1: J Neuroophthalmol. 2012 Jan 31; [Epub ahead of print] 

Diagnostic Uncertainty Due to Optic Disc Drusen.

Vaphiades MS.

Departments of Ophthalmology, Neurology and Neurosurgery, University of Alabama
at Birmingham, Birmingham, Alabama.

ABSTRACT: An 80-year-old woman developed swollen optic nerves with hemorrhages
associated with optic disc drusen. Hemorrhagic complications of optic disc
drusen are discussed.

PMID: 22297266  [PubMed - as supplied by publisher]

2: J Neuroophthalmol. 2012 Jan 31; [Epub ahead of print] 

Relapsing Dorsal Midbrain Syndrome Following Interventions for Hydrocephalus in
Aqueductal Stenosis.

Apkarian AO, Garton HJ, Wesolowski J, Trobe JD.

Departments of Ophthalmology and Visual Sciences (Kellogg Eye Center) (AOA,
JDT), Neurology (JDT), Neurosurgery (HJLG, JDT), and Radiology (Neuroradiology)
(JW), University of Michigan Medical Center, Ann Arbor, Michigan.

ABSTRACT: Dorsal midbrain syndrome (DMS) is a recognized clinical manifestation
of increased intracranial pressure (ICP) associated with ventricular
enlargement, especially in shunt malfunction, but the mechanism by which DMS
occurs in this setting is unsettled. We report a patient with
triventriculomegaly attributed to aqueductal narrowing by a tectal mass who went
through 2 cycles of developing and resolving DMS promptly after undergoing
interventions that altered the size of the posterior third ventricle and
proximal aqueduct but probably did not markedly alter ICP. This case provides
additional evidence that DMS in this setting is caused by deformation of the
dorsal midbrain region produced by rapid expansion of the posterior third
ventricle or proximal aqueduct.

PMID: 22297265  [PubMed - as supplied by publisher]

3: J Neuroophthalmol. 2012 Jan 26; [Epub ahead of print] 

Hyperbaric Oxygen Therapy in the Treatment of Radiation Optic Neuropathy.

Malik A, Golnik K.

Department of Ophthalmology, Cincinnati Eye Institute, University of Cincinnati,
Cincinnati, Ohio.

ABSTRACT: Radiation optic neuropathy (RON) is characterized by the rapid onset
of painless irreversible vision loss in one or both eyes. We report 4
consecutive patients who presented with unilateral or asymmetric bilateral RON.
All had bilateral optic nerve enhancement on MRI and were treated with
hyperbaric oxygen (HBO) and oral corticosteroids. In the less affected eye, 2 of
the 4 patients had preservation of vision, while in the other 2 patients, vision
declined. No patient showed improvement in the more symptomatic eye, and in 2
patients, there was a significant decline in visual acuity. Our findings suggest
that prompt treatment with HBO and oral corticosteroids may result in visual
preservation in the less affected eye despite the presence of optic nerve
enhancement on MRI.

PMID: 22286188  [PubMed - as supplied by publisher]

4: J Neuroophthalmol. 2012 Jan 26; [Epub ahead of print] 

Paralysis of Accommodation With Preserved Pupillary Function as the Initial
Manifestation of Guillain-Barre Syndrome.

Fausett BV, Trobe JD.

Departments of Ophthalmology and Visual Sciences (BVF, JDT) and Neurology (JDT),
University of Michigan Medical School, Ann Arbor, Michigan.

ABSTRACT: A 7-year-old boy who complained of blurred reading vision was found on
ophthalmologic examination to have subnormal distance visual acuity in both eyes
and a hyperopic refractive error that was deemed too small to explain his
symptoms. Within days, he developed speech and gait deficits that led to a
diagnosis of Guillain-Barre syndrome (GBS). Inpatient examination confirmed a
profound binocular loss of accommodation with preservation of iris sphincter
function and eye movements. This is only the second detailed report of
accommodative loss with sparing of the pupil as the initial clinical
manifestation of GBS.

PMID: 22286187  [PubMed - as supplied by publisher]

5: J Neuroophthalmol. 2012 Jan 19; [Epub ahead of print] 

Is Leber Hereditary Optic Neuropathy Treatable? Encouraging Results With
Idebenone in Both Prospective and Retrospective Trials and An Illustrative Case.

Sabet-Peyman EJ, Khaderi KR, Sadun AA.

