Clinical Pearls Facial Weakness and Rash Frank Birinyi, MD

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1 1144 ACADEMIC EMERGENCY MEDICINE DEC 1996 VOL 3/NO 12 Clinical Pearls Facial Weakness and Rash Frank Birinyi, MD I FIGURE 1. The patient on arrival to the ED, attempting to smile and wrinkle his forehead. I FIGURE 2. The vesicular lesions of the patient s neck and external ear.... From The Ohio Stare University, Columbus, OH, Depainnent of Emergency Medicine (FB); and Mount Carmel Medical Center, Mount Carme1 East Hospital, Columbus, OH, Department of Emergency Medicine (FB). Photographic critique: Michael A. Morris, University of Arkansas for Medical Sciences, Little Rock, AR. Section editor: Lawrence B. Stack, ML2 Depanmenr of Emergency Medicine, Vaiiderbilt University Medical Center, Nashville, Th? Received: December 22, 1995; revision received: March ; accepted: March 20, 1996; updated: April 21, Address for correspondence and reprints: Frank Birinyi, MD, 6558 Masefield Streer, Worthington, OH esi@netset.com Chief Complaint: Facial weakness and rash. History of Present Illness: This 24-year-old graduate student presents to the ED with a complaint that he woke up this morning and looked in the mirror and one side of his face did not work. Nine days earlier he noticed pain in the left neck and postauricular region. Two days later he developed a vesicular rash around and below the ear on the left. In the hours prior to his ED evaluation, he noted some weakness of the left side of the face that progressed to almost complete paralysis. He notes that his eyelid does not shut completely and that his eye dries out. Other symptoms include mild dysgeusia and mild hyperacusis. He denies viral prodrome, facial or oral numbness,

2 Facial Weakness and Rash, Birinyi 1145 dizziness, dysphagia. hoarseness, extremity weakness, or dysphasia. There is no history of cancer or stroke. His only medicine is ranitidine. 300 mg, at bedtime for hyperacidity. There is no risk factor for immunosuppression. Physical Examination: The patient is alert, oriented, and cooperative. Vital signs include temperature 36.9"C (oral), pulse 62 beats/min, blood pressure 1W80 mm Hg, and respiratory rate 20 breathshin. Cranial nerve exarnination reveals the pupils to be equal and reactive to light. The visual fields are normal. The fundi are normal. The extraocular muscles are intact. Corneal sensation is present and symmetric. Facial symmetry testing shows good strength in the right side of the face but a peripheral facial nerve weakness of the left (Fig. 1). The patient can partially close the left eye, and Bell's phenomenon (turning upward of the globe during attempted eye closure) is present. He has normal sensation over the face. His tongue and palate are Symmetric. There is no bruit over the head or neck. The neck strength is good. There are vesicular and crusting lesions around the left ear and the left side of the neck (Fig. 2). Manual muscle testing is normal. Deep tendon reflexes are 2+ and symmetric. Sensory examination is normal to light touch, pinprick, temperature, and passive motion. Cerebellar testing is normal. There is no pronator drift. Toes are down-going. The remainder of the physical examination is normal. Ancillary Studies: The complete blood count, serum electrolytes, BUN, creatinine, and glucose are normal. HIV testing is negative. (The correct diagnosis and discussion Qppear on page J

3 Communication Strategies for Specific Situations, Torten et al an autopsy: its importance for patients and physicians. Am J Med. 1989; 86: Knopp R. Practicing cricothyrotomy on the newly dead. Ann Emerg Med. 1995; 25: Iserson K. Law versus life: the ethical imperative to practice and teach using the newly dead emergency department patient. Ann Emerg Med. 1995; Goldblatt AD. Don t ask, don t tell: practicing minimally invasive resuscitation techniques on the newly dead. Ann Emerg Med. 1995; 25~ Olsen J, Spiliger S, Windisch T. Feasibil- gency department. Ann Emerg Med. 1995; 25: ity of obtaining family consent for teaching Key words: physician -patient communicacricothyrotomy on the newly dead in the emer- tion; medical education; patient interview. Clinical Pearls (cont. from page 1145) Diagnosis: Ramsay Hunt syndrome (herpes zoster cephalicus, herpes zoster oticus, herpes zoster auricularis). Discussion: Dr. J. Ramsay Hunt, an American neurologist, described in 1907 the syndrome of herpes zoster associated with facial palsy. The syndrome now bears his name [i.e., the Ramsay Hunt syndrome (RHS)]. This condition should not be confused with another neurologic syndrome ( dyssynergia cerebellaris myoclonica ), which also carries his name. The frequency of herpes zoster in patients with facial weakness has been calculated to be 6-12%.2.3 Women are affected slightly more than men, and the overall incidence is higher in the fifth decade and bey~nd.