TEMPORAL LOBE HERNIATION AND CHIASMOPATHY DURING DOPAMINE AGONIST THERAPY FOR PROLACTINOMA.

Background: Dopamine agonists (DA) are first line treatment for prolactinomas. Optic chiasm herniation can rarely occur during therapy, while brain herniation is very uncommon. Case Reports: A 34 yo woman presented with headaches and vision changes. Prolactin (PRL) was 4300 ng/mL. MRI showed a 4.5 cm pituitary adenoma with chiasm compression. After 3 months, PRL decreased to 201 ng/mL while patient was taking CAB 0.75 mg twice a week. MRI showed ~30% tumor reduction with medial temporal lobe herniation and encephalocele. CAB was stopped and she underwent surgical debulking and encephalocele repair. Histopathology confirmed prolactin tumor. CAB 0.75 mg twice a week was resumed.A 50 yo man had incidental detection of a sellar mass after trauma. MRI showed 3.6 cm tumor with minimal contact of right optic nerve, and PRL 3,318 ng/ml. He received CAB 0.5mg twice a week with PRL improvement to 26 ng/mL after 1 month. After 2 months ophthalmology exam showed new left superotemporal depression. PRL was 68 ng/mL and MRI showed 35% mass reduction and new inferior displacement tethering of the chiasm. CAB dose was decreased to 0.25 mg twice a week. Conclusion: Our cases illustrate that rapid biochemical and radiographic response to DA therapy in large prolactinomas warrants close clinical and neuro-ophthalmologic follow-up. We recommend repeating the MRI 3 months after initiation of DA therapy or sooner in case of new mass effect manifestations. Decision regarding DA dose reduction or chiasmopexy for visual field deficits needs to be multi-disciplinary and on a case-to-case basis.

Differing presenting features of idiopathic intracranial hypertension in the UK and US.

AIM: Demographic factors potentially influencing the presentation and severity of idiopathic intracranial hypertension (IIH) in the US vs. UK populations include obesity and ethnicity. We aimed to compare the presenting features of IIH between populations in the UK and US tertiary referral centres, to assess what population differences exist and whether these cause different presentations and impact on visual function. METHODS: Clinical data were collected on 243 consecutive UK IIH patients and 469 consecutive US IIH patients seen after 2012 in two tertiary centres. Visual function was defined as severe visual loss when Humphrey visual field mean deviation was <-15 dB, when Goldmann visual fields showed constriction or when visual acuity was <20/200. RESULTS: US patients were more commonly of self-reported black race (58.9% vs. 7.1%) than UK patients, but had a similar mean body mass index (38.3 ± 0.63kg/m2 UK vs. 37.7 ± 0.42kg/m2 US; p = 0.626). The UK cohort had lower presenting Frisén grade (median 1 vs. 2; p < 0.001) and severe visual loss less frequently (15.4% vs. 5%; p = 0.014), but there was no difference in mean cerebrospinal fluid-opening pressure (CSF-OP) (35.8 ± 0.88cmH2O UK vs. 36.3 ± 0.52cmH2O US; p = 0.582). African Americans had poorer visual outcomes compared with US whites (19.4% vs. 10% severe visual loss; p = 0.011). Visual function was weakly associated with CSF-OP (R2 = 0.059; p = 0.001), which was similar between UK and US patients. CONCLUSIONS: The UK and the US cohorts had a similar average presenting BMI. However, the worse presenting visual function in the US IIH cohort was partially attributable to differences in the black populations in the two countries.

Noninvasive Assessment of Intracranial Pressure Status in Idiopathic Intracranial Hypertension Using Displacement Encoding with Stimulated Echoes (DENSE) MRI: A Prospective Patient Study with Contemporaneous CSF Pressure Correlation.

