The Cost-Effectiveness of Tocilizumab (Actemra) Therapy in Giant Cell Arteritis.

BACKGROUND: Tocilizumab (Actemra) is a humanized anti-interleukin-6 receptor antibody that has been used as a steroid-sparing agent in giant cell arteritis (GCA). Although the clinical effects are well described in GCA, the cost-effectiveness of the use of tocilizumab in GCA is ill defined. The purpose of this study was to determine the cost-effectiveness of tocilizumab in GCA compared with prednisone alone. METHODS: A retrospective study of 32 patients with biopsy-proven GCA comparing prednisone alone (16 patients) and prednisone with tocilizumab (16 patients) was performed. The cost for tocilizumab therapy for 26 weeks with mild and severe side effects (Groups 1 and 2, respectively) and for 52 weeks with mild and severe side effects (Group 3 and 4, respectively) was compared with estimated costs of mild and severe steroid-induced side effects (Groups 5 and 6, respectively). Statistical analysis between groups was conducted using independent sample t tests. RESULTS: Three out of the 4 group combinations of tocilizumab with prednisone demonstrated a statistically significant (P < 0.05) difference in cost compared with prednisone alone for GCA. Group 2 (26-week tocilizumab therapy with severe steroid-induced side effects), with no statically significant difference in price when compared with steroid therapy alone and far fewer side effects, demonstrated the potential use of tocilizumab in GCA therapy. As expected, longer treatment duration with tocilizumab was associated with greater cost. With respect to side effect severity, the number of side effects of steroid therapy was inversely associated with difference in cost between tocilizumab therapy and steroid side effect treatment. CONCLUSION: This study demonstrates that combination therapy of tocilizumab and prednisone is significantly more expensive than steroids alone with or without accounting for the cost of steroid-induced side effects in treated GCA. The difference in cost between the 2 therapy types is directly related to tocilizumab therapy duration and inversely related to the number or severity of steroid side effects. Patients with GCA who require a shorter duration of steroid therapy and are at risk for a high number of side effects from steroid use may be potential candidates for tocilizumab therapy, from an economic perspective.

Restricted Diffusion in the Optic Nerve Head After Shock-Induced Anterior Ischemic Optic Neuropathy.

ABSTRACT: Shock-induced anterior ischemic optic neuropathy (SIAION) is a known type of optic neuropathy in patients who experienced shock related to different etiologies such as anemia and severe intradialytic hypotension like in our patient. Nonarteritic anterior ischemic optic neuropathy (NAION) is the most common acute unilateral known type of optic neuropathy in older patients with vasculopathic risk factors such as hypertension, diabetes mellitus, and obstructive sleep apnea. Although SIAION and NAION are similar optic neuropathies due to ischemia and, in particular, hypotension, they may have different pathogenic mechanisms (e.g., acute shock or intradialytic hypotension vs nocturnal hypotension), laterality (e.g., unilateral vs bilateral), and severity (e.g., light perception or worse vision). We presented a case with restricted diffusion on the apparent diffusion coefficient and the diffusion weighted imaging confined to the optic disc head in a patient with pallid edema after intradialytic hypotension. Although DWI of the optic nerve is neither 100% specific nor 100% sensitive for ischemia, we believe that restricted diffusion of the optic nerve head in our case is a clinico-radiologic correlate to pallid edema in SIAION.

Localizing Thalamomesencephalic Afferent and Efferent Pupillary Defects.

ABSTRACT: A 42-year-old Algerian man presented for binocular oblique diplopia, hypersomnolence with drop attacks, bilateral hearing loss, and thoracic pain. He had a right thalamomesencephalic hemorrhage due to an underlying cavernous malformation treated with subtotal surgical resection. On neuro-ophthalmic examination, the patient had a left relative afferent pupillary defect and a right oculosympathetic efferent pupillary defect (i.e., Horner syndrome) in addition to other thalamomesencephalic eye and neurologic signs (right fourth nerve palsy, hearing loss, hemiparesis, and thalamic pain). Clinicians should recognize the localizing value of this unique constellation of mesencephalic afferent and efferent pupillary defects.

Neuro-Ophthalmic Manifestations of Intracranial Malignancies.

