Treatment of amyotrophic lateral sclerosis patients by autologous bone marrow-derived hematopoietic stem cell transplantation: a 1-year follow-up.

BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder characterized by progressive loss of spinal cord and cortical motoneurons. Despite improved understanding of the mechanisms underlying ALS, in clinical practice the management of ALS remains essentially supportive and focused on symptom relief. However, over the past few years stem cell research has expanded greatly as a tool for developing potential new therapies for treating incurable neurodegenerative diseases. METHODS: Thirteen patients with sporadic amyotrophic lateral sclerosis (SALS) were included in this study, and bone marrow (BM)-derived hematopoietic progenitor stem cells were used. We selected patients with bulbar involvement and severe loss of movement. Our aim was to put the stem cells into the end of the brain stem and at the beginning of the spinal cord because the blood-brain barrier is intact in ALS and this region was the most affected part in our patients. Under general anesthesia, a total laminectomy was performed at the C1-C2 level. Stem cells were injected to the anterior part of the spinal cord. RESULTS: During the follow-up of 1 year after stem cell implantation, nine patients became much better compared with their pre-operative status, confirmed by electro neuro myography (ENMG). One patient was stable without any decline or improvement in his status. Three patients died 1.5, 2 and 9 months, respectively, after stem cell therapy as a result of lung infection and myocardial infarction (MI). DISCUSSION: These results show that stem cell therapy is a safe, effective and promising treatment for ALS patients.

Treatment of chronic spinal cord injured patients with autologous bone marrow-derived hematopoietic stem cell transplantation: 1-year follow-up.

BACKGROUND: Transplanted bone marrow (BM) cells have been found to improve neurologic disease in central nervous system (CNS) injury models by generating neural cells or myelin-producing cells. The results in treated patients and animal models suggest that BM cells could potentially be used as a therapy for spinal cord injury (SCI) patients. METHODS: Nine patients with chronic complete SCI with American Spinal Injury Association (ASIA) Impairment Scale (ASIA) grade A were included in this study. They were treated with autologous BM-derived hematopoietic progenitor stem cell transplantation without any serious complications. All patients completed the protocols successfully. RESULTS: Three weeks after the operation all patients' movements and sensations were improved. All patients had ASIA grade B or C after the operation. DISCUSSION: We used autologous hematopoietic progenitor stem cells in order to avoid the problems associated with immunologic rejection and graft-versus-host (GvH) reactions, which are frequently caused by allografts. The advantage of this type of cell therapy is that it is not associated with carcinogenesis, which sometimes occurs with embryogenic stem cell therapy. To evaluate the patients we used neurologic impairment scales (ASIA scores), pre- and post-operative Somato Sensorial Evoked Potential (SSEP) assessments and pre- and post-operative Magnetic Resonance Imaging (MRI). All the data showed that BM-derived autologous stem cell therapy is effective and safe for the treatment of chronic SCI.

Critically located cavernous malformations.

INTRODUCTION: The aim of this study was to evaluate the clinical results of patients who underwent resection with the aid of microsurgical techniques and stereotactic and image-guided surgery for critically located cavernous malformations which still represent a considerable surgical challenge due to the close proximity of vital and eloquent structures. METHODS: Between 1997 and 2003, 12 patients with critically located cavernous malformations (CMs) underwent surgical resections at Ankara University Hospital. CMs of the pons (n=3), medulla oblongata (n=1), cavernous sinus (n=3), motor cortex (n=4) and the newly defined superior cerebellar peduncle (n=1) were treated using image-guidance and advanced microsurgical principles. Preoperative assessment was done with CT, MRI and angiography. Lesion locations, clinical presentations and outcome were analyzed. The surgical approach was chosen as lateral suboccipital (n=4), parietal (n=4), cranio-orbitozygomatic (n=3) and retrosigmoid (n=1). RESULTS: All CMs were readily identified and completely removed with no permanent morbidity and mortality. The immediate outcome after surgery was improved for 8 patients (66.6%). Long-term outcome was unchanged for one patient and a proved good surgical outcome for three patients, during the mean follow-up period. DISCUSSION: Stereotactic methods together with image-guidance and microsurgical techniques allow the creation of most effective and safe corridors to access the CMs in eloquent regions with a minimization of tissue manipulation and low risk of permanent neurological deficit.

Tuberculosis of the craniocervical junction: a case report.

Craniocervical tuberculosis (TB) is very rare. Despite the use of magnetic resonance imaging (MRI) and cranial tomography (CT), diagnosis of craniocervical tuberculosis is frequently difficult. In this study, we present a craniocervical tuberculosis abscess case which demonstrates the role of transoral surgery for both diagnosis and treatment.

