Clinical profile of 80-year-old and older thyroid eye disease patients.

PURPOSE: Thyroid eye disease (TED), an autoimmune orbital disorder, follows a time-of-onset bimodal peak: 40-44 and 60-64 years for women, 45-49 and 65-69 years for men. TED, however, can also commence in old age. The study's purpose was to evaluate TED in octo- and nonagenarians. METHODS: Medical records of 19 ≥ 80 years geriatric patients at time of diagnosis were compared to 122 TED patients, aged 20-79. A second analysis was performed after subdividing the control group into two age groups, ≤ 40 ("young group," 16 patients) and 41-79 years ("middle-aged group," 106 patients). RESULTS: The geriatric group's mean age was 84 years (80-94), 11 males and 8 females. Mean follow-up time was 16 months. Compared to the controls, the geriatric patients smoked less (p = 0.012), were more often hypothyroid (p = 0.019), and had concurrent myasthenia gravis (p = 0.02) at time of diagnosis. Diplopia was the most common presenting symptom among the elderly (p = 0.005) and proptosis among the controls, specifically the young group (p = 0.027). Bilateral signs were more common among seniors (p = 0.049). Optic neuropathy was diagnosed in 10% of the geriatric group (2/19) and 11% of middle-aged group (12/106), all being resolved after steroids or orbital decompression. Active disease (clinical activity score (CAS) score = > 3) was more common among the middle-aged group (p = 0.024) while the geriatric patients tended towards higher TED severity grades. Orbital decompression and eyelid repositioning surgeries were more common among the middle-aged group. Strabismus surgeries were more common among seniors. CONCLUSIONS: TED among octo- and nonagenarians has unique patterns, with different demographic features, more exposed to diplopia, hypothyroidism, association with myasthenia gravis, and bilateral involvement. Special attention should be given when medically managing this subgroup.

The "Eyelet Sign" as an MRI Clue for Inflammatory Brown Syndrome.

BACKGROUND: Brown syndrome is characterized by a restrictive elevation deficit of the affected eye in adduction. Besides the well-known congenital form, different acquired etiologies including inflammation, trauma, and surgery may prevent the superior oblique (SO) tendon from gliding freely through the trochlea on attempted upgaze. We present MRI findings in pediatric and adult patients with inflammatory acquired Brown syndrome. METHODS: Retrospective review of clinical and MRI findings of 6 patients (4 children: median age 8.4 years [range 6.1-8.7]; 2 adults: age 46.4 and 51.1 years). Median follow-up was 23 months (range 1-52). RESULTS: In all 6 patients, orbital MRI demonstrated inflammatory changes of the SO tendon-trochlea complex. A striking feature was circumferential contrast enhancement of the trochlea with central sparing where the tendon passes, reminiscent of an eyelet. In all cases, the motility restriction improved either spontaneously or with systemic anti-inflammatory treatment. Although both adult patients had a history of known seronegative spondyloarthritis, there was no associated systemic condition in the children in our series. CONCLUSIONS: Both in children and in adults, MRI can provide evidence of inflammatory changes located at the trochlea-tendon complex in acquired Brown syndrome here referred to as the "eyelet sign," which may be helpful in confirming the clinical diagnosis and guide appropriate treatment.

The Role of Neuro-Ophthalmologists in the Care of Patients With Neurofibromatosis Type 2.

Neurofibromatosis type 2 (NF2) is a rare genetic disorder, affecting the central nervous system and leading to various degrees of disability. Its hallmark is bilateral vestibular schwannomas that invariably lead to progressive hearing loss. Specific ophthalmic abnormalities in patients with NF2 may help to establish an early diagnosis. These include juvenile cataract, epiretinal membrane, combined hamartoma of the retina and the retinal pigment epithelium, optic disc glioma, and optic nerve sheath meningioma. In addition, intracranial tumors may produce a variety of neuro-ophthalmic abnormalities that have the potential to impair visual function, such as postpapilledema optic atrophy, compression of the visual pathways, keratopathy, ocular motor cranial nerve palsies, and amblyopia. Care of NF2 patients is best provided by interdisciplinary medical teams including a neuro-ophthalmologist.

Practice Preferences: Temporal Artery Biopsy versus Doppler Ultrasound in the Work-Up of Giant Cell Arteritis.

