PARANEOPLASTIC OPTIC NEUROPATHY AS AN INITIAL CLINICAL MANIFESTATION OF SMALL CELL LUNG CANCER. A CASE REPORT.

Paraneoplastic optic neuropathy (PON) is a very rare condition. In this study, a case of PON whose first complaint was painless vision loss in one eye is presented. In the follow-up of our case, optic neuropathy developed in the fellow eye. Electromyography examination performed due to diffuse body pain and motor loss in the left extremity is compatible with peripheral sensorimotor polyneuropathy. Lung biopsy was planned due to EMG result and and lymphadenopathy detection in thorax computed tomography (CT). The biopsy result of the patient was reported as nonspecific hyperplasia. As the patient's complaints increased, the paraneoplastic antibody panel was requested and CV2 / CRMP5 antibody was found positive. Thereupon, as a result of repeated biopsy, our patient was diagnosed with small cell lung cancer. We think that paraneoplastic optic neuropathy should be considered in the differential diagnosis in patients with advanced age, smoking, painless subacute vision loss, optic disc swelling, and we should insist on research in this direction as in our case.

Intracranial chondrosarcoma: a case report and review of the literature.

INTRODUCTION: Chondrosarcoma is a rare malignant tumor originating from cartilagenous tissue. It tends to localize in the epiphysis of long bones and pelvic bones. Only 7% of the all chondrosarcomas originate in the craniocervical region. CASE REPORT: A 25-year-old man was admitted to our hospital with headache and dizziness. Physical examination and neurological examination were normal. Skull radiography revealed a radioopaque lesion in the right parieto-occipital region. Cranial computed tomography and magnetic resonance imaging showed a mass lesion including calcification areas and homogenous contrast enhancement in the right parieto-occipital region. The tumor was removed totally by a microsurgical technique. A classical type chondrosarcoma was confirmed histopathologically. The patient was discharged with no neurological deficit. DISCUSSION: Intracranial chondrosarcoma has been first reported by Mott in 1899. Primary intracranial chondrosarcomas, constitute only less than 0.16% of all brain tumors. Most of them are located at the skull base. The choroid plexus, dura mater and brain parenchyma are extremely rarely invaded. Meningiomas, solitary fibrous tumor, chordoma, hemangiopericytoma, metastasis and vascular malformations should be considered as differential diagnoses. Radical surgical removal of the tumor is the preferred management procedure. Chemotherapy and radiotherapy may by added as adjuvant therapy. We present the case of a patient with an intracranial chondrosarcoma, who has treated successfully with surgical removal.

Cervical segment nervus vagus neurofibroma with associated neurofibromatosis type 1: a case report.

INTRODUCTION: Neurofibroma occurs as an isolated or multiple lesions frequently associated with neurofibromatosis type 1 (NF-1). Neurofibroma is a benign peripheral nerve sheath tumor composed of a variable mixture of Schwann, perineurial-like, and fibroblastic cells, as well as ones with features intermediate between these various cells, immersed in a collagenous or myxoid matrix. CASE REPORT: A 10-year-old boy visited the outpatient clinic with complaints of swelling and pain on the left side of his neck, which has been present for a year. A mass was discovered adjacent to the left internal carotid artery (ICA) and was removed by a microsurgical technique. No postoperative neurological deficit was identified and the pathological diagnosis was once more reported as neurofibroma. DISCUSSION: Nerve sheath tumors may be observed in patients with von Recklinghausen's disease, although vagal neurofibromas are noted more frequently than schwannomas among these patients. For unknown reasons, neoplasms associated with von Recklinghausen's disease occur more frequently on the right side. Cervical vagal neurofibromas most often present as asymptomatic, slowly enlarging, lateral neck masses. When symptoms are observed, hoarseness is the most common. Dysphonia, dyspnea, dysphagia, cough, syncopal episodes, tongue weakness and atrophy, and Horner's syndrome have also been reported as presenting complaints.

