First demonstration of the effectiveness of peptide receptor radionuclide therapy (PRRT) with 111In-DTPA-octreotide in a giant PRL-secreting pituitary adenoma resistant to conventional treatment.

In prolactin-secreting giant adenomas, cabergoline treatment is the first line approach. Surgery and/or radiotherapy are indicated when the tumour is resistant to medical treatment and continues growing, causing visual field impairment. Data concerning other therapeutic approach are scanty. Although PRL-secreting tumours may express somatostatin receptors type 2, 3 and 5, somatostatin analogs treatment is generally ineffective and peptide receptor radionuclide therapy (PRRT) has never been reported. A 58 year-old woman complaining of severe neurological symptoms caused by a giant prolactinoma, relapsing after surgery and not-responding to dopamine-agonists and octreotide LAR treatment, underwent four cycles of PRRT with 111-Indium-DTPA-octreotide with remarkable tumour shrinkage and a significant improvement in clinical conditions. No side effects were reported. This is the first report on the effectiveness and safety of PRRT with radio-labelled somatostatin analogs in a patient with aggressive giant prolactinoma resistant to conventional treatment.

Five-level one-piece laminoplasty for extensive tumors of the lumbar spine.

The authors describe the surgical method and results of nonexpansive one-piece multivel laminoplasty of the whole lumbar spine, for microsurgical resection of extensive spinal tumors. This technique allows a very comfortable approach to the dura and intradural content, as well as a perfect replacement of the posterior spinal arch with supporting elements and an optimal reconstruction of the spinal anatomy. A nonexpansive whole lumbar one-piece laminoplasty was performed for resection of extensive multilevel lumbar tumors. The authors report an illustrative case of a patient who initially presented with a three-year history of numbness on both legs and progressive difficulty in walking. Two months before admission, he complained of bilateral sciatica and rectourinary dysfunction. A spinal magnetic resonance imaging (MRI) documented an intradural tumor extending from L2 to S1. The patient underwent a nonexpansive whole lumbar one-piece laminoplasty and microsurgical removal of the intradural lesion. The postoperative course was uneventful, the sensory disturbances and bilateral sciatica early recovered while rectourinary disturbance gradually improved up to a complete resolution at one year follow-up. The authors believe that multilevel laminoplasty rather than laminectomy is the technique of choice as a posterior procedure for extensive lumbar spinal tumors. With this technique, it is possible to obtain a very confortable approach to the dura and intradural content, as well as a perfect replacement of the posterior spinal arch with supporting elements and an optimal reconstruction of the normal spine. Moreover, this method prevents postoperative instability and deformity and avoids the so called post-laminectomy epidural membrane.

Malignant teratoma in Klippel-Feil syndrome: a case report and review of the literature.

INTRODUCTION: Klippel-Feil syndrome is characterized by a congenital fusion of cervical vertebrae. Intracranial teratomas are nongerminomatous germ cell tumors and they account for 0.3 to 0.9% of all intracranial tumors. Teratomas with malignant transformation refer to lesions which give rise to malignant cancer of somatic type. The association between tumors of dermoid origin and Klippel-Feil malformation is extremely rare. Only 23 other cases have so far been reported, and only one case of dermoid tumor with areas of dedifferentiation on squamous cell carcinoma has been described. CASE PRESENTATION: We report the case of a 72-year-old white man with a 2-year history of gait and balance disturbances. A brain magnetic resonance imaging revealed a fourth ventricle neoplastic process with infiltrative features. He was operated through a suboccipital craniectomy with a C1 laminotomy and bilateral vertebral artery transposition. At 6-months follow-up, magnetic resonance imaging showed an early regrowth of the fourth ventricle tumor, with the same radiological features. CONCLUSIONS: Patients with Klippel-Feil malformation could develop posterior fossa dermoid tumors. The malignant potential of such tumors must be considered and surgery is recommended. Particular attention must be focused on the histopathological analysis in order to identify possible foci of malignant transformation.

In vivo evidence that erythropoietin has a neuroprotective effect during subarachnoid hemorrhage.