To determine whether temporal artery biopsy (TABx) or Doppler ultrasound (US) of the temporal artery is the preferred confirmatory test for giant cell arteritis, an online survey of ophthalmologists and neurologists in North America, Europe and Israel was conducted in 2019; Canadian rheumatologists were also included. There were 406 survey participants with an estimated survey response rate of 18%. Ninety-four per cent of North American practitioners preferred TABx compared with 74% of their European counterparts. Two per cent of North American practitioners preferred Doppler US versus 24% of European physicians. Regional differences were statistically significant (p < .001).

Repetitive ocular vestibular evoked myogenic potential stimulation for the diagnosis of myasthenia gravis: Optimization of stimulation parameters.

OBJECTIVE: To determine the most effective stimulation parameters for the diagnosis of ocular myasthenia gravis (MG) using repetitive ocular vestibular evoked myogenic potentials (oVEMP) for quantification of the extraocular muscle response decrement. METHODS: Repetitive bone-conducted oVEMPs were elicited in 18 MG patients and 20 healthy subjects. We compared four different stimulus repetition rates (20 Hz, 30 Hz, 40 Hz, 50 Hz) and 100 Hz continuous stimulation, as well as recordings from the inferior oblique muscles and the lateral rectus muscles to determine the most sensitive and specific oVEMP parameters for decrement detection. RESULTS: Repetitive stimulation at all tested repetition rates with recordings from inferior oblique muscles allowed for effective differentiation between MG patients and healthy subjects. Among all repetition rates, 30 Hz showed a trend towards superiority, with a sensitivity of 71% and a specificity of 94% (area under the curve (AUC) 0.88) when using the smaller decrement of the two eyes and -10% as cutoff. Considering the larger decrement for analysis (-9% as cutoff), sensitivity increased to 82%, but specificity decreased to 78% (AUC 0.81). CONCLUSIONS: Our study demonstrates, that repetitive oVEMP stimulation elicits a robust decrement in the inferior oblique muscles of MG patients at repetition rates between 20 Hz and 50 Hz, with a probable optimum at 30 Hz. SIGNIFICANCE: Repetitive inferior oblique oVEMP stimulation with optimal stimulus parameters facilitates early and accurate diagnosis of ocular MG.

Management of Peripapillary Choroidal Neovascular Membrane in Patients With Idiopathic Intracranial Hypertension.

OBJECTIVE: To report the clinical features and treatment outcomes of patients with peripapillary choroidal neovascular membrane (CNVM) secondary to idiopathic intracranial hypertension (IIH). METHODS: Retrospective, multicenter chart review of patients diagnosed with peripapillary CNVM in the course of the treatment and follow-up of IIH. RESULTS: Records were reviewed from 7 different institutions between 2006 and 2016. Ten patients (13 eyes) with a diagnosis of IIH and at least 3 months of follow-up developed CNVM. Three of the total 10 patients developed bilateral CNVM. The mean time from the diagnosis of IIH to CNVM diagnosis was 41 months. Mean follow-up period was 8 months after diagnosis of CNVM. All patients were treated with acetazolamide for IIH. Seven eyes were observed, and 6 eyes were given anti-vascular endothelial growth factor (anti-VEGF) injections, including bevacizumab, ranibizumab, and aflibercept. All CNVMs regressed with subretinal fibrosis, and visual acuity improved in most patients. Papilledema resolved in only 1 eye, while the other 12 eyes had persistent papilledema at last follow-up. CONCLUSIONS: Peripapillary CNVM, a rare complication of IIH, often resolves spontaneously with treatment of IIH. In vision-threatening and/or persistent cases, intravitreal anti-VEGF treatment may be a safe and effective therapeutic option.

Immune-mediated conditions affecting the brain, eye and ear (BEE syndromes).

The triad of central nervous system symptoms, visual disturbance and hearing impairment is an oft-encountered clinical scenario. A number of immune-mediated diseases should be considered among the differential diagnoses including: Susac syndrome, Cogan syndrome or Vogt-Koyanagi-Harada disease; demyelinating conditions such as multiple sclerosis or neuromyelitis optica spectrum disorder; systemic diseases such as systemic lupus erythematosus, Sjögren syndrome or Behcet disease and granulomatous diseases such as sarcoidosis. In this article, we coin the term 'BEE syndromes' to draw attention to the various immune-mediated diseases that affect the brain, eye and ear. We present common disease manifestations and identify key clinical and investigation features.

