Determination of tibial somatosensory evoked potentials predicts detrusor sphincter dyssynergia in children with neurogenic bladder dysfunction.

INTRODUCTION: Tibial somatosensory evoked potentials (SEP) are used to identify the neurological status and tethered cord (TC) in patients with spina bifida (SB). Its significance in contributing to the interpretation of urodynamics to determine bladder status is unknown. This study aimed to determine the correlation between SEP and urodynamics in children with SB. MATERIAL AND METHODS: SEP and urodynamic results, for differential diagnosis of TC, were evaluated. SEP scores were correlated with urodynamic findings. SEP results were scored from 1 to 6, with 1, denoting a favorable score and 6, an unfavorable score. Age, gender, detrusor, and sphincter activities in urodynamics were noted. Results were analyzed using the χ2 test and logistic regression analysis. Receiver operating characteristic (ROC) curve was formed to get a valid threshold for the SEP score to predict the urodynamic condition. RESULTS: There were 44 SB patients for whom SEP was done for differential diagnosis of TC. Fifteen patients who did not meet the inclusion criteria were excluded from the study. SB aperta was present in 17 patients and occulta in 12, respectively. The patients had a mean age of 6.6 ± 3.2 years. There were 13 boys and 16 girls. A strong correlation was found between high SEP scores and detrusor sphincter dyssynergia (p < 0.001). A SEP score over 3.5 was found to be 93% sensitive and 73% specific to predict this correlation. There was no relationship between detrusor activity and SEP scores (p = 0.18). DISCUSSION: Tibial SEP is an important noninvasive adjunct tool for the diagnosis of TC in patients with SB. Urodynamic studies are the gold standard in the evaluation of bladder status in neurogenic bladder dysfunction due to SB. Detrusor sphincter dyssynergia may be regarded as a sign of severe spinal cord injury in these patients. CONCLUSION: Our findings suggest that in children with neurogenic bladder, high SEP scores may predict the presence of detrusor sphincter dyssynergia but not the status of detrusor function while providing pathophysiological evidence for neural injury.

Prevalence of inguinoscrotal pathologies and risk factors in a cohort of 388 children with spina bifida.

BACKGROUND: There is limited information about the prevalence and risk factors of inguinal hernia and undescended testis in patients with spina bifida (SB). The aim of this study was to identify the properties and prevalence of inguinoscrotal diseases in these patients. METHODS: A questionnaire was completed by parents of patients with the diagnosis of SB in our center. Together with demographic data, presence an of inguinal hernia, side, operation history, presence of ventriculoperitoneal (VP) shunt, type of SB aperta or occulta, recurrence and presence of undescended testis were questioned. Patients were grouped into 2 as SB aperta and occulta. The prevalence of these pathologies and their clinical properties were evaluated. RESULTS: In this study, 388 patients were evaluated. Of these, 238 patients had SB aperta and 150, SB occulta. There was no significance in comparison of gender. The prevalence of inguinal hernia was 12.6% in general. A hernia was noted in 37 SB aperta patients (15.6%) whereas this was seen in 12 of the SB occulta patients (8%) (p=0.029). When there was a VP shunt, hernia prevalence was 21.5% and when there was no shunt, this ratio was 7.1% (p=0.0001). Prevalence of inguinal hernia was 21.8% in males and 3.2% in females (p=0.0001). When there was a VP shunt with SB aperta the prevalence was 21.9% and when a VP shunt was present with SB occulta, this number was found to be 13.3% (p=0.006). The prevalence of undescended testis was 17.7% and there was no difference between SB aperta and occulta patients. CONCLUSIONS: Inguinal hernia and undescended testis are more frequent in SB patients when compared to the normal population. VP shunts and male gender may be risk factors for inguinal hernia in these children. These findings may imply neurological factors in the etiology of inguinal hernia and undescended testis.

Changes in the cuff pressure in neonates in the absence of nitrous oxide.

