Central Diabetes Insipidus Associated with Orbital Apex Syndrome.

Orbital apex syndrome (OAS) manifests as multiple cranial nerve palsies caused by an abnormal nerve response to inflammation or other processes. Central diabetes insipidus (CDI) is characterized by deficient synthesis or secretion of antidiuretic hormone. A 62-year-old woman underwent myringotomy for otitis media with effusion. Two months after the procedure, symptoms of hearing loss had not improved, and she underwent left tympanoplasty and mastoidectomy. After surgery, she presented with left ocular pain and visual loss. Neurologic examination revealed ptosis, total ophthalmoplegia, and a relative afferent pupillary defect on the left eye. Magnetic resonance imaging showed an asymmetric contrast-enhancing lesion in the left orbital apex and left cavernous sinus, with adjacent dural thickening and enhancement. OAS was diagnosed, and steroid treatment was started. During the regular follow-up period, she reported polyuria, and CDI was diagnosed. Treatment with intranasal desmopressin 10 µg twice daily was started, and symptoms greatly improved. The mechanism underlying the association of CDI with OAS is unclear, and further research is needed. The present case suggests that polyuria in OAS should alert neurologists and ophthalmologists to possible CDI.

Laminin-521 Protein Therapy for Glomerular Basement Membrane and Podocyte Abnormalities in a Model of Pierson Syndrome.

Background Laminin α5ß2γ1 (LM-521) is a major component of the GBM. Mutations in LAMB2 that prevent LM-521 synthesis and/or secretion cause Pierson syndrome, a rare congenital nephrotic syndrome with diffuse mesangial sclerosis and ocular and neurologic defects. Because the GBM is uniquely accessible to plasma, which permeates endothelial cell fenestrae, we hypothesized that intravenous delivery of LM-521 could replace the missing LM-521 in the GBM of Lamb2 mutant mice and restore glomerular permselectivity.Methods We injected human LM-521 (hLM-521), a macromolecule of approximately 800 kD, into the retro-orbital sinus of Lamb2-/- pups daily. Deposition of hLM-521 into the GBM was investigated by fluorescence microscopy. We assayed the effects of hLM-521 on glomerular permselectivity by urinalysis and the effects on podocytes by desmin immunostaining and ultrastructural analysis of podocyte architecture.Results Injected hLM-521 rapidly and stably accumulated in the GBM of all glomeruli. Super-resolution imaging showed that hLM-521 accumulated in the correct orientation in the GBM, primarily on the endothelial aspect. Treatment with hLM-521 greatly reduced the expression of the podocyte injury marker desmin and attenuated the foot process effacement observed in untreated pups. Moreover, treatment with hLM-521 delayed the onset of proteinuria but did not prevent nephrotic syndrome, perhaps due to its absence from the podocyte aspect of the GBM.Conclusions These studies show that GBM composition and function can be altered in vivovia vascular delivery of even very large proteins, which may advance therapeutic options for patients with abnormal GBM composition, whether genetic or acquired.

Masquerading optic neuritis.

A 54-year-old woman presented to the ophthalmology emergency department with a 10-day history of blurred vision. The best-corrected visual acuities and Ishihara colour vision were bilaterally reduced with a left relative afferent pupillary defect. Slit-lamp examination was otherwise normal. Retrobulbar optic neuritis (ON) was presumed as she had suffered with this previously and was known to have multiple sclerosis (MS). She was recalled the following week for visual field (VF) testing, which was not available at the time of presentation. VFs demonstrated an incongruous left homonymous hemianopia. She was immediately referred to the medical team to investigate for a stroke, which was subsequently excluded. Thereafter, a trial of pulsed methylprednisolone was commenced, resulting in near complete resolution of the hemianopia. This case demonstrates not only the importance of VF testing, but also how ON may present with any field defect, including mimicking a stroke, a point valuable to ophthalmologists and medics alike.

Evidence of specific cognitive deficits in patients with chronic low back pain under long-term substitution treatment of opioids.

