Lyme disease, also termed Bannwarth's syndrome, is caused by an infection with the spirochete Borrelia burgdorferi transmitted by tick bites. It is a systemic disease affecting the skin, nervous system, heart, and joints. Symptoms may be mild and can include fever, fatigue, migratory arthritis, myalgia, headache, meningismus, lymphadenopathy, and skin lesions. Erythema migrans, a characteristic skin lesion found in Lyme disease, is defined as an enlarging, annular erythematous skin lesion seen in half of infected patients (2).
TABLE 1 Systemic Disease Overview
Paget's disease Fibrous dysplasia Osteopetroses
Amyotrophic lateral sclerosis Mixed connective tissue disease Polyarteritis nodosum Wegener's granulomatosis Pseudotumor cerebri Kawasaki syndrome Amyloidosis
Neurologic involvement may present as meningitis, encephalitis, peripheral neuropathy, myelitis, or cranial neuropathy. Cranial neuropathies are quite common and found in 60% of patients (3). Facial paralysis is the most common neurologic manifestation affecting 10% to 31% of patients (4,5). Recent studies have demonstrated 2.2% of all patients with facial paralysis have serologic evidence of Lyme disease (3). In Western Europe, Lyme disease is now the leading cause of acute facial nerve paralysis in children and represents the most common etiology for facial paralysis in endemic areas (2). Bilateral facial nerve involvement is also common in Lyme disease and is present in 13% to 25% of cases (3,5). Children with Lyme disease have a higher incidence of facial paralysis than adults. Studies cite up to 55% involvement of the facial nerve in children (3), with an increased incidence of bilateral facial nerve involvement (2).
The mechanism of injury to the facial nerve results from direct axonal damage by the spirochetes (2). Diagnosis is made by measuring IgM and IgG titers for antibodies against B. burgdorferi. Although the IgM titers are present first, patients may not seroconvert for three to four weeks. Furthermore, the IgM response quickly dissipates. IgG antibody titers develop in patients around six to eight weeks after infection and remain elevated until the infection resolves. Unfortunately, facial nerve involvement typically occurs early in Lyme disease before seroconversion can occur (2). Additional laboratory findings can include cerebrospinal fluid (CSF) pleocytosis, elevated CSF protein, and increased erythrocyte sedimentation rate (ESR), but these are all nonspecific findings.
Treatment involves prolonged antibiotic therapy to eradicate the causative spirochetes. B. burgdorferi is highly sensitive to tetracycline but only moderately sensitive to penicillin. A standard treatment is 2g or 100mg/kg ceftriaxone daily for 14 days or doxycycline 100 mg b.i.d. for four weeks. Other antibiotics used include erythromycin and imipenem. Corticosteroids are given to treat severe carditis and arthritis. It is important to note that patients can be reinfected after curative treatment.
The prognosis for facial nerve recovery is quite good, even in the absence of antibiotic therapy. One investigation demonstrated 78% of cases with facial involvement recovered completely within three months; the remaining 22% had some residual weakness with grade I—II House-Brachmann paresis (5).
Syphilis is a systemic infection caused by the spirochete Treponema pallidum, a facultative anaerobic bacterium. Syphilis infections are extremely diverse in their presentation and are known for their ability to mimic almost any disease. Syphilis can be classified into one of five stages: primary, secondary, congenital, latent, and tertiary (6). Primary syphilis is seen with the initial infection and usually shows a chancre at the site of inoculation. Patients are typically asymptomatic. Secondary syphilis is a systemic infection with patients exhibiting fever, headache, chills, arthralgias, malaise, and photophobia. Congenital syphilis involves vertical transmission of the infection to the fetus. The spectrum of afflictions in congenital syphilis is extremely diverse and can be delayed in its presentation until adulthood. In the latent phase, patients have serologic evidence of the infection but are otherwise free of any clinical signs or symptoms. Some patients progress to tertiary syphilis marked by destructive lesions involving any organ system. Tertiary syphilis has three types: cardiovascular, mucocutaneous, and neurosyphilis. Neurosyphilis ultimately affects approximately 10% of untreated patients, with the central nervous system directly damaged by the spirochetes (7). Common sequelae of neurosyphilis include injury to the dorsal columns, so-called tabes dorsalis, as well as interstitial keratitis and cranial neuropathies. Cranial neuropathies most commonly affect the facial and cochleovestibular nerves. Facial paresis or paralysis is often found with unilateral or bilateral involvement (7). Facial paralysis almost always occurs within one year of primary infection and is usually a complete paralysis. The likely cause of damage to the facial nerve is an obliterative endarteritis of the terminal nutrient arterioles. This causes ischemia, inflammation, and ultimately, necrosis of the facial nerve (8).
Diagnosis is confirmed by various serologic tests. The venereal disease research laboratory (VDRL) or rapid plasma reagin (RPR) tests are used for screening, but both tests have a high false-positive rate. The fluorescent treponemal antibody absorption (FTA-ABS) test is a more reliable test that has high sensitivity but remains positive even after the spirochetes have been eradicated. Demonstration of the spirochetes in CSF or a positive VDRL CSF test may be required for definitive diagnosis in some cases. Treatment of neurosyphilis involves long-term penicillin (six weeks) as well as prednisone for months (30-60 mg q.d.). The prognosis for facial nerve recovery is variable but usually good with early antibiotic therapy. A direct relation between duration of paralysis and recovery was observed in a literature review of all cases of facial involvement since 1945 (9). A more detailed discussion of syphilis can be found in Chapter 15.
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