Mehmet Tatli

Posterior epidural migration of sequestered lumbar disc fragment causing cauda equina syndrome

Posterior epidural migration of free disc fragments is rare, and posterior migration of the free fragments causing cauda equina syndrome is exceptionally rare. This report describes a 53-year-old man with disc fragment extrusion at the levels of L3–4 and a 54 -year-old man with disc fragment extrusion at L5–S1 intervertebral space. The patients responded well to the operative therapy with complete relief of the symptoms. The pathological examination confirmed that the specimen was a degenerated intervertebral disc. Early surgery should be the first choice of therapy in patients with large posteriorly migrated sequestered disc fragments, to prevent severe neurological deficits such as cauda equina and conus medullaris syndromes.

Unusual Presentation of Cervical Spinal Intramedullary Arachnoid Cyst in Childhood: Case Report and Review of the Literature

The authors report a 7-year-old girl who presented with progressive quadriparesis which had started a month before admission. Magnetic resonance imaging of the spine revealed an intramedullary cystic lesion extending from C2 to C4. After performing a C2–5 laminectomy, the cyst was drained and anatomically fenestrated with the subarachnoid space by a 1-cm vertical median myelotomy without using a stent. The histopathological findings revealed arachnoid cyst. In the first month after operation, the neurological deficits disappeared gradually, except for a slight left upper paresis. Cervical spinal intramedullary arachnoid cyst is extremely rare and should be considered in the differential diagnosis of intramedullary cystic lesions in childhood. Recovery is possible after subtotal excision and fenestration of the cyst to allow communication with the subarachnoid space even if neurological deficits are present for a long time.

Comparison of the effects of maternal protein malnutrition and intrauterine growth restriction on redox state of central nervous system in offspring rats

Both maternal protein malnutrition and intrauterine growth restriction (IUGR) have deleterious effects on brain development, but a comparison of these effects has not been previously reported. The objectives of this study were to investigate and compare the effects of both factors on the oxidative status of the central nervous system (CNS), including the spinal cord, in offspring rats. We evaluated various parameters of oxidative status and antioxidant enzyme activities of superoxide dismutase and catalase (CAT) in different regions of the CNS from 60-day-old rats subjected to prenatal and postnatal protein restrictions [middle protein restriction 12%, severe protein restriction (SPR) 4%] or IUGR produced by uterine artery ligation. Furthermore, we compared these study groups to each other and to control rats fed an isocaloric 24% protein diet. Results were analyzed using one-way ANOVA followed by Tukeys post hoc test. Both protein restrictions and IUGR altered various parameters of oxidative status. In all evaluated structures, protein restrictions resulted in increases in thiobarbituric acid-reactive substances level and index of lipid peroxidation (P<0.001), and in decreases in antioxidant enzyme activities (P<0.005). IUGR also increased lipid peroxidation levels in the blood samples (P<0.04) and protein oxidative damage in the cerebellum and cerebral cortex (P<0.005); however, no effects were detected on the spinal cord. The greatest decrease in CAT activity was in the cerebellum of rats fed with SPR diet (P<0.001). This study suggests that not only severe but also middle protein malnutrition have deleterious effects on CNS structures, including the spinal cord. Protein restriction has a greater effect on the redox state of the CNS than IUGR.

Atretic Parietal Cephalocele

A 3-month-old girl was admitted to the hospital with a 2.5 ! 3 ! 2 cm, posterior parietal vertex midline cystic lesion. The smooth lesion was a round pulsating swelling. Neurological examination was within normal limits according to the patient’s age. Her delivery had been via the spontaneous vaginal route with no accompanying anomalies. There was no family history of congenital central nervous system lesions. Magnetic resonance imaging (MRI) was performed, which demonstrated a posterior parietal extra-axial lesion on sagittal T 1 -weighted images. The lesion was isointense similar to cerebrospinal fluid (CSF) on T 1 -weighted sequences ( fig. 1 ). At surgery, a smooth mass was found filled with CSF, which contained no neural or glial tissue within the lesion. No associated venous structure or any other visible venous abnormality was detected. The lesion was excised totally at the dural junction, dura and overlying skin were repaired. A postoperative MRI showed no abnormality except normal postoperative changes ( fig. 2 ). The patient had an uneventful recovery and was discharged without any complication.

Apparently novel genetic syndrome of pachygyria, mental retardation, seizure, and arachnoid cysts†

We report on an apparently new syndrome in a consanguineous family with seven members, three of whom have cerebral anomalies including pachygyria and arachnoid cysts along with mental retardation and seizures. The two patients with seizure disorders also had multiple enlarged perivascular spaces seen in the white matter of the centrum semiovale. Our data provide a contribution to the accumulating knowledge on familial cerebral anomalies including arachnoid cysts and lissencephaly. Given the lack of mutation in known lissencephaly genes such as LIS1, 14‐3‐3ε, and DCX, this syndrome may constitute a new phenotype with autosomal recessive inheritance.