To ascertain in vivo whether recombinant human erythropoietin has a neuroprotective effect on the cortex during subarachnoid hemorrhage, 56 rabbits were divided into the following groups: Group 1 control sham operated plus placebo (n=14; saline solution - NaCl 0.9%); Group 2 control sham operated plus recombinant human erythropoietin (n=14); Group 3 subarachnoid hemorrhage plus placebo (n=14); Group 4 subarachnoid hemorrhage plus recombinant human erythropoietin (n=14; intraperitoneal administration of recombinant human erythropoietin immediately after inducing subarachnoid hemorrhage). In none of the Groups 1 and 2 animals was subarachnoid hemorrhage induced. In Group 3 rabbits, an increase in locomotor activity (open field apparatus) was observed 24, 48 and 72 h after surgery, and the mortality rate was 42.9% within 72 h after surgery, and, no increase in locomotor activity was observed in Group 4 rabbits, which survived for at least 72 h. Our findings suggest that recombinant human erythropoietin may be of benefit in the treatment of subarachnoid hemorrhage.

Spasmodic torticollis due to neurovascular compression of the spinal accessory nerve by the anteroinferior cerebellar artery: case report.

OBJECTIVE AND IMPORTANCE: Spasmodic torticollis is a neuromuscular disorder characterized by uncontrollable clonic and intermittently tonic spasm of the neck muscles. We report a case of spasmodic torticollis attributable to neurovascular compression of the right XIth cranial nerve by the right anteroinferior cerebellar artery (AICA). CLINICAL PRESENTATION: A 72-year-old man with a 2-year history of right spasmodic torticollis underwent magnetic resonance imaging, which demonstrated compression of the right XIth cranial nerve by an abnormal descending loop of the right AICA. INTERVENTION: The patient underwent microvascular decompression surgery. During surgery, it was confirmed that an abnormal loop of the right AICA was compressing the right accessory nerve. Compression was released by the interposition of muscle between the artery and the nerve. CONCLUSION: The patient's postoperative course was uneventful, and his symptoms were fully relieved at the 2-year follow-up examination. This is the first reported case of spasmodic torticollis attributable to compression by the AICA; usually, the blood vessels involved are the vertebral artery and the posteroinferior cerebellar artery.

Peritumoral edema in meningiomas: microsurgical observations of different brain tumor interfaces related to computed tomography.

Although generally benign tumors, meningiomas may be associated with extensive peritumoral brain edema as seen on computed tomographic scans. Fifty-two patients with intracranial meningiomas were studied, and the hypodense areas on computed tomographic scans were related to the intraoperative microsurgical findings and to the sizes of the tumors. We have identified three kinds of tumor-brain interfaces characterized by different difficulties in microsurgical dissection: smooth type, transitional type, and invasive type. These different microsurgical interfaces seem to correlate very precisely with computed tomographic images of halo-like and finger-like hypodense areas, allowing prediction of the microsurgical effort to be made in the surgery of meningiomas. The size of the tumor seems to be important in our subjects in determining the amount of edema produced. Indeed, a positive correlation (P < 0.001) was found between the sizes of the tumors and the extent of peritumoral hypodensity. A positive correlation (P < 0.002) also has been found between grade of edema and cortical penetration. Cerebral cortex disruption was systematically observed by us in invasive-type meningiomas and in 3 of 21 cases (14.3%) in transitional-type meningiomas. No penetration was observed in smooth-type meningiomas.

Do spinal meningiomas penetrate the pial layer? Correlation between magnetic resonance imaging and microsurgical findings and intracranial tumor interfaces.

