Atypical Acute Retroviral Syndrome Presenting as a Facial Palsy

Introduction

Acute retroviral syndrome (ARS) refers to signs and symptoms of acute human immunodeficiency virus (HIV) infection.¹ Clinical suspicion for ARS is often based on signs and symptoms believed to be typical of ARS. However, 70% of patients present with typical ARS and 30% present with atypical ARS,¹ which both include a broad range of signs and symptoms. Fig. 1 illustrates data from 12 studies that assessed frequencies of 19 signs and symptoms believed to be typical of ARS. Yet, frequencies of these typical signs and symptoms differed greatly across studies and the most frequent signs and symptoms are nonspecific (e.g., fever, malaise/fatigue, pharyngitis, myalgia, headache). Table 1 lists data from a study of 70 patients with atypical ARS.¹ Among just 70 patients, there were 32 unique presentations and seven patients were entirely asymptomatic. The variety of signs and symptoms, or lack thereof, associated with typical and atypical ARS makes the clinical diagnosis of ARS challenging.

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Fig. 1 Diverse signs and symptoms associated with typical acute retroviral syndrome. Data are from a study that describes primary data and reviews 11 published studies.¹ Black dots represent frequencies of signs and symptoms as reported by the studies. Red bars represent median frequencies of the signs and symptoms. Note that median frequencies do not give weight to the number of patients included in each study (range: 12–378, median = 32).

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Case Report

Second Encounter

The patient returned 2 and a half years later, reporting white spots on the right side of his tongue for 1 year and left side of his tongue for several weeks. Physical examination revealed a corrugated, white plaque on the right side of his tongue (Fig. 2A) and circular, white plaque with a 5 mm diameter on the left side of his tongue (Fig. 2B). A punch biopsy of the left-sided tongue lesion was positive for squamous hyperplasia and negative for dysplasia and neoplasm, suggesting oral hairy leukoplakia. An HIV-1 antibody immunoassay was positive, HIV-1 polymerase chain reaction revealed 5.52 log₁₀ copies/mL (reference, < 1.30 log₁₀ copies/mL), and CD4 cell count was 101 cells/μL (reference, 365–2087 cells/μL). He was referred to an infectious disease specialist and started on antiretroviral therapy.

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Fig. 2 Photos of the patient’s tongue lesions taken several months after the second encounter. (A) The right lesion remained unchanged since the second encounter. (B) During the second encounter, a punch biopsy removed the entire left lesion. The pictured lesion developed during the following months.

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Discussion

Because of the broad range of presentations, clinical examination has limited capacity to identify acute HIV infection.² Thus, organizations like the Centers for Disease Control and Prevention and United States Preventive Services Taskforce call for extensive HIV screening (i.e., at least one lifetime test and repeat testing for those with increased risk).³⁴ Still, some HIV-positive patients will slip through cracks in screening guidelines. It falls on primary care providers to catch these patients, preferably during acute HIV infection, to interrupt transmission and reduce HIV-related morbidity and mortality.⁵

During the first encounter, our patient presented with left-sided facial weakness and left- and right-sided facial sensory deficits. The latter finding suggests involvement of both trigeminal nerves, which is consistent with reports of additional cranial nerve deficits in patients with Bell’s palsy.⁶ However, no risk factors or symptoms suggested HIV infection. Although we did not investigate the potential for an HIV infection at this time, the patient likely had an acute HIV infection, as he experienced flu-like symptoms (i.e., typical ARS) before the visit and soon after developed the facial palsy (i.e., atypical ARS). By the second encounter, his HIV infection had progressed to acquired immunodeficiency syndrome (AIDS), at which time he presented with oral hairy leukoplakia. The majority of facial palsies are idiopathic (i.e., Bell’s palsy),⁷ but ∼0.1% are caused by HIV.⁸ Although few reports describe facial palsies at the time of ARS, there is a clear connection between facial palsies and HIV infections.⁸⁹¹⁰

Conclusion

We suggest that facial palsies should pique clinical suspicion for HIV, especially in the context of recent or concurrent flu- or mononucleosis-like illness.