Can Baseline Computed Tomography Scans Be Used to Identify Patients at High Risk of Vision Loss due to Terson Syndrome?

PURPOSE: To determine if routinely performed computed tomographic (CT) scanning in patients with aneurysmal subarachnoid hemorrhages (aSAHs) is sufficient to identify patients at high risk of vision loss due to Terson syndrome (TS). METHODS: Consecutive patients with a diagnosis of aSAH admitted to the neurologic intensive care unit of a regional referral hospital over a 3-year period were prospectively evaluated. Head CT scans performed in the emergency department were assessed for the presence of a "crescent sign" (evidence of significant subinternal limiting membrane hemorrhage). Dilated funduscopic examinations were performed by an ophthalmologist, masked to the results of the CT scan, to identify retinal and vitreous hemorrhages consistent with TS. Retinal hemorrhages were categorized according to size-those smaller than 2 mm in diameter were deemed low risk (lrTS) for vision loss and those larger than 2 mm in diameter were deemed high risk (hrTS) for vision loss. RESULTS: One hundred seventeen patients with aSAH were enrolled in the study. The overall incidence of TS was 24.9% (29 of 117 patients; 12 were bilateral). Compared to patients without TS, those with TS had a higher Fisher Hemorrhage Grade and a lower mean (±standard deviation) GCS score (8.66 ± 4.97 vs 12.09 ± 1.10; P < 0.001). The CT crescent sign was positive in 7 patients (6.0%), 6 (5.1%; 2 were bilateral) of whom were found to have hrTS. Of the 110 patients without a CT crescent sign, 88 (75.1%) patients did not have TS, 21 had lrTS, and 1 patient had hrTS in one eye. The CT crescent sign was highly sensitive (85.7%) and specific (99.1%) for diagnosing hrTS. CONCLUSION: The CT crescent sign is a highly sensitive and specific marker for hrTS. CT scanning may replace routine ophthalmologic examinations to identify patients at risk of vision loss due to aSAH.

Myelin Oligodendrocyte Glycoprotein Antibody-Positive Optic Neuritis: Clinical Characteristics, Radiologic Clues, and Outcome.

PURPOSE: To characterize the clinical phenotype of myelin oligodendrocyte glycoprotein antibody (MOG-IgG) optic neuritis. DESIGN: Observational case series. METHODS: Setting: Multicenter. Patient/Study Population: Subjects meeting inclusion criteria: (1) history of optic neuritis; (2) seropositivity (MOG-IgG binding index > 2.5); 87 MOG-IgG-seropositive patients with optic neuritis were included (Mayo Clinic, 76; other medical centers, 11). MOG-IgG was detected using full-length MOG-transfected live HEK293 cells in a clinically validated flow cytometry assay. MAIN OUTCOME MEASURES: Clinical and radiologic characteristics and visual outcomes. RESULTS: Fifty-seven percent were female and median age at onset was 31 (range 2-79) years. Median number of optic neuritis attacks was 3 (range 1-8), median follow-up 2.9 years (range 0.5-24 years), and annualized relapse rate 0.8. Average visual acuity (VA) at nadir of worst attack was count fingers. Average final VA was 20/30; for 5 patients (6%) it was ≤20/200 in either eye. Optic disc edema and pain each occurred in 86% of patients. Magnetic resonance imaging showed perineural enhancement in 50% and longitudinally extensive involvement in 80%. Twenty-six patients (30%) had recurrent optic neuritis without other neurologic symptoms, 10 (12%) had single optic neuritis, 14 (16%) had chronic relapsing inflammatory optic neuropathy, and 36 (41%) had optic neuritis with other neurologic symptoms (most neuromyelitis optica spectrum disorder-like phenotype or acute disseminated encephalomyelitis). Only 1 patient was diagnosed with MS (MOG-IgG-binding index 2.8; normal range ≤ 2.5). Persistent MOG-IgG seropositivity occurred in 61 of 62 (98%). A total of 61% received long-term immunosuppressant therapy. CONCLUSIONS: Manifestations of MOG-IgG-positive optic neuritis are diverse. Despite recurrent attacks with severe vision loss, the majority of patients have significant recovery and retain functional vision long-term.

Preoperative visual field deficits in temporal lobe epilepsy.

Surgical resection and laser thermoablation have been used to treat medically refractory epilepsy with good results. However, they are not without risk. One of the most commonly reported complications of temporal lobe surgery is contralateral superior homonymous quadrantanopsia. We describe a patient with quadrantanopsia discovered as part of our recently modified protocol to workup patients prior to epilepsy surgery. This field cut was subtle and not detected on routine neurological examination. While we understand that this is a single case, we advocate for more judicious preoperative visual field examinations to truly characterize the incidence of postoperative visual field lesions.

A novel ANO3 variant identified in a 53-year-old woman presenting with hyperkinetic dysarthria, blepharospasm, hyperkinesias, and complex motor tics.

