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Background Nutritional visual defects are apparently uncommon nowadays in developed nations. Retinal change-related visual defects caused by hypovitaminoses may be underdiagnosed. Aim of the study To investigate the retinal structural and functional changes in a patient with multivitamin deficiency before and during vitamin supplementation. Methods A 51-year-old female had been on vegetarian diet as a child, and on restrict vegan diet during the last 2 years, developing severe bilateral deterioration of visual function and polyneuropathy. Blood test revealed low levels of vitamin A, B6 and D. The patient underwent examinations with optical coherence tomography (OCT), computerized visual field examination (VF), electroretinography (ERG), visual evoked potentials (VEP) and neurography before and after vitamin supplementation. Results Visual acuity (VA) was 20/1000 and VF examination showed central scotoma in both eyes. Color vision was significantly affected. Full-field ERG showed normal rod and cone function, but a clearly reduced central peak was registered in multifocal ERG (mf-ERG), indicating impaired fovea function. VEP showed delayed latency and low amplitude of P100 in both eyes. Neurography showed sensory polyneuropathy. OCT showed significant thinning of macular ganglion cell plus inner plexiform layer (GCIPL) with rapid progression. Retinal nerve fiber layer (RNFL) was preserved and normal, which is in contrast to neuroinflammatory conditions. After 2.5 years of multivitamin supplementation, the visual functions were improved. GCIPL thickness was stable without further deterioration. Conclusions Multivitamin deficiency results in progressive thinning of GCIPL with severe visual deterioration. In contrast to neuroinflammation, RNFL is preserved and normal. Stabilized GCIPL during vitamin supplementation was associated with improved visual function. OCT provides a sensitive and objective measure for differential diagnosis, monitoring retinal change and response to therapy.

Objective: Investigate the temporal development of EEG and prognosis. Methods: Prospective observational substudy of the Target Temperature Management trial. Six sites performed simplified continuous EEG-monitoring (cEEG) on comatose patients after cardiac arrest, blinded to treating physicians. We determined time-points of recovery of a normal-voltage continuous background activity and the appearance of an epileptiform EEG, defined as abundant epileptiform discharges, periodic/rhythmic discharges or electrographic seizure activity. Results: 134 patients were included, 65 had a good outcome. Early recovery of continuous background activity (within 24 h) occurred in 72 patients and predicted good outcome since 55 (76%) had good outcome, increasing the odds for a good outcome seven times compared to a late background recovery. Early appearance of an epileptiform EEG occurred in 38 patients and 34 (89%) had a poor outcome, increasing the odds for a poor outcome six times compared to a late debut. The time to background recovery and the time to epileptiform activity were highly associated with outcome and levels of neuron-specific enolase. Multiple regression analysis showed that both variables were independent predictors. Conclusions: Time to epileptiform activity and background recovery are independent prognostic indicators. Significance: Patients with early background recovery combined with late appearance of epileptiform activity may have a good outcome.

International audience; Background: Whilst modern awake intraoperative mapping has been widely accepted and implemented in the last decades in neuro-oncology, sparse reports have been published on the safety and efficiency of this approach in epilepsy surgery.Method: This article reports four cases with different locations of epileptogenic zones as examples of possible safe and efficient resections.Result: The results of the resections on seizure control were Engel 1 (no disabling seizures) in all cases and no patient experienced significant neurological deficits.Discussion: The discussion focuses on aspects of the future of epilepsy surgery in a hodotopical paradigm.

