Case Presentation: A 70-year-old man with a history of testicular cancer in remission, diabetes mellitus type 2 (HbA1c:6.2), and isolated left CN VI and VII palsy presented with double vision and left-sided eye pain. One week prior to admission, the patient reported difficulty opening his left eye and double vision. Lubricant eye drops failed to alleviate his symptoms. On the day of admission, he awoke with severe 10/10 pain behind his L eye and was unable to open it. On arrival, the patient was afebrile with normal vital signs. His physical exam revealed a completely shut left eye. When his eyelid was lifted, his left eye was noted to be down and out. His left pupil was larger than right pupil. Initial labs revealed a corrected calcium of 12.2 mg/dL and Cr of 2.47 mg/dL (baseline 0.9). PTH was <6. Urinalysis was not significant for infection, but revealed a urine Na of 98 mMol/L and urine osmolality of 325 mOsm/kg. MRI Brain and MR Angiography were unrevealing for intracranial abnormalities. Lumbar puncture demonstrated WBC count of 6, RBC count of 2, glucose of 60 mg/dL, and protein of 34 mg/dL. Workup for vasculitis, including ANA, ANCA, myeloperoxidase antibody, and serine proteinase, were within normal limits. CT Chest, Abdomen/Pelvis revealed right hilar and mediastinal adenopathy. Labs sent earlier this admission returned showing 1,25-dihydroxy vitamin D = 82 pg/mL [18-72] and angiotensin converting enzyme (ACE) = 243 [9-67]. The patient was diagnosed with sarcoidosis. His calcium normalized with intravenous fluids, calcitonin, and pamidronate. He was empirically started on a prednisone taper and was scheduled for an outpatient endobronchial ultrasound (EBUS) for hilar lymph node biopsy.
Discussion: Sarcoidosis is a rare systemic granulomatous disorder, of which the etiology and pathogenesis remains unknown. Typically, sarcoidosis involves the pulmonary system; however, up to 5-10% of patients present with neurologic involvement.Neurosarcoidosis has a variety of different presentations, including headaches, seizures, and mononeuropathies. Clinical diagnosis can be difficult, given the non-specificity of the manifestations. Therefore, it is important to keep this diagnosis in the differential, especially in patients in which the index of suspicion is high. This patient had three separate episodes of unexplained cranial nerve neuropathies, hypercalcemia, and hilar adenopathy, all of which suggested the possibility of a single unifying diagnosis. This case focuses on a new diagnosis of sarcoidosis, when the presenting complaint was a cranial nerve neuropathy. Cranial nerve neuropathies consist of approximately 66% of the neurological manifestations of systemic sarcoidosis. The neuropathies can be unilateral or bilateral, and are often recurrent. This case illustrates the importance of maintaining a broad differential in patients with recurrent neurologic findings, without a convincing diagnosis.
Conclusions: Neurosarcoidosis is a rare variant of sarcoidosis and can present with a variety of different manifestations. Diagnosis of this disease can be challenging, given the non-specificity of presenting symptoms. This case is an example of a unique presentation of neurosarcoidosis, which can be used as a teaching point in further elucidating the presenting symptoms and management of this disease.