OBJECTIVE: To study the relationships between spinal dura-arachnoid and tumor-cord interfaces in spinal meningiomas and to investigate whether a disruption of the pial layer and penetration of the tumor in the spinal cord occurs. METHODS: Fifteen patients with histologically proven meningiomas underwent magnetic resonance imaging (MRI) preoperatively. All patients underwent microsurgery. The histological characteristics of the tumors were compared with MRI and microsurgical findings. RESULTS: At surgery, the peritumoral hypointense rim revealed by MRI in 10 of 15 patients corresponded to a well-defined cerebrospinal fluid-containing space confined between the outer arachnoidal layer and the inner leptomeningeal layer. In those patients in whom the hypointense peritumoral rim was absent, the inner layer was either difficult to identify or clearly absent, and the blood vessels were extremely adherent to the tumor, requiring a more cautious dissection. Penetration of the tumors through disruption of the pial surface was not documented. CONCLUSION: Previous anatomic and electron microscopy studies demonstrated, in human spinal meninges, the presence of an intermediate layer attached to the inner aspect of the arachnoid, extending laterally over the dorsal surface of the spinal cord and arborizing over the nerve roots and blood vessels. The intermediate layer is not present in human cerebral leptomeninges. The presence/absence of this layer might explain the hypointense rim detected by MRI and might also explain why no penetration and no peritumoral edema is observed in spinal meningiomas as compared with intracranial meningiomas.

Primary cervical melanoma with brain metastases. Case report and review of the literature.

Primary intramedullary melanoma is a very rare tumor that occurs most frequently in the middle or lower thoracic spinal cord. The authors present a case of primary cervical cord melanoma that developed in a 62-year-old man who was surgically treated and subsequently underwent radiation therapy. Clinical and histogenetic features of this neoplasm and results of chemo-. radio-, and immunotherapy are reported. Both "dysembryogenetic" and "mesodermal" hypotheses on the origin of primary spinal melanoma are discussed.

Unusual MRI finding of multiple adenomas in the pituitary gland: a case report and review of the literature.

The simultaneous occurrence of multiple adenomas in the pituitary gland is a rare event. We report the coexistence of three non functioning pituitary microadenomas in a 37-year-old woman, referred to us for oligomenorrhea and headache. Biochemical evaluation revealed prolactin (131 U/liters), follicle-stimulating hormone (4.1 U/liters), luteinizing hormone (3.9 U/liters), 17beta-estradiol (74 pg/mL), free (2.0 pg/mL) and total testosterone (0.5 ng/mL), dehydroepiandrosterone-sulfate (3.5 microg/mL), 17OH-progesterone (0.8 ng/mL), cortisol (13.1 microg/dL), free triiodothyronine (4.8 pmol/L), free thyroxine (18.5 pmol/liters), thyrotropin (1.6 mU/L), and growth hormone (0.2 ng/mL) levels in the normal range, as for as the response to dynamic endocrine tests. MRI showed an enlarged sella turcica, occupied by three distinct hypointense areas that measured less than 5 mm in diameter in the left, medium and right side of the pituitary, respectively. This finding was confirmed 6 months later by a second MRI that revealed also a light increase in microadenomas dimensions. The patient, therefore, underwent neurosurgery by transfenoidal approach. Histologic examination showed no morphologic differences between the specimens obtained from the different microadenomas. Immunohistochemistry evaluation revealed a positive staining for the common alpha-subunit of glycoproteic hormones and negative for the other pituitary hormones tested, while electron microscopy showed cells with a poor secretory apparatus and a variable grade of cell differentiation. In conclusion, we report the fifth case described with multiple pituitary adenomas diagnosed in vivo and the first with three coexisting tumors revealed by MRI before neurosurgery. The occurrence of multiple pituitary tumors emphasizes the role of pituitary and extrahypophiseal factors in the clonal expansion of genetically altered cells.

Cerebellopontine angle lipoma. Case report.

A cerebellopontine angle (CPA) lipoma in a 17 year-old woman is reported. The patient was admitted for evaluation of episodic vertigo, preauricolar pain and progressive hearing loss on the right ear. Auditory brain stem evoked potentials revealed an interwave latency increase on the right ear. Computerized Tomography and Magnetic Resonance Imaging showed a low density lesion and high intensity signal, respectively, in the right CPA. Reports of surgically treated lipomas of the CPA are extremely rare. Our patient underwent a right lateral suboccipital craniotomy and a subtotal resection of the lesion. Histological examination revealed a richely vascularized lipomatous tissue traversed by nerve fibers. The surgical indication for such rare lesion is discussed.

Asymptomatic aneurysms. Literature meta-analysis and indications for treatment.