Optic neuritis (ON) causes axonal loss as reflected by thinning of retinal nerve fiber layer (RNFL) and can be tracked by optical coherence tomography (OCT) about 6 months after ON onset, when swelling of optic nerve head (ONH) has vanished. Changes of macular ganglion cell layer (GCL) thickness provide another window to track the disease process in ON. GCL thinning over time in relation to RNFL change after ON remains elusive. Using OCT, we followed 4 patients with acute unilateral isolated ON for more than 9 months. A diagnosis of multiple sclerosis (MS) was established in all 4 patients. First follow-up was 2-3 weeks after ON onset, and thereafter every 2-3 months. RNFL swelling peaked during first month after acute ON, followed by rapidly reduced swelling (pseudoatrophy) during following 2 months, and thereafter successively vanished 6 months after ON onset. GCL thinning was observed 1-3 months after ON onset, i.e. already during optic disk swelling and before real RNFL thinning. The results imply that quantifying GCL thickness provides opportunities to monitor early axonal loss and ON-to-MS progression, and facilitates distinguishing real atrophy from pseudoatrophy of RNFL after acute ON.

Awake craniotomy for brain tumours Awake neurosurgery is a useful method in lesions near eloquent brain areas, particularly low-grade gliomas.The aim is to maximise tumour resection and preserve neurological function. We performed 40 primary awake surgeries and 8 residual surgeries. Patients were operated awake throughout the procedure or with a laryngeal mask and general anaesthesia during the opening stage and then awake during intracerebral surgery. Language and motor function were mapped with direct cortical stimulation, motor evoked potential and standardised neurological testing. Radiologically, complete resection was achieved in 18 out of 40 patients in the primary surgeries. Full neurological recovery at three months was observed in 29 patients. Of the 11 patients with persisting neurological deficits at three months, symptoms were present preoperatively in 9 patients. We conclude that awake surgery, combined with intraoperative neurophysiological methods, is a safe method to improve treatment for low-grade gliomas.

Objective: Seizures with an aura of a "sensed presence," a religious emotion, or feelings of euphoria (ecstatic seizures) are characterized by heightened self-awareness. A previous case report on a patient with epilepsy and "sensed presence" as an aura described hypoperfusion in both temporal lobes and a local ictal increase in the left frontoparietal area. A reexamination of the data was suggested by a recent study of patients with ecstatic seizures, which proposed that hyperactivation of the left anterior insula might be a potential cause. Methods: We reanalyzed the laboratory data on the case with "sensed presence" aura using a fusion of SPECT and MR images of the brain, which had not previously been available, and a close examination of the subdural ictal EEG registrations. Results: Examination of the ictal EEG recordings from subdural strip electrodes implanted subtemporally and temporally on both sides showed that seizure activity occurred first at the most medial subtemporal electrode on the left side. From an anatomical point of view, this electrode position is close to the ventral aspect of the left anterior insula, and it is possible that the seizure activity was initiated there. Reexamination of the SPECT data after fusion with contemporary MR images clearly indicated that the region of strong hyperactivation overlies the left anterior insula. Hyperactive regions also appear on the midinsula bilaterally. Together with the neurophysiological ictal EEG, this evidence supports a reinterpretation that this aura of "sensed presence" can be attributed to hyperactivation of the left anterior insula. Conclusion: The present findings support the proposal that ecstatic seizures or "sensed presence" auras can originate from the left anterior insula, a region that has been suggested to engender self-awareness associated with positive feelings.