The Most Common Causes of Eye Pain at 2 Tertiary Ophthalmology and Neurology Clinics.

BACKGROUND: Eye pain is a common complaint, but no previous studies have determined the most common causes of this presenting symptom. Our objective was to determine the most common causes of eye pain in 2 ophthalmology and neurology departments at academic medical centers. METHODS: This was a retrospective cross-sectional analysis and chart review at the departments of ophthalmology and neurology at the University Hospital Zurich (USZ), University of Zürich, Switzerland, and the University of Utah (UU), USA. Data were analyzed from January 2012 to December 2013. We included patients aged 18 years or older presenting with eye pain as a major complaint. RESULTS: Two thousand six hundred three patient charts met inclusion criteria; 742 were included from USZ and 1,861 were included from UU. Of these, 2,407 had been seen in an ophthalmology clinic and 196 had been seen in a neurology clinic. Inflammatory eye disease (conjunctivitis, blepharitis, keratitis, uveitis, dry eye, chalazion, and scleritis) was the underlying cause of eye pain in 1,801 (69.1%) of all patients analyzed. Although only 71 (3%) of 2,407 patients had migraine diagnosed in an ophthalmology clinic as the cause of eye pain, migraine was the predominant cause of eye pain in the neurology clinics (100/196; 51%). Other causes of eye pain in the neurology clinics included optic neuritis (44 patients), trigeminal neuralgia, and other cranial nerve disorders (8 patients). CONCLUSIONS: Eye pain may be associated with a number of different causes, some benign and others sight- or life-threatening. Because patients with eye pain may present to either a neurology or an ophthalmology clinic and because the causes of eye pain may be primarily ophthalmic or neurologic, the diagnosis and management of these patients often requires collaboration and consultation between the 2 specialties.

Natural history of primary paediatric optic nerve sheath meningioma: case series and review.

PURPOSE: To study the natural history, clinical and radiological characteristics of primary paediatric optic nerve sheath meningioma (PPONSM). METHODS: Retrospective study of eight paediatric patients who were treated between 1994 and 2016 at the University Hospital Zurich, Switzerland and the Royal Adelaide Hospital, Australia. Clinical records and imaging studies were reviewed. RESULTS: The mean age at presentation was 11 years (range: 6-17 years). There were six female patients and two male patients. 2/8 patients had associated neurofibromatosis type 2. Patients were followed up for 71-297 months (mean 156±70 months). 6/8 patients were observed through the course of their disease and 2/8 patients were treated with radiotherapy. 2/8 patients who were observed had minimal change in vision and did not experience tumour growth after long-term follow-up. CONCLUSIONS: This is the largest PPONSM case series with long-term data on patients treated conservatively. We highlight that a small subset of these tumours are indolent and can be managed using observation alone.

Ocular vestibular evoked myogenic potentials as a test for myasthenia gravis.

OBJECTIVE: To explore whether ocular vestibular evoked myogenic potentials (oVEMP) can be used to detect a decrement in the extraocular muscle activity of patients with myasthenia gravis (MG). METHODS: Twenty-seven patients with MG, including 13 with isolated ocular and 14 with generalized MG, and 28 healthy controls participated. We applied repetitive vibration stimuli to the forehead and recorded the activity of the inferior oblique muscle with 2 surface electrodes placed beneath the eyes. To identify the oVEMP parameters with the highest sensitivity and specificity, we evaluated the decrement over 10 stimulus repetitions at 3 different repetition rates (3 Hz, 10 Hz, and 20 Hz). RESULTS: Repetitive stimulation at 20 Hz yielded the best differentiation between patients with MG and controls with a sensitivity of 89% and a specificity of 64% when using a unilateral decrement of ≥15.2% as cutoff. When using a bilateral decrement of ≥20.4% instead, oVEMP allowed differentiation of MG from healthy controls with 100% specificity, but slightly reduced sensitivity of 63%. For both cutoffs, sensitivity was similar in isolated ocular and generalized MG. CONCLUSION: Our study demonstrates that the presence of an oVEMP decrement is a sensitive and specific marker for MG. This test allows direct and noninvasive examination of extraocular muscle activity, with similarly good diagnostic accuracy in ocular and generalized MG. Thus, oVEMP represents a promising diagnostic tool for MG. CLASSIFICATION OF EVIDENCE: This study provides Class III evidence that oVEMP testing accurately identifies patients with MG with ocular symptoms (sensitivity 89%, specificity 64%).