~.~ The initial symptoms in RHS include a viral prodrome and pain in and around the ear. Occasionally vomiting, diarrhea, and fever are present. Facial weakness and herpes zoster infection usually develop concurrently about 1 week later. The facial weakness is of the lower-motorneuron type and is identical to that seen with idiopathic (Bell s) facial nerve palsy. Weakness of both the upper and lower face is present. Asymmetry and muscle weakness are appreciated when the patient is asked to wrinkle the forehead or raise the eyebrows, close the eyes tightly, pucker the lips, blow out the cheeks, and smile. Subtle findings include differences in the forehead creases and the levels of the eyebrows, a blink lag, effacement of the nasolabial fold, lower teeth showing, sclera showing (because of a lower lid sag), and talking out of one side of the mouth. Other signs and symptoms in RHS, as in Bell s palsy, include aberrant taste (dysgeusia, ageusia), dry eye, hearing loss (sensorineural or conductive), hyperacusis (phonophobia), and disturbed vestibular function. A variety of other neurologic signs (facial and tongue numbness, corneal anesthesia, dysphagia, inability to elevate the palate, hoarseness) may be associated with RHS secondary to the communications the facial nerve makes with cranial nerves V, IX, and X. Thus, RHS may manifest as a cranial polyneuropathy. Following the facial nerve, the (descending) order of frequency of involvement of the other cranial nerves is VIII, IX, V, and X.4 The rash of RHS is herpes zoster caused by the varicella-zoster virus. The rash develops on and around the ear (including the mastoid) and also can involve the face, neck, shoulders, tongue, buccal mucosa, palate, uvula, and larynx. This distribution is dependent on which sensory ganglia are involved. Nerves that communicate with the facial nerve may be involved, including the cranial nerves as well as the cervical plexuses from cervical nerves 2, 3, and 4. The clinical course of RHS is variable. The rash (erythema, vesicles) and facial weakness usually appear con- c~rrently.~ Facial weakness progresses to its maximum by days.2* Complete paralysis occurs in 67% of patients and is particularly seen in older patients; 80% of patients >50 years old have complete paralysk2 All patients show some degree of recovery, and maximum recovery is noted by 6-12 months. Recovery is related to the maximal degree of loss of function and to age. Complete recovery without complications has been noted in 66% of patients with partial weakness, and 10% of patients with complete paralysis. Older patients have a poorer recovery. In general, when compared with Bell s palsy, RHS has worse prognosis. Most (>go%) patients with Bell s palsy make satisfactory recoveries, compared with 60% of RHS patients5 Resolution of the zoster infection itself occurs over days.6 Other long-term complications of RHS include tearing, spasm, synkinesis (mass facial motion) of the eyelids and mouth, ptosis of the eyebrow, and dryness of the Post-herpetic neuralgia may develop, although usually not to a severe degree. Anatomy: In his original paper, Dr. Hunt attributed the syndrome to reactivation of the dormant varicella-zoster virus within the geniculate ganglion; he described geniculate ganglionitis. In light of the anatomy, the otolaryngologic findings of RHS (and Bell s palsy) are fairly clear to understand (Fig. 3). The facial nerve, along with cranial nerve VIII, follows a horizontal and lateral course through the internal auditory meatus and petrous portion of the temporal bone to the inner ear. Subsequently, the nerve travels between

4 1154 ACADEMIC EMERGENCY MEDICINE DEC 1996 VOL 3/NO 12 I FIGURE 3. The facial nerve and its branches as it courses through the temporal bone. the cochlea and semicircular canals and then makes an abrupt posterior turn. This posterior turn forms the genu, the site of the geniculate ganglion. A clinically important branch from the geniculate ganglion, the greater petrosal nerve, carries secretory fibers to the lacrimal gland. Beyond the geniculate ganglion, the facial nerve travels posteriorly and then turns downward, toward its exit from the skull through the stylomastoid foramen. Clinically important nerve branches of this vertical portion of the facial nerve include the stapedius, the chorda tympani, and a small sensory branch (that travels with a branch of the vagus nerve) to the ear. The stapedius branch innervates the stapedius muscle, which dampens, via the stapes, sounds >80-85 db. The chorda tympani carries both taste and