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Poster 190: Atypical Presentation of Inflammatory Myopathy Related to Dermatomyositis: A Case Report
Abstracts / PM R 9 (2017) S131-S290 Poster 190: Atypical Presentation of Inflammatory Myopathy Related to Dermatomyositis: A Case Report Jason Roth, DO (New York University School of Medicine, New York, NY, United States), Walter Alomar-Jimenez, MD, Mark V. Ragucci, DO 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 to biopsy results being available, patient was treated with 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 of motion were 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 inflammatory myopathy that affects 4-10/1,000,000 annually. Symptoms include skin changes and proximal and pharyngoesophageal muscle weakness. Dermatomyositis may be associated with interstitial lung disease and malignancy. 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 191: Immune-Mediated Necrotizing Myopathy Exacerbated by Statin Use: A Case Report Aileen Hilario, DO (SUNY Downstate Medical Center, Brooklyn, NY, United States), Haris Choudry, MD, Marcel Bayol, MD Disclosures: Aileen Hilario: I Have No Relevant Financial Relationships To Disclose Case/Program Description: A 54-year-old African American woman with hypertension, hyperlipidemia, and recent left thalamic stroke with no residual deficits presented with progressive weakness in all four extremities. On admission, her creatine kinase (CK) was elevated to 25,000 and atorvastatin was discontinued. Patient’s baseline CK was noted to be 1995, prior to initiating atorvastatin and aspirin for secondary stroke prevention. She underwent a right quadriceps biopsy, which revealed necrotizing myopathy with concern for immune-mediated necrotizing myopathy (IMNM). She was treated with 5 days of solumedrol, 5 days of intravenous immunoglobulin, then started on mycophenolate mofetil and prednisone. She was unable to perform active range of motion of her bilateral hip and knee flexors due to severe proximal weakness. She required maximum assistance for bed mobility, but ambulated 30 feet
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with a rolling walker and contact-guard assistance. Patient was transferred to acute inpatient rehabilitation to address her functional limitations. With immunomodulating agents and cessation of statin therapy, patient’s CK, liver function tests, and lactate dehydrogenase trended down slowly. Weekly rituximab infusions were added to her regimen. Functionally, she progressed to supervision for transfers, stair negotiation, and ambulation of 100 feet with a rolling walker and 200 feet with a wide-based quad cane. Setting: Acute Inpatient Rehabilitation Facility. Results: Patient tested strongly positive for HMG CoA Reductase antibodies, which are associated with statin-induced necrotizing myopathy. Her right quadriceps biopsy revealed necrotizing myopathy without significant inflammation or chronic myopathic features. Discussion: IMNM shows minimal inflammation, but can occur in association with statin use. If associated with statin therapy, myopathy typically progresses despite discontinuing the medication. HMG CoA reductase antibodies are positive in many patients, as in this case. Conclusions: This case demonstrates IMNM, which was exacerbated by statin use for secondary stroke prevention. Level of Evidence: Level V Poster 192: Recurrent Transverse Myelitis with Underlying Neuromyelitis Optica Triggered by West Nile Virus Infection: A Case Report. Joseph A. Wong, MD (Rahway, NJ, USA), Jane Han, MD, Iqbal H. Jafri, MD Disclosures: Joseph A. Wong, MD: I Have No Relevant Financial Relationships To Disclose Case/Program Description: Patient is a 65-year-old woman with history of diabetes, hypothyroidism, and psoriasis who was admitted for progressive lower extremity weakness. Magnetic resonance imaging (MRI) spine revealed diffuse extensive central spinal cord edema with enhancement. Cerebrospinal fluid was positive for West Nile virus (WNV) immunoglobulin G. She was diagnosed with transverse myelitis and admitted to acute inpatient rehabilitation for comprehensive therapy for muscle weakness along with medical management of bowel and bladder incontinence and painful muscle spasms. Several days after discharge, she was readmitted to acute care for recurrent transverse myelitis supported by MRI findings of progressive signal alterations of spine. Due to the recurrent and extensive transverse myelitis, patient was diagnosed with neuromyelitis optica (NMO). Months later, she again developed recurrence and was readmitted for rehabilitation. Setting: Acute inpatient rehabilitation. Results: Patient’s overall functional level decreased with each recurrence of NMO. However, functional level improved each time after comprehensive rehabilitation, with most recent admission AMPAC (Activity