Malignant optic glioma - the spectrum of disease in a case series.

PURPOSE: Malignant optic glioma of adulthood is a rare, invasive neoplasm of the anterior visual pathway with 66 cases reported in the literature. It presents as anaplastic astrocytoma (WHO grade III) or glioblastoma (WHO grade IV). The present case series covers the spectrum of disease manifestations, discusses neuroradiological findings, and reviews the current literature. METHODS: Retrospective case series of five patients from three tertiary referral centers and literature review. RESULTS: Visual loss with or without pain was the presenting symptom in all patients (two women, three men). Two patients were initially misdiagnosed as optic neuritis, and one patient as atypical non-arteritic anterior ischemic optic neuropathy (NAION). A neoplastic disease was suspected in the two remaining patients. MRI features were iso- to hypointensity on T1-weighted native images, contrast enhancement, and hyperintensity on T2-weighted images. Biopsy was generally diagnostic; however, one patient required two biopsies for diagnosis. The series includes an exceptional case of intraocular tumor extension and vitreous spread. The disease was lethal within one to two years in all patients. CONCLUSIONS: Malignant optic glioma is a diagnostic challenge and remains a devastating and lethal disease. Advances in the understanding of tumor biology have yet failed to translate into effective treatment regimens.

Effects of extraocular muscle surgery in children with monocular blindness and bilateral nystagmus.

BACKGROUND: Monocular infantile blindness may be associated with bilateral horizontal nystagmus, a subtype of fusion maldevelopment nystagmus syndrome (FMNS). Patients often adopt a significant anomalous head posture (AHP) towards the fixing eye in order to dampen the nystagmus. This clinical entity has also been reported as unilateral Ciancia syndrome. The aim of the study was to ascertain the clinical features and surgical outcome of patients with FMNS with infantile unilateral visual loss. METHODS: In this retrospective case series, nine consecutive patients with FMNS with infantile unilateral visual loss underwent strabismus surgery to correct an AHP and/or improve ocular alignment. Outcome measures included amount of AHP and deviation at last follow-up. RESULTS: Eye muscle surgery according to the principles of Kestenbaum resulted in a marked reduction or elimination of the AHP. On average, a reduction of AHP of 1.3°/mm was achieved by predominantly performing combined horizontal recess-resect surgery in the intact eye. In cases of existing esotropia (ET) this procedure also markedly reduced the angle of deviation. A dosage calculation of 3 prism diopters/mm was established. CONCLUSIONS: We advocate a tailored surgical approach in FMNS with infantile unilateral visual loss. In typical patients who adopt a significant AHP accompanied by a large ET, we suggest an initial combined recess-resect surgery in the intact eye. This procedure regularly led to a marked reduction of the head turn and ET. In patients without significant strabismus, a full Kestenbaum procedure was successful, while ET in a patient with a minor AHP was corrected by performing a bimedial recession.

Prediction of the clinical outcome of cavernous sinus lesions in children.

To identify predictive findings in children with nontraumatic acquired cavernous sinus lesions, a retrospective study of the clinical course of 4 of our own patients and 17 more children found in an extensive literature search was performed. Mean age was 8.7 years. Malignancy was found in 11 of 21 children (6 female, 15 male). Of these 21 children, 9 were cured, 8 are either in remission or their course is unknown, and 4 died. Eight of the nine cured patients were diagnosed as having had Tolosa-Hunt syndrome. Of the four deceased children, three had a very short course (1 to 4 months) and were diagnosed with malignant lymphoma (n = 2) and rhabdomyosarcoma (n = 1). One patient died from a brain tumor other than the initially diagnosed T-cell lymphoma in the cavernous sinus after a follow-up of 8 years. MRI should be the preferred imaging technique-even if it is not conclusive in many cases-and every possible diagnostic effort should be made before using corticosteroids. No clinical or radiological signs other than rapid deterioration seem to be predictable of a malignant cavernous sinus lesion with poor outcome. Thus, close follow-up is recommended in children with signs and symptoms indicative of an acquired lesion in the cavernous sinus.

Vitreous deformation during eye movement.