sensory afferents from the tongue. After the facial nerve exits the stylomastoid foramen, it enters the parotid gland. Differential Diagnosis: Facial weakness with associated vesicles establishes the diagnosis of RHS. The absence of rash, however, in the ED patient who presents with acute facial weakness does not completely rule out RHS.' There are several considerations in this regard. First, the vesicles may not have erupted yet, since the zoster may appear up to 10 days after the weakness develops. Second, in the early stages, only erythema may be present. Third, vesicles may be present, but in a location not easily appreciated on cursory inspection, such as behind the ear or on the oral mucosa. Fourth, vesicles may have appeared and already resolved by the time of the patient's presentation. Therefore the ear and particularly the skin behind the ear, the concha, tongue, buccal mucosa, palate, and uvula should be examined closely for vesicles, erythema, or small crusted lesions. As in zoster elsewhere in the body, the rash is char- acterized by a papulovesicular eruption on an erythematous base. A rash that is limited to a small area may be herpes simplex type I. If the rash is recurrent, herpes simplex type I is more likely, since zoster is rarely recurrent. In those situations in which zoster is recurrent, leukemia should be strongly considered.* If a patient's facial weakness is not associated with a rash, other etiologies should be entertained. Although facial weakness in ED patients is frequently diagnosed as idiopathic (Bell's) palsy, Bell's palsy remains a diagnosis of exclusion. Ninety other conditions have been reported to cause facial weakness.' These include trauma (basal skull fracture, facial injury), infection (otitis media, chickenpox, Lyme disease), and metabolic etiologies (diabetes, hypertension).' Significant etiologies for emergency physicians to consider include tumor and cerebrovascular infarct. A cerebrovascular infarct should be suspected with lower facial weakness and an intact upper face. Speech may be slurred secondary to tongue weakness. Paresis of the upper extremity, beginning with the thumb, fingers, and hand, suggests a middle cerebral artery infarct. Anterior cerebral artery infarcts, on the other hand, usually affect the lower extremity. A patient with a history of cancer (particularly breast, lung, ovarian, prostate, thyroid, or kidney) and new-onset facial palsy must be suspected to have metastatic tumor.' Cranial nerves V-XI1 should be carefully evaluated because each of these nerves traverses the temporal bone. Although a prolonged and slowly progressive or relapsing course is suggestive of tumor, the onset of symptoms (sudden or delayed, complete or incomplete) is not diagnostic in differentiating the causes of facial weakness. Half of patients with Bell's palsy may present with a complete and sudden onset; 40% of patients with facial weakness secondary to a facial nerve tumor present in similar fashion. Both Bell's palsy and facial nerve tumor also may manifest as recurrent (ipsilateral) facial weakness.' Treatment: Acyclovir is widely mentioned in the literature for the management of RHS. However, no study to date has proven its benefit. There is no literature to support the use of acyclovir in the normal, immunocompetent host. Acyclovir is indicated, however, in patients with immunosuppression or encephalitis. It is generally well tolerated IV and the dose is' 5-10 mg per kg, 3 times a day for 7-10 days.'o-ls Steroids are used by some; their benefit is equivocal and there is no universal agreement as to their effica~y.~*'~~'~ Their use is presumed to result in a lower incidence of complete paralysis and fewer long-term complication~.~ Doses of prednisone reported in the literature are usually about 60 mg a day initially. The total duration of treatment is usually days, including a 1-week taper,3.6j5,16 Surgery to decompress the facial nerve has

5 ~~ Facial Weakness and Rash, Sirinyi 1155 been recommended for some patients, but its efficacy also is unproven. Eye care is an important component of treatment to avoid drying and resultant corneal ulceration, perforation, and potential loss of the eye. In patients with facial weakness, the eye is vulnerable to drying out for several reasons. There may be incomplete eye closure, absence of Bell s phenomenon, a paralytic ectropion of the lower lid, loss of corneal sensation, and decreased tear production. Patients with the BAD syndrome (lack of Bell s phenomenon, Anesthetic cornea, Dry eye) almost always have eye complications, and close ophthalmology followup is necessary. For patients with eye symptoms (pain, redness, or irritation), the goal of therapy is to keep the globe