A 47-year-old man comes to the emergency department after waking up this morning with a right-sided facial droop. The patient is unable to fully close his right eye. He has had no headache, neck stiffness, extremity weakness, or numbness but reports fatigue and low-grade fevers for the past several weeks. He has no chronic medical conditions and takes no medications. The patient occasionally uses alcohol and is sexually active with multiple women. Vital signs are within normal limits. Physical examination shows flattening of the right nasolabial fold, drooping of the right corner of mouth, and right-sided hyperacusis. Sensation to touch on the face is normal bilaterally. The remainder of the neurologic examination is normal. The parotid glands are enlarged bilaterally, and scattered enlarged cervical lymph nodes are present. Inspection of the external acoustic meatus and tympanic membrane shows no abnormalities. The lungs are clear to auscultation and heart sounds are normal. Laboratory results are as follows:
| Complete blood count | |
| Hemoglobin | 13 g/dL |
| Platelets | 260,000/mm3 |
| Leukocytes | 8,200/mm3 |
| Serum chemistry | |
| Sodium | 140 mEq/L |
| Potassium | 3.8 mEq/L |
| Creatinine | 0.8 mg/dL |
| Calcium | 11 mg/dL |
| Glucose | 100 mg/dL |
Which of the following is the most likely cause of this patient's facial paralysis?
Manifestations of sarcoidosis | |
Pulmonary |
|
Cutaneous |
|
Ophthalmologic |
|
Neurologic |
|
Cardiovascular |
|
Gastrointestinal |
|
Other |
|
*Manifestations of Löfgren syndrome. AV = atrioventricular; LFT = liver function test. | |
This patient with new-onset facial nerve palsy, hypercalcemia, bilateral parotid gland swelling, and constitutional symptoms likely has sarcoidosis with extrapulmonary manifestations. Although lung involvement (eg, hilar lymphadenopathy) is common in sarcoidosis, it is often (>50%) asymptomatic, and patients may present with extrapulmonary disease.
Neurologic complications (caused by granulomatous infiltration and inflammation) occur in up to 10% of patients. Cranial mononeuropathy is common, with peripheral facial nerve palsy being a classic manifestation presenting with facial droop; inability to close the eye; disappearance of the nasolabial fold; and hyperacusis, a hypersensitivity to sounds due to paralysis of the stapedius muscle (innervated by the facial nerve). Other neurologic manifestations include neuroendocrine disturbances (eg, central diabetes insipidus) and generalized seizures.
Additional extrapulmonary manifestations seen in this patient include bilateral parotid gland swelling (exocrine gland infiltration), hypercalcemia (1-alpha hydroxylase production by macrophages in lung granulomas), and constitutional symptoms (eg, fever). Diagnosis is by biopsy of the most accessible lesion, and first-line treatment includes prednisone.
(Choice A) Herpes zoster oticus (Ramsay Hunt syndrome) causes a triad of ear pain, facial paralysis, and vesicular rash over the auricle, external auditory canal, or face. Preherpetic neuralgia can precede the rash by 1-5 days. This patient has no neuropathic pain; moreover, his hypercalcemia and parotid gland swelling make herpes zoster oticus unlikely.
(Choice B) Although idiopathic Bell palsy presents with acute onset (hours to a day) of facial paralysis (as seen in this patient), it is a diagnosis of exclusion and would not cause parotid gland swelling and hypercalcemia.
(Choice C) Lyme disease can cause facial nerve palsy and can present with acute facial droop, fevers, and constitutional symptoms. However, parotid gland enlargement and hypercalcemia are not typically seen.
(Choice D) Symptomatic meningitis from neurosyphilis can cause cranial nerve abnormalities, typically in the first year after infection. This patient does not have findings of meningitis and early syphilis (eg, headache, stiff neck, rash). Moreover, neurosyphilis does not explain parotid gland swelling and hypercalcemia.
(Choice E) Parotid gland tumors are associated with facial palsy, a painless intraparotid mass, and cervical lymph node swelling. These tumors are slow growing and cause gradual, unilateral disability, rather than the acute, bilateral symptoms seen in this patient. Hypercalcemia is not expected.
Educational objective:
Sarcoidosis is a systemic granulomatous disorder that can involve the nervous system, presenting as facial nerve palsy. Other common extrapulmonary manifestations of sarcoidosis include lymphadenopathy, hypercalcemia, and parotid gland swelling.