BACKGROUND: To describe the various neuro-ophthalmic presentations, key exam features, and clinical findings associated with 5 common primary and secondary intracranial malignancies. EVIDENCE ACQUISITION: Retrospective PubMed search and review of published case reports, case series, observational studies, book chapters, and review articles examining the neuro-ophthalmic features of intracranial malignancies including primary glial neoplasms (e.g., glioblastoma multiforme), primary and secondary lymphoma, intracranial metastases, carcinomatous/lymphomatous meningitis, and intracranial germ cell tumors. The search strategy used to perform the retrospective review included the aforementioned tumor type (e.g., glioblastoma multiforme) and the following terms and Boolean operators: AND ("visual loss" OR "papilledema" OR "diplopia" OR "ophthalmoplegia" or "neuro-ophthalmology" OR "proptosis"). RESULTS: The rate of growth and the location of an intracranial tumor are essential factors in determining the neuro-ophthalmic presentation of certain intracranial malignancies. Primary malignant brain glial neoplasms commonly present with visual afferent complaints (e.g., unilateral or bilateral visual acuity or visual field defects, bitemporal or homonymous hemianopsia), pupil abnormalities (relative afferent pupillary defect), and optic atrophy or papilledema. Primary intraocular lymphoma (with or without central nervous system lymphoma) typically presents as a painless bilateral vitritis. Secondary intracranial malignancies have variable afferent and efferent visual pathway presentations. Carcinomatous/lymphomatous meningitis is associated with diplopia (e.g., multiple ocular motor cranial neuropathies with or without vision loss from papilledema or compressive/infiltrative optic neuropathy). Intracranial germ cell tumors can present with a chiasmal syndrome or dorsal midbrain syndrome. CONCLUSION: Intracranial malignancies can present with neuro-ophthalmic symptoms or signs depending on topographical localization. Specific neuro-ophthalmic presentations are associated with different malignant intracranial tumors. Clinicians should be aware of the common malignant intracranial tumors and their associated clinical presentations in neuro-ophthalmology.

Checkerboard Visual Field Defect in Occipital Stroke.

A 74-year-old man with vasculopathic risk factors presented to the emergency room with a chief complaint of peripheral vision loss resulting from an intracranial hemorrhage in his right parietal and occipital lobes. Urgent craniotomy and ventriculostomy led to a stable clinical condition with subsequent development of a crossed quadrant homonymous hemianopsia (checkerboard visual field) due to a new right parieto-occipital infarct superimposed on a prior left occipital infarct. This uncommon visual field defect represents juxtaposed homonymous quadrantanopias that produce a striking checkerboard appearance that is almost pathognomonic for bilateral occipital lesions.

Familial Cavernous Hemangioma.

A 30-year-old woman presented with diplopia after resection of an intracranial cavernous malformation. Fundus examination showed an asymptomatic intraocular cavernous hemangioma of the retina. Clinicians should be aware of the potential coexistence of intraocular and intracranial cavernous malformations; the presence of both should suggest familial etiology. As with other intracranial and intraocular vascular lesions (e.g., arteriovenous malformation in Wyburn-Mason syndrome, retinal hemangioblastoma in von Hippel Lindau disease, and choroidal hemangioma in Sturge-Weber syndrome), the presence of a vascular lesion in either location should prompt evaluation for additional pathology.

Sixth nerve palsy in sporadic Burkitt Lymphoma.

PURPOSE: To describe a unique presentation of Central Nervous System Burkitt Lymphoma. OBSERVATIONS: A 59-year-old male presented with new onset binocular horizontal diplopia five days after initial presentation with abdominal distension, weight loss, and night sweats. He was diagnosed with Burkitt Lymphoma with base of skull metastasis that was initially visible only on PET scan and subsequently resolved with chemotherapy. CONCLUSIONS AND IMPORTANCE: Burkitt Lymphoma (BL) is an aggressive type of B-cell, non-Hodgkin, lymphoma that arises due to a translocation of the MYC proto-oncogene. Although central nervous system (CNS) involvement has been described previously with BL, isolated sixth nerve palsy as the initial sign of CNS metastasis is rare. Suspicion should remain high for metastatic disease in patients presenting with acute-onset neurologic complaints even when initial imaging is negative as timely treatment can prevent poor outcomes.