Cranial Nerves Ii–Xii Not Intact: Palsies to Remember

1Mercy Hospital and Medical Center, Chicago, IL
2University of Chicago, Chicago, IL

Meeting: Hospital Medicine 2011, May 10-13, Dallas, Texas.

Abstract number: 1011

Case Presentation:

A 33‐year‐old white woman with type 2 diabetes presented with left‐sided facial weakness, dysphagia, xerostomia, and decreased hearing on the left. She was presumptively diagnosed with Bell's palsy and empirically treated with a course of steroids. She did not complete the course because of hyperglycemia. She presented to our emergency room after noticing worsening of her symptoms. On physical exam, her vital signs were normal except for tachycardia. Abnormalities on the neurologic exam included decreased hearing on the left and an inability to close her eyes, left more than right. Initial labs showed hypercalcemia but were otherwise unremarkable. Computed tomography of the head without contrast and magnetic resonance imaging with and without contrast of the brain were normal. Lumbar puncture showed no abnormalities. Serum ACE level was mildly elevated. Chest radiograph showed bilateral hilar lymphadenopathy (Fig. 1). Computed tomography of the thorax with contrast showed symmetric mediastinal and hilar lymphadenopathy. Flexible laryngoscopy showed left vocal cord paralysis with left recurrent laryngeal nerve palsy. A slit‐lamp ophthalmologic exam was normal. A swallowing study for dysphagia demonstrated signs of gross aspiration and decreased pharyngeal contraction. A mediastinal lymph node biopsy showed noncaseating granulomatous inflammation consistent with sarcoidosis in the paratracheal lymph nodes. She was treated with high‐dose oral steroids, and her dysphagia and facial weakness improved over 7 days. She was discharged home with long‐term steroid therapy. She has been followed in the neurology clinic and continued to improve gradually.

FIGURE 1. Chest radiograph with lymphadenopathy.

Discussion:

The most common manifestation of neurosarcoidosis includes cranial nerve palsies with peripheral facial nerve palsy presenting in up to 50% of cases. Facial nerve palsy in neurosarcoidosis can be unilateral in 65% of cases and bilateral in 35% of cases. The most serious cranial neuropathies involve the optic and vestibular nerves. Optic disc edema, retrobulbar optic neuropathy, and optic neuropathy can be found and vestibular dysfunction can also be seen. Pathogenesis involves granulomatous infiltration of the cranial nerve nucleus or the cranial nerve. Approximately 15% of patients also develop peripheral nervous system dysfunction, leading to mononeuropathies and polyneuropathies, as well as restless leg syndrome in addition to a decrease in pain and temperature sensations. The definitive diagnosis of neurosarcoidosis is a biopsy of the nervous system. Diagnostic criteria are listed in Table 1. Therapy involves high‐dose prednisone with a slow taper in addition to a chronic dose if symptoms do not resolve. Infliximab and azathioprine can also be used; however, bone marrow suppression is an adverse reaction. Radiotherapy can be used for refractory neurosarcoidosis.

TABLE 1 Diagnostic Criteria Adapted From Zajicek et al.

Conclusions:

Cranial nerve disorders present in 50%–75% of patients with neurosarcoidosis and involvement of nearly every cranial nerve has been reported.

Disclosures:

A. Desai ‐ none; M. Kasarla ‐ none; S. Reddy ‐ none

To cite this abstract:

Desai A, Kasarla M, Reddy S. Cranial Nerves Ii–Xii Not Intact: Palsies to Remember. Abstract published at Hospital Medicine 2011, May 10-13, Dallas, Texas. Abstract 1011. Journal of Hospital Medicine. 2011; 6 (suppl 2). https://www.shmabstracts.com/abstract/cranial-nerves-iixii-not-intact-palsies-to-remember/. Accessed July 19, 2019.

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