Retinal detachment results in visual loss and requires surgical treatment. The risk of retinal detachment depends, among other factors, on the vitreous rheology, which varies with age. To date, the viscoelasticity of the vitreous body has only been measured in cadaver eyes. However, the ex vivo and in vivo viscoelasticity may differ as a result of the effect of intravitreal membranes. Therefore, an MRI method and appropriate postprocessing tools were developed to determine the vitreous deformation and viscoelastic properties in the eyes of living humans. Nineteen subjects (eight women and 11 men; mean age, 33 years; age range, 14-62 years) gazed at a horizontal sinusoidal moving target during the segmented acquisition of complementary spatial modulation of magnetization images. The center of the lens and the scleral insertion of the optic nerve defined the imaging plane. The vitreous deformation was tracked with a dedicated algorithm and fitted with the commonly used viscoelastic model to determine the model parameters: the modified Womersley number a and the phase angle b. The vitreous deformation was successfully quantified in all 17 volunteers having a monophasic vitreous. The mean and standard deviation of the model parameters were determined to be 5.5 ± 1.3 for a and -2.3 ± 0.2 for b. The correlation coefficient (-0.76) between a and b was significant. At the eye movement frequency used, the mean storage and loss moduli of the vitreous were around 3 ± 1 hPa. For two subjects, the vitreous deformation was clearly polyphasic: some compartments of the vitreous were gel-like and others were liquefied. The borders of these compartments corresponded to reported intravitreal membrane patterns. Thus, the deformation of the vitreous can now be determined in situ, leaving the structure of the intravitreal membranes intact. Their effect on vitreous dynamics challenges actual vitreous viscoelastic models. The determination of the vitreous deformation will aid in the quantification of local vitreous stresses and their correlation with retinal detachment.

Retinal disorders.

The retina represents part of the central nervous system (CNS). After modifying the neural signal, the axon of the last neuron enters the optic nerve and leaves the eye. In most cases of retinal disease leading to visual loss, the diagnosis will be made by an ophthalmologist after examining the ocular fundus. Some retinal disorders, however, might not be detectable at the time of examination. Those patients will be referred to a neurologist for "unexplained visual loss" when suspecting a lesion behind the optic nerve. Moreover, knowledge of potential retinal abnormalities is useful for the neurologist when seeing patients with CNS disease, which can manifest itself also in the retina. This chapter aims to give an overview about retinal disorders causing no or only few retinal abnormalities, those associated with neurological diseases, as well as the most important retinal diseases involving the tissues of the ocular fundus (vitreous body, retina, pigment epithelium, and the choroid). The most frequently used examination techniques and diagnostic tools are described. Tumors, vascular disease, especially diabetic retinopathy, age-related macular degeneration, chorioretinal inflammatory and toxic disorders, paraneoplastic retinopathies, inherited retinal dystrophies, and retinal involvement in CNS disease such as phakomatoses and multiple sclerosis are discussed.

Pituitary apoplexy: re-evaluation of risk factors for bleeding into pituitary adenomas and impact on outcome.

OBJECTIVE: To assess frequency, symptoms and outcome of pituitary apoplexy (PA) among pituitary adenoma patients, to gain better insight into risk factors for bleeding into pituitary adenoma and to estimate the sequelae of PA by means of a matched control group. METHOD: By reviewing charts of 574 patients with pituitary adenoma, we analysed incidence, symptoms and outcome of PA and potential risk factors for developing PA by means of a control group (patients with pituitary adenoma without PA). RESULTS: In total, 42 suffered from PA, all had macroadenomas; 30/217 male (14%) and 12/179 female (7%) macroadenoma patients, 32/194 patients with clinically non-functioning (16.5%) and 10/202 with clinically active (5.0%) macroadenoma were affected. Antithrombotic therapy predisposed patients to PA (P=0.026), diabetes mellitus and hypertension did not (P=1.00). Patients with PA and pituitary adenoma patients without PA had similar frequencies of hypopituitarism (45 vs 48%, P>0.05) and visual field defects (38 vs 55%, P>0.05), but ophthalmoplegia was significantly more common (76 vs 5%, P<0.001) in patients with PA. Nearly all patients were treated by surgery; most recovered from ophthalmoplegia, whereas visual function improved only moderately. Endocrine outcome was worse in patients with PA than in patients without PA. CONCLUSIONS: Male sex and characteristics of the adenoma itself (especially tumour size and tumour type) rather than patient's cardiovascular risk factors such as diabetes and hypertension seem to predispose to PA; antithrombotic therapy may also be important.

