Aplasia ras homologous member I gene and development of glial tumors.

The ARHI (aplasia Ras homologue member I, also known as DIRAS3) gene shows 60.0% sequence homology to the Ras proto-oncogene and was the first mater-nally-imprinted tumor suppressor gene identified in the Ras family. It is constitutively expressed from the paternal allele in normal breast, ovary, heart, liver, pancreas, thyroid and brain tissues, and is lost or markedly down-regulated primarily in breast, ovarian, pancreas and thyroid tumor tissues. We have investigated the expression, LOH (loss of heterozygosity) and methylation status of this gene in glial tumors and peripheral blood samples of 21 patients, and in seven normal brain tissue samples. Gene expression by real time reverse transcriptase polymerase chain reaction (RT-PCR) was found to be increased in 14 and decreased in seven of the 21 tumors. The LOH was detected by fragment analysis, using five labeled polymorphic markers specific for the 1p31 region, in two of the tumors. Methylation status of the CpG island I, II and III was evaluated using COBRA (combined bisulfite restriction analysis) and RFLP (restriction fragment length polymorphism) in 21 tumors and also a hypermethylated healthy volunteer as a positive control, revealed that only two tumors had hypermethylation in CpG island I (of which one also had LOH). These results suggest that LOH and hypermethylation may be one mechanism of silencing the ARHI gene expression and development of glial tumor development.

Somatotropin adenoma and resistance to thyroid hormone.

Resistance to thyroid hormone (RTH) is a rare disease characterized by non-suppressed TSH in spite of high free thyroid hormone levels. Up to date, in the literature, there are more than 600 RTH cases, but co-incidental hypophyseal adenoma was reported in only 1 case. In the literature, despite reported cases with thyrotropinoma accompanying RTH, we could not find a case with somatotropinoma accompanying RTH. Here, we report a 34-yr-old male patient, who was admitted to the hospital with complaints of dyspnea, chest pain, and palpitation in 2003. His alpha- subunit value was normal and the alpha-subunit/TSH molar ratio was <1. His response to TRH stimulation test was normal. His TSH level was suppressed in the T3 suppression test. Hypophyseal magnetic resonance imaging showed a 6-mm hypophyseal microadenoma. Levels of all anterior hypophyseal hormones, including GH and IGF-I, were normal. Oral glucose tolerance test (OGTT)-GH suppression test was normal. The patient was followed with the diagnosis of RTH and incidental hypophyseal adenoma. After 3 yr, because of high levels of IGF-I: 901 ng/ml (68-324), the OGTT-GH suppression test was reported and no suppression was detected. Thus, the patient was referred to surgery with the pre-diagnosis of RTH and acromegaly. Immunohistochemistry was showed as strong GH staining with low Ki 67 index while TSH and other anterior hypophyseal hormones stainings were negative. Post-operative thyroid hormones were still high.

Ultra rapid spontaneous resolution of acute posttraumatic subdural hematomas in patient with temporal linear fracture.

We report a case of 57 year-old man with documented posttraumatic acute subdural hematoma and a linear temporal bone fracture. He suffered from a blunt head injury and presented with sudden loss of consciousness. Within 2 hours he became alert and oriented. Follow-up CT scan of brain 2 hours after the initial one showed resolution and redistribution of the subdural hematoma. To our knowledge, this is the first case in the literature about spontaneous resolution of an acute subdural hematoma in a patient with a linear fracture and the fastest resolution period. In this article, the authors discuss the underlying pathophysiology of this uncommon phenomenon.

Traumatic epidural haematoma of the posterior fossa in childhood: 16 new cases and a review of the literature.

A series of 16 children (nine males, seven females) with traumatic epidural haematoma of the posterior fossa (TEHPF) is presented. All patients had a history of mild to moderate trauma to the occiput. The causes were falls in 15 and traffic accident in one case. Four of the 16 cases had depressed fractures of the occipital bone. Surgical intervention was performed in all cases. In 14 patients the outcome was good, one patient had moderate disability and one died. Our study revealed that early diagnosis and urgent surgical intervention may give the patient a chance of total recovery and craniotomy with haematoma evacuation is an appropriate surgical technique as in the case of supratentorial extradural hematoma.

Intracranial Haemorrhage Probably Due to an Angiographically Occult AVM after Carotid Stenting. A Case Report.

