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A 17-Year-Old Boy With Facial Palsy and Hyperacusis

2008; Lippincott Williams & Wilkins; Volume: 27; Issue: 7 Linguagem: Inglês

10.1097/inf.0b013e31816bce9f

1532-0987

Zainab Malik, Nathan Litman,

Otolaryngology and Infectious Diseases

A 17-year-old African American boy presented to the emergency department (ED) in November complaining, "I can't move my face." He had woken up that morning unable to move the right side of his face, and denied fever, headache, rash, constitutional symptoms, or any history of trauma. He was diagnosed with Bell's palsy and was discharged home on oral acyclovir and a prednisone taper. Three days later, he returned to the ED with persistent facial paralysis, now accompanied by a 2-day history of headaches and vomiting. He also complained of pain in his right ear, sensitivity to light and sound, a "pins and needles" sensation over the right side of his face, and a tingling sensation on his tongue. His medical history was significant for surgical drainage of a pilonidal cyst 2 weeks before the current illness, for which he was prescribed amoxicillin-clavulanic acid for 7 days. He had a history of varicella at the age of 5 years and of occasional cold sores on his upper lip. He had received all of his childhood vaccines, and tuberculin skin test had been negative 1 year ago. He lived in the Bronx, had no history of recent travel, and denied tick bites or exposure to animals. He was a junior in high school and worked as a lifeguard in the summer. He denied sexual activity or use of illicit drugs or alcohol. Upon admission to the hospital, he had a temperature of 38.2°C, heart rate of 110 beats/min, respiratory rate of 20 breaths/min, and blood pressure of 127/75 mm Hg. On physical examination, he was awake, alert, and complained of a frontal headache. He preferred to be in a quiet dark room, complaining that regular ambient noise was too loud. There were 2 crusted cold sores on his right upper lip and drooping of the right side of his face, with flattening of the ipsilateral nasolabial fold. His pupils were equal and reactive bilaterally, but he was unable to completely close the right eye, to wrinkle his forehead on the right side, or to puff out his right cheek. He had an asymmetric smile. There were no vesicles on his face or oral mucosa, but otoscopic examination demonstrated a crusted lesion in his right ear canal. Tympanic membranes were normal bilaterally. Neck stiffness was present, but Kernig and Brudzinski signs were negative. The remainder of his physical examination was normal. A computed tomography scan of the head was normal. Complete blood count showed a white blood cell (WBC) count of 4400/mm3 with 65% segmented neutrophils, 19% lymphocytes, and 16% monocytes. Hemoglobin was 13 g/dL and platelet count was 172,000/mm3. Urinalysis, serum electrolytes, blood urea nitrogen, creatinine, bilirubin, alkaline phosphatase, alanine transaminase, and aspartate transaminase were normal. Total protein was 7.5 g/dL and albumin 4 g/dL. A lumbar puncture revealed clear cerebrospinal fluid (CSF) with 32 WBC/mm3 (7% segmented neutrophils, 91% lymphocytes, and 2% monocytes), 25 red blood cells (RBC)/mm3, protein of 45 mg/dL, and glucose of 47 mg/dL. Gram stain of the CSF showed no organisms and no cells, and latex agglutination testing was negative. CSF polymerase chain reaction for herpes simplex virus and CSF viral culture were negative. Serum enzyme linked immunosorbent assay for Lyme disease was also negative. The patient was empirically treated with ceftriaxone and acyclovir, and he was continued on a prednisone taper. Headache and neck stiffness resolved within 24 hours of hospital admission, and ceftriaxone was discontinued once the CSF culture was negative for bacterial pathogens. A comprehensive evaluation for infectious etiologies was performed, leading to his diagnosis. Denouncement Varicella-zoster virus (VZV) was cultured from the patient's ear canal lesions. Based on this result and his clinical presentation, he was diagnosed with Ramsay Hunt syndrome (RHS). In addition, enzyme linked immunosorbent assay and Western blot for human immunodeficiency virus (HIV) were positive. His CD4+ lymphocyte count was 28 cells/μL and HIV viral load was 414,555 copies/mL. Based on the CD4 count of <200 cells/μL, the patient was also diagnosed with acquired immunodeficiency syndrome (AIDS). On further questioning about risk factors for HIV acquisition, the patient disclosed that he was sexually active with 2 female partners, and did not use condoms. The patient was treated with intravenous acyclovir (ACV) and oral prednisone for 7 days. Prophylaxis for Mycobacterium avium complex and Pneumocystisjiroveci was initiated with azithromycin and trimethoprim-sulfamethoxazole (TMP-SMX), respectively. HIV genotyping demonstrated the absence of resistance mutations, and the patient was started on a regimen of efavirenz, emtricitabine, and tenofovir 6 weeks after his acute illness. Six months after starting highly active antiretroviral therapy, HIV viral load became undetectable, and CD4 count increased to 220 cells/μL. Now 2 years later, the patient continues to have sensitivity to sound and right-sided lower motor neuron (LMN) paralysis of the face. In 1907, James Ramsay Hunt described a syndrome of facial