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Acute Cranial Nerve VI Palsy Secondary to Presumed Paraneoplastic Syndrome Brittany McNeely, O.D.; Anthony Van Alstine, O.D., M.S., FAAO Abstract: Complex cancer patient presents with acute onset diplopia and severe headache. Ocular examination reveals isolated cranial nerve VI palsy OS. After thorough workup, paraneoplastic syndrome is the most likely etiology. I. Case History 1) Patient demographics: 69 year old Native American male 2) Chief complaint: Sudden onset diplopia and new, severe headache in the orbital and temporal region OS for 3 days 3) Ocular history: Negative. The patient had a completely normal eye exam 3 weeks prior to presentation. 4) Medical history: The patient has a history of colon cancer with metastasis to the lung evidenced on CT. At the time of presentation, the patient was not undergoing treatment with chemotherapy. Despite prior chemotherapy treatment with Folfiri and Panitumumab, the pulmonary mass was continuing to enlarge as evidenced on serial CT exams. The patient also had rising CEA-mono levels, indicating increased antigen secretion by the tumor. A new chemotherapy treatment regimen was to be initiated in the coming weeks. The patient also has a history of Hypertension, Hyperlipidemia, Cholecystitis, and Anemia. 5) Medications: Lorazepam, Loperamide, Ondasetron, Aspirin, Hydrochlorothiazide, Losartan, Ibuprofen 6) Other salient information: This patient has no history of diabetes, and his hypertension had been well-controlled with systemic medication over the last several months. The patient reported the worst headache of his life surrounding the left orbit and temporal region. History was positive for malaise, but negative for jaw claudication and painful palpation of the temporal artery. The patient was taking a reprieve from chemotherapy due to significant cutaneous side effects and general improved well-being without chemotherapy. II. Pertinent findings 1) Clinical: Presenting VA was 20/25+ OD and 20/20 OS. Pupil testing was normal. Extraocular motility testing and cover test revealed a cranial nerve VI palsy OS. The patient did not report a significant increase in pain with abduction OS. Anterior segment findings were unremarkable. Posterior segment examination revealed a healthy retina and normal optic nerves. 2) Physical: The patient reported the worst headache of his life, and the patient’s affect was consistent was consistent with this complaint. 3) Laboratory studies: Following presentation in the eye clinic, ESR and CRP were ordered to rule out giant cell arteritis. Both the ESR and CRP were slightly elevated, with an ESR of 35 mm/hr and CRP of 1.44mg/dL. Hemoglobin A1c was 5.9. Blood pressure reading was slightly elevated at 146/86 mmHg. Over the next several days, extensive laboratory tests were ordered. CBC showed elevated lymphocytes and granulocytes. MRSA screening, ANA, and Rheumatoid factor were negative. Urinalysis and Thyroid testing with TSH and T3 were normal. CEA-mono levels had elevated significantly from 2.84 to 3.33 over the previous 6 weeks. Previously, the CEA-mono level had been steadily increasing by 0.1-0.2 every 2-3 months or remaining stable between measurements. 4) Radiology studies: MRI studies showed no compressive etiology of the brain or orbits. Chest X-ray showed a mass in the left lung measuring 3.2mm x 2.9mm. Previous mass measurement was 3.1mm x 2.8mm 6 weeks prior. The tumor had been steadily increasing in size since diagnosis 9 months prior. 5) Others: Ophthalmology and Thoracic Surgery were consulted for a potential temporal artery biopsy due to the patient complaint, location of the pain, cranial nerve VI palsy, and slightly elevated ESR and CRP. It was determined that artery biopsy would be performed if imaging and further lab testing showed no other etiology for the presentation. The patient was admitted to the hospital the day after presentation due to dizziness while at the hospital for further evaluation. During his stay, the patient was evaluated extensively by Neurology. MRI and CT showed no brain or orbit malformations, and all other testing for ischemic and compressive etiology was normal. Rapid CEA-mono elevation, indicating rising cancer enzymes, was the only significant change in the patient’s medical history. The patient did not have a history of recent illness or any other new neurological symptoms. While ESR and CRP were slightly elevated, the patient has a history of complex cancer, and the levels were not elevated to sufficient levels