SUMMARY: Angiographically occult vascular malformations refer to cerebrovascular malformations that are not demonstrable on technically satisfactory cerebral angiography. Authors herein present a very unusual intracranial bleeding complication related to an angiographically occult vascular malformation after extracranial carotid artery stenting procedure. A 52-year-old male patient admitted to the hospital with 2 episodes of amaurosis fugax in the left eye. Cervical carotid angiography and bilateral carotid Doppler ultrasonography revealed a 98% stenosis of the left internal carotid artery just distal to the bifurcation. Post-stenting control cervical carotid angiography revealed neither any residual stenosis nor a developmental venous anomaly. The patient developed left pupil dilatation with loss of consciousness two hours after the neurovascular intervention. Emergent cranial CT showed acute subdural haematoma, intracerebral and subarachnoid haemorrhage with massive midline shift. He underwent an emergent craniotomy with left temporal lobectomy. Abnormal cortical vascular structures with prominent engorgement were remarkable over the posterior temporal cortex. Histopathological studies confirmed the diagnosis of an occult AVM. Classically, these lesions are not visualized with angiography.Our patient's cerebral angiography and MR investigations were all normal. To our knowledge this is the first case in literature in which intracranial haemorrhage (acute subdural haematoma, intracerebral haematoma, SAH) occurred due to hyperperfusion of angiographically occult vascular malformation.

Endovascular treatment of kissing aneurysms at the fenestrated basilar artery. Case report with literature review.

BACKGROUND: Basilar artery fenestrations are most commonly located in the proximal basilar trunk close to the vertebrobasilar junction and may harbor saccular aneurysms. Surgical treatment of such aneurysms has been reported in several cases previously, despite the difficulty of the surgical approach. Endovascular treatment may provide a more convenient treatment alternative in such cases. This case is also interesting in that two discrete aneurysms arise from each limb of the fenestration. CASE REPORT: A 20-year-old man presented with acute subarachnoid hemorrhage. He had two kissing aneurysms at the fenestrated proximal basilar artery as demonstrated by MRA and selective angiography. The two aneurysms were successfully treated with GDCs via the contralateral vertebral arteries. One-year control angiogram showed stable occlusion. CONCLUSION: Aneurysms at the fenestrated basilar artery may be effectively treated with endovascular coil occlusion. The occurrence of multiplicity and the treatment alternatives are discussed, with relevant literature review.

Intraradicular disc herniations in the lumbar spine and a new classification of intradural disc herniations.

STUDY DESIGN: A case report of intraradicular disc herniation. Intraradicular disc herniation is a special type of intradural disc herniations. In this report, we present the tenth case of intraradicular lumbar disc herniation and suggest a new classification for intradural disc herniations. CASE REPORT: A 32-year-old male was admitted to hospital having experienced pain in the lower back and right leg for 1 month prior to admission. Neurological examination revealed weakness of the extensor hallucis longus, positive Laségue's sign, decreased ankle reflex in his right lower extremity, and bilateral paravertebral muscle spasm. Magnetic resonance imaging (MRI) revealed a disc herniation with a posterolateral extruded fragment on the right at the level of the L5-S1 space. He underwent L5 laminectomy. During the operation, the right S1 root was found to be swollen and immobile. A longitudinal incision was made in the dura of the right S1 root and an intradural free disc fragment was removed, and the S1 root was relieved. The patient was free of pain postoperatively. CONCLUSION: We suggest a new classification for intradural disc herniations with this unusual case presentation and review the literature for pathogenesis, clinical picture, diagnosis and treatment.

Cervical tuberculous spondylitis associated with systemic lupus erythematosus.

STUDY DESIGN: A case report of cervical tuberculous spondylitis associated with systemic lupus erythematosus (SLE). Infection is a frequent problem in SLE, especially in patients hospitalised with the complications of the disease. Tuberculous spondylitis very rarely occurs in SLE patients, and cervical involvement has not been previously reported. CASE REPORT: A 54-year-old female patient was admitted to our hospital with a complaint of neck pain radiating to her shoulder of 2 months' duration. The neurological examination was completely normal and radiological investigations revealed narrowing, angulation and destruction of the end plates of the 5th and 6th cervical vertebrae. She has received corticosteroid and colchicine treatment for the diagnosis of SLE during the last 10 years. The anterior cervical approach was used and pyogenic material was debrided from the C5-6 intervertebral space, and an otogenous bone graft with a Smith Robinson type fusion was performed. CONCLUSION: High doses of corticosteroids are implicated as a risk factor for infection in SLE patients. Early diagnosis and appropriate medical and surgical treatment, as well as increased awareness of higher susceptibility to opportunistic infections, such as tuberculous spondylitis, are keystones for decreasing morbidity and mortality in patients with SLE.

Parafalcine chondrosarcoma: an unusual localization for a classical variant. Case report and review of the literature.

