A 6-year-old girl presents with bilateral lower leg swelling, progressive fatigue,and ultimately, refusal to walk secondary to severe pain, which began a month ago.
She had a respiratory illness along with mouth sores one month prior to development of these symptoms. There is no history of fever, rash, travel or sick contacts. Family history is significant for leukemia, breast cancer, colon cancer, and osteoarthritis.
She is afebrile and has normal vital signs.Her lower extremity examination shows non-pitting edema over both ankles and knees. There is no tenderness or crepitus on palpation of her lower extremities. She moves her lower extremities spontaneously when lying in bed and has normal strength. She has pain with full extension of knees as well as with dorsiflexion of feet. There is no spinal tenderness, or limitation in range ofmotion of her back and hips. Sensations and deep tendon reflexes are intact bilaterally. She has significant apprehension when asked to walk, and bears weight briefly on her toes. In addition, she demonstrates marked emotional lability.
Laboratory results are as follows: WBC count 18.7K/mL (71% neutrophils, 0%
Bands), Hgb 10.4 g/dL, a platelet count 611 K/mL, and a normal peripheral smear.
Serum electrolytes, BUN, creatinine, liver enzymes, LDH, uric acid, creatinine kinase, aldolase and antineutrophil cytoplasmic antibodies (ANCA) are within normal limits. Her erythrocyte sedimentation rate is 127 mm/hr (0–10 mm/hr) and C-reactive protein is 5.8 mg/dL (0.0–0.9 mg/dL).MRI of the lower extremities reveals normal osseous structures with edema within the popliteal fossae and patchy muscular and fascial edema bilaterally .
Evaluation for infectious processes, including bacterial cultures of blood and stool, respiratory viral panel, PPD, and serologic evaluation for Bartonella,
Parvovirus B19, Coccidiomycosis, EBV, CMV and mycoplasma
were negative. A bone scan was normal. An MRI of the brain and spine performed, after consultation with neurology because of behavioral changes, to rule out secondary CNS involvement, were normal.
She remains nonambulatory during first few days of hospitalization, until further evaluations reveal the diagnosis.
What do you think this investigation which was requested?
What is the diagnosis?
What is the treatment?
Because of markedly elevated inflammatory markers and inability to ambulate, our differential diagnosis included infectious, post-infectious, rheumatologic, and oncologic processes.
some of these have been excluded by the above mentioned investigation.
the test which reveal the diagnosis : ASO and DNAse B titers results came back at 447 IU/mL (0–99 IU/mL) and 340 (</ ¼ 170),
Because of markedly elevated inflammatory markers and inability to ambulate, our differential diagnosis included infectious, post-infectious, rheumatologic, and oncologic processes.
some of these have been excluded by the above mentioned investigation.
the test which reveal the diagnosis : ASO and DNAse B titers results came back at 447 IU/mL (0–99 IU/mL) and 340 (</ ¼ 170),
respectively. Thus a diagnosis of post streptococcal myositis
and fasciitis was made.
She was started on oral amoxicillin and non-steroidal antiinflammatory medications. In addition, physical therapy
was initiated, and within three days she showed marked
improvement. She was discharged home to complete a tenday
course of amoxicillin. At her one month follow up, she was back to her baseline activity level without pain, and her ASO titer and ESR had normalized.
Lessons for the Clinician:
• It is important to consider post-streptococcal related myalgia/myositis in the differential diagnosis of children presenting with diffuse muscle and fascial inflammation.
• Post-streptococcal related myalgia/myositis can have atypical
presentations, such as isolated myalgia and can mimic other serious diseases.
• Serum levels of muscle enzymes are normal in patients with post-streptococcal myositis.
Remember :
Post-streptococcal myositis has been reported in patients ranging is ages from 6 to 39 years and there has been a slight female predominance. All patients have presented with severe, debilitating myalgia, generally affecting the proximal muscles of the upper and lower extremities.
Patients generally have a neutrophil predominant leukocytosis,
mild anemia, thrombocytosis, elevated inflammatory markers, normal muscle enzymes and elevated ASO titer.
MRI would remain the modality of choice for establishing
diagnosis, and in our case indeed revealed inflammation
of both muscle and fascia without any bony involvement.
Imaging studies were not performed in the previously reported cases.
Treatment strategies have been variable, but generally included use of anti-inflammatory medications, antibiotics against group A Streptococcus and occasionally steroids for
refractory cases.
Response to treatment is generally good, with most patients returning to baseline along with resolution of serologic evidence of inflammation.
and fasciitis was made.
She was started on oral amoxicillin and non-steroidal antiinflammatory medications. In addition, physical therapy
was initiated, and within three days she showed marked
improvement. She was discharged home to complete a tenday
course of amoxicillin. At her one month follow up, she was back to her baseline activity level without pain, and her ASO titer and ESR had normalized.
Lessons for the Clinician:
• It is important to consider post-streptococcal related myalgia/myositis in the differential diagnosis of children presenting with diffuse muscle and fascial inflammation.
• Post-streptococcal related myalgia/myositis can have atypical
presentations, such as isolated myalgia and can mimic other serious diseases.
• Serum levels of muscle enzymes are normal in patients with post-streptococcal myositis.
Remember :
Post-streptococcal myositis has been reported in patients ranging is ages from 6 to 39 years and there has been a slight female predominance. All patients have presented with severe, debilitating myalgia, generally affecting the proximal muscles of the upper and lower extremities.
Patients generally have a neutrophil predominant leukocytosis,
mild anemia, thrombocytosis, elevated inflammatory markers, normal muscle enzymes and elevated ASO titer.
MRI would remain the modality of choice for establishing
diagnosis, and in our case indeed revealed inflammation
of both muscle and fascia without any bony involvement.
Imaging studies were not performed in the previously reported cases.
Treatment strategies have been variable, but generally included use of anti-inflammatory medications, antibiotics against group A Streptococcus and occasionally steroids for
refractory cases.
Response to treatment is generally good, with most patients returning to baseline along with resolution of serologic evidence of inflammation.
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