paralysis, inner ear dysfunction, periauricular pain, and herpetiform vesicles of the pinna, now known as herpes zoster oticus or RHS. After primary infection with VZV, the virus becomes latent in the sensory ganglia, and reactivation of VZV from the geniculate ganglion precipitates the signs and symptoms found with RHS. These clinical symptoms include LMN facial palsy resulting from infection of motor fibers originating from the motor nucleus of cranial nerve (CN) VII as they pass through the geniculate ganglion; vesicles and loss of taste sensation over the anterior 2/3 of the tongue caused by inflammation of cell bodies of fibers located within the geniculate ganglion; decreased lacrimation and salivation from inflammation of parasympathetic fibers in the greater petrosal and chorda tympani nerves; vesicular eruption over the pinna because of inflammation of cell bodies of fibers of CN VII within the geniculate ganglion; and hyperacusis caused by inflammation of the stapedial nerve fibers within the geniculate ganglion.1 Some patients may present with LMN facial palsy and vesicles on the pinna and tongue with symptoms suggestive of other cranial neuropathies including CN V, VIII, IX, and X. Hunt suggested that because cranial nerve ganglia comprise a chain, inflammation of a single ganglion could extend to others, leading to additional cranial neuropathies.2 VZV reactivation, manifesting as zoster, occurs as a result of a decline in cell-mediated immunity to VZV associated with disease or normal aging. Varicella infection during the first year of life is a risk factor for zoster in childhood because of low cell-mediated immune responses in infancy.3 In addition, the incidence of herpes zoster in HIV-infected children, even in the post-highly active antiretroviral therapy era, is much higher than that of healthy children: 1.11 versus 0.1 per 100 person-years.4,5 Among HIV-infected children, the risk of developing zoster is much higher in those with CD4 lymphocyte percentage of <25% at the time of the primary varicella infection, compared with children with higher CD4 percentages.6 HIV-infected patients who develop VZV reactivation also tend to have more advanced HIV disease; a CD4 cell count of <200 cells/μL is an independent predictor of complications caused by VZV.7 Common complications after VZV reactivation in healthy children include bacterial skin infections, uveitis and keratitis, and meningoencephalitis.4 Postherpetic neuralgia, a common sequela of VZV reactivation in adults, is rarely seen in children. Immunosuppressed patients, including those with HIV, are at risk for severe complications after VZV reactivation including recurrent zoster, acute retinal necrosis, radiculitis, and myelitis. In a recent population-based study of children hospitalized with herpes zoster in Germany, immunocompromised children were significantly more likely to develop generalized herpes zoster of the skin compared with healthy children (26% versus 4%; P = 0.006).4 RHS, whereas an uncommon manifestation of zoster in childhood, has been reported in 7% of children presenting with acute peripheral facial nerve paralysis in Japan.8 There are several case reports describing RHS among HIV-infected patients.9,10 The combination of ACV and prednisone is commonly used for treatment of Bell's palsy. In a recent study of patients with Bell's palsy of no identifiable cause,11 early treatment with prednisolone alone when compared with no treatment, significantly improved the chances of complete recovery at 3 months (83.0% versus 63.6%, P < 0.001) and at 9 months (94.4% versus 81.6%, P < 0.001). Addition of ACV to prednisolone did not further improve the outcome over the use of prednisolone alone. ACV used alone, did not show any benefit. However, in this study patients with VZV infection were excluded. Specific data on treatment of RHS suggest that when the combination of ACV and prednisone was initiated within 72 hours of the onset of facial paralysis, these patients had a significantly higher rate of recovery from facial paralysis compared with those whose treatment was initiated after 7 days (75% versus 30%).12 Although HIV testing is not routinely recommended for children or adolescents with herpes zoster, it is important to consider causes of impaired cell-mediated immunity when faced with a patient with a severe complication of VZV reactivation. With the changing face of the HIV epidemic in the United States, an increasing proportion of HIV-infected individuals are young men and women who are unaware that they are at risk for HIV; hence the utility of risk-based testing has decreased.13–15 In 2006, the Centers for Disease Control revised guidelines for HIV testing of adults, adolescents, and pregnant women,16 recommending routine diagnostic HIV testing and "opt-out" screening as part of routine medical care for all adults and adolescents. With opt-out screening, HIV testing can be performed after notifying the patient, and assent is inferred unless the patient declines testing. In addition, the consent for HIV testing is incorporated into the patient's general consent for medical care, and a separate consent form for HIV testing is no longer recommended. These recommendations will serve to destigmatize the HIV screening process and will lead to early diagnosis and treatment of HIV in adolescents.