Department of Ophthalmology (EJS-P, KRK, AAS), Doheny Eye Institute, Keck School
of Medicine, University of Southern California, Los Angeles, California.

ABSTRACT: A 31-year-old woman developed subacute bilateral visual loss over a
2-week period. Two months later, the diagnosis of Leber hereditary optic
neuropathy (LHON) 11778/ND4 was established and the patient was treated with 900
mg of idebenone daily. Over the ensuing 9 months, visual acuity improved from
20/200 to 20/25 in each eye with near-total resolution in visual field
abnormalities. Our case report is in agreement with 2 large published series of
patients with LHON treated with idebenone, raising hope for treatment of this
visually devastating mitochondrial disorder.

PMID: 22269948  [PubMed - as supplied by publisher]

6: J Neuroophthalmol. 2012 Jan 19; [Epub ahead of print] 

Is Intravitreal Bevacizumab an Effective Treatment Option for Nonarteritic
Anterior Ischemic Optic Neuropathy?

Prescott CR, Sklar CA, Lesser RL, Adelman RA.

Department of Ophthalmology and Visual Science (CRP, CAS, RLL, RAA), Yale
University School of Medicine, New Haven, Connecticut.

ABSTRACT: Nonarteritic anterior ischemic optic neuropathy (NAION) causes sudden
profound loss of vision with no known cause or cure. Various treatment
modalities, both surgical and pharmacologic, have been tried without success.
The purpose of our retrospective study was to evaluate the effect of
intravitreal bevacizumab (Avastin) as a treatment option for NAION. We evaluated
demographics of 5 patients and compared visual acuity and automated visual
fields prior to and following intravitreal bevacizumab injection. Visual acuity
at presentation was 20/20 in 4 of 5 patients and 20/150 in 1. Visual acuity
improved to 20/40 in the patient who presented with decreased acuity and
decreased slowly in 3 patients and rapidly in 1. All patients presented with
variable visual field defects: 1 improved slightly, 3 progressed, and 1 remained
stable. One patient subsequently developed NAION in the fellow eye. These
results are consistent with the natural course of the disease, and bevacizumab
did not appear to have a dramatic effect on the clinical outcome in this small
series of patients with NAION.

PMID: 22269947  [PubMed - as supplied by publisher]

7: J Neuroophthalmol. 2012 Jan 19; [Epub ahead of print] 

Spinal Leptomeningeal Lymphoma Presenting as Pseudotumor Syndrome.

Ahmed RM, King J, Gibson J, Buckland ME, Gupta R, Gonzales M, Halmagyi GM.

Departments of Neurology (RMA, GMH), Haematology (JG), and Neuropathology (MEB,
RG), Royal Prince Alfred Hospital, Sydney, Australia; and Departments of
Neurology (JK) and Neuropathology (MG), Royal Melbourne Hospital, Melbourne,
Australia.

ABSTRACT: We describe 2 patients with inexorably progressive pseudotumor
syndrome (intracranial hypertension without mass lesion or ventriculomegaly)
both initially misdiagnosed as having idiopathic intracranial hypertension and
who were eventually found to have spinal leptomeningeal lymphoma. Neither had,
at any time, any clinical signs of a spinal cord or root lesion. We discuss the
possible implications of these observations regarding the diagnosis and
mechanism of the pseudotumor syndrome.

PMID: 22269946  [PubMed - as supplied by publisher]

8: J Neuroophthalmol. 2012 Jan 20; [Epub ahead of print] 

Central Retinal Artery Occlusion and Recurrent Papillitis in a Patient With
Incomplete Behcet Disease:  A Response.

Zhang X, Tian G.

Department of Neurology Beijing Tongren Hospital Capital Medical University
Beijing, China.

PMID: 22269945  [PubMed - as supplied by publisher]

9: J Neuroophthalmol. 2012 Jan 20; [Epub ahead of print] 

Visual Pathway Axonal Loss in Benign Multiple Sclerosis:  A Longitudinal Study.

Galetta KM, Graves J, Talman LS, Lile DJ, Frohman EM, Calabresi PA, Galetta SL,
Balcer LJ.

Departments of Neurology (KMG, JG, LST, DJL, SLG, LJB), Ophthalmology (SLG,
LJB), and Epidemiology (LJB), University of Pennsylvania School of Medicine,
Philadelphia, Pennsylvania; Department of Neurology (EMF), University of Texas
Southwestern Medical Center at Dallas, Dallas, Texas; and Department of
Neurology (PAC), Johns Hopkins University School of Medicine, Baltimore,
Maryland.

