Oral methylphenidate for the treatment of refractory facial dystonias.

Oral methylphenidate (Ritalin, Novartis) has been reported to alleviate symptoms of benign essential blepharospasm in an off-label application. This series presents 3 patients with refractory periorbital and facial dystonias, including blepharospasm, apraxia of eyelid opening, and oromandibular dystonia unresponsive to standard treatments who experienced a response to oral methylphenidate therapy. While the mechanisms for facial dystonias have not been elucidated, there is evidence to suggest that they are on the spectrum with Parkinson disease. Given the role of dopamine loss in the pathogenesis of Parkinson, the authors' speculate that methylphenidate may be acting on the pathway directly involved in facial dystonias. To the authors' knowledge, this is the first report of a case of successful treatment of blepharospasm refractory to upper eyelid myectomy with methylphenidate monotherapy.

The last ride of Henry II of France: orbital injury and a king's demise.

Jousting was a popular pastime for royalty in the Renaissance era. Injuries were common, and the eye was particularly at risk from the splinters of the wooden lance. On June 30, 1559, Henry II of France participated in a jousting tournament to celebrate two royal weddings. In the third match, Gabriel de Montgomery struck Henry on the right shoulder and the lance splintered, sending wooden shards into his face and right orbit. Despite being cared for by the prominent physicians Ambroise Paré and Andreas Vesalius, the king died 10 days later and was found to have a cerebral abscess. The wound was not explored immediately after the injury; nevertheless, wooden foreign bodies were discovered in the orbit at the time of autopsy. The dura had not been violated, suggesting that an infection may have traveled from the orbit into the brain. Nostradamus and Luca Guarico, the astrologer to the Medici family, had prophesied the death of Henry II of France, but he ignored their warnings and thus changed the course of history in Renaissance Europe.

Ocular toxicity in BRAF mutant cutaneous melanoma patients treated with vemurafenib.

PURPOSE: To determine the frequency of ocular adverse effects associated with vemurafenib (PLX4032) treatment for metastatic cutaneous melanoma. DESIGN: Retrospective review of the clinical study reports from the clinical pharmacology, phase 1, phase 2, and phase 3 trials of vemurafenib. METHODS: The vemurafenib clinical trials were a multicenter series involving adult patients with histologically confirmed, BRAF(V600) mutation-positive, unresectable, stage IIIC or IV melanoma. A total of 855 patients were enrolled in the trials: 568 patients were treated with vemurafenib and 287 patients were treated with dacarbazine. RESULTS: Among the 568 patients treated with vemurafenib, ocular adverse effects developed in 22% (95% confidence interval [CI], 18.5-25.6). The most common ocular diagnosis was uveitis (4.0%; 95% CI, 2.6-6.0), followed by conjunctivitis (2.8%; 95% CI, 1.6-4.5) and dry eyes (2.0%; 95% CI, 1.1-3.7). All were successfully managed while vemurafenib therapy was continued. CONCLUSIONS: Ocular adverse events and symptoms may be seen in more than one-fifth of patients being treated with vemurafenib. However, vemurafenib can be continued while the ocular symptoms are being managed. The pathogenesis of ocular symptoms in this patient population is unclear; additional studies are necessary.

Bilateral lacrimal gland disease: clinical features of 97 cases.