BACKGROUND: Intracranial chondroid tumors are infrequently seen in neurosurgical practice. These tumors usually arise from cartilaginous synchondroses at the base of the skull, but occasionally from the pluripotential mesenchymal cells of the meninges. We present here a case of classic low-grade giant chondrosarcoma of the falx cerebri. This is only the second case of this variant reported in this location, and we summarize the diagnostic criteria with a brief review of literature. CASE REPORT: A 56-year-old female patient was admitted to the hospital with a history of progressive right-sided weakness occurring in the last 8 months and a recent grand mal seizure. Radiological evaluation demonstrated a large extra-axial mass in the left parafalcine area, suggesting a possible meningioma. An anterior interhemispheric approach enabled gross total removal of the tumor and a histologic diagnosis of a low-grade classic chondrosarcoma was made. The patient is currently stable and has shown no evidence of recurrence in more than 3 years without any adjuvant treatment. CONCLUSIONS: Intracranial cartilaginous tumors include classical, mesenchymal and myxoid chondrosarcomas in addition to benign chondromas. Parafalcine localization should be considered for all these variants as well as for meningiomas, hemangiopericytomas, solitary fibrous tumors, and meningeal metastatic carcinomas. Detailed radiological evaluation, light microscopic and ultrastructural analyses, and immunocytochemistry are essential for correct diagnosis. In contrast to mesenchymal and myxoid types, the prognosis of classic variants is usually good and does not require adjuvant treatment modalities if a radical resection of the tumor can be obtained. Increased documentation of clinical, radiological, and histologic findings as well as response to treatment modalities will provide a better understanding of the pathophysiology of these rare tumors, and highlight the optimum treatment strategies

Neurocutaneous melanosis associated with Dandy-Walker malformation. case report and review of the literature.

Neurocutaneous melanosis is a rare dysmorphogenesis associated with single or multiple giant pigmented cutaneous nevi and diffuse involvement of the leptomeninges anywhere in the central nervous system (CNS). It is interesting that almost 8-10% of patients had associated Dandy-Walker malformation in the literature, suggesting a common origin of the developmental abnormalities. In this article, we present a 2-year-old patient with neurocutaneous melanosis associated with Dandy-Walker malformation. We reviewed the literature and discuss the pathogenesis based on the preferred hypotheses so far.

Endoscopic evacuation of sacral hydatid cysts: case report.

A case of hydatid disease of the intra and parasacral area is reported. This rare localization was probably due to previous abdominal hydatid cyst surgery. The patient underwent surgery, with a small opening in the SI lamina and the cysts were removed totally with the aid of a rigid endoscope. The surgical technique used and MRI findings are discussed.

The effect of pinealectomy and foetal pineal transplantation of collagen ageing in rats.

Recent progress in pineal research emphasized the importance of this circumventricular organ in the phenomenon of ageing as well. The present study was undertaken to elucidate the effects of pineal gland on collagen ageing by spectrofluorometrically measuring collagen-cross-link bound fluorescein. Experiments were performed on three different age groups of rats (3-4 weeks, 8-10 weeks, 15 and more weeks). Fluorescein values were found to increase causally by age in the control group. Eight weeks after sham operation of all the groups fluorescein level increments were shown to be parallel with the values of normal physiological ageing values. In experimental groups; in 3-5 weeks old rats pinealectomy produced a significant premature collagen ageing and this was found to be completely reversed by foetal pineal gland transplantation, but the reversal was limited to a sham operation level. On the other hand although a premature collagen ageing was found to be induced 8 weeks after pinealectomy in 8-10 weeks old rats with a statistical significance both within subject values and between sham operation and pinealectomy, foetal pineal gland transplantation was found not to reverse the premature ageing induced by pinealectomy. In 15 weeks old and older groups of rats, pinealectomy was found to facilitate the intrinsic ageing phenomenon of collagen with statistical significance again both within subject values and between sham operation and pinealectomy. Foetal pineal gland transplantation was not performed in this age group of rats. On interpretation of the data of pinealectomy against sham operation for all the age groups, the maximum degree of percentage increase in collagen-cross-link bound fluorescein was found in 3-5 weeks old rats (%218). The percentage increase in fluorescein values was found to be %170 and %126 in 8-10 weeks old and 15 weeks old and older rat groups, respectively. Consequently, we have experimentally shown the induction of collagen ageing by pinealectomy and the restorative competence of foetal pineal gland transplantation in the present study. The results seem to be impressive and details of the ageing process are the subject of further research.

The effect of the pineal gland on liver regeneration in rats.