BACKGROUND: Changes in the pressure of cuffed neonatal size tracheal tubes (TT) during anaesthesia without nitrous oxide are not well described. We determined whether the cuff pressure changes over time in neonates under general anaesthesia without nitrous oxide. METHODS: The airways of thirty neonates were secured with a high volume low pressure cuffed TT for meningocele surgery. The cuff was manually inflated until there was no audible leak and maintained at 10-15 cm H2O throughout by monitoring the pressure continuously with both a manometer and a pressure transducer. At baseline, the cuff pressure was assessed in the supine and then prone positions. During surgery, if the pressure exceeded 15 cm H2O, the cuff was deflated to < 15 cm H2O and if it was < 10 cm H2O, the cuff was inflated to 10-15 cm H2O. The time interval between corrections and the number of corrections were recorded. RESULTS: The cuff pressures in 18 neonates (60%) required correction during surgery. The cuff pressure exceeded 15 cm H2O in nine neonates (30%) and was corrected. The cuff pressures in 13 neonates were less than 10 cm H2O and required correction. The gender, weight, height, and duration of anaesthesia did not differ significantly between neonates who required correction of the cuff pressure and those who did not. Mean cuff pressures were similar at 15, 45, and 75 minutes of anaesthesia. CONCLUSIONS: In 60% of neonates undergoing surgery in the prone position under general anaesthesia without nitrous oxide, the cuff pressure exceeded 15 cm H2O. In such cases, cuff pressure should be monitored continuously throughout the surgery.

Glottic views using a Miller size 0 blade are superior to those from a Macintosh size 0 blade in neonates: a randomized trial.

INTRODUCTION: Both the Miller and Macintosh blades are commonly used during laryngoscopy in infants and children, although the glottic views have not been compared in neonates. This study compared the glottic views with the Miller and Macintosh size 0 blades in neonates when the blades were placed above and below the epiglottis. MATERIAL AND METHODS: Forty anaesthetized and paralyzed neonates undergoing elective surgery were enrolled and randomized to either the Miller or Macintosh size 0 blade. Two glottic views were obtained in random order in each neonate and were photographed using the same blade: lifting the epiglottis first then the tongue base or vice versa. The percentage of glottic opening (POGO) scores were evaluated with each view. The POGO scores and cardiorespiratory variables were then analysed. RESULTS: The POGO scores using the Miller blade to lift both the epiglottis and the tongue base were similar, whereas the scores using the Macintosh blade to lift the epiglottis were greater than those to lift the tongue base. The POGO scores using the Miller blade in both positions were significantly greater than those using the Macintosh blade in the corresponding positions (P = 0.0001). The heart rate using the Miller blade was greater than that using the Macintosh blade (P = 0.0001). CONCLUSIONS: In neonates, the glottic views using the Miller size 0 blade to lift both the epiglottis and the tongue base were deemed to be excellent and superior to those using the Macintosh blade in both positions.

Urodynamic Study Findings Prior to Myelomeningocele Repair in Neonates.

INTRODUCTION: Myelomeningocele (MMC) is the most common cause of neurogenic bladder dysfunction in children. Neurogenic bladder dysfunction is developed before birth due to autonomous nervous system affected prenatally in patients with MMC. The aim of this study was to share urodynamic study findings before MMC repair and to discuss the correlation with neurological evaluation. MATERIALS AND METHODS: We prospectively studied 37 patients who underwent surgery for MMC repair in our institution in the first 20 h of their lives between 2013 and 2016. All patients were evaluated by a neurosurgeon, neonatologist, and pediatric surgeon. Urodynamic study was performed in first 18h of life before MMC repair in all patients. Lesion level, occurrence of hydrocephalus, neurological functions, spinal deformities, and urodynamic study results were analyzed. RESULTS: The study included 18 female and 19 male patients. Overactive detrusor was detected in 22 patients, and hypoactive detrusor was detected in 5 patients. Overactive sphincter muscle was detected in 32 patients, and hypoactive sphincter was detected in 2 patients. Detrusor-sphincter dyssynergia was present in 34 patients. CONCLUSION: Detailed analysis of urodynamic study findings in larger patient groups may be important to understand the physiopathology of prenatal damage in patients with MMC.