BACKGROUND: Cervical dystonias have a variable presentation and underlying etiology, but collectively represent the most common form of focal dystonia. There are a number of known genetic forms of dystonia (DYT1-27); however the heterogeneity of disease presentation does not always make it easy to categorize the disease by phenotype-genotype comparison. CASE PRESENTATION: In this report, we describe a 53-year-old female who presented initially with hand tremor following a total hip arthroplasty. The patient developed a mixed hyperkinetic disorder consisting of chorea, dystonia affecting the upper extremities, dysarthria, and blepharospasm. Whole exome sequencing of the patient revealed a novel heterozygous missense variant (Chr11(GRCh38): g.26525644C > G; NM_031418.2(ANO3): c.702C > G; NP_113606.2. p.C234W) in exon 7 in the ANO3 gene. CONCLUSIONS: ANO3 encodes anoctamin-3, a Ca+2-dependent phospholipid scramblase expressed in striatal-neurons, that has been implicated in autosomal dominant craniocervical dystonia (Dystonia-24, DYT24, MIM# 615034). To date, only a handful of cases of DYT-24 have been described in the literature. The complex clinical presentation of the patient described includes hyperkinesias, complex motor movements, and vocal tics, which have not been reported in other patients with DYT24. This report highlights the utility of using clinical whole exome sequencing in patients with complex neurological phenotypes that would not normally fit a classical presentation of a defined genetic disease.

An update on eye pain for the neurologist.

Pain in and around the eye with or without an associated headache is a common presenting complaint to the neurologist. Although the main causes for eye pain are easily diagnosed by simple examination techniques that are readily available to a neurologist, sometimes the etiology is not as obvious and may require a referral to an ophthalmologist. This article summarizes and updates our prior review in Neurologic Clinics on this topic and includes (1) ocular and orbital disorders that produce eye pain with a normal examination, (2) neurologic syndromes with predominantly ophthalmologic presentations, and (3) ophthalmologic presentations of selected headache syndromes.

Isolated palsies of cranial nerves III, IV, and VI.

In this article, isolated palsies of cranial nerves III, IV, and VI are addressed. After discussion of the pertinent clinical anatomy of cranial nerves III, IV, and VI, the isolated involvement of each of these oculomotor nerves is defined. Based on a review of the literature, methods of evaluation and follow-up of patients presenting with diplopia from lesions of these cranial nerves are presented.

Purtscher-like retinopathy in a patient with HELLP syndrome.

PURPOSE: To report the antepartum presentation of Purtscher-like retinopathy and hemolysis, elevated liver enzymes, low platelets (HELLP) syndrome that resulted in severe permanent visual loss. DESIGN: Interventional case report. METHODS: A 25-year-old primigravida patient at 38.5 weeks gestation presented with severe bilateral loss of vision. Immediate hospitalization with complete evaluation, urgent medical treatment, and cesarean section was performed. RESULTS: Ophthalmoscopic evaluation showed bilateral Purtscher-like retinopathy. Laboratory studies revealed elevated liver enzymes, thrombocytopenia, and evidence of intravascular coagulation consistent with HELLP syndrome. Despite the successful delivery of a healthy baby, the patient developed permanent visual loss. CONCLUSION: Purtscher-like retinopathy with permanent visual loss can occur antepartum in patients with HELLP syndrome.

Localizing forms of nystagmus: symptoms, diagnosis, and treatment.

Nystagmus is a rhythmic oscillation of the eyes that may be acquired or congenital. The symptoms of acquired nystagmus include blurred vision and oscillopsia. Most patients with congenital nystagmus do not have oscillopsia symptoms. Several forms of nystagmus have localizing value and any neuroimaging should be directed to the topographic localization suggested by the morphology of the nystagmus and any accompanying localizing signs. Several optical, medical, and surgical treatments have been used with some success in specific forms of nystagmus.

Case studies in neuro-ophthalmology for the neurologist.

In summary, neurologists should be aware of emergent neuro-ophthalmic conditions: (1) temporal arteritis (GCA), (2) IIH, (3) intracranial shunt malfunction, (4) pituitary apoplexy, and (5) pupil-involved TNP. Earlier recognition and treatment of these disorders makes a difference in final out-come. Appropriate evaluation and management may be vision or life saving.

Optical coherence tomography in a case of bilateral neuroretinitis.

A 42-year-old man had fever, chills, and bilateral visual loss. Visual acuity was markedly subnormal OU and ophthalmoscopy disclosed optic disc swelling with retinal thickening extending into the macula OU, findings consistent with neuroretinitis. Fluorescein angiography revealed optic disc leakage and submacular accumulation of dye OU without retinal vascular leakage. Optical coherence tomography (OCT) showed outer plexiform layer retinal edema and subfoveal detachments. There was evidence of active human immune deficiency virus and cytomegalovirus infections. Several weeks after multidrug therapy, sequential OCT scans documented resolution of the outer plexiform edema and submacular detachments in parallel with improved visual acuity. The OCT findings support the theory that submacular detachments in neuroretinitis result from diffusion of fluid from the optic disc to the outer plexiform layer and through the outer limiting membrane to the subretinal space.

Imaging for neuro-ophthalmic and orbital disease.