BACKGROUND AND PURPOSE: Intracranial pressure is estimated invasively by using lumbar puncture with CSF opening pressure measurement. This study evaluated displacement encoding with stimulated echoes (DENSE), an MR imaging technique highly sensitive to brain motion, as a noninvasive means of assessing intracranial pressure status. MATERIALS AND METHODS: Nine patients with suspected elevated intracranial pressure and 9 healthy control subjects were included in this prospective study. Controls underwent DENSE MR imaging through the midsagittal brain. Patients underwent DENSE MR imaging followed immediately by lumbar puncture with opening pressure measurement, CSF removal, closing pressure measurement, and immediate repeat DENSE MR imaging. Phase-reconstructed images were processed producing displacement maps, and pontine displacement was calculated. Patient data were analyzed to determine the effects of measured pressure on pontine displacement. Patient and control data were analyzed to assess the effects of clinical status (pre-lumbar puncture, post-lumbar puncture, or control) on pontine displacement. RESULTS: Patients demonstrated imaging findings suggesting chronically elevated intracranial pressure, whereas healthy control volunteers demonstrated no imaging abnormalities. All patients had elevated opening pressure (median, 36.0 cm water), decreased by the removal of CSF to a median closing pressure of 17.0 cm water. Patients pre-lumbar puncture had significantly smaller pontine displacement than they did post-lumbar puncture after CSF pressure reduction (P = .001) and compared with controls (P = .01). Post-lumbar puncture patients had statistically similar pontine displacements to controls. Measured CSF pressure in patients pre- and post-lumbar puncture correlated significantly with pontine displacement (r = 0.49; P = .04). CONCLUSIONS: This study establishes a relationship between pontine displacement from DENSE MR imaging and measured pressure obtained contemporaneously by lumbar puncture, providing a method to noninvasively assess intracranial pressure status in idiopathic intracranial hypertension.

[Diplopia: An important symptom in internal medicine!] / Diplopie : un symptôme important en médecine interne !

Diplopia is defined as "double vision" when looking at a single object. Monocular diplopia is related to an ocular disorder and must be differentiated from binocular diplopia which is secondary to ocular misalignment. The examination of the patient with binocular diplopia is often challenging for non-specialists. However, a careful and systematic clinical examination followed by targeted ancillary testing allows the clinician to localize the lesion along the oculomotor pathways. The lesion may involve the brainstem, the ocular motor nerves III, IV or VI, the neuromuscular junction, the extraocular ocular muscles, or the orbit. Causes of binocular diplopia are numerous and often include disorders typically managed by internal medicine such as inflammatory, infectious, neoplastic, endocrine, and metabolic disorders. In addition to treating the underlying disease, it is important not to leave diplopia uncorrected. Temporary occlusion of one eye by applying tape on one lens or patching one eye relieves the diplopia until more specific treatments are offered should the diplopia not fully resolve.

Functional and simulated visual loss.

Nonorganic visual loss (NOVL) is the cause of a large number of referrals to neurologists and ophthalmologists and is a frequent area of overlap between neurologists, ophthalmologists, and psychiatrists. NOVL is the presence of visual impairment without an organic cause for disease despite a thorough and comprehensive investigation. A diagnosis of NOVL requires both the absence of any findings on examination and proof of the integrity and functioning of the visual system. Although sometimes a challenging diagnosis to make, there are a number of techniques and maneuvers which can be utilized fairly easily, either at the bedside or in the clinic, to help determine if a patient has NOVL. In some instances specialized testing, such as formal visual field testing, optical coherence tomography, visual evoked responses, electroretinogram, and various imaging modalities (magnetic resonance imaging) are performed to help determine if the cause of visual loss is organic or nonorganic. Once a diagnosis of NOVL is made, treatment centers around reassurance of the patient, close follow-up, and, if necessary, referral to a psychiatrist, as these patients may have underlying psychiatric disorders and a preceding strong emotional event leading to the current symptoms, and may be more likely to develop depression and anxiety.

Idiopathic intracranial hypertension: Diagnosis, monitoring and treatment.

INTRODUCTION: Idiopathic intracranial hypertension (IIH) is a disorder typically affecting young, obese women, producing a syndrome of increased intracranial pressure without identifiable cause. STATE OF THE ART: Despite a large number of hypotheses and publications over the past decade, the etiology of IIH is still unknown. There continues to be no evidence-based consensus or formal guidelines regarding management and treatment of the disease. Treatment studies show that the diagnostic lumbar puncture is a valuable intervention beyond its diagnostic importance, and that weight management is critical. However, many questions remain, regarding the efficacy of acetazolamide, cerebrospinal fluid (CSF) shunting procedures, optic nerve sheath fenestration, and cerebral transverse venous sinus stenting. Identification of subgroups of patients at high-risk for irreversible visual loss, such as black patients, men, morbidly obese patients, and patients with fulminant IIH, helps determine management approaches and refine follow-up strategies. PERSPECTIVE: Better understanding of the pathophysiology and ongoing clinical trials will hopefully help inform treatment strategies over the next few years.

