Facial Nerve Palsy 7 James M
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Facial Nerve Palsy 7 James M. Gilchrist Abstract Facial neuropathy is the most common cranial neuropathy, due to its extensive course and multiple sites of potential injury. The causes of facial neuropathy are many, but 70% are diagnosed as Bell’s palsy, an idiopathic syndrome but increasingly being associated with herpes simplex virus infection as the cause of the majority of cases. Ramsay Hunt syndrome (herpes zoster oticus) is the second most common cause. Facial neuropa- thy causes weakness of the muscles of facial expression on the ipsilateral side, and can be distinguished from a central, or upper motor neuron, caused by the involvement of forehead muscles. Taste, hearing, salivation, lacrimation, and sensation over the ipsilateral ear and the face may also be disturbed. Diagnosis can be confi rmed by electrodiagnostic testing or MRI but is often not necessary. Treatment is directed at the underlying cause. In cases of Bell’s palsy a short course of steroids has been shown effective, if started within 72 h of onset. Treatment with antiviral agents against herpes virus is probably best reserved for patients with severe or complete facial neuropathy or those with Ramsay Hunt syndrome. Keywords Antiviral agents • Bell’s palsy • Herpes simplex • Herpes zoster • Lyme • Ramsay Hunt syndrome • Seventh cranial neuropathy • Steroids J. M. Gilchrist , MD () Neurology , Warren Alpert Medical School of Brown University, Rhode Island Hospital , Providence , RI , USA e-mail: [email protected] K.L. Roos (ed.), Emergency Neurology, DOI 10.1007/978-0-387-88585-8_7, 133 © Springer Science+Business Media, LLC 2012 134 J.M. Gilchrist Introduction Pathophysiology and Pathogenesis The facial nerve, also known as the seventh cra- Facial nerve palsy cannot truly be understood nial nerve, is the most commonly diagnosed without at least some knowledge of the anatomy cranial neuropathy. The reasons for this lie in its of the seventh cranial nerve (Fig. 7.1 ). The motor anatomy and its obviousness. The facial nerve has root of the facial nerve arises in the facial nucleus both an intracranial and an extracranial course, in the lateral pons. Fibers leaving the nucleus taking three signifi cant bends along the way and execute a loop (the internal genu of the facial traversing several potential points of diffi culty nerve) around the nucleus of the abducens nerve, (lateral pons, cerebellopontine cistern, the bony traveling medially from below to above the sixth facial canal which it shares for part of the way nerve nucleus before exiting at the pontomedul- with the acoustic nerve, the inner ear, the parotid lary junction. The motor root then traverses the g land). And facial nerve palsy is the absolute cerebellopontine cistern to enter the internal manifestation of being “in your face.” The facial acoustic canal. The sensory root of the facial nerve provides innervation for muscles of facial nerve is separate from the motor root between the expression, which is a primary mode of express- brainstem and the internal acoustic canal and is ing emotion in humans and an important aspect of often called the nervus intermedius. Within the nonverbal communication, as well as a major aes- nervus intermedius are sensory fi bers which end thetic component of beauty. As such, we are espe- in the spinal nucleus of cranial nerve V, and para- cially attuned to facial movement and any sympathetic fi bers. asymmetry is immediately evident and upsetting. In the internal acoustic canal, the now-whole Patients do not sit at home and wait out a new facial nerve is accompanied by the eighth cranial facial palsy. They set out for the emergency room nerve. The nerves travel to the meatal foramen soon after onset and with considerable anxiety. (“porous acusticus”), which is divided into bony compartments, to then enter the facial canal, also called the fallopian canal. The meatus is the nar- Epidemiology rowest portion of the entire facial canal, less than 0.7 mm [ 8 ] . The facial nerve then enters the laby- Facial neuropathy is a common neurologic syn- rinthine portion of the facial canal, so called drome, and 70% will be diagnosed as Bell’s palsy because it is close to the superior semicircular [ 1 ] . The annual incidence of Bell’s palsy is 25 per canal. The labyrinthine canal ends at the genicu- 100,000 [ 2 ] with a lifetime risk of 1 in 60–70 [ 3 ] . late ganglion, where the cell bodies of the sensory Males and females are affected equally and the and parasympathetic neurons are located. The frequency per decade of life is remarkably simi- greater petrosal nerve branches off at the genicu- lar between age 10 and over 70 years of age [ 2 ] . late ganglion to head to the sphenopalatine and There is no preference between right and left pterygopalatine ganglia, from whence it supplies sides, though bilateral involvement is rare, at the lacrimal gland with parasympathetic innerva- 0.3–2% [ 4 ] . tion. From the geniculate ganglion, the facial Ramsay Hunt syndrome is the second