moist. Depending on the degree of dryness, this can be accomplished by voluntarily blinking, the use of eye drops and ointments, and protective devices. Taping plastic film (Saran wrap) over the orbit at night is effective to keep the globe moist. Patients who continue to be symptomatic with exposure keratitis in spite of medical therapy may require implantation of a gold weight in the upper lid to improve upper lid closure. Tightening of the lower lid for paralytic ectropion also may be required. Nonopioid analgesics may be given for pain because opioids are rarely required for pain control. For patients in whom the rash is significantly symptomatic (e.g., painful), steroid cream may be prescribed for the zoster 4 times a day for 5-10 days, or until the lesions clear. Importantly, patients with facial weakness due to facial nerve palsy should be reassured they are not having a stroke. They can be released from the ED and instructed to follow up with an otolaryngologist in 1-2 weeks. Should the facial weakness subsequently worsen, however, the patient should obtain prompt follow-up. Clinical Pearls: The RHS consists of facial nerve weakness and herpes zoster infection involving the ear; face, neck, shoulders, or oral mucosa. It may mangest as a cranial nerve polyneuropathy. Patients with facial nerve weakness should be carefilly examined for associated rash (erythema, vesicles, crusted lesions). Patients with facial nerve weakness (without rash) should be carefully evaluated to eliminate the diagnoses of tumor and cerebrovascular infarct. Patients with facial nerve weakness should be examined for the BAD syndrome (lack of Bell s phenomenon, Anesthetic cornea, Dry eye) and ophthalmologic follow-up should be arranged, if indicated. There is no literature to support the use of acyclovir in the immunocompetent host. The benejts of steroids are unclear. Photographic Critique (by Michael Morris): Figures 1 and 2 are very good clinical photographs. Background control is excellent; exposure and lighting are good. Because of the care taken at the time of photography, the presenting symptoms are patently evident to the viewer. The overall appearance of the 2 photographic illustrations is quite professional and illustrates well the signs outlined in the text. The only suggestions to improve these photographs would be to move in closer on Figure 1 to increase the size of the face in the final illustration, remove the patient s gown (in both views) to eliminate the only distracting element in the 2 photographs, and use a rim light (from behind the patient) to provide some separation between the patient s hair and the black background. Figure 3 was drawn by Tim Vojt. The author thanks Dr. Mark May for reviewing the manuscript. I REFERENCES 1. Hunt JR. On herpetic inflammations of the geniculate ganglion. A new syndrome and its complications. J Nerv Ment Dis. 1907; 34: Devriese PP, Moesker WH. The natural history of facial paralysis in herpes zoster. Clin Otolaryngol. 1988; 13: Robillard RB, Hilsinger RL, Adour KK. Ramsay Hunt facial paralysis: clinical analysis of 185 patients. Otolaryngol Head Neck Surg. 1986; 95~ Aviel A, Marshak G. Ramsay Hunt syndrome: a cranial polyneuropathy. Am J Otolaryngol. 1982; 3: May M, Podvinec M, Ulrich J, Peiterson E, Klein SR. Idiopathic (Bell s) palsy, herpes zoster cephalicus and other facial nerve disorders of viral origin. In: May M (ed). The Facial Nerve. New York: Thieme. 1986, pp Hirsch BE. Infections of the external ear. Am J Otolaryngol. 1992; 13: Hunt JR. A further contribution to the herpetic inflammations of the geniculate ganglion. Am J Med Sci. 1908; May M. Differential diagnosis by history, physical findings, and laboratory results. In: May M (ed). The Facial Nerve. New York: Thieme, 1986, pp May M, Klein SR. Differential diagnosis of facial nerve palsy. Otolaryngol Clin North Am. 1991; Rothschild MA, Drake W. Scherl M. Cephalic zoster with laryngeal paralysis. Ear Nose Throat J. 1994; 73: O Driscoll IU, McShane DP. Herpes zoster oticus-diagnosis and treatment. Irish Med J. 1992; 85: Uri N, Greenberg E, Meyer W, Kitzes-Cohen R. Herpes zoster oticus; treatment with acyclovir. Ann Otol Rhino1 Laryngol. 1992; 101: Dickins JRE, Smith JT, Graham SS. Herpes zoster oticus: treatment with intravenous acyclovir. Laryngoscope. 1988; 98: Inamura H, Aoyagi M, Tojirna H, Koike Y. Effects of aciclovir in Ramsay Hunt syndrome. Acta Otolaryngol Suppl (Stockh). 1988; Stafford FW, Welch AR. The use of acyclovir in Ramsay Hunt syndrome. J Laryngol Otol. 1986; 100: Adour KK. Medical management of idiopathic (Bell s) palsy. Otolaryngol Clin North Am. 1991; 24: Key words: herpes zoster; Bell s palsy; Ramsay Hunt syndrome; herpes zoster oticus; dermatology.