Communication in science.

Science must have a common language. For centuries, Latin language carried out this job, but the progress in computer technology and internet world through the last 20 years, began to produce a new language with the new century; the computer language. The information masses, which need data language standardization, are the followings; Digital libraries and medical education systems, Consumer health informatics, Medical education systems, World Wide Web Applications, Database systems, Medical language processing, Automatic indexing systems, Image processing units, Telemedicine, New Generation Internet (NGI).

Comprehensive microsurgical anatomy of the jugular foramen and review of terminology.

The microsurgical anatomy of the jugular foramen was studied in 12 formalin preserved cadavers (24 foramina) and 40 dry-skulls (80 foramina). The jugular foramen was exposed by microsurgical dissection with drilling from a superior to inferior direction. Observations regarding dural architecture of the jugular foramen and relationships between neurovascular structures passing through the foramen were noted in cadavers. Normal bony construction of the foramen and its variational anatomy were examined in dry-skull specimens. Using photographs and drawings, the anatomy of the jugular foramen is presented and related terminology is discussed in the light of a literature review.

Anteromedial approach to the orbit.

This study evaluated the surgical results of the anteromedial approach for treatment of orbital lesions in 16 patients. Pre- and postoperatively, all patients underwent a complete physical examination focusing on the head and neck area including a thorough ophthalmologic evaluation, computerized tomography, and magnetic resonance imaging. The surgical approach was limited to a medial orbitotomy in five patients; the remaining 11 patients underwent a medial orbitotomy combined with an external sphenoethmoidectomy. The tumor was removed completely without damaging the intraorbital neurovascular structures in all but one patient whose recurrent clival chordoma extended beyond the limits of an extracranial approach. Fibro-osseous lesions, cavernous hemangiomas, and dermoid cysts were the most common pathologies. The follow-up ranged from 18 to 48 months, and no patient has shown evidence of a recurrence. One patient with a clival chordoma received radiation therapy. The lateral nasal skin incision healed with acceptable cosmetic results. The anteromedial approach to the orbit provides a wider working space and direct exposure while protecting neurovascular structures.

Zygomatic anterior subtemporal approach for lesions in the interpeduncular cistern.

The interpeduncular cistern is a difficult region to approach through conventional methods due to its deep location and important adjacent neurovascular structures. Therefore, it is usually difficult to expose the region sufficiently. Technical problems associated with various surgical approaches have led to emergence of combined approaches and their modifications (i.e., the removal of the zygomatic arch). In addition, a frontotemporal craniotomy is reported to provide a wide exposure of the anterior temporal base, thus allowing oblique access to the interpeduncular cistern with minimal brain retraction. This study describes clinicians' experience and the surgical results of 24 patients who underwent a zygomatic anterior subtemporal approach.

Magnetic resonance imaging and computerized tomography images in a case of pituitary abscess.

A 29-year-old male patient with clinical manifestations of panhypopituitarism and diabetes insipidus is presented. The clinical and laboratory evaluation of the pituitary reserve confirmed a total pituitary insufficiency. Computed tomography (CT) scan demonstrated a low-density lesion suggesting a pituitary tumor with suprasellar extension, and magnetic resonance imaging (MRI) revealed a pituitary mass with decreased signal intensity on T1-weighted images and capsular contrast enhancement after gadolinium injection. The surgical procedure was the treatment of choice. During surgery a large amount of purulent material was removed. With light microscopy, chronic non-specific inflammation and a pituitary abscess capsule were demonstrated in the tissue. The results of the cultures revealed coagulase negative staphylococcus. The post-operative course was uneventful and, as the patient was pituitary-deficient, he was on replacement therapy and was being followed-up. Pituitary abscess is an exceptional lesion in the literature and despite the advent of CT and MRI, its preoperative diagnosis still remains difficult. However, the presence of an intrasellar expansive process with liquid center and contrast enhanced outline should suggest the possibility of an abscess. Sellar round cystic mass isointense or hypointense to grey matter on T1, high intensity signal on T2, or opposite pattern with a peripheral rim enhancement following gadolinium injection, and diabetes insipidus may all be suggestive of a pituitary abscess.

The neurosurgical management of prolactinomas.

