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.

Bilateral Scalp Necrosis in Giant Cell Arteritis.

ABSTRACT: Giant cell arteritis (GCA) is a medium-to-large vessel vasculitis of the elderly. Common constitutional clinical features include headache, scalp tenderness, and jaw claudication. Severe unilateral or bilateral visual loss is the most feared ophthalmic complication of GCA. Scalp necrosis is a known ischemic complication of GCA with approximately 100 cases reported in the literature to date. We report a case of scalp pain and an erythematous cutaneous lesion in the distribution of ophthalmic division of the trigeminal nerve that mimicked herpes zoster ophthalmicus. A temporal artery biopsy was positive for GCA, and small vessel arteritis was seen at the time of simultaneous skin biopsy. To the best of our knowledge, this is the first such report in the English language ophthalmic literature.

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.

Corticosteroid Usage in Giant Cell Arteritis.

Giant cell arteritis (GCA) is a condition that can cause irreversible visual loss if untreated. While corticosteroids remain the mainstay of treatment to prevent visual loss, the type, dose, route, and duration of corticosteroid treatment of GCA remain controversial. Our study surveyed neuro-ophthalmologists to determine commonly prescribed dosages of corticosteroids for the treatment of GCA with or without visual loss. For patients with acute visual loss, 52% would use intravenous (IV), 46% would use IV or oral and 2% would use oral corticosteroids. Seventy-three per cent would use 500 to 1000 mg IV methylprednisolone in this group. For patients with GCA without acute visual loss, 67% would use the oral route, 30% would use IV or oral, and 3% indicated they would use IV route of treatment. Seventy-five per cent would use 1.0 to 1.5 mg/kg oral prednisone in this group. Our results suggest a majority but not a complete consensus for route and dose of corticosteroid treatment in GCA and confirm conventional recommendations for high dose IV corticosteroids for GCA with visual loss and lower dose oral regimens for GCA without visual loss.

Spaceflight associated neuro-ocular syndrome.

PURPOSE OF REVIEW: Several decades of long duration space flight missions by the National Aeronautics and Space Administration has revealed an interesting and unique constellation of neuro-ophthalmic findings now called spaceflight associated neuro-ocular syndrome (SANS). The unique space environment of microgravity produces novel physiological changes and derangements that present a challenge to astronauts in current and future long duration space missions. Although the precise mechanism of SANS is not fully understood, in this review, we examine recent developments that may to help explain possible causes and potential countermeasures. RECENT FINDINGS: The cause of SANS is still largely unknown. A growing body of evidence implicates multiple factors that contribute to the development of SANS including cephalad fluid shifts, increased intracranial pressure, venous/lymphatic stasis, inflammation, metabolism, axoplasmic stasis and radiation exposure. SUMMARY: The pathologic mechanism behind SANS may be multifactorial and may be amenable to different countermeasures for prevention and management of SANS.

Remote Pontine Hemorrhage After Left Frontal Lobe Meningioma Resection Presenting as Bilateral Internuclear Ophthalmoplegia.

Postoperative hemorrhages are relatively common complications of surgical procedures including craniotomies, and these typically occur at or near the operative site. Bleeding in remote areas (e.g., posterior fossa) after supratentorial craniotomy can occur and may be associated with a high morbidity and mortality. Although remote cerebellar hemorrhage after craniotomy is well described, remote pontine hemorrhage is less common. We describe a bilateral internuclear ophthalmoplegia due to an RPH after otherwise uncomplicated resection of a frontal meningioma. Clinicians should be aware that neuro-ophthalmic findings can occur from hemorrhages remote from the operative site.

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.

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.

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.

Arteritic Orbital Ischemia Producing Afferent and Efferent Pupillary Defects.

A 67-year-old woman presented with acute loss of vision to no light perception (NLP), a right afferent pupillary defect, and anisocoria with a nonreactive and dilated pupil in the right eye. Fundus examination showed pallid optic disc edema and a central retinal artery occlusion (CRAO) in the right eye. A temporal artery biopsy showed giant cell arteritis (GCA). Orbital involvement in GCA has been reported previously. However the combination of an afferent and efferent pupillary defect, NLP vision, pallid disc edema, and a CRAO in an elderly patient is likely a unique clinical combination that should strongly suggest GCA. Clinicians should be aware of the myriad presentations of GCA, including orbital ischemia.

Transient vision loss after optic nerve sheath fenestration.

Optic nerve sheath fenestration (ONSF) is a well-known, relatively safe, and effective surgical treatment for visual loss related to papilledema. Visual loss following ONSF is uncommon but can occur from direct surgical trauma, ischemic optic neuropathy, orbital or intrasheath hemorrhage, or arterial occlusion. Transient severe (e.g., light perception (LP) or no light perception (NLP) visual loss) after ONSF is uncommon but has been reported. We describe a case of LP vision following uncomplicated ONSF with orbital imaging demonstrating significant postoperative inflammation and slow recovery after intravenous steroid treatment. We discuss the various mechanisms for this phenomenon after ONSF. To our knowledge, this is the first clinico-radiologic description with follow up and recovery of this postoperative complication of ONSF to be reported in the English language ophthalmic literature.

Update: the Miller Fisher variants of Guillain-Barré syndrome.

PURPOSE OF REVIEW: This article will update and review the Miller Fisher variants (MFV) of Guillain-Barré syndrome (GBS) including the clinical presentation, diagnostic testing, and treatment. RECENT FINDINGS: Although the diagnosis of GBS and MFV can be made on clinical grounds, cerebrospinal fluid (CSF) analysis, nerve conduction studies, imaging (e.g. ultrasound and MRI), and serologic testing can help to confirm the diagnosis. Some patients may need immunotherapy with either intravenous immunoglobulin (IVIg) or plasma exchange, and recent studies suggest that complement inhibition combined with IVIg could be of benefit, but further studies are needed to prove efficacy. SUMMARY: GBS is characterized by an acute, ascending polyneuropathy, ataxia, areflexia, and CSF albuminocytologic dissociation. The MFV of GBS is associated with ophthalmoplegia. Clinicians should have high index of suspicion for MFV of GBS in patients with acute ophthalmoplegia in order to establish the diagnosis, perform appropriate evaluation, and start treatment. SDC VIDEO LINK:.

Acute Vision Loss After Ophthalmic Artery Embolization of Meningioma.

A 40-year-old man presented with acute-onset painless visual loss after preoperative embolization for a large skull base meningioma through the ophthalmic artery. We describe the clinical presentation of an isolated ipsilateral afferent and efferent pupillary defect in this patient who had radiographic documentation of embolization material in the intraorbital ophthalmic artery. Although ophthalmic and retinal artery occlusions have been described previously after endovascular embolization, our patient had a unique clinicoradiologic presentation.

Shared Features of the Heimann-Bielshowsky Phenomenon and Ocular Neuromyotonia.

We describe a case of unusual ocular movement with features of both the Heimann-Bielshowsky Phenomenon and Ocular Neuromyotonia that might suggest that they share some common pathophysiology or be on a spectrum of abnormal firing of ocular motor cranial nerves. We are the first to propose such a relationship.