Optical coherence tomography (OCT) in optic neuritis and multiple sclerosis.

Optical coherence tomography (OCT) is a non-invasive imaging technique routinely used in ophthalmology to visualize and quantify the layers of the retina. It also provides information on optic nerve head topography, peripapillary retinal nerve fiber layer thickness, and macular volume, which correlate with axonal loss. These measurements are of particular interest in optic neuropathies and in multiple sclerosis, and OCT parameters are now used as endpoints in neurologic clinical trials.

Atypical idiopathic intracranial hypertension: normal BMI and older patients.

BACKGROUND: Idiopathic intracranial hypertension (IIH) typically affects young, obese women. We examined 2 groups of atypical patients with IIH: those with a normal body mass index (BMI) and those at least 50 years of age. METHODS: A retrospective cohort study of 407 consecutive adult patients with IIH with known BMI from 3 centers was undertaken. Demographics, associated factors, visual acuity, and visual fields were collected at presentation and follow-up. RESULTS: We identified 18 IIH patients (4%) with normal BMI and 19 (5%) aged 50 years or older at the time of diagnosis who were compared with the remainder of the cohort. Medication-induced IIH was more frequent in patients with IIH with normal BMI (28 vs 7%, p = 0.008). No patient with IIH with a normal BMI had severe visual loss in either eye (0 vs 17%, p = 0.09). Older patients with IIH had a lower BMI, but were still generally obese (33 vs 38, p = 0.04). Older patients were less likely to report headache as initial symptom (37 vs 76%, p < 0.001) and more likely to complain of visual changes (42 vs 21%, p = 0.03). Treatment of any type was less likely in older patients (significant for medications: 74 vs 91%, p = 0.004), and they were more likely to have persistent disc edema at last follow-up (median Frisén grade: 1 vs 0, p = 0.002), but had similar, if not better, visual outcomes compared with younger patients. A case-control study did not identify any new medication or risk factor associations. CONCLUSIONS: Patients with normal body mass index and those 50 years or older make up a small proportion of patients with idiopathic intracranial hypertension (IIH), but appear to have better visual outcomes than more typical patients with IIH.

[Vascular transient monocular visual loss]. / Les troubles visuels monoculaires transitoires d'origine vasculaire.

Diagnosis and management of transient monocular visual loss is an emergency. Ocular conditions causing transient visual loss are routinely managed by ophthalmologists. Vascular transient monocular visual loss may result from emboli, hypoperfusion, vasospasm, or venous congestion. Evaluation focuses on the carotid arteries, ophthalmic arteries, the aortic arch, the heart, and rarely hypercoagulable states. Secondary prevention of ischemic events is essential in order to prevent permanent visual loss as well as cerebral ischemic and cardiovascular death. Aggressive treatment of vascular risk factors is usually associated with antiplatelet agents. Anticoagulant and carotid surgery are only rarely required after vascular transient monocular visual loss.

[Transient visual loss: a practical approach]. / Les troubles visuels transitoires : démarche diagnostique.

Transient vision loss results from a variety of disorders including benign dry eye as well as vision- or life-threatening disorders such as giant cell arteritis. The goal of the initial evaluation is to determine the most likely mechanism based on the past medical history, characteristics of visual loss, and ophthalmologic and neurologic examinations. The ophthalmologist is often the first physician consulted and plays an essential role. Indeed, identification of the mechanism of transient visual loss allows the care-taker to properly decide on the work-up, its timing (emergency or otherwise), and subsequent treatment.

Pain in ischaemic ocular motor cranial nerve palsies.

