Kedar K. Adour

The true nature of Bell's palsy: analysis of 1,000 consecutive patients.

In a series of 1502 patients seen in our Facial Paralysis Research Clinic 1048 were diagnosed as having Bells palsy. Review of clinical, epidemiologic, and laboratory data, plus review of the literature, has led to the conclusion that Bells palsy is an acute benign cranial polyneuritis probably caused by reactivation of the herpes‐simplex virus, and the dysfunction of the motor cranial nerves (V, VII, X) may represent inflammation and demyelinization rather than ischemic compression. Spinal fluid analysis suggests that the disease is a phenomenon of the central nervous system with secondary peripheral neural manifestations. With our presently available information, treatment of a viral disease with an anti‐inflammatory agent is rational. Prednisone treatment started within the first week of the disease can restore better function to the paralyzed face than is achieved without such therapy, and facial nerve decompression has been unnecessary.

Bell's Palsy Treatment with Acyclovir and Prednisone Compared with Prednisone Alone: A Double-Blind, Randomized, Controlled Trial

In a double-blind study, we compared the final outcome of 99 Bells palsy patients treated with either acyclovir-prednisone (53 patients) or placebo-prednisone (46 patients). For patients receiving acyclovir, the dosage was 2,000 mg (400 mg 5 times daily) for 10 days. Electrical tests included electroneurography and the maximal stimulation test. Univariate comparisons of outcome and electrical tests between the two groups were made with χ2 analysis, Fishers exact test, and t-tests. The outcome in acyclovir-prednisone-treated patients was superior to that in placebo-prednisone-treated patients. Treatment with acyclovir-prednisone was statistically more effective in returning volitional muscle motion (recovery profile of 10; p = .02) and in preventing partial nerve degeneration (p = .05) than placebo-prednisone treatment. The t-tests indicated that the recovery profile and index means were significantly better for the acyclovir-treated group (recovery profile t = 1.99, p = .051; recovery index t = 2.10, p = .040). We conclude that acyclovir-prednisone is superior to prednisone alone in treating Bells palsy patients and suggest that herpes simplex is the probable cause of Bells palsy.

Ramsay Hunt Facial Paralysis: Clinical Analyses of 185 Patients:

In a prospective study of 1507 patients, evaluated consecutively for facial palsy in the Cranial Nerve Research Clinic at the Kalser Permanente Medical Center, Oakland, California, between 1966 and 1976, 185 cases (12%) were diagnosed as Ramsay Hunt syndrome. In 46 cases (25%), the diagnosis of herpes zoster was confirmed by acute and convalescent serum titers for varicella-zoster virus. In 139 cases (75%), viral titers were not performed and the diagnosis was based on the characteristic clinical presentation of the Ramsay Hunt syndrome. The data were subjected to multivariate analysis evaluating age, sex, race, signs and symptoms at onset, severity of paralysis, associated medical problems with concomitant neurologic deficits, and response to therapy. These were compared with data of 1202 patients with Bells (herpes simplex) palsy. The facial palsy of Ramsay Hunt syndrome was found to be more severe, to cause late neural denervation, and to have a less favorable recovery profile than Bells (herpes simplex) facial palsy. Prognostic factors and treatment recommendations are discussed.

Recurrent bell's palsy: Analysis of 140 patients

Of 1,700 patients with facial paralysis seen in a retrospective study from 1969 through 1977 and 280 patients seen prospectively from 1983 through 1986, 7.1% had recurrence of Bells palsy. In this group, the frequency of ipsilateral recurrence was equal to that for contralateral recurrence. The mean age at onset of Bells palsy was 33.0 years; Bells palsy recurred a mean of 9.8 years later. Recurrent facial paralysis did not indicate a worse prognosis for recovery regardless of which side was affected. There was no statistical difference between results for male patients or female patients, nor was there a statistically significant sex predominance, except in the age group 10 to 19 years. In our results, computed tomography (CT) scan in patients with recurrent Bells palsy detected no facial‐nerve neuroma. Of 77 patients followed a mean of 33 years after the first episode (range, 2.8 to 60 years), none showed progressive facial‐nerve dysfunction or any signs of tumor. We conclude that an ipsilateral recurrence of facial paralysis without documented evidence of a tumor does not warrant a transmastoid decompression of the facial nerve. The results of our analysis were verified prospectively as well as retrospectively. A new classification system is introduced for ease of computer analysis and for simplified discussion of recurrent facial paralysis.