Evaluation of Neurological Examination, SEP Results, MRI Results, and Lesion Levels in Patients Who Had Been Operated for Myelomeningocele.

OBJECTIVE: Myelomeningocele is the most severe and the most frequent form of spina bifida. Most of the myelomeningocele patients undergo operations in new-born age. In terms of life quality and rehabilitation, follow-up's of these patients in the growth and development period after the operation is critical. In our study, our aim is to emphasize the correlation of SEP results with MRI results and clinical features of the myelomeningocele patients. MATERIALS AND METHODS: In our study, we included 36 patients who had undergone myelomeningocele operation and have been followed-up in Istanbul Bilim University Florence Nightingale Hospital, Spina Bifida Research and Treatment Centre. Posterior tibial nerve SEP was performed on each patient and neurological examinations were done in the same session. Results were compared with clinical functional lesion levels, levels of fusion defect and ambulation levels. In order to evaluate SEP results, we used age-related reference values from Boor et al.'s study in 2008. Patients were grouped as normal, unilaterally prolonged, bilaterally prolonged, unilaterally lost, and bilaterally lost. RESULTS: The correlations of posterior tibial nerve SEP results were significant with ambulation levels (r = 0.428, P < 0.01), clinical functional lesion levels (r = 0.477, P < 0.01) and fusion defect levels (r = -0.528 P < 0.05). The lumbar SEP results were only significantly correlated with functional lesion levels (r = 0.443 P < 0.05). CONCLUSIONS: Radiological studies are insufficient when evaluating the functionality of the central nervous system. To fully evaluate the functionality and watch the neurological development with accuracy, especially in operated patients, electrophysiological studies should be an indispensable part of myelomeningocele follow-ups.

Transsphenoidal surgery for pituitary adenomas in pediatric patients: a multicentric retrospective study.

INTRODUCTION: Pediatric pituitary adenomas are rare lesions. Incidence is reported between 1 and 10% of all childhood brain tumors and between 3 and 6% of all surgically treated adenomas. Although pituitary adenomas present with symptoms of hormone hypersecretion or neurological disruptions secondary to mass effect, they are almost constantly benign. Characteristics of patients may vary in different studies according to age, gender, size of adenoma, hormonal activity, and recurrence rates. METHODS: Data on consecutive pediatric patients who were operated for pituitary adenoma with endoscopic endonasal transsphenoidal surgery (EETS) and transsphenoidal microsurgery (TMS) in the Department of Neurosurgery, Istanbul University Cerrahpasa Medical Faculty, Istanbul, Turkey, in the Neurosurgical Unit of the San Matteo Hospital, Pavia, Italy, and in the Division of Neurological Surgery Department of Biotechnology and Life Sciences, University of Insubria-Varese, ASST Sette Laghi, Ospedale di Circolo e Fondazione Macchi, Varese, Italy, between July 1997 and May 2018, were analyzed. Twenty-seven patients (11 males and 16 females), who were 18 years old or younger at the time of surgery, were included in the study. Medical records, images, and operative notes of patients were retrospectively reviewed. RESULTS: There were 16 females (59.3%) and 11 males (40.7%). Mean age was 15.3 ± 3.3 (4-18). Thirty-two surgical procedures were performed for 27 patients (6 children required second operation). Thirteen patients (48.14%) had Cushing's disease (CD), 5 patients (18.5%) had growth hormone (GH)-secreting adenoma, 5 patients (18.5%) had prolactinoma, and 4 patients (14.8%) had non-functional adenoma. Twenty-two patients (81.4%) met remission criteria, and 5 patients (18.5%) did not meet remission criteria. Four patients met remission criteria after the second operation. CONCLUSION: Transsphenoidal approach affords effective release of mass effect and not only restoration but also perpetuation of normal endocrine functions in the majority of pediatric pituitary adenoma patients. Satisfactory results are reported with both EETS and TMS in the literature. Despite the technical difficulties in pediatric age, transsphenoidal resection of adenoma is still the mainstay treatment that provides cure in pediatric patients.