Autopsy and angiographic studies indicate that cerebral aneurysms occur in an estimated 0.2% to 8.9% of the general population. Modern noninvasive imaging techniques now detect these lesions in ever increasing numbers, and neurosurgeons are faced with a difficult decision about whether to recommend surgical treatment of incidentally discovered asymptomatic unruptured aneurysms (AA). While in the past an expectant management was generally recommended for AA, elective surgery is now advocated because of the risk of bleeding of the untreated aneurysm and the low operative morbidity and mortality rates of modern microneurosurgery. We performed a meta-analysis of the literature on morbidity and mortality from elective surgery for AA to obtain a more generalizable estimate of operative risk than is currently available. Our meta-analysis contained 19 series consisting of 343 cases of elective surgery for AA. There was a total of 3 deaths for a combined mortality rate of 0.87%, and a total of 15 deficits for a combined morbidity rate of 4.3%. These results compare favorably with the 10.3% natural history cumulative risk of mortality for unruptured aneurysms. The authors' personal experience with AA management was also reviewed and a particularly significant illustrative case is briefly reported. We recommend treatment for all asymptomatic aneurysms incidentally discovered particularly if: a) the aneurysm size is greater than 5 mm and arterial hypertension is present; b) the patient is otherwise healthy; c) the estimated life expectancy is > or = 10 years.

Late onset of a large cavernous aneurysm after spontaneous disappearance of cerebral arteriovenous malformation. Case report.

A large intracavernous aneurysm (Icav. An) revealed eighteen years after the spontaneous disappearance of a cerebral arteriovenous malformation (AVM) is reported. The main hypotheses to explain the pathogenesis of the association of such malformations and the potential for spontaneous regression of AVMs are reviewed on the light of relevant literature. The multiple congenital vascular anomalies hypothesis is stressed to explain the late onset of Icav. An in this patient.

Combined systemic beta-interferon and thymostimulin administration for metastatic brain tumors. A brief report.

Interferon-beta (IFN-B) plus thymostimulin (Th) were administered for eight weeks, intravenously and by intramuscular route to eight patients with metastatic brain tumors. No patient had a complete response. One had a stable disease and seven a progressive disease during that time. The mechanisms of the action of IFN-B and Th, and the possible therapeutic value are discussed.

Peritumoral subarachnoid hemorrhage in microsurgical transitional interface type meningioma: may the interface play a role?

The evidence of an intracranial hemorrhage from a meningioma, in comparison with bleeding from different intracranial tumours, is very infrequent. The pathophysiological mechanisms that can explain the possibility of bleeding from meningiomas have not been yet completely clarified. We report a case of a left parasagittal meningotheliomatous meningioma, situated in the premotor cortex, presenting with a peritumoral hemorrhage, at the interface between the meningioma and the brain parenchyma, and strictly confined within the subarachnoid space. A detailed immunohystochemical study of the tumour was performed. The neuroradiological and neurosurgical analysis of the tumour-to-brain interface, served to understand the pathophysiology of this uncommon behaviour. Other possible pathomechanisms explaining bleeding from meningiomas, in the light of the more recent literature are discussed.

Trigeminal pain caused by a cerebellopontine-angle lipoma. Case report and review of the literature.

A 16-year-old girl complained of 2-year history of right facial pain, episodic vertigo and progressive hearing loss in the right ear. The facial pain was described as an episodic lancinating event confined to the second and third branch of the right trigeminal nerve. Computed tomography and magnetic resonance imaging revealed a 2 cm lesion in the right cerebellopontine-angle. At surgery, a soft, yellowish mass was found incorporating the 7th and 8th cranial nerves. The anterior-inferior cerebellar artery (AICA) was displaced medially and pushed into the sensory portion of the trigeminal nerve root, causing vascular compression. The hearing loss remained unchanged. The trigeminal pain disappeared over a period of several weeks. Patients can be harmed in an attempt to remove these neurovascular nonmalignant, generally non growing, fatty vascular lumps. Only a partial, meticulous removal should be performed with a maximum effort to decompress the affected nerve.

Radicular compression by lumbar intraspinal epidural gas pseudocyst in association with lateral disc herniation. Role of the posterior longitudinal ligament.