Prevalence of Concurrent Diabetes Mellitus and Idiopathic Facial Paralysis (Bell's Palsy)

Diabetes mellitus was present in 11.4 per cent of 684 patients with Bells palsy, in 28.4 per cent of the sixty-seven with recurrent or bilateral palsy, and in 16.8 per cent of the 440 with palsy who were thirty years or older. Diabetes was present in only 3.8 per cent of 27,399 persons thirty years or older who had never had Bells palsy and who underwent multiphasic health testing. These figures clearly indicate that diabetes is more common among patients with Bells palsy than among persons who have never had that disease; and that the risk of Bells palsy is increased in patients with diabetes. The diabetic patient is more prone than the nondiabetic person to nerve degeneration, and this tendency to nerve degeneration is not age-related. Although 10 per cent of our patients with Bells palsy and known diabetes were younger than thirty-nine years, we now advise screening for blood sugar elevation only for patients who are forty years or older, or who have recurrent or bilateral facial paralysis.

Auditory symptoms associated with herpes zoster or idiopathic facial paralysis.

Auditory symptoms (hyperacusis, tinnitus, decreased hearing) have long been recognized to accompany herpetic or idiopathic facial paralysis. Twenty‐nine percent of 1,080 patients with idiopathic facial paralysis and 37 percent of 172 with herpes zoster oticus facial paralysis had auditory symptoms. Abnormal related sensori‐neural hearing loss was documented in only 11 of these 377 patients with auditory complaints. All of the 11 had a diagnosis of herpes zoster oticus.

Acute Superior Laryngeal Nerve Palsy: Analysis of 78 Cases

Acute superior laryngeal palsy is often clinically unrecognized and frequently overlooked. Yet, this motor paralysis occurs more frequently than facial paralysis. Like acute facial paralysis, superior laryngeal palsy often occurs as part of a cranial polyneuritis that is probably related to herpes simplex virus reactivation. Rotation of the larynx and shortening of one vocal cord have been found in such diverse syndromes as vestibular neuronitis, migraine and tension headaches, unexplained cough, tinnitus aurium, globus hystericus, and carotidynia. Electromyographic studies suggest that laryngeal spasm may be caused by faulty regeneration of the superior, rather than the recurrent, laryngeal nerve.

The Facial Paralysis Prognostic Index

The treatment of facial paralysis requires a reliable estimate of prognosis as well as an accurate diagnosis. Although the majority of patients recover, the degree of subsequent facial function varies. An estimation of the risk of nerve degeneration and subsequent disability is needed to guide therapy and to advise patients. Currently, prognosis is obtained by clinical evaluation of initial signs and symptoms, specific nerve excitability and function testing, and response to medical therapy. This study attempts to identify and quantitate factors that predict the outcome of facial paralysis. In 1214 patients with Bells palsy, we identified factors associated with a severe course or poor outcome. We analyzed the predictive value of each item in an extensive research protocol, estimating the relative contribution to the total risk of unfavorable outcome by means of multiple regression models. With the use of these data, we have developed the facial paralysis prognostic index (FPPI). The FPPI can assist otologists to more accurately estimate outcome based on physical examination and electrical testing.

Herpes Simplex Polyganglionitis

Evidence suggests that many cranial nerve syndromes, such as migraine headache, acute vestibular neuronitis, globus hystericus, carotidynia, acute facial paralysis (Bells palsy), and Menieres disease, are caused by the neurotropic herpes simplex virus (HSV). Because transitory cranial nerve dysfunction during acute HSV infection can be asymptomatic but often occurs in conjunction with mucocutaneous vesicles, we tested five subjects with herpes labialis for cranial nerve dysfunction. Four of the subjects had hypesthesia of the trigeminal nerve (which recurred in two); four, hypesthesia of the glossopharyngeal nerve; and two, hypesthesia of the second cervical nerve. Three of the subjects had positional or spontaneous nystagmus (which recurred in one); one of the subjects had a unilateral, decreased caloric response of 50%. Unilateral weakness of the cricothyroid muscle or the palate occurred in three of the subjects (and recurred in one). Volitional electromyograms were normal in all the subjects, but two of the subjects had increased facial nerve latency (which recurred in one). Similar findings of an acute, transitory nature should suggest to the clinician a viral polyganglionitis caused by HSV infection.

Maximal nerve excitability testing versus neuromyography: Prognostic value in patients with facial paralysis†‡

Neuromyography (NMG) is compared with maximal nerve excitability testing (NET) as a prognostic tool in facial paralysis. Normal latencies, summation muscle action potentials, and test/retest reliability were determined in three groups of subjects. NMG was performed in 33 control subjects of Group 1 to determine test/retest reliability. To determine the range of normal facial nerve conduction latency and amplitude and configuration of the summation muscle action potentials, NMG was performed in 172 otolaryngologic control patients without facial paralysis. NMG arid NET were then compared in Group 3, which consisted of 43 patients with Bells palsy. Fluctuations in NMG were then analyzed in a fourth test series of three subjects from Group 1, in whom summation muscle action potentials at various positions of the electrodes were compared. The high rate of fluctuations observed indicates the need for further assessment of test/retest reliability and standardization of normal values in NMG. Our results indicate that maximal NET is more reliable than NMG for prognosis of facial paralysis.