BACKGROUND: There is a growing number of patients worldwide being treated with long-term opioids for chronic non-cancer pain, although there is limited evidence for their effectiveness in improving pain and function. Opioid-use related adverse effects, especially in cognitive functioning in these patients, are rarely evaluated. OBJECTIVES: The present study investigated the cognitive functions of patients with chronic back pain who underwent long-term opioid treatment in comparison with those patients without opioid usage and healthy controls. STUDY DESIGN: A prospective, nonrandomized, cross-sectional study. SETTING: Multidisciplinary pain management clinic, specialty referral center, University Hospital in Germany. METHODS: In a prospective cross-sectional design, 37 patients with chronic back pain who underwent long-term opioid therapy (OP) were compared with 33 patients with chronic back pain without opioid therapy (NO) and 25 healthy controls (HC). Assessment of primary outcome included cognitive function such as information processing speed, choice reaction time, pattern recognition memory, and executive function. Other data included pain, back function, depression and anxiety, use of medication, and education status. The relationship between cognitive functions and anxiety/depression was analysed. RESULTS: Both patient groups needed significantly longer time in information processing when compared to HC (Group 1: 41.87 ± 20.47 Group 2: 38.29 ± 19.99 Group 3: 30.25 ± 14.19). Additionally, OP patients had significantly reduced spatial memory capacity, flexibility for concept change, and impaired performance in working memory assessment compared to NO patients and HC. The impaired cognitive outcomes were significantly associated with pain intensity, depression scores, and medication use. LIMITATIONS: Limitations include small number of patients with heterogeneous opioid therapy and the nonrandomized observational nature of the study. CONCLUSIONS: Our findings give a differential view into the cognitive changes from chronic back pain with and without long-term opioids treatment. Chronic back pain itself impairs some distinct cognitive functions. Long-term opioid therapy adds further cognitive impairment.

Fibrin membrane pupillary-block glaucoma after uneventful cataract surgery treated with intracameral tissue plasminogen activator: a case report.

BACKGROUND: Fibrin pupillary-block glaucoma is a rare complication after cataract surgery. The treatment for this condition is still controversial, since Nd:YAG laser fibrin membranotomy tends to reocclude and laser peripheral iridotomy entails the risk of damaging the corneal endothelium in the presence of corneal edema associated with elevated intraocular pressure. CASE PRESENTATION: A 62-year-old man with diabetes mellitus developed acute elevation of intraocular pressure with a shallow anterior chamber five days after uneventful cataract surgery. Initially, slit lamp examination provided only limited information due to severe corneal edema. After resolution of corneal edema with systemic glaucoma therapy, a complete fibrin membrane was observed across the pupil by slit lamp examination. Anterior segment optic coherence tomography clearly revealed a thin fibrin membrane covering the entire pupillary space, a shallow anterior chamber, and a deep posterior chamber. The intraocular lens was not observed by anterior segment optic coherence tomography. In contrast, ultrasound biomicroscopy, which has superior penetration depth, was able to visualize the intraocular lens deep in the posterior chamber. Injection of tissue plasminogen activator into the anterior chamber resulted in complete fibrinolysis and released the pupillary block. CONCLUSION: This case suggests that ocular anterior segment imaging modalities, especially ultrasound biomicroscopy, serve as powerful diagnostic tools to identify mechanisms of acute angle closure glaucoma, which is often accompanied by poor intraocular visibility. This is the first reported case of fibrin pupillary-block glaucoma after cataract surgery successfully treated with intracameral tissue plasminogen activator.

Bilateral, simultaneous, uveitis-associated pupillary membranes.

A 48-year-old Caucasian man with an established diagnosis of pathological ankylosing spondylitis with cervical spinal fusion and a lengthy history of acute, recurrent, anterior uveitis presented with decreased vision in both eyes. Biomicroscopic examination revealed bilateral inflammatory pupillary membranes associated with anterior uveitis to be the source of the visual compromise. Aggressive topical anti-inflammatory and mydriatic therapy did not break the pupillary membranes and the patient ultimately underwent surgical resection of the membranes in both eyes. Vision returned to normal in one eye and was only slightly reduced in the fellow eye after a prolonged post-operative period involving multiple ophthalmic surgical procedures. This is the first reported case of bilateral, simultaneous uveitis-associated pupillary membranes.

Ocular damage secondary to intense pulse light therapy to the face.

PURPOSE: To promote awareness and prevention of ocular damage that can occur during Intense Pulsed Light (IPL) treatments of the periocular areas. METHODS: A retrospective chart review was conducted of 2 cases involving ocular damage following IPL procedures that were treated at Bascom Palmer Eye Institute for ocular complications. Routine data were collected during ophthalmic examinations. RESULTS: Case 1: A 36-year-old female presented with eye pain, marked pupillary constriction, and anterior uveitis an hour after receiving IPL treatment to the face. Within 1 month, the damage had progressed to posterior synechiae and iris transillumination defects. She continues to have pain and severe photophobia due to permanent iris atrophy and transillumination that have persisted for years. Case 2: A 27-year-old female presented with severe eye pain, vision disturbances, pupillary defects, and anterior uveitis 3 days after IPL of an eyelid freckle. At 2 months follow up, the iris and pupillary defects remain permanent. The patient continues to suffer from photophobia and pain. CONCLUSIONS: The pigmented iris absorbs light in the same wavelength range of IPL, thus remaining vulnerable to IPL exposure, especially when applied to the periocular area. The fact that IPL is not a laser may give people a false sense of security regarding damage to the eye. The cases presented give evidence that periorbital IPL treatment may permanently affect pigmented intraocular structures. It is imperative for treating physicians to be aware of these hazards and to use appropriate eye protection to prevent ocular damage.