BACKGROUND: The objective was to discuss the neurosurgical management of the prolactinomas. METHODS: Five-hundred-fifty patients suffering from prolactinoma were treated with trans-sphenoidal and transcranial approach. The diagnosis of prolactinoma was based on various degree of high level prolactinemia, galactorrhea, gonodal disturbance, neurological examination and radiological findings. In all cases the adenoma was histologically verified. The patients were investigated according to the anatomo-radiological classification of Hardy and Vesina, and the range of preoperative PRL basal levels. RESULTS: Follow-up was ascertained in 81% of patients who were followed for a mean of 7.2 year (1-10 year). While the total removal percentage was 98% in the group with microprolactinoma, this ratio dropped to 63.9% for macroadenomas and 23.5% for giant adenomas. Early improvement of prolactin level ratio was 81.6% in microprolactinomas, 28.3 in macroadenomas and 11.7% in giant adenomas. Hormonal cure was 64.3% in microadenomas, 6.7% in macroadenomas and 0% in giant adenomas. The ratio of hormonal cure was decreasing in patients with high prolactin levels. In the follow-up recurrence of prolactinomas occurred in 39% of the patients. CONCLUSIONS: Medical treatment is the first step in prolactin secreting adenomas. Trans-sphenoidal microsurgery became popular in treatment of prolactinomas because of low operative morbidity and mortality. Patients with recurrence should be evaluated for second step treatment (surgery, bromocriptine, or radiotherapy).

Anatomical study of the lateral wall of the cavernous sinus, emphasizing dural construction and neural relations.

This study aims to determine the microscopic anatomy of the layers of the lateral wall of the cavernous sinus (CS) and, in particular, intends to examine the location and relations of the dural openings on the deep layer. Forty sides of 20 formalin-fixed and fresh cadavers were dissected and their CS examined. In 12 cases we found an opening on the deep dural layer; however, in four of them the inferolateral trunk of the internal carotid artery (ICA) was identified through these dural openings. We noticed the trochlear nerve making a curve (5% of cases) or lying close to the ophthalmic nerve (12.5%) on the lateral wall. In one case, the triangular area described by Parkinson could not be exposed surgically. Our findings indicate the importance of the heterogeneous courses of the cranial nerves lying on the lateral wall and point to the significance of the dural openings, which can influence the etiology of neoplastic invasions originating from the CS.

Radiological anatomy of the intratemporal course of facial nerve.

Preoperative evaluation of the facial nerve (FN) anatomy within the temporal bone by high-resolution computed tomography (HRCT) helps in minimizing surgical trauma to the nerve. In order to demonstrate the radiological correlation of the intratemporal FN, eight adult, formalin-preserved cadavers were studied by comparing the transaxial and coronal sections of HRCT with anatomic microdissection findings. It was possible to visualize all segments of the FN canal in its intratemporal course. The most difficult part of the FN to demonstrate was the pyramidal section. Anatomic microdissection findings were consistent with the HRCT images. It was concluded that adequate information on the FN anatomy could be obtained from standard HRCT scans.

Lymphocytic hypophysitis and infundibuloneurohypophysitis; clinical and pathological evaluations.

This report describes the clinical and pathological characteristics of two patients with lymphocytic hypophysitis (LHy) and two with infundibuloneurohypophysitis (INHy). Two of the patients were women and two were men, and their ages were between 27 and 38 years old. This disease was not associated with either pregnancy or the postpartum period in the female patients. Two of the patients presented with diabetes insipidus, one with panhypopituitarism and right abducens paralysis and one with headache and galactorrhea. At presentation three of the patients had mild to moderate hyperprolactinemia and one had low prolactin levels. All four had abnormal magnetic resonance imaging (MRI): focal nodular enlarging of the infundibulum and normal hypophysis in one, expanding sellar masses in two, and diffusely thickened stalk with slightly enlarged pituitary gland in one. Three cases showed no sign of adenohypophysial deficiency with stimulation tests. One patient had associated chronic lymphocytic thyroiditis. Of the first three patients, one patient underwent transcranial and two underwent transnasal transsphenoidal (TNTS) surgery for mass excisions since they were thought to have pituitary tumors. Endoscopic endonasal transsphenoidal biopsy was performed in the last one with a suspicion of LHy. The pathological and immunohistochemical examinations revealed lymphocytic infiltration. Hyperprolactinemia resolved with surgery in two patients and one developed diabetes insipidus as a complication. We conclude that LHy and infundibuloneurohypophysitis should be considered in the differential diagnosis of the mass lesions of the sellar region and also should be kept in the mind for the etiopathogenesis of cases of hyperprolactinemia, galactorrhea and diabetes insipidus. In suspected cases endoscopic endonasal biopsy for the histopathological diagnosis can be a safe approach.

