Effectiveness of vagal nerve stimulation (VNS) in patients with drop-attacks and different epileptic syndromes.

PURPOSE: The effectiveness of VNS was evaluated in thirty-nine encephalopatic patients with drug-resistant epilepsy characterized by multiple seizures and drop attacks. Twenty-five patients were affected by severe epilepsy with multiple independent spike foci (SE-MISF) and fourteen patients by Lennox-Gastaut syndrome (LGS). METHOD: Changes in seizure frequency, cognition, adaptive behaviour and quality of life were assessed before and after VNS implant until three years. Outcome assessment for all seizure types included the number of seizures/month and the reduction in seizure frequency rate at each follow-up. Moreover, the effect of VNS on frequency, duration and intensity of drop attacks was separately addressed by a modification of McHugh scale. RESULTS: VNS produced a mean seizure rate reduction of 41% at six months, 50% at twelve months, and 54% at thirty-six months. After one year of stimulation, thirteen patients with SE-MISF (52%) and three patients with LGS (21%) showed a reduction above 50% in all seizures' frequency rate. As for drop attacks, eight patients (20%) gained a reduction above 50%, while seven patients (17%) showed a reduction only in intensity and duration. Cognitive level and adaptive behaviour were unchanged, while a better quality of life was reported in half out of the patients. CONCLUSIONS: VNS had a greater effect in reducing seizures frequency and drop attacks' intensity and duration in SE-MISF patients than LGS patients. An improved quality of life was observed also in those patients who only reduced the intensity and duration of drop attacks.

Cingulate epilepsy in a child with a low-grade glioma.

INTRODUCTION: Anterior cingulate epilepsy is a peculiar epileptic syndrome with a broad range of clinical manifestations, depending on the numerous projections of anterior cingulate into motor systems. Its diagnosis is often delayed, as seizures mostly occur during sleep and are typically misdiagnosed as parasomnias. Moreover, most focal anterior cingulate epilepsies are believed to be cryptogenic or idiopathic, even if there are some reports of anterior cingulate cortical dysplasia, while anterior cingulate neoplasms underlying epilepsy are rare. CASE REPORT: Here, we report a 30-month-old boy who developed, at the age of 20 months, cingulate epilepsy associated with a low-grade oligodendroglial tumor. It must outlined that the clinical presentation was characterized by very frequent and disabling seizures as the only symptom of the disease, while the results of several neuropsychological tests suggested intact intellectual and behavioral abilities. DISCUSSION AND CONCLUSION: Seizures disappeared completely after surgical removal of the lesion and neuropsychological child's performances remained completely normal. Long-term follow-up and observation are essential for evaluating the future clinical course.

Decompressive craniectomy for medically refractory intracranial hypertension due to meningoencephalitis: report of three patients.

BACKGROUND: Meningoencephalitis may sometimes cause medically refractory intracranial hypertension and brain herniation. In such patients death is common. There are a limited number of reports on the use of decompressive craniectomy as a life saving measure in these circumstances with some good results. The aim of the study was to report experience in three further patients. MATERIALS AND METHODS: In a 15-month period, three patients affected by acute meningoencephalitis were surgically treated by decompressive craniectomy at the Department of Neurosurgery of the Polytechnic University of Ancona. In all patients common symptoms at presentation were headache, fever and neck rigidity, rapidly followed by the development of focal neurological deficits and coma. Intracranial pressure monitoring was always performed and correlated with serial CT scan examinations. Because of the development of severe intracranial hypertension refractory to conventional medical treatment, a decompressive hemicraniectomy was performed in two patients and a bifrontal decompressive craniectomy in the third one. Bacterial meningoencephalitis was diagnosed in two patients, viral meningoencephalitis in the remaining one. FINDINGS: One patient died 3 days after surgery. The remaining two completely recovered consciousness, with no residual focal neurological deficit. CONCLUSIONS: Surgery resulted in an immediate reduction of intracranial pressure in two of the three patients with severe meningoencephalitis. Decompressive craniectomy may be a useful option in the management of a patient with medically refractory intracranial hypertension caused by meningoencephalitis. Early intervention may enhance its benefits.

Complications of vagal nerve stimulation for epilepsy in children.

Vagal nerve stimulation (VNS) is a surgical option to treat drug-resistant epilepsy. A few side effects have been described, mainly as anecdotal reports. We analysed our material concerning a juvenile population to identify the most common and most important complications, discussing them with the literature. Thirty-six patients were studied (18 months-18 years old). The children were assessed before the VNS implant and 3, 6, 12, 24 and 36 months after surgery. The mean follow-up was 30 months. Four patients required a second surgery: two for changing the device 3 years after implant; one for revision of an imperfect implant; one for removing a non-functioning device. In one patient a transient vocal cord paralysis was observed. Hoarseness was the main complaint (38.8%). More infrequent was mild sleep apnoea (8.3%), sternocleidomastoid muscle spasm, drooling and snoring in one patient each. Skin scars were reported with a different frequency according to the surgical technique. At variance with the literature reports, we did not observe infections. Side effects of VNS can be minimised, but not avoided completely, with a correct technical procedure, which in turn depends upon a thorough knowledge of vagus nerve anatomy.

