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A Immunosupressed Woman Presenting with Acute Flaccid Journal of the Louisiana State Medical Society CLINICAL CASE OF THE MONTH An Immunosuppressed Woman Presenting with Acute Flaccid Paralysis and Progressive Respiratory Failure Rebeca Monreal, DO; Arturo Vega, MD; Francesco Simeone, MD; Lee Hamm, MD; Enrique Palacios, MD; Marlow Maylin, MD; and Fred A. Lopez, MD (Section Editor) A 68-year-old woman with membranous glomerulo- use. She lived with her husband on the Mississippi Gulf nephritis and hypertension presented in late August with Coast. Previously she was functional and independent with a tremor, weakness of the upper and lower extremities, all activities of daily living. headache and nausea. She had not been feeling well for the On physical examination her temperature was 98.6˚ F previous two weeks and also reported sore throat and nasal (though as high as 101.2˚ F); blood pressure 116/83 mmHg; congestion. Four days prior to admission her temperature pulse rate of 85 beats per minute; and a respiratory rate 12 was 100.8˚ F. Other symptoms included loose stools, head- breaths per minute. She appeared anxious, but was in no ache, fatigue, muscle weakness, dyspnea, dry heaves, and respiratory distress. She was able to follow commands and double vision. She initially saw her primary care physician. move all extremities, but had generalized muscle weakness Her serum creatinine at that time was 3.9 mg/dL (baseline (strength 3/5 throughout) and an intentional tremor of her 2.7) and her creatine phosphokinase (CPK) was 275 U/L. arms bilaterally. Ciprofloxacin by mouth was prescribed and oral hydration Vancomycin, ceftriaxone, and acyclovir were started recommended. Her weakness progressed and her tremor empirically. Computed tomography (CT) scan of the head extended to her facial muscles. She developed difficulty eat- without contrast was unremarkable. Renal ultrasound (US) ing and was admitted to the hospital. There was no weight revealed kidney sizes of 10.6 cm on the right and 8.2 cm on loss, cough, dysuria, arthralgia, or skin rash. the left. Her medical treatment included mycophenolate mofetil, The day after admission her muscle weakness wors- furosemide, valsartan, enalapril, labetalol, and clonidine. ened. Her Achilles and patellar reflexes were absent; She had a hysterectomy and appendectomy remotely in biceps and triceps reflexes were 2+/4+ bilaterally. Her time. Her father had systemic lupus erythematosus. She intentional tremor worsened. She also developed asterixis denied drug allergies, tobacco, alcohol, or recreational drug and myoclonus. Cranial nerve function was intact, and fun- CME INFORMATION TARGET aUDIENCE CREDIT The November/December Clinical Case of the Month The LSMS Educational and Research Foundation is intended for medical students, general practitioners, designates this educational activity for a maximum of pediatricians, medicine subspecialists, emergency one (1) AMA PRA Category 1 CreditTM. Physicians should medicine physicians, radiologists, neurologists, and only claim credit commensurate with the extent of their pathologists. participation in the activity. EDUCATIONAL oBJECTIVES DISCLOSURE The Clinical Case of the Month is a regular educational Drs. Monreal, Vega, Simeone, Hamm, Palacios, and feature. Medical students, residents, postdoctoral Maylin have nothing to disclose. fellows, and faculty collaborate in the preparation of Dr. Lopez discloses that he is a member of the Journal these discussions. After reading this article, physicians Board of Trustees and the Journal Editorial Board. should be better able to identify and understand the pathophysiology, microbiology, epidemiology, clinical presentation, diagnosis and treatment of West Nile Virus infections. Estimated time to complete this activity is one ORIGINAL rELEASE dATE eXPIRATION dATE (1) hour. 11/30/2007 11/30/2008 298 J La State Med Soc VOL 159 November/December 2007 doscopic exam was normal. There was decreased sensation and thalamic region, petechial hemorrhages and cystic to pin-prick and light touch in the distal lower extremities. necrosis. These MRI findings were consistent with WNV- Cerebellar function could not be assessed due to weakness associated encephalitis. and tremor. An electroencephalogram (EEG) done on day five Her condition deteriorated further on the second showed periods of moderate amplitude 5 Hz theta activity hospital day. She became paraparetic, obtunded and alternating with periods of lower amplitude 2-3 Hz delta developed respiratory distress. She was transferred to the activity with brief, less than 0.5 second, attenuations. There intensive care unit (ICU). The presence of shallow breaths was a generalized attenuation of activity with stimulation. and a paradoxical motion of her chest wall prompted The patient was