Measure for Post-Acute Care) score for basic mobility, 28.67 and daily activity, 23.29. Upon discharge, basic mobility improved to 34.52 and daily activity improved to 31.89. Discussion: Transverse myelitis is a neurological disorder characterized by inflammation of the spinal cord usually triggered by viral infection. Recurrent episodes are rare and may suggest an underlying autoimmune disease process such as NMO. NMO occurs when an abnormal response to infection leads to demyelination of spinal cord and optic nerve, leading to progressive weakness and autonomic dysfunction. Our patient’s predisposition to autoimmune disorders, suggested by her past history of psoriasis, may have been a clue to earlier diagnosis of NMO. This is important since each episode of NMO progressively increases disability that necessitates early aggressive rehabilitation and medical management. Conclusions: Consider the possibility of NMO in patients presenting with recurrent transverse myelitis, especially with underlying autoimmune disorders. Level of Evidence: Level V
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with a rolling walker and contact-guard assistance. Patient was transferred to acute inpatient rehabilitation to address her functional limitations. With immunomodulating agents and cessation of statin therapy, patient’s CK, liver function tests, and lactate dehydrogenase trended down slowly. Weekly rituximab infusions were added to her regimen. Functionally, she progressed to supervision for transfers, stair negotiation, and ambulation of 100 feet with a rolling walker and 200 feet with a wide-based quad cane. Setting: Acute Inpatient Rehabilitation Facility. Results: Patient tested strongly positive for HMG CoA Reductase antibodies, which are associated with statin-induced necrotizing myopathy. Her right quadriceps biopsy revealed necrotizing myopathy without significant inflammation or chronic myopathic features. Discussion: IMNM shows minimal inflammation, but can occur in association with statin use. If associated with statin therapy, myopathy typically progresses despite discontinuing the medication. HMG CoA reductase antibodies are positive in many patients, as in this case. Conclusions: This case demonstrates IMNM, which was exacerbated by statin use for secondary stroke prevention. Level of Evidence: Level V Poster 192: Recurrent Transverse Myelitis with Underlying Neuromyelitis Optica Triggered by West Nile Virus Infection: A Case Report. Joseph A. Wong, MD (Rahway, NJ, USA), Jane Han, MD, Iqbal H. Jafri, MD Disclosures: Joseph A. Wong, MD: I Have No Relevant Financial Relationships To Disclose Case/Program Description: Patient is a 65-year-old woman with history of diabetes, hypothyroidism, and psoriasis who was admitted for progressive lower extremity weakness. Magnetic resonance imaging (MRI) spine revealed diffuse extensive central spinal cord edema with enhancement. Cerebrospinal fluid was positive for West Nile virus (WNV) immunoglobulin G. She was diagnosed with transverse myelitis and admitted to acute inpatient rehabilitation for comprehensive therapy for muscle weakness along with medical management of bowel and bladder incontinence and painful muscle spasms. Several days after discharge, she was readmitted to acute care for recurrent transverse myelitis supported by MRI findings of progressive signal alterations of spine. Due to the recurrent and extensive transverse myelitis, patient was diagnosed with neuromyelitis optica (NMO). Months later, she again developed recurrence and was readmitted for rehabilitation. Setting: Acute inpatient rehabilitation. Results: Patient’s overall functional level decreased with each recurrence of NMO. However, functional level improved each time after comprehensive rehabilitation, with most recent admission AMPAC (Activity Measure for Post-Acute Care) score for basic mobility, 28.67 and daily activity, 23.29. Upon discharge, basic mobility improved to 34.52 and daily activity improved to 31.89. Discussion: Transverse myelitis is a neurological disorder characterized by inflammation of the spinal cord usually triggered by viral infection. Recurrent episodes are rare and may suggest an underlying autoimmune disease process such as NMO. NMO occurs when an abnormal response to infection leads to demyelination of spinal cord and optic nerve, leading to progressive weakness and autonomic dysfunction. Our patient’s predisposition to autoimmune disorders, suggested by her past history of psoriasis, may have been a clue to earlier diagnosis of NMO. This is important since each episode of NMO progressively increases disability that necessitates early aggressive rehabilitation and medical management. Conclusions: Consider the possibility of NMO in patients presenting with recurrent transverse myelitis, especially with underlying autoimmune disorders. Level of Evidence: Level V