OBJECTIVE: Bilateral lacrimal gland (LG) disease is a unique presentation that can result from varied causes. We reviewed the diagnoses, clinical features, and outcomes of 97 patients with this entity. DESIGN: Case series. PARTICIPANTS: Ninety-seven patients with bilateral LG disease. METHODS: Retrospective review and statistical analysis using analysis of variance and the Fisher exact test. MAIN OUTCOME MEASURES: Patient demographics, clinical features, diagnostic testing, diagnosis, and treatment. RESULTS: Patient age ranging from 8 to 84 years (mean, 46 years). The predominant gender was female (77%), and race included black (49%), white (38%), and Hispanic (12%) patients. Diagnoses fell into 4 categories: inflammatory (n = 51; 53%), structural (n = 20; 21%), lymphoproliferative (n = 19; 20%), and uncommon (n = 7; 7%) entities. The most common diagnoses included idiopathic orbital inflammation (IOI; n = 29; 30%), sarcoidosis (n = 19; 20%), prolapsed LG (n = 15; 15%), lymphoma (n = 11; 11%), lymphoid hyperplasia (n = 8; 8%), and dacryops (n = 5; 5%). Inflammatory conditions were more likely in younger patients (P<0.05) and in those with pain (P<0.001) and mechanical blepharoptosis (P<0.01) at presentation, whereas lymphoma was more common in older patients (P<0.001) without active signs of inflammation at presentation. Black patients were more likely to have sarcoidosis (P<0.01). Laboratory results showed high angiotensin converting enzyme level being significantly more likely in patients with sarcoidosis (P<0.05). However, sensitivity was limited to 45%, with 25% of patients diagnosed with IOI also demonstrating positive results. Corticosteroid therapy was the treatment of choice in 38 cases, corresponding to resolution of symptoms in 29% and improvement in an additional 32%. Overall, chronic underlying disease was found in 71% of patients, among whom 26% achieved a disease-free state, whereas 3% succumbed to their underlying disease. CONCLUSIONS: The cause of bilateral lacrimal gland disease most commonly was inflammatory, followed by structural and lymphoproliferative. Patient characteristics and clinical presentations were key features distinguishing between competing possibilities. Despite local control with corticosteroids or radiotherapy, underlying disease continued in 71% of patients and led to death in 3%.

Orbital inflammation after dental procedures.

This study reports 3 cases of acute orbital inflammation that occurred within 3 weeks of various dental procedures and offers a possible mechanism as to their cause. The charts of 3 patients were retrospectively examined. Clinical notes, laboratory testing, and imaging studies were reviewed. The cases involved a 36-year old woman, a 61-year-old woman, and a 44-year-old woman who developed acute dacryoadenitis after tooth extraction in the former case and after routine dental cleaning in the latter 2. All cases were initially treated with an oral steroid taper over 6 to 8 weeks. The first 2 cases resolved promptly and have remained quiescent. The last individual had recurrent symptoms prompting lacrimal gland biopsy that demonstrated chronic, nongranulomatous inflammation without monoclonality. The patient subsequently responded to periorbital steroid injection only to have a recurrent bout of inflammation after repeat dental cleaning. Another periorbital steroid injection resulted in resolution of inflammation. The authors propose that a subset of acute orbital inflammation may represent an autoimmune response triggered by dental manipulation. These cases are suggestive of an atypical variant of noninfectious, microbe-induced inflammation.

Blepharospasm and apraxia of eyelid opening associated with anti-Hu paraneoplastic antibodies: a case report.

PURPOSE: To report a case of blepharospasm associated with anti-Hu paraneoplastic antibodies that was treated successfully with botulinum toxin A. DESIGN: Case report. PARTICIPANTS: A 57-year-old man had altered mental status and a 20-pound weight loss at presentation. Evaluation revealed an occult small-cell lung cancer. Despite initiating appropriate chemotherapy, his mental status worsened and over the course of several weeks, he was unable to open his eyes because of forceful orbicularis contractions. Neuroimaging and cerebrospinal fluid studies found no evidence of intracranial metastases. However, his paraneoplastic panel was positive for anti-Hu antibodies. He was diagnosed with paraneoplastic encephalitis and blepharospasm. INTERVENTION: Intravenous Solu-Medrol (Pharmacia & Upjohn Co, Bridgewater, NJ) and periocular injections of botulinum toxin A. MAIN OUTCOME MEASURES: Ocular disease control. RESULTS: Intravenous Solu-Medrol improved his mental status, but did not change his ocular symptoms. Subsequent botulinum toxin A injections allowed spontaneous eyelid opening. CONCLUSIONS: Although paraneoplastic blepharospasm is rare, it is an important diagnosis to be aware of because paraneoplastic disorders often herald an occult tumor. This is the only case of paraneoplastic blepharospasm that the authors know of that was the result of anti-Hu antibodies as well as the only case that was treated with botulinum toxin A.

Comparison of lateral and medial orbital decompression for the treatment of compressive optic neuropathy in thyroid eye disease.