Spinal epidural en-plaque meningioma with an unusual pattern of calcification in a 14-year-old girl: case report and review of the literature.

A 14-year-old girl who presented with a severe sensory-motor-sphincter syndrome was found to be harboring an epidural tumor situated posteriorly in the spinal canal from C5 through C7 levels. The mass had computerized tomography and magnetic resonance imaging features suggesting an unusual stratified architecture, with a conspicuous highly calcific component firmly adherent to the dura and a non-calcific mass surrounding it posteriorly and laterally. Although meningiomas have a low incidence in the first two decades of life, and in the spine they rarely have entirely extradural location at any age, a meningioma was suspected. Intraoperative biopsy confirmed the tumor to be benign, and careful total resection including the whole large dural implant was carried out; the wide dural defect was grafted with fascia lata. A meningothelial meningioma with a largely calcified psammomatous component was diagnosed. The girl made a complete recovery, and is tumor-free 9 years postoperatively. Outcome from surgery for spinal meningiomas can be good, despite the severity of the preoperative condition; however, enplaque and highly calcific tumors still bear a poorer prognosis. Complete resection is mandatory in children, in whom they are extremely rare. Modern imaging techniques help in making a correct initial diagnosis and optimizing surgery in order to provide good results, even in more-challenging cases.

Acute posttraumatic paraplegia caused by epidural hematoma at the vertex.

Vertex epidural hematomas (VEDHs) are well known but uncommon. Their clinical presentation may be misleading and missed by routine CT axial scanning; thus, diagnosis may be delayed, with possibly fatal consequences. We report a case of acute posttraumatic paraplegia caused by a VEDH, which was evident at CT and for which the patient underwent successful surgery.

Multicentric glioma with unusual clinical presentation.

Multiple glioma is a well-recognized but uncommon entity. They are grouped in two categories: multifocal and multicentric gliomas. Multifocal gliomas grow through dissemination along an established route, spreading through commissural pathways, CSF channels, or the blood or by local extension through satellite formation; at the opposite end of the spectrum, multicentric gliomas are widely separated lesions whose simultaneous presence cannot be attributed to any of the above pathways. Reports in the literature refer to single cases or small series of multicentric gliomas, almost always in adult patients, their occurrence in children being even less frequent. We report the case of a 12-year-old boy with multicentric glioma, atypical acute clinical onset and fast growth of three other tumors in 8 months, and then discuss the problems of diagnosis and therapy.

Treatment of malignant gliomas: a new approach.

The aim of this study is to describe the authors' experience with intra-arterial ACNU chemotherapy of malignant gliomas. The prognosis of cerebral malignant gliomas remains poor, whatever traditional therapy is applied. ACNU is a well tolerated nitrosourea with a strong antimitotic effect on neurogenic cells both in vitro and in vivo; this drug has enhanced efficacy when used at high concentrations, particularly as an intraarterial infusion. Seventy-six patients have been studied to date, 68 of whom are evaluable; these patients were treated by intra-arterial infusion of ACNU (100 mg/m2) every 6 weeks, with a mean of 2.5 courses per patient. The objective response (OR) was 28% and analysis of pretreatment factors revealed that survival was influenced by histological grade, other types of therapy applied, and age. In general IAC is well tolerated and the response and survival appear to be better than with systemic chemotherapy.

Tuberculous spondylitis: a retrospective study on a series of 12 patients operated on in a 25-year period.

BACKGROUND: The incidence of tuberculous spondylitis, which had declined steadily for over 40 years in our countries, started increasing again in the eighties, paralleling the resurgence of pulmonary tuberculosis. Therefore it has become a matter of discussion in contemporary literature, because it can be a diagnostic challenge and, in spite of its severe neurological complications, it is a potentially curable illness. METHODS: In this retrospective study the authors report their experience concerning 12 patients operated on in a 25-year period because of serious cord compression from thoracic (9 cases) and cervical (1 case) tuberculous spondylitis or from thoracic tubercular epidural lesion (2 cases). Surgical techniques were selected on the basis of the cause of cord compression. Fusion with autologous bone and metallic osteosynthesis was performed in the cervical case (1986); no other patient received spinal instrumentation, and this can be explained with the consideration that all but one cases of Pott's paraplegia were treated in the years 1968-1977. In all of these cases fusion was achieved by means of plaster jackets and prolonged bed rest. Prolonged chemotherapy was systematically administered. RESULTS: Follow-up data collected in 1995 show good and long-lasting results. CONCLUSIONS: They conclude that surgical treatment is required in case of cord compression and results can be excellent even in presence of severe neurological impairment; spinal instrumentation available in our era should be now considered in order to make rehabilitation earlier and morphologic results more satisfactory.

Predictive factors of callosotomy in drug-resistant epileptic patients with a long follow-up.

