Wilson and Hoxie have pointed out the frequent coexistence of facial asymmetry in adults with congenital or early onset superior oblique palsy and compensatory head tilt or torticollis.
Bell’s palsy may be bilateral, but only rarely is the involvement on the two sides simultaneous. The truly contemporaneous appearance of bilateral facial paralysis (facial diplegia) is most often a manifestation of the Guillain-Barré syndrome (GBS) and may also occur in Lyme disease and rarely, with HIV infection. There are numerous other causes of bilateral facial palsy, all of them infrequent. Keane (1994) listed the idiopathic (now presumably mainly viral) variety, GBS, and meningeal infiltration by tumor as the most common causes, but also found 2 cases of syphilis among 43 patients. The bilateral syndrome has been reported in approximately 7 of every 1,000 patients with sarcoidosis, although our impression is that it is more frequent. When acute in onset and associated with parotid gland swelling from sarcoidosis, it has been referred to as uveoparotid fever, or Heerfordt syndrome. In typical cases of sarcoidosis, the paralysis on each side tends to be temporally separated by weeks or more. Mononucleosis may affect both sides of the face almost simultaneously; this is probably a form of GBS. Bifacial palsy is also a feature of the developmental disorder, Möbius syndrome (see
Chap. 37).
Less common is the inflammatory disorder known as Melkersson-Rosenthal syndrome, consisting of the triad of recurrent facial paralysis, facial (particularly labial) edema, and less constantly, plication of the tongue. The syndrome begins in childhood or adolescence and may be familial. Biopsy of the lip or skin may reveal a granulomatous inflammation. The cause is not known and, despite the cardinal feature of angioneurotic edema, complement levels are normal. A series of biopsied cases has been reported by Elias and colleagues. Causes of recurrent Bell’s palsy are summarized by Pitts and colleagues.
Kennedy syndrome, causes bifacial weakness in addition to bulbar palsy as the disease progresses; preceding facial fasciculations are characteristic. Facioscapulohumeral muscular dystrophy, as the name implies, incorporates facial weakness but would not be mistaken for Bell’s palsy (see Chap. 45). The same is true for the rare form of amyloidosis associated with crystal lattice deposits in the cornea that typically involves both facial nerves.
This obscure disorder occurs mainly in females and is characterized by a disappearance of fat in the dermal and subcutaneous tissues on one or both sides of the face, giving the appearance of facial paresis. It usually begins in adolescence or early adulthood and is slowly progressive. In its advanced form, the affected side of the face is gaunt and the skin thin, wrinkled, and rather dark; the hair may turn white and fall out, and the sebaceous glands become atrophic; the muscles and bones are not involved as a rule. The condition is a form of lipodystrophy, but the localization within a myotome suggests the operation of some neural factor (possibly a growth factor) of unknown nature. A variegated coloration of the iris and a congenital oculosympathetic paralysis are found in some cases. Rarely, certain central nervous system abnormalities referable to the ipsilateral hemisphere (mainly focal seizures, migraine, trigeminal neuralgia, and ventricular dilatation), are conjoined (Hosten). The significance of these associations is unclear. Immunosuppressive treatment can stabilize the clinical course.
Aberrant Effects of Recovery From Facial Nerve Palsy