AIM: Pain is a common feature of microvascular ischaemic ocular motor cranial nerve palsies (MP). The natural history of pain in this condition has not been studied. The purpose of this report is to define the spectrum of pain in isolated MP, with special reference to diabetic versus non-diabetic patients. DESIGN AND METHODS: Retrospective and prospective chart review was performed on 87 patients with acute-onset MP of a single cranial nerve (CN III, oculomotor; CN IV, trochlear; CN VI, abducens) that progressively improved or resolved over 6 months. RESULTS: Five of the 87 patients had two events, making the total number events 92. There were 39 (42.4%) CN III palsies, five (5.4%) CN IV palsies and 48 (52.2%) CN VI palsies. Thirty-six (41%) patients had diabetes. Pain was present in 57 (62%) events. The majority of diabetic and non-diabetic patients had pain. Pain preceded diplopia by 5.8 (SD 5.5) days in one-third of events. There was a trend towards greater pain with CN III palsies, but this was not statistically significant. Patients who experienced severe pain tended to have pain for a longer duration (26.4 (SD 21.7) days compared with 10.8 (SD 8.3) and 9.5 (SD 9) days for mild and moderate pain, respectively). There was no correlation between having diabetes and experiencing pain. CONCLUSIONS: The majority of MP are painful, regardless of the presence or absence of diabetes. Pain may occur prior to or concurrent with the onset of diplopia. Non-diabetic and diabetic patients presented with similar pain characteristics, contrary to the belief that diabetic patients have more pain associated with MP.

Idiopathic intracranial hypertension: a comparison between French and North-American white patients.

OBJECTIVE: To compare French and American white patients with idiopathic intracranial hypertension (IIH), and to determine prognostic factors associated with visual loss. METHODS: Medical records of all consecutive white patients with definite IIH seen between 2001 and 2006 in three French tertiary care medical centers and one American tertiary medical center were reviewed. Demographics, associated clinical features, and visual function at presentation and follow-up were collected. French white patients were compared to American white patients. RESULTS: One hundred and thirty-four patients (66 French, 68 American) were included. American patients were 8.7 times more likely than French patients to have visual acuity 20/60 or worse or visual field constriction (95% CI: 2.1-36.1, p=0.0001). American patients were treated more aggressively than French patients. French patients were older (31 vs. 28 years, p=0.02) and more likely to have anemia (20 vs. 2%, p<0.001). American patients had a longer duration of symptoms prior to diagnosis (12 vs. 4 weeks, p=0.01) and longer follow-up than French patients (26 vs. 11 months, p=0.001). Multivariable analysis found that nationality was an independent risk factor for visual loss. French and American patients did not differ regarding gender proportion, frequency of obesity, sleep apnea, endocrine diseases, or systemic hypertension. Cerebrospinal fluid (CSF) opening pressures were similar in both groups. CONCLUSION: American patients with IIH had worse visual outcomes than French patients despite more aggressive treatment. These differences are not explained by differences in previously known risk factors.

Idiopathic intracranial hypertension in men.

OBJECTIVE: To compare the characteristics of idiopathic intracranial hypertension (IIH) in men vs women in a multicenter study. METHODS: Medical records of all consecutive patients with definite IIH seen at three university hospitals were reviewed. Demographics, associated factors, and visual function at presentation and follow-up were collected. Patients were divided into two groups based on sex for statistical comparisons. RESULTS: We included 721 consecutive patients, including 66 men (9%) and 655 women (91%). Men were more likely to have sleep apnea (24% vs 4%, p < 0.001) and were older (37 vs 28 years, p = 0.02). As their first symptom of IIH, men were less likely to report headache (55% vs 75%, p < 0.001) but more likely to report visual disturbances (35% vs 20%, p = 0.005). Men continued to have less headache (79% vs 89%, p = 0.01) at initial neuro-ophthalmologic assessment. Visual acuity and visual fields at presentation and last follow-up were significantly worse among men. The relative risk of severe visual loss for men compared with women was 2.1 (95% CI 1.4-3.3, p = 0.002) for at least one eye and 2.1 (95% CI 1.1-3.7, p = 0.03) for both eyes. Logistic regression supported sex as an independent risk factor for severe visual loss. CONCLUSION: Men with idiopathic intracranial hypertension (IIH) are twice as likely as women to develop severe visual loss. Men and women have different symptom profiles, which could represent differences in symptom expression or symptom thresholds between the sexes. Men with IIH likely need to be followed more closely regarding visual function because they may not reliably experience or report other symptoms of increased intracranial pressure.

[Management of isolated optic neuritis in France: survey of neurologists and ophthalmologists]. / Prise en charge des névrites optiques isolées en France: enquête auprès des neurologues et des ophtalmologistes.