that would indicate Giant Cell Arteritis. As a result, the temporal artery biopsy was deemed unnecessary and cancelled. The neurologist indicated the etiology was presumed paraneoplastic syndrome due to the complex cancer history, the absence of ischemic or mass etiology, and the rapid increase in CEA-mono measurements. Laboratory testing for paraneoplastic antibodies is pending. The presence of paraneoplastic antibodies can be used to confirm a diagnosis paraneoplastic syndrome; however, literature review indicates that less than 50% of patients with paraneoplastic syndrome will test positive for antibodies. As a result, the absence of these antibodies cannot rule out paraneoplastic syndrome. Lumbar puncture to evaluate elevated white blood cell count can also aid in the diagnosis; however, at this time, the patient has postponed chemotherapy and declined invasive procedures. III. Differential diagnosis 1) Primary/leading: Post viral etiology. However, the patient has no recent history of illness. 2) Others: Giant Cell Arteritis, malignant neoplasm of the brain or orbit, vasculopathy from Hypertension IV. Diagnosis and discussion Elaborate on the condition: 1) Paraneoplastic syndrome is a group of syndromes in which there are neurological abnormalities as a result of a benign or malignant tumor. These syndromes specifically refer to neurological deficits in cancer patients that are not related to metastasis, infection, or side effects of treatment. Paraneoplastic syndromes most commonly result from an immune response to enzymes released by a primary mass, and they can affect any part of the neurological system. In some cases, the patient may not have a formal cancer diagnosis at initial presentation, and paraneoplastic syndrome may be the presenting sign of malignancy. While hematology can reveal antibodies to certain cancer enzymes, over 50% of patients with paraneoplastic syndrome will have no related antibodies found on laboratory testing. As a result, diagnosis is most commonly made based on the patient complaint and clinical presentation. Paraneoplastic syndromes are most common in lung cancer, ovarian cancer, breast cancer, and Hodgkin’s disease. Because any part of the nervous system can be affected by paraneoplastic syndrome, neuro-ophthalmologic findings can be common in this condition. Neuro-ophthalmologic findings can include cranial nerve palsies, opsoclonus, myoclonus, nystagmus, gaze palsies, pupil abnormalities, and optic neuropathy. There are several different mechanisms that can result in neuro-ophthalmological symptoms; however, the most common etiology is encephalitis. 2) Expound on unique features: Cranial nerve VI palsy of uncommon cause in a complex cancer patient with evidence of mass progression with CT and rising cancer enzymes. Most cranial nerve VI palsies are due to ischemic etiology from uncontrolled diabetes or hypertension. Less commonly, these palsies can arise from an intracranial or intraorbital mass or viral etiology. Cranial nerve palsies due to the immune reaction to cancer enzymes in paraneoplastic syndromes are rare, with few cases reported in the literature. V. Treatment, management 1) Treatment and response to treatment: The patient was prescribed Hydrocodone/APAP to help with his headaches, and the patient was given an eye patch to alleviate diplopia. Oncology was consulted due to presumed paraneoplastic etiology. Lab results for paraneoplastic antibodies are pending. At this time, the oncologist has decided to defer treatment due to patient request. The patient has further evaluation with oncology in 1 month, with complete blood work and CT exams. The cranial nerve palsy will continue to be monitored in Optometry for resolution and possible prismatic correction if the palsy does not resolve. 2) Research/Literature review/ Bibliography Ko, M.W., Dalmau, J., Galetta, S.L. Neuro-Ophthalmologic Manifestations of Paraneoplastic Syndromes. J Neuro-Opthalmol. Vol 28., No 1., 2008 Flemmons, M.S., Schiffman, J.S. Cranial Nerve III, IV, and VI palsies in the Cancer Patient. Ophthalmic Oncology. M.D. Anderson Solid Tumor Oncology Series: 6. 2010 Hammon, T.H., McFadzean, R.M., Ironside, J.W. Anti-Hu Paraneoplastic Syndrome Presenting as Bilateral Sixth Cranial Nerve Palsies VI. Conclusion Most cases of cranial nerve VI palsies can be attributed to ischemic etiology, intracranial mass, or intraorbital mass. However, it is critical to consider paraneoplastic syndrome in patients with a known history of cancer, or in the absence of ischemic or mass etiology.