CASE PRESENTATION
An elderly man over 70 years old with Alexander’s disease (AD), a rare
autosomal dominant leukodystrophy, presented to our hospital with fever
and decreased blood oxygen saturation, which began two weeks prior. He
visited the neurology department of our hospital nine years ago due to
unsteady gait, dysarthria, and difficulty in swallowing and had been
diagnosed with AD. Since then, his symptoms, such as ataxia of limbs,
dysarthria, and dysphagia, progressed, and he underwent gastrostomy and
tracheostomy surgery two years ago.
On examination, the patient had a blood pressure of 104/62 mmHg; pulse,
105 bpm; SpO2, 93% in room air; and fever, 37.9 °C. He had difficulties
in swallowing; speaking, which made it difficult for him to complain
about back or abdominal pain; poor coordination; and loss of motor
control due to AD.
Laboratory studies demonstrated a white blood cell (WBC) count of 10,800
(reference value: <8000), erythrocyte sedimentation rate of 84
mm/h (reference value: <10 mm/h), and C-reactive protein (CRP)
level of 5.69 mg/dL (reference value: <0.3 mg/dL). Blood
culture revealed the presence of methicillin-resistant Staphylococcus
aureus. Thoracic and abdominal computed tomography revealed partial
atelectasis in the right lower lobe of the lung and infiltrative shadows
in both lower lobes of the lung, as well as bilateral kidney stones,
bladder stones, and hydronephrosis. An enlarged right psoas major muscle
and iliopsoas muscle with a 70 × 80 mm low intensity area were
observed.(Figure 1A) The margins were enhanced in a ring shape, which
was considered as an abscess. The patient then underwent percutaneous
drainage of the abscess. A total of 350 mL of fluid was aspirated
(Figure 1B) and methicillin-resistant S. aureus was detected. After
drainage, his fever disappeared, his WBC count decreased to 8000, and
CRP to 0.72 mg/dL. However, four months after drainage, the abscess
returned. Laboratory studies revealed a WBC count of 17,400 and CRP
level of 9.23 mg/dL. Percutaneous drainage was performed again, and 400
mL of fluid was aspirated. Similar to the previous instance, the fever
abated, and the inflammatory findings improved after drainage (CRP
level, 0.72 mg/dL). There was no recurrence of the abscess for one year
(Figure 2); however, a year after the second drainage, the patient
expired due to respiratory failure caused by AD.
Image 1
(A)Axial view of abdominal computed tomography showing a gigantic right
iliopsoas abscess (arrow). (B)Abscess more than 350 ml aspirated by
percutaneous drainage.