PURPOSE: To provide an update on imaging of the brain and orbit for ophthalmologists. DESIGN: Literature review. METHODS: A systematic English-language medline search and summary of recent literature on imaging of brain and orbit was performed. RESULTS: Computed tomography and magnetic resonance (MR) scanning are the mainstays for the evaluation of most disorders involving the brain and orbit. Computed tomography angiography and magnetic resonance angiography are relatively newer applications that are useful for the evaluation of arterial and venous disorders. Special sequences such as fat suppression and fluid attenuation inversion recovery are useful techniques for specific ophthalmic indications. Diffusion weighted imaging and perfusion-weighted imaging are improving the evaluation of acute stroke. Functional MRI, positron emission tomography scanning and single photon emission computed tomography may provide useful information regarding brain or tumor metabolism. Magnetic resonance spectroscopy has expanded our knowledge of brain function. Newer imaging studies have improved our diagnostic abilities on many fronts, including new sequences, new applications of imaging studies, and functional imaging of brain. CONCLUSION: New imaging techniques for brain and orbit have an increased potential for improving diagnostic yield. Accurate and timely communication with the neuroradiologist can optimize the prescription and interpretation of imaging in ophthalmology.

Systemic infections of neuro-ophthalmic significance.

Systemic infectious disease may present with or include neuro-ophthalmic findings. Many of these signs and symptoms are related to topographical location of the infectious process,but some entities have distinctive and characteristic features that allow specific diagnosis. This article updates and reviews the neuro-ophthalmic features of infectious disease.

The uveo-meningeal syndromes.

BACKGROUND: The uveo-meningeal syndromes are a group of disorders that share involvement of the uvea, retina, and meninges. REVIEW SUMMARY: We review the clinical manifestations of uveitis and describe the infectious, inflammatory, and neoplastic conditions associated with the uveo-meningeal syndrome. CONCLUSIONS: Inflammatory or autoimmune diseases are probably the most common clinically recognized causes of true uveo-meningeal syndromes. These entities often cause inflammation of various tissues in the body, including ocular structures and the meninges (eg, Wegener granulomatosis, sarcoidosis, Behcet disease, Vogt-Koyanagi-Harada syndrome, and acute posterior multifocal placoid pigment epitheliopathy). The association of an infectious uveitis with an acute or chronic meningoencephalitis is unusual but occasionally the eye examination may suggest an infectious etiology or even a specific organism responsible for a meningeal syndrome. One should consider the diagnosis of primary ocular-CNS lymphoma in patients 40 years of age or older with bilateral uveitis, especially with prominent vitritis, that fails to respond to treatment or who has associated neurologic findings. A paraneoplastic disorder has been described in patients who have combined optic neuritis and retinitis defined serologically by the presence of a paraneoplastic IgG autoantibody CRMP-5-IgG. These patients may have an inflammatory vitritis and may have signs of cerebrospinal fluid inflammation.

Painful ophthalmologic disorders and eye pain for the neurologist.

Neurologists should be aware of the following causes of eye pain: (1)ocular and orbital disorders with or without visible pathology of the eye (eg,redness, corneal opacity, or proptosis); (2) ophthalmologic syndromes associated with headache; and (3) headache syndromes associated with ophthalmologic findings.

Pseudotumor cerebri.

Pseudotumor cerebri (PTC) is a syndrome of increased intracranial pressure without hydrocephalus or mass lesion and with normal cerebrospinal fluid (CSF) composition. Although often considered to be "idiopathic," detailed investigation has revealed a high incidence of venous outflow abnormalities in PTC syndrome patients. The thought that elevated intracranial venous sinus pressure is a "universal mechanism" for PTC syndrome of varying etiologies has been called into question by a study indicating that the increased venous pressure in idiopathic PTC patients is caused by the elevated intracranial pressure and not the reverse, suggesting that "the chicken is the CSF pressure elevation and the egg is the venous sinus pressure elevation." Vitamin A toxicity may play a role in the pathogenesis of idiopathic PTC. The treatment of PTC has two major goals: the alleviation of symptoms and preservation of visual function. When medical therapy fails or when visual dysfunction deteriorates, surgical therapies for PTC should be considered. The two main procedures performed include lumboperitoneal shunt and optic nerve sheath fenestration. Because of the association of PTC with venous sinus hypertension, some authors are considering venous sinus stenting for refractory cases of PTC. It is still unclear if primary treatment of the observed venous stenosis benefits patients with idiopathic PTC. This should be no surprise, as it is not certain whether the stenoses are the cause or the result of idiopathic PTC.

Whipple's disease with destructive arthritis, abdominal lymphadenopathy, and central nervous system involvement.

We describe a patient with Whipple's disease who had an unusual erosive and destructive polyarthritis, massive abdominal lymphadenopathy, asymptomatic central nervous system involvement, and rare manifestations of orbital pseudotumor and orchitis with epididymitis. Taking oral therapy with trimethoprim-sulfamethoxazole he had recurrent flares of orbital pseudotumor, an episode of orchitis with epididymitis, and persistent polymerase chain reaction T. whipplei-positive cerebrospinal fluid. Resolution was achieved with a one month course of intravenous ceftriaxone and a 6 month course of azithromycin, and no relapse occurred during 24 months of followup.