S193 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 treatedwith steroids.
Patient was transferred to acute inpatient rehab without a known eti-
ology 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 abduc-
tion, 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 sig-
nificant 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 associatedwith interstitial lung disease andmalignancy. Diagnosis
is confirmed with muscle enzyme levels, autoimmune antibodies, elec-
tromyography, and muscle biopsy. Treatment involves immunosuppres-
sion. 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 ex-
tremities. 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 performactive 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
with a rolling walker and contact-guard assistance. Patient was trans-
ferred to acute inpatient rehabilitation to address her functional limi-
tations. 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 negotia-
tion, 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 an-
tibodies, which are associated with statin-induced necrotizing myop-
athy. Her right quadriceps biopsy revealed necrotizing myopathy
without significant inflammation or chronic myopathic features.
Discussion:
IMNM shows minimal inflammation, but can occur in as-
sociation 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 Re-
lationships To Disclose
Case/Program Description:
Patient is a 65-year-old woman with his-
tory 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 al-
terations 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 character-
ized 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 auto-
immune disorders.
Level of Evidence:
Level V
S193
Abstracts / PM R 9 (2017) S131-S290