Vertical muscle transposition augmented with lateral fixation (Foster) suture for Duane syndrome and sixth nerve palsy

PurposeTo report the postoperative results of full-tendon vertical rectus transposition (VRT) augmented with lateral fixation suture for the treatment of type 1 Duane syndrome and sixth nerve palsy and to determine whether there was a decrease in the effect of the Foster suture over time.MethodsThis retrospective, consecutive case series included patients who underwent a full-tendon VRT transposition with lateral fixation for type 1 Duane syndrome or sixth nerve palsy. The primary outcome measures included deviation, abnormal head posture(AHP), abduction deficiency, and postoperative binocular single visual field (BSVF).ResultsEighty-seven patients (87 eyes: 40 eyes with Duane syndrome and 47 eyes with sixth nerve palsy) were included in this study. In Duane syndrome patients, the deviation was reduced by a mean of 95%, the AHP was eliminated in 86% of patients, the abduction was improved by 42%, and a useful BSVF of ∼67% of normal was achieved at 1 year post operation. In sixth nerve palsy patients, the deviation was reduced by 99%, the abduction was improved by 59%, and a useful BSVF of ∼71% of normal was achieved at 1 year post operation. In both groups, the improvements in deviation angle and abduction were stable postoperatively. Sixteen patients needed reoperation for undercorrection.ConclusionVRT surgery with posterior fixation is an effective treatment method for complete sixth nerve palsy and Duane syndrome with esotropia, AHP, and abduction deficiency. The procedure carries a small risk of reoperation for undercorrection. The effect of the Foster suture did not decline over time. © 2013 Macmillan Publishers Limited. All rights reserved

DIFFUSE SPIKE-WAVE STATUS OF 9-YEAR DURATION WITHOUT BEHAVIORAL-CHANGE OR INTELLECTUAL DECLINE

Continuous, generalized 2.5 to 3.5-Hz spike-wave (Sm7) discharges were evident in an EEG performed 13 h after a first seizure associated with a viral illness, in an 8-year-old girl. On eye opening, the EEG abnormality was suppressed and replaced by delta activity and brief spike-wave paroxysms. No alteration in consciousness and seizures or behavioral abnormalities accompanied the EEG findings. Based on the EEG, she was diagnosed as having ''diffuse electrical status,'' the rare EEG phenomenon that occurs during wakefulness without associated behavioral changes. Although the child was completely normal clinically, the EEG findings persisted during a 9-year-follow-up period. Ethosuximide (ESM) 1 gr daily resulted in temporary disappearance of the bioelectrical status. During the last 2 years without medication the EEG finding tended to be restricted to the posterior regions

Is Duane retraction syndrome part of the VACTERL association?

Serpil Akar,1 Birsen Gokyigit,1 Isilay Kavadarli,2 Ahmet Demirok11Pediatric Ophthalmology and Strabismus Department, Prof Dr N Resat Belger Beyoglu Education and Research Eye Hospital, Istanbul, 2Department of Ophthalmology, Gaziantep, Kilis State Hospital, Kilis, TurkeyAbstract: We report here a patient with type 1 Duane’s retraction syndrome and multiple congenital abnormalities as a result of the VACTERL association. The presented combination of Duane’s retraction syndrome and the VACTERL association has not been reported in the literature. The present case was instructive for reviewing the continuous spectrum of ocular anomalies that accompany the VACTERL association.Keywords: Duane’s retraction syndrome, VACTERL association, combinatio

Recurrent absence status epilepticus: clinical and EEG characteristics

In order to outline the clinical and EEG characteristics of recurrent absence status epilepticus (ASE), eight cases with more than two attacks of ASE were studied. Their current ages were between 13 and 84 years, and five of the patients were women. There was a history of epilepsy in five of the patients before the first ASE episode. A varying degree of confusion was the main clinical symptom with associated mild motor signs like perioral, eyelid and generalised myoclonus, seen in one, two and four patients respectively. Two of the patients had juvenile myoclonic epilepsy. One patient had an atypical form of childhood absence epilepsy characterised by recurrent ASE attacks on awakening. There were two patients with phantom absences and late onset generalised convulsions, one patient with perioral myoclonia and absences, and finally two patients with eyelid myoclonia with absences, which are proposed syndromes. On the EEGs that revealed the diagnosis of ASE, there was a marked variability of the generalised multispike and wave discharges. The EEG findings appeared to be syndrome-related with some exceptions. IV Clonazepam lead to a dramatic improvement. Our study shows that the majority of recurrent ASE cases do not fit into the International syndrome classification. (C) 2002 Published by Elsevier Science Ltd on behalf of BEA Trading Ltd

Periodic lateralized epileptiform discharges: association with seizures

The clinical features and EEGs of 45 consecutive patients (40 adults and 5 children) who had periodic lateralized epileptiform discharges (PLEDs) were reviewed to determine the relationship between seizures and PLEDs. Focal encephalitis and ischemic stroke were the most frequent underlying processes for adult patients. All of the children, but only six of the adults, had long-lasting cerebral disorders whereas the remaining adults had acute or subacute illness. There were 38 patients (84.4%) experiencing a seizure disorder. Twenty-six of them had their first seizure during their acute illness, as the pattern of PLED was encountered. Eight cases had status epilepticus, and seven of them had epilepsia partialis continue. Nineteen patients had a recent seizure in the day when PLEDs were observed hut not during EEG recording; 12 patients had their seizures within 10 days before the observation of PLED. PLEDs were grouped into three categories with respect to their extensions: lateralized to one hemisphere (n = 22), localized in one region (n = 17) and being prominent over one side with contralateral spread (n = 6). The last group was found to be more closely associated with frequent seizures or status epilepticus than the other two groups, our results showed that PLEDs were highly correlated with recent seizures in the majority of the patients. These EEG findings may be considered as a manifestation of an increased neuronal excitability caused by different etiologies; but not an ictal pattern. (C) 2000 BEA Trading Ltd

A clinically recognizable neuronal migration disorder: Congenital bilateral perisylvian syndrome. Case report with long-term clinical and EEG follow-up

Congenital bilateral perisylvian syndrome (CBPS) is a recently described, neuronal migration disorder, characterized by pseudobulbar palsy, epilepsy and mental retardation and bilateral perisylvian dysplasia. A 15-year-old boy was diagnosed with CBPS according to the typical clinical, and magnetic resonance imaging (MRI) features. The patient was suffering from atypical absence seizures, repeating daily in spite of antiepileptic drug therapy, since age 7 years. He had also experienced rare generalized tonic-clonic seizures and complex partial seizures. Neurological examination showed severe restriction of tongue movements, severe dysarthria, dysphagia, facial diplegia, mild pyramidal signs and moderate mental retardation. A computed tomographic (CT) scan demonstrated bilateral perisylvian enlargement. The diagnosis was corrected with MRI after six years. Frequent irregular generalized spike and wave abnormalities and focal sharp and slow waves over the posterior regions of both hemispheres were shown by electroencephalograms (EFG). The patient was treated with Na-Valproate, carbamazepine and lamotrigine but did now show any significant change in seizure frequency in the eight-year follow-up period. Intractable seizures, mental retardation and particularly congenital pseudobulbar palsy suggest this congenital entity. Those patients who exhibit these typically clinical features, must have MRI