PURPOSE: To compare the efficacy of lateral orbital decompression with medial orbital decompression for the treatment of compressive optic neuropathy (CON) caused by thyroid eye disease (TED). METHODS: A retrospective review of all patients undergoing orbital decompression for TED-associated CON between 2003 and 2008 was conducted. Clinical outcome measures included visual acuity, mean deviation on Humphrey visual field, Ishihara color plate testing, afferent pupillary defect, and Hertel exophthalmometry. A composite CON score was also calculated for each patient based on the 3 visual outcome measures, with a higher CON score reflecting greater disease severity. RESULTS: Twenty-eight eyes of 17 patients were included in the study. Ten orbits underwent lateral wall decompression, and 18 orbits underwent medial decompression. Both groups showed a significant postoperative improvement in visual acuity, Humphrey visual field testing, and color testing at 3 months following the initial surgery. The composite CON score improved 9.04 ± 9.97 points after lateral decompression (p = 0.02) and 9.03 ± 10.84 points after medial decompression (p = 0.003). There was no significant difference in the degree of improvement in any of the visual outcome measures between the lateral and medial groups. There was a statistically significant difference in the amount of proptosis reduction resulting from lateral decompression compared with medial decompression (6.3 versus 3.1 mm, respectively, p < 0.0001). CONCLUSIONS: Lateral orbital decompression and medial orbital decompression are both efficacious for the treatment of TED-associated CON. Lateral decompression also results in a greater proptosis reduction than medial decompression.

Incidence of diffuse lamellar keratitis after LASIK with 15 KHz, 30 KHz, and 60 KHz femtosecond laser flap creation.

PURPOSE: To compare the incidence of diffuse lamellar keratitis (DLK) after laser in situ keratomileusis (LASIK) with flap creation using the 15 kHz (FS15), 30 kHz (FS30), or 60 kHz (FS60) femtosecond laser. SETTING: University-based academic practice, Ann Arbor, Michigan, USA. DESIGN: Retrospective comparative case series. METHODS: Consecutive myopic LASIK cases performed between January 1, 2005, and June 1, 2007, using the IntraLase FS15, FS30, or FS60 femtosecond laser for flap creation were reviewed. Preoperative clinical characteristics, treatment parameters, and intraoperative and postoperative complications were recorded. Statistical comparisons were made using repeated measures analysis, analysis of variance, chi-square, and Fisher exact tests. RESULTS: Five hundred twenty eyes of 274 patients were included in the study. One hundred seventy-six eyes (93 patients) were treated with the FS15 laser, 180 eyes (93 patients) with the FS30 laser, and 164 eyes (89 patients) with the FS60 laser. Seventeen eyes (10%) in the FS15 laser group, 24 eyes (13%) in the FS30 laser group, and 23 eyes (14%) in the FS60 laser group developed DLK. There was no statistically significant difference in the incidence of DLK between the 3 groups (P = .68). CONCLUSION: There was no significant difference in the incidence of DLK between the FS15, FS30, and FS60 groups.

Ultrasonic bone removal versus high-speed burring for lateral orbital decompression: comparison of surgical outcomes for the treatment of thyroid eye disease.

PURPOSE: To evaluate the efficacy of ultrasonic bone removal during lateral orbital decompression for thyroid eye disease. METHODS: Retrospective, comparative, interventional case series of lateral orbital decompressions performed by the senior author for thyroid eye disease between July 2005 and July 2008. Patients were excluded if they had other coexisting orbital conditions or concurrent decompression of other orbital walls. Primary outcome measures included visual acuity, proptosis, lagophthalmos, eyelid retraction, and exposure keratopathy. RESULTS: Thirty-six consecutive lateral orbital decompressions performed by the senior author were reviewed. The Sonopet Omni ultrasonic surgical aspirator was used to remove the lateral wall in 18 cases, and a high-speed drill with a cutting burr was used in the other 18 cases. There was no significant difference between the groups in postoperative visual acuity, proptosis reduction, lagophthalmos, eyelid retraction, exposure keratopathy, or surgical complications. The average reduction in proptosis was 3.9 mm (range, 1-6.5 mm) in the Sonopet group and 4.0 mm (range, 1-6 mm) in the drill group (p = 0.86). In our series, the average surgical case time was slightly shorter in the Sonopet group than in the drill group (104 vs. 118 minutes, p = 0.032). CONCLUSIONS: Ultrasonic bone removal is a safe and effective alternative to high-speed burring during lateral orbital decompression for thyroid eye disease.