Among unusual abnormalities of the lumbar spine reported since the introduction of Computed Tomography (CT), the presence of gas lucency in the spinal canal, known as vacuum phenomenon, is often demonstrated. On the contrary, epidural gas pseudocyst compressing a nerve root in patients with a lateral disc herniation has rarely been reported. We report a case of a 44-year-old man who experienced violent low back pain and monolateral sciatica, exacerbated by orthostatic position, one week before admission. A lumbosacral spine CT showed the presence of vacuum phenomenon associated with a degenerated disc material and a capsulated epidural gas collection with evidence of root compression. A microsurgical interlaminar approach was carried out and, before the posterior longitudinal ligament was entered, a spherical "bubble" compressing the nerve roots was observed. The capsulated pseudocyst was dissected out, peeled off and excised en bloc. A large part of the posterior longitudinal ligament and the lateral disc herniation were removed. Postoperatively the patient was completely free of symptoms. The mechanism of exacerbation of pain was probably due to the increased radicular compression in the upright posture and, besides the presence of a lateral disc herniation, could be related to a pneumatic squeezing of gas from the intervertebral space into the well capsulated sac by the solicitated L4-L5 motion segment. Histological study of the wall of the pseudocyst showed the presence of fibrous tissue identical to the ligament. We conclude that, in case of a lumbar disc herniation, it is recommended to perform a complete microdiscectomy and an accurate removal of the involved portion of posterior longitudinal ligament in order to prevent pseudocystic formations.

Lumbosacral congenital dermal sinus presenting in a 52-year-old man. Case report.

Spinal congenital dermal sinus is a rare entity which results from failure of the neuroectoderm to separate from cutaneous ectoderm during the process of neurulation. This epithelium-lined tract forms a potential communication between the skin surface in the midline along the spine and the deeper tissues. We describe a case of an asymptomatic lumbosacral congenital dermal sinus in a 52-year-old man. Microsurgical excision of dermal tract was performed with no postoperative complications and with a satisfactory cosmetic result. The authors stress the importance of an early neuroradiological and neurosurgical management of the lesion. The embryogenesis of this pathological condition and the possible complications are also discussed.

L-acetylcarnitine as a new therapeutic approach for peripheral neuropathies with pain.

With ST200 as the commercial source of L-acetylcarnitine hydrochloride, 94 patients were enrolled in this study; 31 were assigned to placebo, 31 to ST200 at 0.5 g/die and 32 to ST200 at 1 g/die, the i.m. treatments being injected daily for 15 consecutive days. In general, concerning the efficacy assessment, the administration of ST200 at 1 g/die appeared to be better than ST200 at 0.5 g/die when compared with the placebo administration. Statistically significant differences were revealed by the comparison of ST200 at 1 g/die to placebo, for the following variables: a) total motility as rated at the end of the 15-day study and confirmed by intention-to-treat analysis, b) visual analogue scale for all the patients having observation at day 15, and c) the objective and subjective judgements on efficacy. Safety and tolerability were good over the entire course of the study.

Pallidotomy improves quality of life in selected parkinsonian patients: an Italian report.

Pallidotomy has recently been reconfirmed as effective for otherwise intractable symptoms of Parkinson's disease. Nonetheless almost every aspect of its performance requires choices which are not fully established and may vary between centers. These include: 1) patient selection; 2) choice of imaging modality, 3) choice of anatomic landmarks for targeting the lesion, 4) choice of method for physiologic confirmation of location, 5) choice of lesion size and shape. We present two cases of pallidotomy procedures in Parkinsonian patients that in our knowledge are the first reported in Italy. Our experience and a careful review of the literature led to the following choices: 1) selection of Parkinsonian patients with dominant L-Dopa induced dyskinesia, akinetic and rigidity symptoms, 2) use of CT due to the distortion effects of MRI, 3) use of standard (Laitinen) coordinates combined with an image fusion method using MRI, 4) use of stimulation to gauge distance to internal capsule and optic tract, 5) production of vertical lesion covering internal segment of pallidum. At a 1-year follow-up the results include a 45% drop in UPDRS (Unified Parkinson's Disease Rating Scale) motor score and almost complete resolution of contralateral dopa induced dyskinesias in both patients.