Anesthesia Dolorosa Caused by Penetrating Cranial Injury

Anesthesia dolorosa (AD) is an uncommon complication of surgical treatments for trigeminal neuralgia. Its incidence is around 0.8%. To our best knowledge, AD caused by a penetrating cranial injury has not been reported previously. We report the case of a 31-year-old male patient with left-sided neuropathic keratitis and AD that began 18 years earlier, following a penetrating cranial injury with a knife to the left postauricular area. The patient was successfully treated by a carbamazepine and gabapentin combination. In conclusion, penetrating cranial injury is uncommon but may cause a serious neurologic disturbance. In the differential diagnosis of AD, a penetrating injury should be kept in mind. In these cases, treatments should be effective and immediate; otherwise, this may result in catastrophic consequences such as neurotrophic keratitis and blindness. Ophthalmologists should be aware of these potential problems.

The syndrome of pachygyria, mental retardation, and arachnoid cysts maps to 11p15.

The Syndrome of Pachygyria, Mental Retardation, and Arachnoid Cysts Maps to 11p15 Kaya Bilguvar, Ali K. Ozturk, Fatih Bayrakli, Aslan Guzel, Michael L. DiLuna, Yasar Bayri, Mehmet Tatli, Selahaddin Tekes, Zulfikar Arlier, Katsuhito Yasuno, Christopher E. Mason, Richard P. Lifton, Matthew W. State, and Murat Gunel* Department of Neurosurgery, Yale University School of Medicine, New Haven, Connecticut Program on Neurogenetics, Yale University School of Medicine, New Haven, Connecticut Department of Neurosurgery, Dicle University, Diyarbakir, Turkey Department of Medical Biology and Genetics, Dicle University, Diyarbakir, Turkey Department of Genetics, Yale University School of Medicine, New Haven, Connecticut Child Study Center, Yale University School of Medicine, New Haven, Connecticut Department of Internal Medicine, Howard Hughes Medical Institute, Yale University School of Medicine, New Haven, Connecticut Department of Neurobiology, Yale University School of Medicine, New Haven, Connecticut

Multifocal Ewing’s sarcoma of the brain, calvarium, leptomeninges, spine and other bones in a child

Ewings sarcoma (ES) is a malignant osseous neoplasm that mostly affects children and young male adults, and usually presents as a solitary bony lesion. Multifocal ES of the central nervous system is extremely rare, with an incidence ranging from 1.1% to 4.3%. Clinically, ES may mimic osteomyelitis. In this report, we describe the case of an 11-year-old boy who had multiple calvarial, leptomeningeal, spinal and various other bony lesions of ES, which were diagnosed radiologically and histopathologically. To the best of our knowledge, this is the first time that multiple brain, calvarial and spinal lesions of ES in a single patient have been reported in the English-language literature. We discuss possible mechanisms and differential diagnoses for this rare pathology.

Melkersson-Rosenthal syndrome with partial oculomotor nerve palsy.

Ann Saudi Med 28(2) March-April 2008 www.saudiannals.net 135 Melkersson-Rosenthal syndrome (MRS) is a rare granulomatous disease characterized by orofacial edema, facial nerve palsy, and furrowed tongue (lingua plicata).1,2 This is the classic triad which defines the syndrome, although it is accepted that the presence of two manifestations or one with a granulomatous cheilitis in the eyelid biopsy, is sufficient to make the diagnosis.3 Facial paralysis, most commonly unilateral, may also occur as well as a congenital fissuring of the tongue. It was first described in 1928 by Melkersson as a syndrome of recurrent facial palsy and edema, and Rosenthal later added the third feature of the syndrome, furrowing of the tongue, in 1931.4 The etiology of MRS is unknown, although both genetic and acquired factors have been implicated.2,5 Isolated eyelid edema, particularly bilateral involvement, is also rare, and as such it is usually misdiagnosed as orbital Melkersson-Rosenthal syndrome with partial oculomotor nerve palsy

Posttraumatic intraventricular arachnoid cyst accompanied by pseudomeningoencephalocele in a child.

Growing skull fracture (GSF) is a rare complication of head trauma. A posttraumatic intraventricular arachnoid cyst (AC), neither isolated nor accompanied by a GSF has not been reported previously. A seven-year-old girl was admitted after a severe head injury with a separated right parieto-occipital fracture and contusion. She responded well to conservative therapy. Seven weeks after discharge, she was re-admitted with a large parieto-occipital pseudomeningoencephalocele due to herniation of cerebrospinal fluid and neural tissue to the subgaleal space through the widened fracture defect, an extra-axial cyst at the posterior interhemispheric space and an intraventricular cystic mass. She underwent open surgery, and the intraventricular cystic mass was totally removed. The histological findings were consistent with an AC. One week after dural repair, hydrocephalus developed, and a ventriculo-peritoneal shunt was inserted. She did well during two-year follow-up. The present case is unique as an intraventricular AC following head trauma. When an intraventricular cystic lesion is encountered after severe head trauma, the possibility of an AC should be considered; especially with neighboring contused neural tissue and leptomeningeal cyst formation.