BACKGROUND: Benign multiple sclerosis (MS), traditionally defined as Expanded
Disability Status Scale (EDSS) score /=15-year disease duration, is
thought to follow a milder clinical course. We determined the extent of visual
pathway axonal loss by optical coherence tomography (OCT) retinal nerve fiber
layer (RNFL) thickness in a benign MS cohort and examined the relation to vision
and quality of life (QOL). METHODS: In this longitudinal study of vision in MS
at 3 academic centers, a subset of patients with EDSS, visual function, OCT, and
QOL assessments was analyzed. Low- and high-contrast letter acuity was performed
to assess visual function. RNFL thickness was determined using time-domain OCT.
QOL scales included the 25-Item National Eye Institute Visual Functioning
Questionnaire (NEI-VFQ-25) and Short Form-36 Health Survey. RESULTS: Among 68
patients (135 eyes) studied longitudinally, 13 (26 eyes) had benign MS using
criteria of EDSS score /=15-year disease duration. Benign MS eyes had
as much RNFL thinning (-3.6 mum, P = 0.0008 vs baseline, paired t test) as
typical MS eyes (-3.3 mum, P < 0.0001). Both groups had significant low-contrast
acuity loss. History of optic neuritis (ON) was more frequent in benign MS (69%
vs 33% of eyes). History of ON distinguished benign vs typical MS (P = 0.002)
and correlated with RNFL thickness at baseline (P = 0.002) and disease duration
(P = 0.03) but not EDSS (P = 0.32, logistic regression). NEI-VFQ-25 scores were
also worse for benign MS, accounting for age (75 +/- 21 vs 88 +/- 11, P =
0.005). CONCLUSION: Patients with benign MS have RNFL axonal loss that is as
marked as that of typical MS and have reduced vision and QOL. While overall
neurologic impairment is mild, visual dysfunction, not well captured by the
EDSS, accounts for a substantial degree of disability in benign MS.

PMID: 22269944  [PubMed - as supplied by publisher]

10: J Neuroophthalmol. 2012 Jan 20; [Epub ahead of print] 

Central Retinal Artery Occlusion and Recurrent Papillitis in a Patient With
Incomplete Behcet Disease:  A Comment.

Liu Y.

Aier Eye Hospital of Changsha Changsha, China liuying_usc@hotmail.com.

PMID: 22269943  [PubMed - as supplied by publisher]

11: J Neuroophthalmol. 2012 Jan 12; [Epub ahead of print] 

Nonconvulsive Status Epilepticus Presenting as Epileptic Nystagmus in a Patient
With Herpes Encephalitis.

Lee JH, Nam DH, Oh SY, Shin BS, Seo MW, Jeong SK, Lee JY.

Department of Neurology, Research Institute of Clinical Medicine, Chonbuk
National University, Chonbuk National University Hospital, Jeonju, South Korea.

ABSTRACT: Epileptic nystagmus (EN) is characterized by rhythmic ocular
oscillations or quick, repetitive eye movements secondary to seizure activity.
The fast component of EN is known to be contralateral to the epileptogenic
focus. There are few reports EN occurring patients in nonconvulsive status
epilepticus (NCSE). We report such a patient in the setting of herpes
encephalitis.

PMID: 22246060  [PubMed - as supplied by publisher]

12: J Neuroophthalmol. 2012 Jan 12; [Epub ahead of print] 

Cortical Vision Loss as a Prominent Feature of H1N1 Encephalopathy.

Pula JH, Issawi A, Desanto JR, Kattah JC.

Departments of Neurology and Neuro-ophthalmology (JHP, JCK), Neurosurgery (AI),
and Radiology (JRD), University of Illinois College of Medicine at Peoria,
Peoria, Illinois.

ABSTRACT: A 20-year-old woman infected with the 2009 H1N1 strain of influenza A
developed bilateral visual loss. Brain MRI showed restricted diffusion of the
parietal and occipital lobes, and her spinal fluid did not contain inflammatory
cells. This report describes an unusual case of H1N1 influenza A virus infection
primarily affecting the posterior visual pathways.

