Cerebral white matter abnormalities in patients with charcot-marie-tooth disease.

Here, we report the structural evidence of cerebral white matter abnormalities in Charcot-Marie-Tooth (CMT) patients and the relationship between these abnormalities and clinical disability. Brain diffusion tensor imaging (DTI) was performed in CMT patients with demyelinating (CMT1A/CMT1E), axonal (CMT2A/CMT2E), or intermediate (CMTX1/DI-CMT) peripheral neuropathy. Although all patients had normal brain magnetic resonance imaging, all genetic subgroups except CMT1A had abnormal DTI findings indicative of significant cerebral white matter abnormalities: decreased fractional anisotropy and axial diffusivity, and increased radial diffusivity. DTI abnormalities were correlated with clinical disability, suggesting that there is comorbidity of central nervous system damage with peripheral neuropathy in CMT patients. ANN NEUROL 2017;81:147-151.

A case of odontogenic orbital cellulitis causing blindness by severe tension orbit.

We report a very rare case of odontogenic orbital cellulitis causing blindness by severe tension orbit. A 41-yr old male patient had visited the hospital due to severe periorbital swelling and nasal stuffiness while he was treated for a periodontal abscess. He was diagnosed with odontogenic sinusitis and orbital cellulitis, and treated with antibiotics. The symptoms were aggravated and emergency sinus drainage was performed. On the next day, a sudden decrease in vision occurred with findings of ischemic optic neuropathy and central retinal artery occlusion. Deformation of the eyeball posterior pole into a cone shape was found from the orbital CT. A high-dose steroid was administered immediately resulting in improvements of periorbital swelling, but the patient's vision had not recovered. Odontogenic orbital cellulitis is relatively rare, but can cause blindness via rapidly progressing tension orbit. Therefore even the simplest of dental problems requires careful attention.

Cerebellar White Matter Abnormalities in Charcot-Marie-Tooth Disease: A Combined Volumetry and Diffusion Tensor Imaging Analysis.

Charcot-Marie-Tooth disease (CMT) is a genetically heterogeneous hereditary peripheral neuropathy. Brain volumetry and diffusion tensor imaging (DTI) were performed in 47 controls and 47 CMT patients with PMP22 duplication (n = 10), MFN2 (n = 15), GJB1 (n = 11), or NEFL mutations (n = 11) to investigate for structural changes in the cerebellum. Volume of cerebellar white matter (WM) was significantly reduced in CMT patients with NEFL mutations. Abnormal DTI findings were observed in the superior, middle, and inferior cerebellar peduncles, predominantly in NEFL mutations and partly in GJB1 mutations. Cerebellar ataxia was more prevalent in the NEFL mutation group (72.7%) than the GJB1 mutation group (9.1%) but was not observed in other genotypic subtypes, which indicates that structural cerebellar abnormalities were associated with the presence of cerebellar ataxia. However, NEFL and GJB1 mutations did not affect cerebellar gray matter (GM), and neither cerebellar GM nor WM abnormalities were observed in the PMP22 duplication or MFN2 mutation groups. We found structural evidence of cerebellar WM abnormalities in CMT patients with NEFL and GJB1 mutations and an association between cerebellar WM involvement and cerebellar ataxia in these genetic subtypes, especially in the NEFL subgroup. Therefore, we suggest that neuroimaging, such as MRI volumetry or DTI, for CMT patients could play an important role in detecting abnormalities of cerebellar WM.

Long-term stability and visual outcomes of a single-piece, foldable, acrylic intraocular lens for scleral fixation.

PURPOSE: To report long-term visual outcomes and complications of a new foldable, one-piece, acrylic posterior chamber intraocular lens with closed-loop haptics (C-flex, Rayner, UK) for scleral fixation. STUDY DESIGN: Retrospective interventional case series of 29 eyes of 29 patients who underwent scleral fixation with the C-flex intraocular lens in the absence of capsular support. METHODS: Review of 29 consecutive cases from January 2001 to December 2005 in which scleral fixation was performed with the C-flex intraocular lens. All cases were performed by a single surgeon (S.J.L.) and with an ab externo two-point scleral fixation technique. Preoperative status, intraoperative complications, and postoperative outcomes were analyzed. RESULTS: Mean best corrected visual acuity improved from 20/150 to 20/40 (P < 0.001) at 1 month and to 20/50 (P < 0.001) at mean follow-up of 41 months. Complications included clinically insignificant decentration [1 (3%)], mild intraoperative vitreous hemorrhage [1 (3%)], postoperative hyphema [2 (7%)], and transient elevated intraocular pressure [2 (7%)]. All complications were mild and resolved without the need for reoperation. CONCLUSION: Scleral fixation of the closed-loop, foldable, acrylic C-flex intraocular lens appeared to reduce operative time, was associated with minimal postoperative complications, and resulted in long-term visual improvement.

Suture fixation technique for a single-piece foldable closed-loop intraocular lens.

PURPOSE: We describe a suture fixation technique for a single-piece foldable acrylic closed-loop intraocular lens (IOL) (C-flex, Rayner). METHODS: In our experimental model analyzing the stability of acrylic haptics, we confirmed that the IOL could be in counterpoise without tilt using only a two-point fixation. This new surgical technique was performed in seven patients. RESULTS: The unique haptic design allows easy and secure suture fixation. The clinical outcomes were encouraging. CONCLUSIONS: It is conceivable that better stabilization can be achieved by the broad arc of distal haptic-tissue contact, in addition to suture fixation, using our surgical technique with the C-flex IOL.

Survey of the sevoflurane sedation status in one provincial dental clinic center for the disabled.

BACKGROUND: Sevoflurane sedation in pediatric and disabled patients has the advantage of faster induction and recovery compared to general anesthesia, as well as minimum influence on the respiratory and cardiovascular functions, and airway protective reflexes. This study aimed to evaluate the clinical efficacy of sevoflurane sedation used in dental treatment at one provincial dental clinic center for the disabled. METHODS: We investigated patients' gender, age, reasons for undergoing sedation, medication history prior to treatment, duration of anesthesia, treatment length, type of treatment, and yearly patterns, for 387 cases of dental treatment performed using sevoflurane sedation from January 2013 to October 2016. RESULTS: We analyzed 387 cases (215 male patients, 172 female patients). Male patients aged 20 year or older accounted for 39.0% of all patients, marking the highest proportion. Patient's lack of cooperation was the most common reason for performing dental sedation. Prosthetic treatment was the most frequently practiced, accounting for 174 treatment cases. The mean lengths of the entire treatment and of the dental procedure were 55.2 min and 39.8 min, respectively. CONCLUSIONS: Sevoflurane sedation has the advantage of fast anesthesia induction and recovery compared to general anesthesia; therefore, it can be used efficiently to induce anesthesia in pediatric and disabled patients during short dental procedures, enabling stable treatment of these patients.