Introduction Continuous EEG-monitoring (cEEG) is used to detect electrographic seizures and is a promising prognostic tool in comatose patients after cardiac arrest (CA). The objective of this study was to investigate the temporal development of epileptiform activity and EEG background activity as predictors of prognosis. Methods In this prospective substudy of the Target Temperature Management trial six sites recorded simplified cEEG on comatose patients resuscitated after CA. The cEEG-data was blinded to the treating team and assessed, blinded to outcome and clinical data, at 12, 24, 36, 48 and 60 h after CA. The standardized EEG terminology of the American Clinical Neurophysiology Society was used. The time-points of recovery of a normal-voltage continuous background and the appearance of an epileptiform EEG were determined, the latter defined as abundant epileptiform discharges ( ⩾ 0.1 Hz), abundant periodic or rhythmic discharges or electrographic seizure activity for 30 min. The cEEG-data was not part of the multimodal prognostication protocol for decisions on withdrawal of care. Poor outcome was defined as best Cerebral Performance Category 3–5 until 180 days. Results 134 patients were included and 65 had a good outcome. 103 patients recovered a continuous normal-voltage background. Background recovery within 24 h occurred in 72 patients, 55 (76%) had good outcome. Among the 31 patients with background recovery beyond 24 h, 10 (32%) had good outcome. Thus a late recovery of background increased the odds for a poor outcome six times (sens 55%, spec 85%). All 31 patients that failed to recover their background within the monitoring period (60 h after CA) had a poor outcome (sens 45%, spec 100%). Appearance of an epileptiform EEG occurred in 66 patients. Among 38 patients with an epileptiform EEG within 24 h, 34 (89%) had poor outcome (sens 49%, spec 94%). 28 patients had start of an epileptiform EEG beyond 24 h, 16 had a poor outcome (sens 46%, spec 80%). Thus an early epileptiform EEG increased the odds for a poor outcome seven times. The logistic regression model showed no significant interactions between background and epileptiform activity regarding predictive ability. Thus assessing both the time to recovery of background and the time to appearance of epileptiform activity improved prognostication. 16 of the 65 patients with good outcome had an epileptiform EEG and 14 (88%) of these patients recovered their background at the same time or before the onset of epileptiform activity. Abundant periodic or rhythmic discharges were seen in 3 survivors with good outcome and all had a recovery of background within 24 h and before the onset of discharges. Conclusion Time to epileptiform activity and EEG background recovery are independent prognostic indicators. Patients with early background recovery combined with late appearance of epileptiform activity may have a good prognosis.

An observational study of changes in muscle structure and the relation to muscle strength in juvenile idiopathic arthritis (JIA).Fifteen children and teenagers (eight girls and seven boys) with JIA, aged 9-19 yr (mean age 16.1), were studied. Muscle biopsies were obtained from the anterior tibial muscle and were examined using histopathological and immunohistochemical methods. Muscle fibre types were classified and fibre areas measured. As markers of inflammation, the major histocompatibility complex (MHC) class I and class II and the membrane attack complex (MAC) were analysed. Results were compared with biopsies from the gastrocnemius muscle in 33 young (19-23 yr) healthy controls. Isometric and isokinetic muscle strengths were measured in ankle dorsiflexion. Strength was compared with reference values for healthy age-matched controls. Nerve conduction velocities were recorded in the peroneal and sural nerves.Four of the 15 muscle biopsies were morphologically normal. Eleven biopsies showed minor unspecific changes. Two of these also showed minor signs of inflammation. MHC class II expression was found in 4/15 patients, which was significantly more than in the healthy controls (P = 0.0143). The expression of MHC class I and MAC did not differ from that in the controls. The mean area of type I fibres was lower than that of type IIA fibres in 12/13 biopsies. Muscle strength was significantly reduced in the patient group. There was a significant positive correlation between muscle fibre area and muscle strength. Nerve conduction studies were normal in all cases.Changes in leg muscle biopsies appear to be common in children and teenagers with JIA. The presence of inflammatory cells in the muscle and expression of MHC class II on muscle fibres may be a sign of inflammatory myopathy. There are no findings of type II muscle fibre hypotrophy or neuropathy, as in adults with RA.

The aim of this study was to investigate to what extent women with superficial dyspareunia can be diagnosed for both partial vaginismus (PaV) and vulvar vestibulitis (VVS) and to discover to what extent surface electromyography (sEMG) of the pelvic floor muscles (PFM) can distinguish between women with PaV solely, PaV+ VVS, and asymptomatic women. A total of 224 consecutive women with superficial dyspareunia were examined clinically for both PaV and VVS diagnoses. We examined 47 women with PaV+/-VVS and 27 asymptomatic women with sEMG of the PFM. The results showed that 102/224 women with superficial dyspareunia and 33/47 women with PaV in the sEMG part of the study had both PaV and VVS. All women with VVS had vaginismus, while 42/224 had PaV but not VVS. sEMG measurements revealed no significant differences between the three groups of women (PaV solely, PaV + VVS, and asymptomatic). Almost half of the women with superficial dyspareunia referred to our clinic have both the diagnosis PaV and VVS. sEMG was not a method of any value to distinguish between women with PaV solely, PaV + VVS, or asymptomatic women. The increased tone found clinically in the PFM of women with PaV+/-VVS may be of other origin than electrogenic contractions.