BACKGROUND/AIMS: We investigated the influence of pinealectomy on liver regeneration after partial hepatectomy in rats. METHODS: Thirty rats were allocated into four groups as follows: group 1 in which no operation was done (control), group 2 in which only midline scalp incision and craniotomy was performed (sham operation), group 3 in which pinealectomy was performed and group 4 in which the rats underwent pinealectomy and pineal transplantation. Eight weeks later all the rats underwent 70% hepatectomy. RESULTS: Hepatic regenerative capacity was observed 24 h after PH by bromodeoxyuridine incorporation into DNA and the mitotic index of hepatocytes. The bromodeoxyuridine labeling indices were 45.4, 35.8, 19.9 and 36.8 in groups 1, 2, 3 and 4, respectively. The mitotic indices of the same groups were 31.2, 28.7, 8.3 and 13.4, respectively. Both the bromodeoxyuridine labeling and mitotic indices were significantly lower in the pinealectomized rats than in the control and sham operation groups. CONCLUSIONS: These results suggest that the pineal gland stimulates liver regeneration after partial hepatectomy in rats.

Pulmonary embolism in neurosurgical patients.

BACKGROUND: Pulmonary embolism continues to be a frequent cause of morbidity and mortality in neurosurgical practice. This study was designed to investigate the presence of signs and/or symptoms helpful for an early diagnosis of pulmonary embolism and to evaluate the safety of heparin treatment in neurosurgical patients. METHOD: We retrospectively reviewed 37 patients with pulmonary embolism among 6081 operated patients within the past 8 years at the Department of Neurosurgery, University of Hacettepe. RESULTS: The overall incidence of pulmonary embolism was 0.42% and the mortality rate was 59.4%. The patients with cerebrovascular lesions had a significantly higher risk of pulmonary embolism compared with other neurosurgical pathologies. All patients were treated with heparin and subsequent oral warfarin therapy. None had intracranial or intraspinal bleeding while receiving anticoagulant therapy. CONCLUSIONS: This study demonstrates that pulmonary embolism has a high mortality rate and heparin therapy can be used safely and without fear of intracranial or intraspinal bleeding.

Brucellosis of the spine.

The spinal form of brucellosis is still a disabling disease in many countries outside North America and northern and central Europe. Fifteen consecutive cases of spinal brucellosis diagnosed and treated over a 20-year period were reviewed retrospectively. Six patients were farmers, while 10 patients had a history of ingestion of unpasteurized milk or other dairy products. A high index of suspicion is necessary for the diagnosis, since there are no pathognomonic signs or symptoms. Radiological assessment of the disease was reviewed and highlights in the differential diagnoses were stressed. The diagnosis was based on actual culture of Brucella bacilli in seven patients. The principal treatment of brucellosis of the spine is conservative, namely, immobilization and antimicrobial therapy. We have found both a combination of ofloxacin and rifampin and ofloxacin monotherapy efficient as the early regimens used in this series. Three patients had to undergo surgery, since a diagnosis could not be made in any other way.

Status epilepticus following iohexol myelography.

Iohexol, a contrast medium widely used for myelography, has rarely been reported to cause convulsive disorders. A case of status epilepticus resulting from iohexol myelography is reported and problems of treatment are discussed.

An unusual dural arteriovenous fistula in an infant.

Dural arteriovenous fistula, (AVF), a rare entity, presents most commonly in adults. An 11-month-old boy presented with symptoms of congenital toxoplasmosis associated with an extensive dural AVF of the torcular Herophili and bilateral occlusion of the transverse and sigmoid sinuses. His intracranial venous drainage had become rerouted via the cavernous sinuses to the ophthalmic veins. The relationship of toxoplasmosis and sinus thrombosis to the pathogenesis of dural AVF and their clinical and radiological features are discussed.

Chondrosarcoma of the temporal bone in an infant: case report and review of the literature.

Cranial chondrosarcomas are rare. The majority of cases arise from the base of the skull and are most common in adult life. In this report, an infant with a chondrosarcoma of the temporal bone is discussed. We are not aware of a previously reported case of chondrosarcoma of the temporal bone in this age group.

Occipital depression fractures in childhood. A report of 14 cases.

In this paper, we present 14 cases with depression fracture of the occipital bone in pediatric age. There were 7 males and 7 females. Their ages ranged from 1 year to 15 years with a median of 5.8 years. The most common causes were: falling (8 cases); traffic accidents (2); assault (2 cases). Five of our 14 cases had compound fractures and the remaining 9 had closed fractures. Epidural and subdural hematomas were observed in 5 and 1 cases, respectively. There was significant improvement in 11 cases; two patients died.