Nejat Akalan

Spinal hydatid cysts

Eleven cases of spinal hydatid cyst are reported. The patients were admitted with symptoms of compression of the spinal cord. The cysts were localized epidurally in 10 patients and intradurally but extramedullarily in one, and were confirmed histopatholgically after surgical intervention. The results are compared with those reported in the literature. Six of the patients were female and five were male. Their ages ranged from 10 to 65 years. All of the patients were treated surgically, with an incidence of cyst recurrence of 18% and no mortality. Mebendazole (Vermox) was given to two of the patients after their operations.

Infection as a cause of spinal cord compression: a review of 36 spinal epidural abscess cases.

Summary¶ A retrospective survey of 36 cases treated in the Department of Neurosurgery, Hacettepe University Hospitals since 1970 was performed. Clinical presentation, aetiology and outcome of this rare disease compared to recently published series. All cases were admitted with signs of neural compression. Clinical and laboratory data suggesting an infectious origin were present only in 4 cases. Radiological investigation including magnetic resonance imaging in 10 patients, were not confirmative for an epidural abscess except for two cases. All cases underwent urgent surgical decompression and tuberculous abscess either in granulation or pus form was found in the majority. Overall mortality rate was 5.8%. Outcome was closely related to the neurological condition on admission rather than the underlying infectious origin. When compared with recently reported series, our cases demonstrated a significant divergence in terms of clinical presentation, pathogenesis and outcome. The most probable reason for this discrepancy is that risk factors for compromised immunity or systemic infection were much less than the other series and mycobacterium tuberculosis is the responsible agent in the majority which has a much more favourable outcome than non-spesific infections.

Psychiatric morbidity, quality of life, and disability in mesial temporal lobe epilepsy patients before and after anterior temporal lobectomy

Considerable interest has been focused on the psychiatric complications of medically refractory temporal lobe epilepsy (TLE) before and after epilepsy surgery. The aim of the present study was to evaluate the psychiatric status, quality of life, and level of disability in medically refractory mesial temporal lobe epilepsy (MTLE) patients, a homogenous subgroup of patients with TLE, before and after anterior temporal lobectomy (ATL). The study population consisted of 22 patients with medically refractory MTLE who were candidates for ATL. Patients were examined before surgery as well as in the third and sixth months of the postoperative period. Psychiatric diagnosis was determined by using SCID-I. To rate the severity of psychiatric disorders, BPRS, HDRS, and HARS were employed on each visit. WHO-DAS-II and WHOQOL-BREF were used to determine the level of disability and quality of life. Preoperatively, six patients had a psychiatric diagnosis. Three months after surgery, six of the patients had psychiatric diagnoses. Five of these six patients had not been previously diagnosed. There was no significant difference between preoperative and postoperative follow-up evaluations in terms of HDRS, HARS, and BPRS ratings. With respect to the total scores and domains of WHO-DAS-II, the change in pre- and postoperative evaluations was statistically significant only for the social life attendance domain. There was no significant difference in the mean scores on the WHOQOL-BREF domains or on the first question about general evaluation of quality of life. For the second question on the level of satisfaction with health, the difference between the three ratings was statistically significant. Preoperative and postoperative rates of psychiatric disorders in our sample were low. While social phobia was frequently seen preoperatively, the postoperative period was spearheaded by major depressive disorder. The decrease in disability in attendance to social life and improvement in the quality of health were in concordance with the literature, indicating the positive results of surgical treatment of epilepsy on quality of life. This study suggests that surgical intervention might be one of the causes of postoperative psychiatric disorders in patients with MTLE.

Tectal Gliomas in Children: The Implications for Natural History and Management Strategy

Tumors involving the tectal region constitute a distinct subgroup of brain stem gliomas with an indolent clinical course. Here, we present the clinical and neuroradiologic features of 9 children with tectal tumors. All patients presented with signs and symptoms of hydrocephalus and were managed with ventriculoperitoneal shunt insertion. MRI studies revealed focal hyperintense lesions on T2-weighted images without any contrast enhancement, and no evidence of progression was demonstrated in any patient. We also reviewed the published series of tectal gliomas in the literature to compare with our results. Based on these and other published series, it was concluded that intrinsic tectal gliomas of childhood with sizes less than 2 cm in diameter and without any tumor extension or contrast enhancement constitute a specific subgroup of tectal masses which rarely display invasive clinical behavior and should be managed conservatively. CSF diversion procedures and long-term yearly follow-up examinations with MRI scans are sufficient in these patients.

Spinal injuries in the pediatric age group: a review of 82 cases of spinal cord and vertebral column injuries.

A review of 82 children with spinal cord and/or vertebral column injury treated in our department between 1968 and 1993 showed that 67% of the patients were boys and the average age was 11.4 years. The cause, vertebral level, and type of injury, and the severity of neurological injury varied with the age of the patient. The cause of pediatric injuries differed from that of adult injuries in that falls were the most common causative factor (56%) followed by vehicular accidents (23%). The most frequent level of spinal injury was in the cervical region (57%, 47 patients) followed by the lumbar region (16.5%, 13 patients). In our series, 18% of the patients had complete injury and the overall mortality rate was 3.6%. Eleven children (13%) had spinal cord injury without radiographic abnormality (SCIWORA), whereas 39 (47%) had evidence of neurological injury. Although the spinal injury patterns differed between children and adolescents, the outcome was found to be predominantly affected by the type of neurological injury (P<0.05). Children with complete myelopathy uniformly remained with severe neurological dysfunction; children with incomplete myelopathy recovered nearly normal neurological function. Finally, the authors conclude that most spinal injuries can be successfuly managed with nonoperative therapy. The literature is reviewed as to the treatment and outcome of pediatric spinal injuries.