Long-term results of vertical rectus muscle transposition and botulinum toxin for sixth nerve palsy.

PURPOSE: To report the long-term outcome of full-tendon vertical rectus muscle transposition combined with chemodenervation of the ipsilateral medial rectus muscle for acquired chronic sixth (abducens) nerve palsy. METHODS: A retrospective study of all patients treated for severe abduction deficit with transposition plus botulinum toxin over the course of 11 years. Minimum follow-up was 12 months. Main outcome measures were the surgical result and its stability. RESULTS: A total of 22 patients were included. Mean age at the time of surgery was 41.7 ± 19.1 years (range, 4.5-69). The etiologies for the palsy were head trauma (11), tumor (10), and idiopathic (1). Mean follow-up time was 44.2 ± 37.4 months (range, 12-123). The average distance deviation was 38.1(Δ) ± 11.6(Δ) preoperatively, 4.0(Δ) ± 16.1(Δ) 3 months after the operation (p = 0.0004), and 7.9(Δ) ± 8.8(Δ) at 12 months (p = 0.0003), with no subsequent change. At the final examination, on average 44.2 months after the operation, 13 patients (59%) were within 10(Δ) of alignment, 2 (1%) were overcorrected, and 7 (32%) had vertical deviations. The majority of patients (73%) had no double vision in the primary position. No patient developed anterior segment ischemia. CONCLUSIONS: Vertical rectus muscle transposition combined with intraoperative botulinum toxin injection into the ipsilateral medial rectus muscle improved alignment in patients with complete chronic sixth nerve palsy. While the effects of treatment may have diminished slightly during the first year after surgery, they remained stable thereafter.

Clinical course and prognosis of trochlear nerve schwannomas.

PURPOSE: To delineate the disease course and prognosis of patients with mass lesions of the fourth nerve presumed to be schwannomas. DESIGN: Nonrandomized retrospective case series. PARTICIPANTS: Thirty-seven consecutive cases of presumed trochlear nerve schwannoma from 9 tertiary university neuro-ophthalmology centers. METHODS: Cases were collected, and their clinical characteristics on presentation and follow-up are described. Inclusion criteria were brain magnetic resonance imaging (MRI) with a lesion suggestive of a schwannoma along the course of the fourth nerve. Exclusion criteria were other causes of fourth nerve palsy, such as congenital, traumatic or microvascular; normal (or lack of) initial brain MRI; lack of adequate clinical information; and disappearance of the lesion on subsequent follow-up brain MRI. MAIN OUTCOME MEASURES: Demographics of patients, presence of neurofibromatosis, symptoms on presentation, vertical deviation, lesion size (on presentation and follow-up), length of follow-up, and outcomes of treatment for lesions or diplopia. RESULTS: Seven patients were excluded and of the 30 patients included in our series, patients were predominantly male (77%) with a mean age of 51 years (range 9-102 years). In contrast with prior case reports, almost all of our cases had a fourth nerve palsy on presentation (29/30), often isolated. Mean follow-up was 3.1 years (range 0.2 months to 11.1 years). There was no significant difference between initial and follow-up lesion size (4.4 vs. 5 mm) for patients who did not receive treatment of lesions (P = 0.36). Only 3 patients underwent neurosurgical resection and an additional patient received gamma-knife radiotherapy. The majority of patients (24/30) did not pursue strabismus surgery for vertical diplopia. CONCLUSIONS: Patients with isolated fourth nerve palsy and small lesions of the fourth nerve have a good prognosis and should be followed with serial MRI scans without neurosurgical intervention unless they develop signs of brain stem compression. Most patients with diplopia and benign fourth nerve lesions typical of trochlear nerve schwannoma can adapt with either prism spectacles or no treatment at all, although strabismus surgery can be successful.

[Neuro-ophthalmology essentials]. / Wissenswertes aus der Neuro-Ophthalmologie.

Neuro-Ophthalmology is an interdisciplinary field dealing with afferent and efferent disorders of the visual system caused by nervous system lesions. Modern imaging methods and their broad availability have greatly facilitated our diagnostic capacity. Despite this impressive technological progress thorough knowledge of clinical signs and symptoms still remains crucial in order to achieve a straight forward optimal patient care. Above several relevant neuro-ophthalmic disorders are described. Emphasis is given on correct initial management and indication for referral to a specialized institution.