Differential diagnosis of idiopathic inflammatory trigeminal sensory neuropathy from neuroma with a biopsy: case report.

OBJECTIVE AND IMPORTANCE: Idiopathic inflammatory trigeminal sensory neuropathy (IITSN) is a disorder with the dominant clinical features of trigeminal sensory disturbance; this idiopathic condition follows a benign course in most cases. Recent reports have shown that transient abnormalities, which may mimic those of trigeminal neuromas, can be observed in magnetic resonance imaging scans. Presented here is a case of IITSN that was diagnosed, with cytological and histopathological verification, during the active inflammatory phase of the disease (the first such attempt, to our knowledge). CLINICAL PRESENTATION: A 20-year-old female patient was referred to our hospital with a 2-month history of numbness of the left side of her face, headache, and hemifacial pain attacks. Cranial magnetic resonance imaging scans revealed a mass above and below the foramen ovale, extending into the cavernous sinus. INTERVENTION: A percutaneous biopsy procedure through the foramen ovale was performed; the pathological examination revealed lymphocytes, macrophages, and endothelial cells but no evidence of neoplastic cells. A few days later, the patient was surgically treated using a cranial base approach, the gasserian ganglion was exposed, and the lesion was removed. Pathological examination of the specimens revealed inflammatory changes and fibrosis of the nerve fibers and ganglion cells. Disruption of the myelin around the nerve bundles was detected. Therefore, IITSN was pathologically confirmed during the early stage of the disease. During 3 months of follow-up monitoring, the patient experienced no serious clinical problems. CONCLUSION: IITSN should be suspected in cases of tumors involving the cavernous sinus, and a percutaneous biopsy through the foramen ovale should be performed as part of the differential diagnosis in such cases. This procedure might obviate unnecessary aggressive surgery. In the current case, no neoplastic cells were observed during the examination; only lymphocytes, macrophages, and endothelial cells were observed, on a background of erythrocytes. Lymphocyte-dominant inflammatory infiltration, fibrotic changes, and demyelinization are cardinal histopathological findings observed during the active phase of IITSN.

The jugular foramen: a comparative radioanatomic study.

BACKGROUND: Advances in microsurgical techniques made possible the removal of advanced jugular foramen (JF) lesions, which once had been accepted as unoperable. However, successful surgery requires detailed knowledge of the JF anatomy. METHODS: Sixteen jugular foramina in eight formalin-preserved adult cadavers were scanned with axial and coronal high resolution computed tomography (HRCT) prior to dissection. After craniectomy and removal of brain tissue, the relationships of the neurovascular structures in the JF were determined by drilling the temporal bones from superior to inferior on planes parallel to the skull base. RESULTS: No bony partition of the JF was observed. A dural band consistently divided the JF into two parts. Anterior to it was the glossopharyngeal nerve (IX) while the vagus (X) and accessory (XI) nerves were located posteriorly. There was a notch in which the IX nerve entered the JF. It was also identified on the CT scans and defined as the glossopharyngeal recess. The IX nerve made a genu within the JF in all specimens. Then, it ran inferiorly through a bony canal in three specimens (18.75%), and through an incomplete bony canal in two (12.5%), which were also defined on the CT images. The inferior petrosal sinus ran through a sulcus anteromedial to the glossopharyngeal recess. The posterior meningeal artery was found to be located between the X and XI nerves within the JF. CONCLUSIONS: This study revealed a complex and highly variable pattern of the relationships of the neurovascular structures in the JF, and their HRCT images correlated well with the anatomic microdissections.

Lymphocytic infundibuloneurohypophysitis presenting as diabetes insipidus in a man.

We report a patient with diabetes insipidus, whose sella magnetic resonance imaging revealed a normal hypophysis with a focal nodular thickening of the infundibulum and lack of hyper-intense signal of the normal neurohypophysis. The histopathologic examination of the lesion showed a lymphoplasmacytic, predominantly lymphocytic, infiltration. A diagnosis of lymphocytic infundibuloneurohypophysitis was made, by the exclusion of other infiltrative, granulomatous diseases.

Tumours of the lateral ventricle. A retrospective review of 112 cases operated upon 1970-1997.