PMID: 22246059  [PubMed - as supplied by publisher]

13: J Neuroophthalmol. 2012 Jan 12; [Epub ahead of print] 

Evaluation and Calibration of a Binocular Infrared Pupillometer for Measuring
Relative Afferent Pupillary Defect.

Shwe-Tin A, Smith GT, Checketts D, Murdoch IE, Taylor D.

Ophthalmology Department (AS-T, GTS), The Great Western Hospital, Wiltshire,
England; Quanticate Ltd. (DC), Hertfordshire, England; Institute of
Ophthalmology (IEM), London, England; and Procyon Instruments Ltd. (DT), London,
England.

BACKGROUND: Binocular infrared pupillometry allows an estimate of the relative
afferent pupillary defect (RAPD), designated the pupillometric RAPD (pRAPD). We
calibrated the pRAPD of a commercially available pupillometer against neutral
density filters (NDFs) of known attenuation. The performance of the pupillometer
using its own proprietary algorithm is assessed and compared to that of
alternative algorithms. METHODS: The pRAPDs of 50 healthy volunteers were
measured with each of 4 filters of known attenuation: 0.0, 0.3, 0.6, and 0.9 log
units, positioned unilaterally in the light stimulus pathway. The filter values
were plotted against the pupillometer output, and the slope and intercept were
used to determine a calibration factor. Corrected pRAPD results were used to
assess physiological ranges of pRAPD. The sensitivity and specificity to 0.3 log
unit differences between increasing filter densities using receiver operator
characteristic (ROC) curves. RESULTS: The calibrated physiological pRAPD ranged
from 0 to 0.22 log units. The area under the ROC curve for detecting unilateral
simulated pRAPD of 0.3 log units, the simulated disease progression from 0.3 to
0.6 log units, and a further progression from 0.6 to 0.9 log units by NDFs was
0.99 (95% confidence interval [CI], 0.95-1.00), 0.86 (95% CI, 0.78-0.92), and
0.79 (95% CI, 0.70-0.87), respectively. The optimum discrimination was for
detecting a unilateral simulated pRAPD of 0.3 log units; sensitivity and
specificity was 98% (95% CI, 88%-99%). CONCLUSION: The commercially available
pupillometer detects the RAPD induced by the NDFs with high sensitivity and
specificity. The results suggest that it is best for detecting unilateral early
disease but potentially useful for assessing progression of disease.

PMID: 22246058  [PubMed - as supplied by publisher]

14: J Neuroophthalmol. 2012 Jan 3; [Epub ahead of print] 

Idiopathic Intracranial Hypertension:  Relation Between Obesity and Visual
Outcomes.

Szewka AJ, Bruce BB, Newman NJ, Biousse V.

Departments of Ophthalmology (AJS, BBB, NJN, VB), Neurology (BBB, NJN, VB), and
Neurological Surgery (NJN), Emory University School of Medicine, Atlanta,
Georgia.

BACKGROUND: Increased body mass index (BMI) has been associated with increased
risk of idiopathic intracranial hypertension (IIH), but the relationship of BMI
to visual outcomes in IIH is unclear. METHODS: A retrospective chart review of
all adult cases of IIH satisfying the modified Dandy criteria seen at our
institution between 1989 and 2010 was performed. Demographics, diagnostic
evaluations, baseline visit and last follow-up examination data, treatment, and
visual outcome data were collected in a standardized fashion. Groups were
compared, and logistic regression was used to evaluate the relationship of BMI
to severe visual loss, evaluating for interaction and controlling for potential
confounders. RESULTS: Among 414 consecutive IIH patients, 158 had BMI >/=40
(World Health Organization Obese Class III) and 172 had BMI 30-39.9. Patients
with BMI >/=40 were more likely to have severe papilledema at first
neuro-ophthalmology encounter than those with a lower BMI (P = 0.02). There was
a trend toward more severe visual loss in 1 or both eyes at last follow-up among
those patients with BMI >/=40 (18% vs 11%, P = 0.067). Logistic regression
modeling found that 10-unit (kilogram per square meter) increases in BMI
increased the odds of severe visual loss by 1.4 times (95% confidence interval,
1.03-1.91, P = 0.03) after controlling for sex, race, diagnosed hypertension,
and diagnosed sleep apnea. CONCLUSION: Our finding of a trend for severe
papilledema and visual loss associated with increasing BMI suggests that very
obese IIH patients should be closely monitored for progression of visual field
loss.

PMID: 22217456  [PubMed - as supplied by publisher]