Sarcoidosis - CNS and thoracic manifestations

Case contributed by Haiying Chen
Diagnosis almost certain

Presentation

Subacute onset of bilateral uveitis and right-sided Bell's palsy.

Patient Data

Age: 40 years
Gender: Male

There is abnormal nodular enhancement in the labyrinthine, tympanic, mastoid and perhaps the extracranial segments of the right facial nerve compared to the left in the post contrast T1 weighted images.

No other abnormal leptomeningeal or parenchymal signal or enhancement elsewhere. 

Post contrast CT chest and abdomen demonstrates bilateral bulky mediastinal and hilar lymphadenopathy with the largest node in the right posterior hilar region. No calcification or central hypodensity within the enlarged lymph nodes. There is also an enlarged lymph node adjacent to the gastro-esophageal junction. 

The lung parenchyma is unremarkable without lesion or scarring. 

The abdominal organs appear normal. 

Case Discussion

Initial workup demonstrated elevated serum ACE (angiotensin-converting enzyme) level and bilateral hilar lymphadenopathy on chest X-ray, which raised the suspicion for sarcoidosis and prompted further imaging. 

The patient underwent an endobronchial ultrasound-guided biopsy of one of the mediastinal lymph nodes. Histopathology revealed non-necrotizing granulomatous inflammation and was negative for malignancy or acid fast bacilli staining. 

In this patient, the abnormal enhancement in the right facial nerve identified on MRI could be one of the manifestations of neurosarcoidosis as a cranial neuropathy. 

Up to 50% of patients with neurosarcoidosis develop peripheral facial nerve palsy. Other cranial neuropathies can involve the optic nerve and the vestibulocochlear nerve 1.

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