L-2-hydroxyglutaric aciduria and brain tumors in children with mutations in the L2HGDH gene: neuroimaging findings.

L-2-Hydroxyglutaric aciduria (L-2-HGA, MIM 236792) is a rare autosomal recessive neurodegenerative disorder characterized by psychomotor delay, cerebellar and extrapyramidal signs and subcortical leukoencephalopathy with basal ganglia and dentate nuclei involvement. Mutations in the gene L2HGDH ( C14ORF160/DURANIN/) have been identified as causative for L-2-HGA. A feature disproportionally associated with L-2-HGA is the development of malignant brain tumors. In our cohort of 40 patients with L-2-HGA, two developed medulloblastoma and glioblastoma multiforme during the course of the disease. Two missense mutations in two patients were identified in the L2HGDH gene in exon 3 (c.292C-->T) and in exon 7 (c.887T-->A). Both mutations were present in the homozygous state. Serial MR imaging findings as well as MR spectroscopy imaging is reported in a patient who developed glioblastoma multiforme.

Arachnoid cyst associated with subdural hematoma: report of three cases and review of the literature

IntroductionArachnoid cysts are usually found incidentally and are generally asymptomatic. They are thought to be developmental anomalies, and their etiology is unknown. Some complications such as subdural hematoma, subdural hygroma, and intracystic bleeding can occur after minor traumas.Case reportHere, we report three cases. Case 1 has subdural hematoma associated with subdural hygroma, case 2 has subdural hematoma after a birth delivery, and case 3 has intracystic bleeding associated with subdural hematoma.DiscussionWe suggest to treat the complicating event in our patients and have chosen burr-hole evacuation for treatment.

Investigation of HSV-1, HSV-2, CMV, HHV-6 and HHV-8 DNA by real-time PCR in surgical resection materials of epilepsy patients with mesial temporal lobe sclerosis

OBJECTIVE The objective of this study is to investigate the presence of viral DNAs of HSV-1, HSV-2, HHV-6, HHV-8, and CMV in hippocampus of the patients with mesial temporal lobe epilepsy (MTLE) syndrome. METHODS Pathological specimens were obtained from 33 patients with MTLE undergone temporal lobectomy with amygdalo-hippocampectomy due to intractable seizures. Autopsy materials from the hippocampus of 7 patients without neurological disease were used as controls. The data was also correlated with the clinical history of patients including febrile convulsions, age, and history of CNS infections. Real-time polymerase chain reaction method was performed for detection of DNAs of these viruses. RESULTS HHV-6, HSV-1 and HHV-8 were detected in the hippocampus of 3, 2 and 1 patients with MTLE respectively. None of the hippocampus of patients with MTLE was positive for DNA of HSV-2 and/or CMV. Three patients with positive HHV-6 DNAs had febrile convulsions and family history for epilepsy. None of our control specimens showed PCR positivity to any of the 5 tested viruses. CONCLUSIONS Our study is the first to report the presence of HHV-8 viral genome in the brain tissue of patient with MTLE. Viral DNAs were detected in a total of 18% of the patients in this study; we can conclude that activity of the latent virus in patients with hippocampal sclerosis should be more extensively studied to establish its role in active infection.

Serial analysis of gene expression in the hippocampus of patients with mesial temporal lobe epilepsy

Hippocampal sclerosis constitutes the most frequent neuropathological finding in patients with medically intractable mesial temporal lobe epilepsy. Serial analysis of gene expression was used to get a global view of the gene profile in human hippocampus in control condition and in epileptic condition associated with hippocampal sclerosis. Libraries were generated from control hippocampus, obtained by rapid autopsy, and from hippocampal surgical specimens of patients with mesial temporal lobe epilepsy and the classical pattern of hippocampal sclerosis. More than 50,000 tags were analyzed (28,282, control hippocampus; 25,953, hippocampal sclerosis) resulting in 9206 (control hippocampus) and 9599 (hippocampal sclerosis) unique tags (genes), each representing a specific mRNA transcript. Comparison of the two libraries resulted in the identification of 143 transcripts that were differentially expressed. These genes belong to a variety of functional classes, including basic metabolism, transcription regulation, protein synthesis and degradation, signal transduction, structural proteins, regeneration and synaptic plasticity and genes of unknown identity of function. The database generated by this study provides an extensive inventory of genes expressed in human control hippocampus, identifies new high-abundant genes associated with altered hippocampal morphology in patients with mesial temporal lobe epilepsy and serves as a reference for future studies aimed at detecting hippocampal transcriptional responses under various pathological conditions.

Multilevel myelopathy in Maroteaux-Lamy syndrome and review of the literature

An 18-year-old male with Maroteaux-Lamy syndrome was presented with spastic quadriparesis. Magnetic resonance imaging of whole spinal canal revealed stenosis at multiple levels of cervical, thoracic and lumbar regions. By the guidance of combined evaluations of neurological examination, neuroradiological and electrophysiological findings, the most responsible spinal segment was detected each time he developed myelopathy and he underwent craniocervical, cervical and thoracolumbar decompressions consecutively. Ligamentum flavum hypertrophy was found to be the principal pathology responsible for the cord compression and myelopathy for all levels. The etiology of myelopathy and priority of the level for which decompression should be done in diffuse spinal stenosis were discussed with the literature review of Maroteaux-Lamy syndrome.