Multiple aneurysms of the distal posterior inferior cerebellar artery: two case reports.

INTRODUCTION: The distal segment of the PICA (posterior inferior cerebellar artery) is a very rare localization for the occurrence of intracranial aneurysms. They are almost always presented with SAH (subarachnoid hemorrhage). Hydrocephalus often develops following intraventricular hemorrhage (IVH). The distal PICA segment is a quite difficult localization for neurosurgical procedures. The authors could not find any case report presenting multiple aneurysms on a distal segment of PICA in the literature. CASE 1: A 62-year-old male patient was admitted to the emergency room with complaints of sudden headache, vomiting and blackout of consciousness. Early CT scans demonstrated SAH and intraventricular hemorrhage (IVH). In his medical history, SAH had been determined in our clinic 3 month previously. He had carried out conservative treatment ever since the distal located PICA aneurysm had been determined. There was no determined symptom except for moderate neck stiffness in his physical examination. In his neurological examination, he was stuporous and he localized the pain in his left arm. A ventriculo-atrial shunt operation was performed after the external ventricular drainage which was applied at the acute period of hydrocephalus in his previous SAH. Both of the left distal PICA aneurysms were clipped via a left lateral suboccipital craniotomy. The patient was discharged from the hospital without neurological deficit in the seventh day postoperatively. His control examination was normal and there was not any fixed pathological filling in the control cerebral digital subtraction angiography (DSA). CASE 2: A 49-year-old female patient was admitted to the emergency service with the complaint of headache, dizziness and vomiting. She was neurologically intact except for neck stiffness. A cranial CT demonstrated triventricular hydrocephalus and a hemispheric cerebellar hematoma, which was about 1 x 1.5 cm in diameter. The cerebellar DSA was normal. The patient was discharged with no neurological impairment. In the examination of the control cerebral DSA, two aneurysmatic fillings were determined in the distal segments of right posterior inferior cerebellar artery (PICA). Both of the aneurysms were clipped through a microsurgical approach. No neurological deficit was determined in the postoperative early and late stages. In the control cerebral DSA, no aneurysmatic filling was seen. DISCUSSION: Distal PICA aneurysms constitute only 0.28-1.4% of all intracranial aneurysms. Most of the patients present with SAH and along with this; intraventricular hemorrhage is seen and later on, hydrocephalus develops. As for treatment, surgery and endovascular interference methods can be applied.

Osteoblastoma of the temporal bone: a case report.

Benign osteoblastoma is an unusual primary bone tumor. It affects usually long bones or the vertebral column. Osteoblastoma affecting the calvarium is extremely rare. A 23-year-old man presented with a two-month history of swelling and tenderness in the right temporal region. Computed tomography revealed an expansile, lytic lesion in the left temporal bone. T1- and T2-weighted magnetic resonance (MR) images demonstrated an isointense lesion in the temporal bone. After gadolinium DTPA injection, MR images revealed dense enhancement. The histopathological findings proved that the lesion was a benign osteoblastoma. To date, very few cases of the benign osteoblastoma arising in the calvarium have been reported in the literature. We discuss diagnostic and management aspects of this uncommon tumor.

Hydatid cysts in uncommon locations in the same patient: simultaneous cardiac and spinal involvement.

INTRODUCTION: The spine and cardiac cavities are uncommon locations for hydatid disease. Spinal and cardiac hydatid cases separately constitute only 0.5-1% of all hydatid cases. We reported a case with spinal and cardiac involvement simultaneously. Clinical and radiological findings of this unique case are discussed. CASE REPORT: A thirty-four-year-old male patient was admitted to hospital with pain at the low back and left thigh. He had undergone surgery for lumbosacral hydatidosis twice before. Cysts had been removed totally in the last operation. The disease relapsed with widespread involvement of the sacropelvic region. During the investigation, we diagnosed a cyst in the cardiac cavity, incidentally. The cyst in the cardiac cavity was removed totally. DISCUSSION: Hydatid disease is still an endemic disease in South America and some Mediterranean countries including Turkey. Cysts rarely involve the spine and cardiac cavities. The simultaneous involvement of the sacropelvic region and the cardiac cavity is an extremely rare condition. Cardiac cysts have a poor prognosis. Prompt surgical extraction of the cyst is a critical mainstay of the management.