Ocular complications of laser-assisted eyebrow epilation.

PURPOSE: To report a series of patients with ocular complications associated with laser-assisted eyebrow hair removal. PATIENTS AND METHODS: Case reports of three patients with eye pain and photophobia following laser epilation of the eyebrow region. The eye examination included visual acuity, slit-lamp examination, tonometry and fundoscopy. The follow-up period was 3 months. RESULTS: Each patient had conjunctival hyperaemia in one or both eyes and anterior chamber pigmentary cells. One patient presented with posterior synechiae, which did not respond to treatment. CONCLUSIONS: Laser epilation of the eyebrows may result in anterior uveitis as well as irreversible damage to the iris.

Videonystagmography to assess eyelid dynamic disorders.

OBJECTIVES: Apply a videonystagmography (VNG) device to study indirectly blinking in patients with essential blepharospasm. PATIENTS AND METHODS: A VNG device was used to study the pupillary occlusion. It was recorded and analyzed as indirect parameters of blinking in 23 essential blepharospasm on day 0, month 1, and month 3 following botulinum toxin injection using VNG. RESULTS: Botulinum toxin significantly reduced pupillary occlusions (PO) frequency (9.6 PO/min on D0 and 4.7 PO/min on M1 (p = 0.004), and average PO time (0.95 sec on D0 and 0.58 sec on M1 (p = 0.03) for PO higher than 0.3 sec. On the other hand, it does not have any action on frequency (32.4 PO/min on D0 and 31.3 PO/min on M1 (p = 0.7) and average PO time (0.16 sec on D0 and 0.15 sec on M1 (p = 0.4) of PO lower than 0.3 sec. CONCLUSION: Video acquisition computer-assisted makes it possible to measure blinking parameters indirectly in real time and to assess essential blepharospasm objectively. The protocol analysis of PO is attractive for indirect analysis of blinking.

Hyoscine skin patches for drooling dilate pupils and impair accommodation: spectacle correction for photophobia and blurred vision may be warranted.

Hyoscine skin patches diminish salivation by their anticholinergic action. The aim of reporting this case series is to present the ophthalmic side effects in children, and to highlight the precautions to take. Five children (two males, three females; age range 8-18y) with quadraplegic cerebral palsy (Gross Motor Function Classification System Level V) and profound intellectual impairment, wearing hyoscine skin patches to control excessive salivation, were examined. Binocular visual acuity, pupil reaction, pupil diameter, and dynamic retinoscopy were recorded before and after instilling guttae cyclopentolate 1%. The accommodative responses were compared with the expected norms. Spectacle correction was provided for refractive error and to compensate for lack of accommodation. Tinted lenses were provided for photophobia. Visual assessment was repeated 6 months later. All children had dilated pupils with a mean diameter of 7.8mm (range 7-9mm) before instilling guttae cyclopentolate. Mean pupil constriction to bright light was only 1.8mm (range 1-2mm). Mean resting accommodation was 0.45 dioptres (D; range 0-1D) and no accommodative responses were detected on dynamic retinoscopy. Three children were photophobic. Visual behaviour was seen to improve after the use of appropriate spectacles. Photophobia was relieved by tinted lenses. The anticholinergic effects of hyoscine skin patches can cause photophobia and impair vision due to pupil dilatation and paralysis of accommodation. Appropriate spectacles are recommended.

Marcus Gunn Jaw-Winking synkinesis in a neonate.

This report describes a 9-day-old female infant admitted for hypocalcemic seizures. The hypocalcemia was treated, but the infant was noted to have vesico-ureteral reflux and a mild ptosis of her left eye with rhythmic blinking during bottle feeding. The eye findings were subsequently diagnosed as Marcus Gunn jaw-winking Synkinesis.

Interdisciplinary treatment and ophthalmological findings in Parry-Romberg syndrome.

Parry-Romberg syndrome is a rare pathologic process, characterized by progressive hemifacial atrophy. A case of Parry-Romberg syndrome with ocular involvement is reported. A 27-year-old male patient with Parry-Romberg syndrome was interdisciplinary investigated and treated. For reconstruction of hemifacial soft tissues a free vascular parascapular graft was performed. Opthalmological findings included an evident enophthalmos and an eyelid lag with keratopathy and epiphora. Furthermore pupillary disturbances and endothelial precipitates were detectable. Complete fundus examination showed a unilateral optic disc swelling, central vitreous opacities and peripheral pigmentary disturbances. In the presented case of Parry-Romberg syndrome a rare association to ophthalmological involvement could be found. Beside the enophthalmos and eyelid alterations, also a panuveitis with papillitis should be treated by steroid therapy.