Neurosurgical treatment of Cushing disease in pediatric patients: case series and review of literature.

AIM: Pituitary adenomas are rare in childhood in contrast with adults. Adrenocorticotropic hormone (ACTH)-secreting adenomas account for Cushing's disease (CD) which is the most common form of ACTH-dependent Cushing's syndrome (CS). Treatment strategies are generally based on data of adult CD patients, although some difficulties and differences exist in pediatric patients. The aim of this study is to share our experience of 10 children and adolescents with CD. PATIENTS AND METHOD: Medical records, images, and operative notes of 10 consecutive children and adolescents who underwent transsphenoidal surgery for CD between 1999 and 2014 in Cerrahpasa Faculty of Medicine were retrospectively reviewed. Mean age at operation was 14.8 ± 4.2 years (range 5-18). The mean length of symptoms was 24.2 months. The mean follow-up period was 11 years (range 4 to 19 years). RESULTS: Mean preoperative cortisol level was 23.435 µg/dl (range 8.81-59.8 µg/dl). Mean preoperative ACTH level was 57.358 µg/dl (range 28.9-139.9 µg/dl). MR images localized microadenoma in three patients (30%), macroadenoma in four patients (40%) in our series. Transsphenoidal microsurgery and endoscopic transsphenoidal surgery were performed in 8 and 2 patients respectively. Remission was provided in 8 patients (80%). Five patients (50%) met remission criteria after initial operations. Three patients (30%) underwent additional operations to meet remission criteria. CONCLUSION: Transsphenoidal surgery remains the mainstay therapy for CD in pediatric patients as well as adults. It is an effective treatment option with low rate of complications. Both endoscopic and microscopic approaches provide safe access to sella and satisfactory surgical results.

Major Depression and Obsessive-compulsive Disorder Treated with Deep Brain Stimulation of Bilateral Nucleus Accumbens: The First Case of Turkey.

Deep brain stimulation (DBS) is a new alternative treatment for treatment-resistant major depression (MD) and obsessive-compulsive disorder (OCD). Various DBS targets were defined for MD and OCD. Nucleus accumbens (NAcc) comes out among the other targets in patients with MD and comorbid OCD when physiopathology and limited side effects are taken into account. We report a 27-year-old male with MD and OCD who was treated by bilateral NAcc-DBS. The aim of this study is to discuss NAcc as a DBS target in patients with MD and OCD and to report the first case of a psychiatric disorder treated with DBS in Turkey.

Analysis of Denver Neurodevelopmental Screening Test Results of Myelomeningocele, Hydrocephalus, and Microcephaly Patients.

CONTEXT: Spina bifida, hydrocephalus, and similar congenital central nervous system (CNS) anomalies take origin from embryologic stages weeks before birth, but assessment and follow-up of these patients are important to figure and predict the effects of these anomalies on child's neurodevelopment. AIMS: To evaluate of multiple groups of congenital CNS anomalies in the neurodevelopment level. SETTINGS AND DESIGN: The study was conducted at a research and treatment center for spina bifida patients. MATERIALS AND METHODS: The study group included 348 patients with a mean age of 15.4 (±15.1) months, who had spina bifida aperta, hydrocephalus, and microcephaly. Patients with other known intracranial conditions were excluded. The subjects were evaluated into five groups: Group 1, 88 patients with congenital hydrocephalus; Group 2, 48 patients with congenital hydrocephalus and ventriculoperitoneal shunt; Group 3, 148 patients with microcephaly; Group 4, 30 patients who were operated for spina bifida aperta; and Group 5, 39 patients who were operated for spina bifida aperta and also had ventriculoperitoneal shunt implantation. Denver Developmental Screening Test II was used to assess patients' neurodevelopment levels. STATISTICAL ANALYSIS USED: Pearson's chi-square and Fisher's exact tests were used for data analysis. Group comparisons were also made in pairs with chi-square test according to Bonferroni corrections. Frequency of abnormal findings was significantly correlated with age (P = 0.014). RESULTS: Total score differences of five groups appeared to be statistically significant according to Pearson's chi-square test (P = 0.000). When we compared groups in pairs, abnormal results were significantly frequent in shunted groups (P < 0.01). CONCLUSIONS: Our results suggested that shunt-dependent hydrocephalus caused serious neurodevelopmental impairments in patients.