The aim of this study was to present the failure rate and normal values for motor latency of the anal sphincter after magnetic sacral stimulation (LMSS) using a modified recording technique. A bipolar sponge electrode was placed in the anal canal for recording. A ground electrode was placed in the rectum to reduce stimulus artifact. Magnetic stimulation was induced through a twin coil energized by a Maglite-r25 generator. Two groups were examined: 14 healthy volunteers and 14 patients with a spinal cord injury (SCI) above the conus. Nine of 56 studies (16%) failed. There were no significant differences in latency between right- and left-sided stimulation or between the healthy group and the SCI patients. As described, LMSS measurements are minimally invasive and have a low failure rate. They may be used to test the integrity of the distal motor pathway in patients with bladder or bowel dysfunction who may benefit from continuous electrical sacral root stimulation.

PURPOSE: The aim of this controlled study was to examine whether it was feasible to use magnetic stimulation as a new diagnostic tool to evaluate the motor function of the sacral roots and the pudendal nerves in patients with fecal incontinence. PATIENTS AND METHODS: Nineteen consecutive patients (17 females) with a median age of 67 (range, 36–78) years referred for fecal incontinence and 14 healthy volunteers (six females) with a median age of 42 (range, 23–69) years were examined. Latency times of the motor response of the external anal sphincter were measured after electric transrectal stimulation of the pudendal nerve and magnetic stimulation of the sacral roots. RESULTS: The success rates of pudendal nerve terminal motor latency and sacral root terminal motor latency measurements were 100 and 85 percent, respectively, in the control group and 94 and 81 percent, respectively, in the fecal incontinence group. Median left pudendal nerve terminal motor latency was 1.88 (range, 1.4–2.9) milliseconds in the control group and 2.3 (range, 1.8–4) milliseconds in the fecal incontinence group (P

Introduction Awake craniotomy with neurophysiological monitoring is often performed in patients with brain tumours in close proximity to functional brain areas (speech, motor, internal capsule). We describe our experience in 35 patients. Materials and methods 35 patients with brain tumours adjacent to eloquent areas were operated on during a time frame of five years. Among these, 23 patients had oligodendroglioma grade 2, 4 patients had astrocytoma grade 2, 1 patient had oligodendroglioma grade 3, 2 patients had oligoastrocytoma grade 2, 4 patients had glioblastoma and 1 patient had Multiple Sclerosis. 14 brain tumours were located as follows: left frontal, 11 right frontal, 2 left temporal, 2 left parietal, 4 right frontotemporal (insula), 2 left frontotemporal (insula). The sleep-wake-sleep procedure was used for most of the patients. Direct cortical stimulation (DCS) was used to map the motor cortex and language areas. Median nerve SSEP was used to localize the central sulcus. Subcortical electrical stimulation was used to localize the motor tract during tumour resection. EEG was recorded to monitor seizure discharges. Monitoring of motor function was done by manual testing of motor performance and DCS. Monitoring of language function was performed by talking to the patients, counting and picture naming. Results 21 patients had transient neurological deficitswhich full recovered. 1 patient had permament neurological deficit. 2 patients were converted from awake to asleep craniotomy due to acute psychological distress and seizure during surgery. Neurophysiological monitoring was helpful and feasible in all cases, helping the surgeon to detect functional brain areas thus enabling him to resect the brain tumour with high security. Seizure during DCS occurred in one patient. Conclusion Neurophysiological monitoring during awake craniotomy is feasible and reproducible. There were no major side effects during monitoring. After introducing short train stimulation for language area mapping no seizures occurred.