Kawasaki Disease

This disease, named after the Japanese pediatrician who first described it in 1967, consists of a systemic vasculitis that manifests with coronary vessels aneurysms and is accompanied by high fever, skin rash, and enlarged neck lymph nodes. Other symptoms, such as painful and swollen joints, abdominal pain, diarrhea, irritability, and headaches, can also be observed. Most reports in the literature come from Asian countries and involve children under the age of 5. Facial nerve involvement is possible as a neurological manifestation of Kawasaki disease, presumably due to ischemia resulting from inflammation of the surrounding arteries. In the majority of cases, FP is unilateral, but bilateral involvement has also been reported.3

Kawasaki disease is a rare disease in which an infectious or genetic cause can be suspected; however, as yet no specific etiology has been identified. Diagnosis is not helped by any laboratory test and relies mainly on the age group involved (mostly children) and the presence of the heart disease. Aspirin and high-dosage intravenous gamma globulin are usually the treatment of choice that decreases, in the majority of the cases, the morbidity of the disease.

Neuroborreliosis or Lyme Disease

This pathological entity has been named after the report from a city in Connecticut (i.e., Lyme), where it was provoked in a school community by the bite of an insect (genus Ixodes, commonly called ticks) that caused multiple neurological involvement.4 Facial nerve palsy is part of the syndrome along with other typical signs, like redness, fatigue, malaise, and fever. The insect′s bite produces a bacterial infection via the spirochete Borrelia burgdorferi, mostly carried by white-tailed deer and white-footed mice ( Fig. 7.1 ).

Neuroborreliosis (NBD) usually evolves in three stages: the first is characterized by erythema migrans at the site of the bite. This is often described as a “bull′s eye” rash: a flat or slightly raised red spot at the site of the tick bite. This can be quite large and expanding in size with a clear arc in the center. In the second phase, days to weeks later, dissemination of the infection takes place and neurological symptoms may be seen; the third phase (months to years later) only develops in untreated subjects and corresponds to the chronic stage of the disease. Ten percent of patients with NBD develop unilateral or bilateral FP during the second or third stage of the disease, which usually resolves completely, although electrophysiological testing may reveal an incomplete facial recovery in a certain number of affected subjects.5

NBD must be considered when AFP occurs in the northern hemisphere and during summertime. These cases are often bilateral. A correct diagnostic work-up should include, among suspected cases, serologic testing like single step VIsE (IR6) enzyme-linked immunosorbent assay for detection of immunoglobulin G antibodies to Borrelia burgdorferi peptide, and of immunoglobulin G and immunoglobulin M antibodies to sonicate Borrelia burgdorferi antigens.6 This test has been shown to express positivity during all stages of the disease, be less time-consuming, and as reliable as the formerly recommended two-tier test, which may, instead, be negative in the early stages.7 Treatment consists of 10 to 14 days of antibiotics (ceftriaxone, 2 g/day; doxycycline, 100 mg twice a day; or azithromycin, 500 mg/day) that allow complete remission of the disease when administered in the early, first stage of NBD.

Melkersson-Rosenthal Syndrome

This rare disease of unknown etiology (with supposed genetic predisposition) consists of a triad of symptoms: recurrent orofacial edema (mainly the upper lip), relapsing facial palsy, and fissured (folds and furrows) tongue. Interestingly enough, Melkersson in 1928 described the coexistence of recurrent facial palsy with angioneurotic edema, and Rosenthal (1931) added the fissured tongue to the clinical picture that was eventually named after both of them by Luscher in 1949. The most frequent sign is fissured tongue (granulomatous cheilitis of Miescher), while association with FP occurs in half of the patients and the complete triad is only present in 25% of patients. This condition usually starts in the second decade of life, and the manifestations usually occur sequentially and alternating (rarely simultaneously). Bilateral, sequential FP with relapses generally coincides with the side of facial swelling. Melkersson-Rosenthal syndrome may at times conceal Crohn disease or sarcoidosis. Attacks may occur as frequently as days apart or be separated by years. Swelling may persist and increase after recurrent attacks, eventually becoming permanent. Treatment is symptomatic and may include medication therapies with nonsteroidal anti-inflammatory drugs and corticosteroids to reduce swelling, as well as antibiotics and immune-suppressants. Facial nerve decompression in its entire course has also been proposed to avoid or reduce the number of relapsing episodes.8