Between the years 1970 and 1997, 112 patients with tumors of the lateral ventricle were operated on at the University of Ankara, School of Medicine, Department of Neurosurgery. Seventy-one patients (63.4%) were male and 41 patients (36.6%) female. Headache (35.7%), nausea and vomiting (22.3%) were the most common presenting complaints. Papilloedema (42.9%), motor and sensory loss (25%) were the most common findings at neurological examination. Complete tumor removal was accomplished in 38.4% of the patients. Histopathologically, the most commonly seen types of the tumor were ependymoma (25%) and astrocytoma (21.4%). Among the various approach, the anterior transcortical (53.6%) and the posterior transcortical (16%) were the most commonly used. Eleven patients were reoperated for tumor recurrence. After surgery, radiation therapy was also performed on fourty-two patients. The morbidity and mortality rates were considerably higher before 1976 when the use of microneurosurgical techniques was introduced. After this, our morbidity and mortality rates decreased dramatically. The overall surgical mortality rate was 7.1% before 1976; during the last 10 years (n:46), it was 6.5%. In this report, our choice of operative approaches and the results will be discussed.

Branches of the intracavernous internal carotid artery and the blood supply of the intracavernous cranial nerves.

With the increasing frequency of surgical operations to the cavernous sinus greater knowledge of the microanatomy of the cavernous sinus has become necessary. The most frequently seen complications during cavernous sinus surgery involve impairment of cranial nerves. This can occur due to direct damage or ischemia. For these reasons, it is important to know the arterial supplies to the cranial nerves in the cavernous sinus and the anatomy of these branches as well. 15 formaline fixed adult cadavers were used in this study. Before the dissections, the internal carotid artery and vertebral artery were filled with coloured latex on both sides. In this report, the intracavernous branches of internal carotid artery (I.I.C.A.) were identified based on the principles of Nomina Anatomica (1989) and compared with others. In our study we found that the segment of the abducens nerve which lies in Dorello's channel was supplied by the meningeal branch; from the point at which it pierces the cerebellar tentorium, the trochlear nerve is supplied by the tentorial cerebellar artery; the posterior cerebellar artery supplies the proximal segment of the oculomotor nerve that proceeds to the oculomotor triangle. Except for these, all the cranial nerves that were located on the lateral wall of the sinus cavernosus are supplied by the tentorial marginal branch and the branches of the lateral trunk.

An anatomical study of the tympanic branch of the glossopharyngeal nerve (nerve of Jacobson).

Our study was aimed to examine the anatomic relationships of the tympanic branch of the glossopharyngeal nerve (GPN), namely the Jacobson's nerve (JN). The JN is the first branch of the GPN after having passed the jugular foramen. It contributes to the tympanic plexus on the promontory. It transmits secretory innervation to the parotid gland. Its possible role in the regulation of the middle ear pressure has also been hypothesized in terms of animal studies. Using microdissection techniques and high-resolution computed tomography (HRCT) scanning, the anatomic relationships and course of the JN were examined in eight formalin-preserved cadavers (16 sides). A morphometric analysis related to the JN was also performed both in the 16 cadavers and 40 dry-skull specimens. The JN emerged from the inferior ganglion of the GPN in all specimens. The mean distance between the ganglion and the genu of the GPN was 11.3 mm. The inferior 2/3 of the tympanic canal (TC) followed a vertical course, and then it ran anteromedially with an angle of 160 degrees to 170 degrees. The mean length of the TC was 9.5 mm. The TC was well-defined in all axial HRCT scans. In 2 cases the JN was entirely encased in a bony canal in the middle ear. A double JN was observed in one case. This study gives an additional information regarding the anatomy of the JN.

Tentorial meningiomas.

We report our experience with and long-term results of 37 patients with tentorial meningiomas who underwent surgery between 1972 and 1993. The average age was 43 years, and the mean duration of symptoms was 36 months. Headache (83.8%) and extremity or gait ataxia (35.1%) were the most common complaints. On neurological examination, signs of elevated intracranial pressure and cerebellar deficits (51.4%) were the most common findings, followed by third nerve involvement (35.1%). Computed tomography, angiography, and, in recent years, magnetic resonance imaging were used as diagnostic tools and for planning the surgical procedure. According to the primary site of attachment, the tentorial meningiomas were divided into three subgroups: medial, lateral, and falcotentorial. The lateral and medial tumors, with mainly supratentorial development, were approached from above by using a temporal, temporooccipital, or parietooccipital craniotomy. For tumors developing mainly in the posterior cranial fossa, suboccipital craniectomy was performed. In six patients who showed medial tentorial and petrous apex attachment, a combined subtemporal transpetrosal and retromastoid approach was performed. In 31 patients, the tumors were totally removed, and, in 6 patients, only subtotal excision could be done. Seven patients had postoperative complications, but only one of them died of severe brain edema. Our mortality rate was 2.7%. In this article, appropriate preoperative studies, surgical techniques, and surgical results are discussed.