DBS in pediatric patients: institutional experience.

INTRODUCTION: DBS is initially used for treatment of essential tremor and Parkinson's disease in adults. In 1996, a child with severe life-threatening dystonia was offered DBS to the internal globus pallidus (GPi) with lasting efficacy at 20 years. Since that time, increasing number of children benefited from DBS. PATIENTS AND METHODS: We retrospectively evaluated our database of patients who underwent DBS from 2011 to 2017. All patients ≤ 17 years of age at the time of implantation of DBS were included in this series. Subjective Benefit Rating Scale (SBRS), Hoehn Yahr Scale (HYS), Fahn Marsden Rating Scale (FMRS), Clinical Global Impressions Scales (CGI), and Yale Global Tic Severity Scale (YGT) were used to evaluate clinical outcome. RESULTS: Between May 2014 and October 2017, 11 children underwent DBS procedure in our institution. Six of them were female and five of them were male. Mean age at surgery was 11.8 ± 4.06 years (range 5-17 years). In our series, four patients had primary dystonia (PDY) (36.3%), three patients had secondary dystonia (SDY) (27.2%), two patients had JP (18.1%), and two patients had Tourette Syndrome (TS) (18.1%). Two JP patients underwent bilateral STN DBS while the other nine patients underwent bilateral GPi DBS. SBRS scores were 1.75 ± 0.5 for patients with PDY, 3 ± 0 for patients with JP, 2.5 ± 0.7 for patients with TS, and 2 ± 1 for patients with SDY. Mean FMRS reduction rate was 40.5 for patients with dystonia. Significant improvement was also defined in patients with TS and JP after DBS. None of the patients experienced any intracerebral hemorrhage or other serious adverse neurological effect related to the DBS. Wound complications occurred in two patients. CONCLUSION: There are many literatures that support DBS as a treatment option for pediatric patients with medically refractory neurological disorders. DBS has replaced ablative procedures as a treatment of choice not only for adult patients, but also for pediatric patients. Wound-related complications still remain the most common problem in pediatric patients. Development of smaller and more flexible hardware will improve quality of children's life and minimize wound-related complications in the future.

Morphometric Analysis of Dose-dependent Effect of Progesterone on Experimental Vasospasm-induced Rat Femoral Arteries.

OBJECTIVE: Our aim of this study was to determine effective doses of progesterone which has a vasodilatory effect during the early stage of vasospasm. Cerebral vasospasm (CV) is a predominant cause of morbidity and mortality which develops following subarachnoidal hemorrhage (SAH). Etiopathogenesis of CV is multifactorial. Despite many previously performed studies on this issue, the mechanism by which blood and blood products in the subarachnoidal space induce CV has not been clarified yet. MATERIALS AND METHODS: In our study, we used "Rat Femoral Artery Vasospasm Model" introduced by Okada et al. Thanks to easy procurement and maintenance of rats. Rats were divided into four groups as: Group 1 (n = 8; control group), Group 2 (n = 8; vasospasm group), Group 3 (n = 8; vasospasm + 3 mg/kg progesterone group), and Group 4 (n = 8; vasospasm +15 mg/kg progesterone group). Progesterone which is an endogenously synthesized natural steroid was preferred in our study. Progesterone increases the production of vasodilatory epoxyeicosatrienoic acid by acting on its binding sites termed as pregnane X receptor. It decreases the intracellular influx of Ca2+ by blocking the functioning of L-type channels in smooth muscle cells. It manifests another vasodilatory effect by decreasing expression of TxA2 receptor. In our study, at the end of the 7th day, where the most intense vasospasm is seen, 1 cm pieces were excised from the femoral arteries and histopathologically examined under light microscope. RESULTS: Vascular walls of three vasospasm-induced groups were relatively thicker when compared with the control group. Drug-treated groups were not different from each other. Vascular walls of the groups treated with lower and higher doses of the drug were thinner when compared with the vasospasm group without any statistically significant difference between groups (P > 0.05). Luminal cross-sectional areas of the drug-treated groups did not differ from each other. Mean luminal cross-sectional areas of the control and the drug-treated groups were larger than that of the vasospasm group without any statistically significant intergroup difference (P > 0.05). CONCLUSION: Based on the results of our study, progesterone did not exert protective effects on vascular wall thickness, while histopathological examination of luminal cross-sectional areas revealed its vasodilatory effects without any statistically significant difference between groups. Starting from the study results obtained, we think that its potential use as a preventive agent against the development of post-SAH CV requires conduction of multicentered, placebo-controlled, randomized, and double-blind studies.