treated supportively in the ICU, but intubation and initiation of mechanical ventilation. no improvement in her neurological deficits was noted. Cerebrospinal fluid (CSF) examination showed red One month later and still ventilator-dependent, she was blood cell count (RBC) 1180, white blood cell count (WBC) transferred to a long-term acute care facility. Two and 114 (16% polymorphonuclear neutrophilic leukocytes a half months later, after many complications including (PMN), 59% lymphocytes, 25% monocytes), protein 78.3 recurrent episodes of ventilator-acquired pneumonia, mg/dl, glucose 78mg/dl, and negative Gram stain, India sepsis, acute renal failure, fluid and electrolyte imbalances, ink, potassium hydroxide (KOH), and acid-fast stains. and Clostridium difficile-associated colitis, she remained Cultures for herpes virus (HSV), cytomegalovirus (CMV), ventilator-dependent, with persistent paralysis and a tuberculosis, and fungi were obtained and later found to waxing and waning level of consciousness. In consultation be negative. An arbovirus panel, including West Nile Virus with her family and based on her previously expressed (WNV) serology, was requested. Serology was negative for wishes, care was withdrawn, and she expired. California encephalitis, eastern equine encephalitis, western equine encephalitis, and St. Louis encephalitis. Serum and DISCUSSION CSF studies targeting the presence of WNV infection were positive. This case of confirmed WNV encephalitis and acute Magnetic resonance imaging (MRI) of her brain, with flaccid paralysis (AFP) in an immunosuppressed patient and without contrast, (Figures 1, 2), was obtained on hospital was complicated by respiratory failure and ventilator day four and demonstrated meningeal enhancement, a high- dependency. Outlook for neurological recovery remained density focal lesion in the left caudate nucleus and thalamus poor despite prolonged supportive care. (post contrast administration). Small-vessel white-matter The WNV belongs to the flavivirus family, which disease was also noted. An MRI done for comparison nine also includes Japanese encephalitis, St Louis encephalitis, days later showed increased intensity in the basal ganglia Kunjin virus, and Murray River Valley encephalitis (found A B C Figure 1. MR Axial Sections (A) Fluid Attenuation Inversion Recovery revealed abnormal signal intensity in the left basal ganglia, (B) T2 Weighted image revealed an abnormal signal intensity in the left basal ganglia (arrow), and (C) T1 post-contrast demonstrated a focal area of abnormal enhancement in the region of the globus pallidus on the left. J La State Med Soc VOL 159 November/December 2007 299 Journal of the Louisiana State Medical Society A B C D E F Figure 2. MRI follow-up nine days after previous examination (Figure1) revealed significant progression with bilateral abnormal intensities in the basal ganglia, more pronounced on the left and associated with a minimal hemorrhagic component. Axial sections: (A) T1 Weighted image, (B) T2 Weighted image (arrow), (C) Fluid Attenuation Inversion Recovery and (D) Diffusion Weighted image. Abnormal enhancement post-contrast is identified in both basal ganglia, more pronounced on the left (E, axial section; and F, coronal section). in Australia). WNV was first isolated in the West Nile cases, 177 (4.1%) were fatal. The areas in the United States province of Uganda in 1937. According to the Centers for with a caseload of more than 275 included California (278), Disease Control (CDC), a total 4,269 cases were reported Colorado (345), Idaho (996) and Texas (354). In Louisiana, in the United States in the year 2006. These were divided 180 cases were reported in 2006. In the New Orleans area, into three categories: 1) WNV fever, 1,459 cases (a milder 17 cases were reported in Orleans Parish and 14 in Jefferson disease without neurologic involvement); 2) encephalitis/ Parish. Of note, 12 cases were reported in Harrison County, meningitis, 2,616 cases (neuroinvasive); 3) clinically Mississippi.1 unspecified, 194 patients. Of the 2,616 neuroinvasive cases, 101 had acute flaccid paralysis. Of all the 4,269 reported 300 J La State Med Soc VOL 159 November/December 2007 Transmission mechanical ventilation ranged from less than 1 to 6 days. The primary route of transmission is through the bite The duration of mechanical ventilation in survivors ranged of an infected Culex mosquito (although 36 other species of from 21 to 135 days, with a median of 49 days. The long-term mosquito also have been known to carry WNV). Mosquitoes management of this complication can be challenging.5 acquire WNV by feeding on infected birds that serve as the reservoir for the virus. Birds can remain viremic for Diagnosis up to two weeks. WNV is known to infect other animals The cerebrospinal
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