Cauda equina syndrome caused by primary lumbosacral and pelvic hydatid cyst: a case report.

INTRODUCTION: Hydatid disease occurs in humans as a result of faeco-oral contamination and spinal echinococcosis is rare even in areas where echinococcosis is endemic. Hydatid cyst primarily occurs in the liver and lungs. Bone involvement constitutes only 0.5-2% of all hydatidoses. About half of the bone involvement occurs in vertebrae. The thoracic spine is the most common site of the spinal hydatidosis. Primary hydatid cysts of the lumbar and sacral spinal canal are very rare. CASE REPORT: We present a 31-year-old man with cauda equina syndrome caused by a primary hydatid cyst of the lumbosacral and pelvic areas. He had been admitted to hospital with left foot and low back pain three years ago. Magnetic resonance imaging revealed an intraspinal hydatid cyst extending from L2 to S2. The cyst had been totally removed. He was symptom-free for three years. After three years, he presented with acute cauda equina syndrome. His neurological examination revealed total plegia of dorsal flexion of the left foot and perianal hypoaesthesia. MRI showed lumbosacral and pelvic hydatidosis again. After total removal of the cyst, his neurological status revealed immediately relief. DISCUSSION: Hydatid cyst is an important health problem in some countries including Turkey. Bone involvement is seen in only 0.5-2% of cases. Furthermore sacral and lumber vertebral involvement is extremely rare. We presented a case with a spinal hydatid cyst which classified as a combination of intraspinal extradural, vertebral and paravertebral forms according to the Braitwate and Lees classification. Surgical excision and additional medical treatment is still the most effective treatment. Cysts located intraspinally have a tendency to rupture spontaneously. For this reason the high recurrence rate (30- 40%) is still a major problem in management.

Agenesis of the left internal carotid artery associated with right anterior cerebral artery A1 segment bifurcation aneurysm: a case report.

Agenesis of the internal carotid artery occurs in less than 0.01% of the population. Intracranial aneurysms accompany agenesis in 25-43% of the patients. We present a unique patient with a saccular aneurysm of the anterior communicating artery associated with agenesis of contralateral internal carotid artery.

Image-guided microneurosurgical management of small arteriovenous malformation: role of neuronavigation and intraoperative Doppler sonography.

INTRODUCTION: The goal of this study was to evaluate the efficacy and reliability of neuronavigation and intraoperative microvascular Doppler sonography (MDS) for identifying afferent (feeding) and efferent (draining) vessels as well as for controlling the totality of the surgical resection of arteriovenous malformations (AVMs). METHODS: Between June 2000 and November 2005, twenty-five patients with small arteriovenous malformations (grades I-III) underwent microsurgical removal at our institution. A passive-marker-based neuronavigation system (Brain Lab, Munich, Germany), and an intraoperative MDS (Multi Dop X system, DWL, Germany) were used in this surgery. Blood flow velocities (BFV) in afferent and efferent vessels were recorded before and after removal of AVM. The preoperative neurological status and postoperative outcome were recorded. Patient follow-up monitoring ranged from 4 months to 3 years (mean: 16 months). RESULTS: The calculated registration accuracy of the neuronavigation computer ranged between 0.2-1.7 mm (mean: 1.1 mm). Before AVM removal the mean BFV of afferent vessels was 56.5+/-13.4 (28-98 cm/s) and the PI varied by 0.40+/-0.11 (0.25-0.66), after AVM removal these values reduced to 4.8+/-0.8 cm/s and 0.26+/-0.05, respectively. Similarly, before AVM removal, the mean BFV of efferent vessels was 13.5+/-4.5 (4-20 cm/s) and PI was 0.4+/-0.2 (0.34-0.56), after AVM removal both BFV and PI were not recorded. Complete removal of the AVMs was accomplished in 24 (96%) out of 25 patients which was confirmed with postoperative digital subtraction angiography (DSA). While there was no mortality, three patients (12%) had a worsening in their neurological status after surgery. CONCLUSION: Image-guided microneurosurgery with intraoperative MDS is a safe, effective, and reliable method for identifying the afferent and efferent vessels and for confirming the complete resection of AVMs. These benefits of image-guided microsurgery were most apparent for small, deep-seated AVMs that were not visible on the surface of the brain. In addition these techniques reduce the operative time and blood loss during AVM resection.