Surgical treatment of patients with myelomeningocele-related spine deformities: study of 26 cases.

INTRODUCTION: Scoliosis, kyphosis, and sacral agenesis (SA) are common spine deformities in myelomeningocele (MMC) patients. Surgery of spine deformities in MMC patients is associated with various difficulties as infection, pathological skin breakage, instrumentation failure, and neurological deterioration. The purposes of this study are to share our clinical experience and discuss different surgical techniques which are defined in the literature. PATIENTS AND METHOD: We retrospectively evaluated our database of patients with MMC who underwent surgical procedures for spine deformities from 2014 to 2016. Demographic and clinical data, surgical parameters, surgical techniques and levels, pre- and postoperative deformity angles, level of posterior fusion defect, spinal malformations, neurological evaluation of lower extremities and complications were collated. We divided the cases into three groups according to the type of deformities. The groups were lumbar kyphosis (Group 1), congenital scoliosis (Group 2), and paralytic scoliosis (Group 3). RESULTS: There were 26 patients in the study. Fifteen patients were male and 11 patients were female. The median age of the patients was 8.03 (range = 3-17 years) at the time of operation. There were 10 patients in Group 1, 7 patients in Group 2, and 9 patients in Group 3. In Group 1, preoperative kyphosis angle varied between 51° and 160°, with an average of 95.7°. In Group 2, preoperative Cobb angle varied between 57° and 150°, with an average of 106.6°. Kyphosis was present in 4 patients. Preoperative kyphosis angle varied between 74° and 140°, with an average of 93°. In Group 3, preoperative Cobb angle varied between 45° and 145°, with an average of 72.5°. CONCLUSION: Spinal deformity in children with MMC has been considered to cause severe disability. Surgical treatment is a challenging procedure with a wide spectrum of complications, but can provide good correction of spinal deformity and pelvic obliquity, and improve the quality of life. Self-growing systems which are a new alternative to traditional growing rod systems, must be considered to preserve growing potential of spine.

Shunt revision rates in myelomeningocele patients in the first year of life: a retrospective study of 52 patients.

PURPOSE: Shunt placement indications are stringent and require confirmation of clinical and radiological evidence of hydrocephalus (HC). The aim of this study was to determine the rate of shunting and discuss the outcome in the first year of life in patients with myelomeningocele (MMC) on the basis of review of the literature. METHODS: All patients who underwent postnatal repair of MMC at our institution between March 2014 and March 2015 were evaluated. Patients were only included if they underwent both MMC repair and ventriculoperitoneal (VP) shunt insertion at our institution and were followed up for at least 12 months. The mean ages for repair of MMC, MMC levels, timing of VP shunt placement, shunt revisions, and causes of shunt revisions were documented. RESULTS: Fifty-two patients with MMC were included in this study. The average gestational age at birth was 38 weeks. The level of MMC was thoracolumbar in 13 cases, 11 times lumbar, 21 times lumbosacral, and 7 times sacral. Thirty-one patients (59.61%) suffered from hydrocephalus and required placement of a shunt. When we evaluate the lesion levels of patients who require shunting, 13 cases were thoracolumbar, 6 cases were lumbar, and 11 cases were lumbosacral. None of the sacral cases needed VP shunt. Seven patients (13.4%) had shunt revision within the first year of life. The cause of shunt revision was wound problem in one patient (1.9%), underdrainage in two patients (3.8%), infection in three patients (5.7%), and mechanical obstruction in another one patient (1.9%). CONCLUSION: MMC closure and management of the associated HC are one of the most basic, but never simple, legs of the pediatric neurosurgery around the world. As clinicians and neurosurgeons, we are obligated to analyze recent evidences and evaluate present approaches to achieve optimization in this subject until further technologies or approaches became more advantageous for our patients.

