: Comprehensive 21-day pain management program. Results: Physical therapy was most helpful to this patient to increase strength and range of motion to her lower extremities. Along with central-acting muscle relaxers and proper pain medication, the patient made progress with mobility and pain reduction. Discussion: The confirmation of the multiple sclerosis diagnosis explains the 3 relapses she had during her youth. The increased muscle tone due to the multiple sclerosis limited her ROM at the hips and knees producing back spasms and pain around the buttocks and inguinal areas. Conclusions: This unique case of relapsing remitting multiple sclerosis which has stayed in remission for more than 35 years presented with lower extremity spasticity and low back pain.