Factors leading to hydrocephalus after aneurysmal subarachnoid hemorrhage.

Hydrocephalus is not a rare complication following aneurysmal subarachnoid hemorrhage. Hydrocephalus following subarachnoid hemorrhage can progress acutely (0-3 days), subacutely (4-13 days) or chronically (after 13 days). The predisposing factors leading to hydrocephalus after subarachnoid hemorrhage are not known exactly. This study assessed the predictive factors for the development of this condition. All patients presenting with subarachnoid hemorrhage between 1992-2001 were evaluated. All of them had initial computed tomography (CT) and hydrocephalus was diagnosed on CT scans. Age, gender, preexisting diabetes mellitus and hypertension, neurological state according to the Hunt and Hess scale at admission, Fischer grade on CT, the presence of intraventricular hemorrhage and localization of aneurysm were analyzed to see if there was any meaningful relationship between hydrocephalus and these factors. One hundred and fourteen patients with aneurysmal subarachnoid hemorrhage were evaluated. The incidence of hydrocephalus was 28.1 %. The incidence for acute hydrocephalus was 18.4 %, for subacute 5.2 % and for chronic 4.3 %. Sixty-nine percent of patients with hydrocephalus were graded as 3, 4 or 5 according to the Hunt and Hess scale on admission. Fifty-five percent of patients with hydrocephalus were graded as 3 and 4 according to Fisher grade on initial CT scan. Preexisting diabetes, higher Fisher grade and intraventricular hemorrhage were statistically significant predictors for the development of hydrocephalus. But only preexisting diabetes and higher Fisher grade were independent predictors according to multivariate analyses.

Cerebral vasculitis and ischaemic stroke in Behçet's disease: report of one case and review of the literature.

INTRODUCTION: Behçet's disease (BD) is a multisystemic, recurrent, inflammatory disorder. Neurological involvement is well-known but cerebral vasculitis and ischaemic stroke are unusual. CASE DESCRIPTION: A 43-year-old male patient presented with acute left hemiparesis, he had recurrent oral aphthae and scrotal ulcerations. Two episodes of transient brainstem ischaemia and an episode of right hemiparesis were reported in the past 2 years. Cranial magnetic resonance (MR) imaging showed a right striatocapsular infarction and multiple segmental stenosis, fusiform enlargement and beading of the arteries of the polygone of Willis were seen on angiography. Cerebro-spinal fluid (CSF) examination disclosed lymphocytic pleocytosis. Skin pathergy test was positive. A diagnosis of BD with cerebral vasculitis was made and immunosuppressive therapy was started. Some improvement of the arterial lesions on MR angiography and normalization of CSF were observed after 1 year of treatment. DISCUSSION: Low grade chronic meningo-encephalitis is the core neuropathological process in neuro-Behçet's disease. Nevertheless BD is a systemic disease known to cause vasculitis and can exceptionally lead to cerebral vasculitis and brain infarction. While BD is usually not part of the differential diagnosis of cerebral vasculitis, it should be borne in mind especially in endemic areas of the disease and in patients from these areas.