most canal takes its fi rst external genu, and is called the common cause of facial neuropathy and can occur tympanic segment. The nerve to the stapedius in children (16.7% of unilateral facial palsies) muscle branches off within the tympanic canal. and adults (18.1% of facial palsies) with nearly The tympanic segment ends as the facial canal equal frequency, though less so in children under makes its second external bend, this time at a 90° the age of 6 [ 5 ] . Zoster sine herpete looks to be angle. The fi nal intracranial part of the facial especially common in children between 6 and canal is called the mastoid segment and proceeds 15 years of age and may account for over a third in a vertical descent to the stylomastoid foramen of all facial neuropathies in that age group [ 6, 7 ] . where the facial nerve becomes extracranial. 7 Facial Nerve Palsy 135 Fig. 7.1 Anatomy of the seventh cranial nerve The chorda tympani branches off in the mastoid the geniculate ganglion [ 10 ] . Increased rates of segment and then enters the tympanic cavity HSV genome fragments by PCR were seen in the before joining the lingual nerve. It sends pregan- saliva of patients with Bell’s palsy [ 11 ] , and PCR glionic parasympathetic fi bers to the sublingual also identifi ed such fragments in human genicu- and submaxillary glands. The chorda tympani late ganglia at autopsy [ 12, 13 ] . Murakami found also conveys taste from the anterior two-thirds of active HSV sequences in the endoneurial fl uid of the tongue. Once the facial nerve is extracranial, the facial nerve in patients with Bell’s palsy [14 ] , it divides into the posterior auricular branch and facial neuropathy was induced in animals by which supplies sensation to the auricle and pinna reactivating HSV [ 15– 18 ] . Reactivation of the of the ear, although it also has motor fi bers. Other HSV virus presumably causes infl ammation of terminal branches control muscles of facial the facial nerve in the facial canal, initially caus- expression, as well as branches to the digastric ing demyelination, but if severe, causing axonal and stylohyoid muscles. damage and Wallerian degeneration distal to the The potential causes of facial neuropathy are lesion [19 ] . protean (Table 7.1 ), as are the related pathophysi- Ramsay Hunt fi rst described the condition ologies. There is mounting evidence that Bell’s which bears his name in 1907 [ 20 ] . Ramsay Hunt palsy is associated with herpes simplex (HSV) syndrome is defi ned as a peripheral facial neu- infection. McCormick fi rst postulated a connec- ropathy accompanied by an erythematous vesicu- tion with HSV infection in 1972 [ 9 ] , and several lar rash of the ear (herpes zoster oticus) or mouth lines of evidence support that up to 79% of Bell’s [ 20 ] . The infection involves the contiguous facial palsy is caused by reactivated HSV infection in nerve in the tympanic segment, leading, as in 136 J.M. Gilchrist Table 7.1 Causes of unilateral seventh cranial neuropathy Relatively common Parasitic (trichinosis, Bell’s palsy (idiopathic) neurocystercercosis) Herpes simplex virus Infl ammatory Ramsay-Hunt syndrome (herpes zoster) Leukemia (children) Lyme Infections Zoster sine herpete HIV seroconversion Trauma Osteomyelitis of skull base Diabetes mellitus Otogenic infections Pregnancy Parotitis/abscess Guillain–Barré syndrome Mastoiditis Sarcoid Leprosy Neoplastic meningitis Poliomyelitis Pontine infarct Mycoplasma Pontine hemorrhage Infl uenza Multiple sclerosis Miscellaneous Brainstem tumor Benign intracranial hypertension Facial neuropathy from forceps/birth trauma Melkersson–Rosenthal Acoustic neuroma Amyloidosis Relatively less common Wegener’s granulomatosis Pontine Polyarteritis Encephalitis Sjogren’s syndrome Abscess HTLV-1 Congenital/postnatal Hereditary neuropathy with Mobius syndrome pressure palsies (HNPP) Hemicranial microsomia Familial Bell’s Congenital lower lip paralysis CIDP Kawasaki disease Charcot-Marie-Tooth Albers-Schoenberg (osteopetrosis) Histiocytosis X Infantile hypercalcemia Interferon therapy Cardiofacial syndrome Sclerosteosis Tumor Ethylene glycol intoxication Meningioma Wernicke–Korsakov syndrome Cholesteatoma Stevens-Johnson syndrome Metastatic Neurinoma Parotid tumor Meningitis Infectious Bacterial Fungal Tuberculous Syphilis Bell’s palsy, to infl ammation, edema, demyelina- Only 11 had CSF pleocytosis, and nine had ele- tion, and potentially, axonal damage. Ramsay vated protein concentration, and he suggested Hunt syndrome can be seen in the absence of a that meningitis might occur concomitantly with rash (zoster sine herpete) when accompanied by facial neuropathy but not as a cause of facial neu- either a fourfold rise in varicella zoster virus ropathy [ 22 ] . (VZV) antibody or detection of VZV DNA [ 20 ] . This may occur in anywhere from 2.4% to 19% [ 5, 21 ] of patients who otherwise might have Clinical Features been thought to have Bell’s palsy. The cause of Lyme facial neuropathy is likely Facial neuropathies can affect either sex and not from meningitis. Halperin examined the CSF occur at any age [2 ] .