BACKGROUND: Acute isolated optic neuritis is often the first manifestation of multiple sclerosis (MS). Despite the results of several clinical trials its management remains controversial. With the advent of new disease-modifying agents for the treatment of MS, management of isolated optic neuritis has become more complicated. The goal of this study was to evaluate the current clinical practice of French ophthalmologists and neurologists in the management of acute isolated optic neuritis, and to evaluate the impact of recently published randomized clinical trials on their practice. METHODS: A survey, including 24 questions on the diagnosis and treatment of acute isolated optic neuritis was sent to all neurologists and to a sample of ophthalmologists in France. RESULTS: The responses of 655 neurologists and 141 ophthalmologists were analyzed. This study shows mostly that patients initially present more frequently to ophthalmologists, and are subsequently referred to neurologists. Most optic neuritis patients undergo a brain MRI and a lumbar puncture. Although most patients receive high dose intravenous steroids, up to 15% of neurologists and 21% of ophthalmologists still recommend oral prednisone (1 mg/kg per day). Steroids are often prescribed for the wrong reason, including to improve final visual acuity or decrease the risk of MS. Disease modifying agents are sometimes prescribed outside of the official French recommendations. CONCLUSION: The evidence-based guidelines are only partially followed by practitioners managing patients with acute optic neuritis.

[Age-related macular degeneration and risk of stroke]. / Dégénérescence maculaire liée à l'âge et risque d'accident vasculaire cérébral.

Age-related macular degeneration (ARMD) and stroke are common causes of disability in subjects over 40 years of age. The etiology of ARMD remains unknown and numerous epidemiological studies have suggested a relationship between vascular risk factors and ARMD. A direct relationship between stroke and ARMD has also been suggested. An update on the association between ARMD and stroke is discussed in this article.

Racial differences in idiopathic intracranial hypertension.

OBJECTIVE: To evaluate racial differences in idiopathic intracranial hypertension (IIH). METHODS: Medical records of all consecutive patients with definite IIH seen between 1989 and 2006 were reviewed. Demographics, associated factors, and visual function at presentation and follow-up were collected. Black patients were compared to non-black patients. RESULTS: We included 450 patients (197 black, 253 non-black). Obesity, systemic hypertension, anemia, and sleep apnea were more common in black patients than in non-black patients (p

The "sunglasses sign" predicts nonorganic visual loss in neuro-ophthalmologic practice.

OBJECTIVE: The aim of our study was to evaluate whether wearing sunglasses (the "sunglasses sign") can be used by neuro-ophthalmologists to predict nonorganic visual loss (NOVL) in their patients. METHODS: We prospectively collected information on all new patients seen by us over 13 months. We included all patients who ultimately received a diagnosis of NOVL, and all patients wearing sunglasses in our clinic. We recorded demographics, iris color, number of positive review of systems, ocular examination, precipitating event or trauma, workers' compensation claims, disability and lawsuit related to the visual loss, and the reason for wearing sunglasses. RESULTS: Among the 1,377 consecutive new patients seen in our clinic during the study, 34 patients wore sunglasses, among whom 7 (20.6%) had organic visual loss. During the study period, 59 patients were diagnosed with NOVL, among whom 27 (45.8%) wore sunglasses. The sensitivity of wearing sunglasses for NOVL was 0.46 (95% CI 0.33 to 0.59). The probability that a patient walking into our clinic had NOVL was 0.043 (95% CI 0.033 to 0.055); it increased to 0.79 (95% CI 0.62 to 0.91) in sunglasses patients. The specificity of sunglasses for the diagnosis of NOVL was 0.995 (95% CI 0.989 to 0.998). At least one of the following characteristics (highly positive review of systems, workers' compensation claim, disability, and lawsuit) was found in 26 of 27 (96.3%) of NOVL patients wearing sunglasses and in none of the sunglasses patients with organic neuro-ophthalmic disorders. All 7 sunglasses patients with organic neuro-ophthalmic disorders had reasonable ophthalmic explanations for wearing sunglasses. CONCLUSION: The "sunglasses sign" in a patient without an obvious ophthalmic reason to wear sunglasses is highly suggestive of nonorganic visual loss.