Health-related quality of life in non-paraplegic (ambulatory) children with myelomeningocele.

PURPOSE: Evaluation of the effects of ventriculoperitoenal shunt and incontinence presence on health-related quality of life of ambulatory myelomeningocele patients. METHODS: The study group included 35 myelomeningocele patients, between 5 and 18 years old (mean age = 9.6), who were neonatally operated. All patients were ambulatory. The Child Edition of the Child Health and Illness Profile (CHIP-CE) used to evaluate the patient group. Seventeen patients were using clean intermittent catheterization and nine patients had ventriculoperitoneal shunt. RESULTS: The CHIP-CE has five domains, and in satisfaction, resilience and achievement domains, significant lower scores were obtained from our study group. In terms of clean intermittent catheterization use, we got significantly lower scores on satisfaction, resilience and achievement domains (p < 0.05). According to the presence of ventriculoperitoneal shunt, we found lower scores in satisfaction, resilience, risk avoidance and achievement domains but the differences were not significant (p > 0.05). No significant difference was spotted according to gender and age. CONCLUSIONS: Continence problems have important effects on life quality of myelomeningocele patients. Incontinency should always be considered as a major variable in health-related quality of life evaluations.

Holmes' Tremor with Shoulder Pain Treated by Deep Brain Stimulation of Unilateral Ventral Intermediate Thalamic Nucleus and Globus Pallidus Internus.

A 21-year-old male was admitted with severe right arm and hand tremors after a thalamic hemorrhage caused by a traffic accident. He was also suffering from agonizing pain in his right shoulder that manifested after the tremor. Neurologic examination revealed a disabling, severe, and irregular kinetic and postural tremor in the right arm during target-directed movements. There was also an irregular ipsilateral rest tremor and dystonic movements in the distal part of the right arm. The amplitude was moderate at rest and extremely high during kinetic and intentional movements. The patient underwent left globus pallidum internus and ventral intermediate thalamic nucleus deep brain stimulation. The patient improved by more than 80% as rated by the Fahn-Tolosa-Marin Tremor Rating Scale and Visual Analog Scale six months after surgery.

Foramina parietalia permagna: familial and radiological evaluation of two cases and review of literature.

PURPOSE: Foramina parietalia permagna is a variable intramembranous ossification defect of the parietal bones. Foramina parietalia permagna have an autosomal dominant inheritance, and it is showed that mutations in chromosome 5 and 11 are causing this anomaly. Enlarged parietal foramina occurs extremely rare. They are usually asymptomatic, but occasional headache, vomiting, pain over unprotected cerebral cortex, and seizures may be experienced by the patients. In the literature, some associated congenital bony defects, soft tissue pathologies, underlying neuronal deficits, and vascular variations have been described. METHODS: We report two cases of foramina parietal permagna with their pedigrees and genetic analysis. RESULTS: In case 1, cytogenetic analysis revealed a mutation of the ALX4 gene and all of the members of the family diagnosed with FPP. MRI revealed inferior vermian cerebellar hypoplasia. Surgery was not considered. In case 2, cytogenetic analysis could not be obtained because of financial reasons. Cranial MRI revealed hypoplastic right transverse sinus and sigmoid sinus, with a persistent parafalcine sinus. Surgery was not considered. CONCLUSION: Despite of its rarity, genetic background and some important associated anomalies make foramina parietalia permagna more than an uncommon insignificant genetic disorder.