Some possible factors to predict outcome after callosotomy were investigated in a personal series composed of 36 patients. Twenty-seven of them were submitted to anterior callosotomy, 1 to posterior callosotomy and the remaining 8 patients to two stage complete division of corpus callosum. All factors, either positive or negative, do not appear completely reliable; they can be envisaged only in relative terms and do not seem to be relevant to make surgical decision or to discard surgery. In author's series, dealing specifically with drop-attacks, it clearly appeared that abrupt falls to the ground unpreceded by other epileptic features did definitely better. This was the only statistically significant datum.

Modifications in morphology of epileptic seizures after callosotomy.

The callosotomy on drug-resistant epileptic seizures may abolish or reduce their frequency and may modified the morphology of different kind of seizures. Our series is composed of 36 patients. Drop-attacks and complex partial seizures (CP) were present in all patients, generalized tonic-clonic seizures (GTC) in 16 (44.4%) and simple partial seizures (SP) and myoclonic attacks (MY) in 6 patients each. We found two kinds of modifications: a) lateralization of hypotonia which becomes hemisomatic, with lateral bending to the hypotonic side; b) different way of falling, due not only to the lateralization of hypotonia but also to the gradual impairment of muscle tone. Modifications in CP mainly concerned automatisms, which disappeared in 39.1% of patients, while 34.8% of them had a significant reduction in automatisms; this reduction consisted in simplification of automatic movements and in shorter duration of seizures.

EEG Patterns after callosotomy.

In 36 patients with drug-resistant epilepsy submitted to anterior callosotomy (27 cases), to two-stage total callosotomy (8 cases) and to posterior callosotomy (1 case) the EEG variations concerning background activity, focal activity and sharp-waves (SW) bisynchronous activity were evaluated. EEG modifications observed after callosotomy are the following: background rhythm tends to be better organised as spectral analysis demonstrated, this finding usually coincide with reduction of bisynchronous discharges. It appears that improvement in background activity cannot be correlated with outcome, but it seems to be to some extent since at the same time cognitive functions also seem to improve; however, this last aspect need to be checked in much larger series. The number and location of EEG foci do not change, but they appear to be more active; this is likely to depend only on the concomitant reduction of bisynchronous activity. No correlation seems to exist between the number and the location of foci, which are generally multiple. Lateralization of bisynchronous discharges as well as the reduction of their frequency and duration were observed. However, the clinical course is quite different: in some patients we have achieved good clinical responses in others postoperative results were poor. Lateralization of bisynchronous discharges is never absolute, on the grounds that in prolonged recordings bisynchronous discharges are nearly always present. Bisynchronous discharges in some cases are alternatively predominant in both hemispheres even within minutes or seconds. It was observed that after certain time, generally some months, lateralized discharges tend to generalize again, confirming that corpus callosum is replaced in discharge diffusion by other structures (brain-stem, diencephalon).

Mutism in 36 patients who underwent callosotomy for drug-resistant epilepsy.

Thirty-six drug-resistant epileptic patients submitted to callosotomy were studied. Anterior callosotomy was performed in 27 patients, total two-stage callosotomy was performed in 8 patients and 1 patient had only posterior callosotomy. We found mutism in 10 patients (2 after complete callosotomy and 8 after anterior section). The patients did not speak, but the comprehension was present: they were able to carry out orders and to write. Their attitude to the environment was characterized by complete indifference. The mutism was always transient, lasting from 4 to 25 days (mean 7 days). Regression of mutism was always complete. We think that this complication should be chiefly attributed mainly to surgical manipulation, even if it is impossible to completely exclude a multi-factorial etiology.

Meningioma-like cerebral relapse of Hodgkin's disease.

A case of Hodgkin's disease with isolated cerebral relapse is reported here. The peculiar presentation features are reported and the difficulties related to the differential diagnosis are discussed.

Percutaneous twist-drill craniostomy for the treatment of chronic subdural haematoma.

A consecutive series of 65 adult patients with chronic subdural haematoma was reviewed to ascertain the efficacy of twist-drill craniostomy and closed-system catheter drainage. In all patients but two the percutaneous evacuation of the haematoma alone was enough to bring about their recovery. Infections or neurological complications were never observed. Pneumocephalus was likewise absent in all patients. Therefore this technique should be recommended as the "first intention" management of the disease, particularly in elderly patients.

Evolution of neuropsychological changes after partial callosotomy in intractable epilepsy.

Early neuropsychological changes following partial anterior callosotomy were evaluated in 15 patients with epilepsy (10 secondary generalized and 5 Lennox-Gastaut syndromes) by comparing their performances 1 month before surgery and then 15-20 and 90-100 days postoperatively without modifying the anticonvulsant treatment. The following neuropsychological and motor functions were tested: memory, attention, visuo-motor ability, posture, motor dexterity, language, praxis and gnosis. Social behaviour was also investigated. The main results are as follows: (i) most cognitive functions showed no significant variation; (ii) motor organization was still slightly impaired at the second check-up after the surgery; (iii) improvements in social behaviour and posture were frequently observed at the final evaluation. The analysis of individual cases highlights the influence of the extent of commissurotomy, lesions related to the surgical procedure and preoperative brain damage in determining the post-surgical profile. On this basis, the short-term neuropsychological cost of partial callosotomy appears to be low and seems to depend mostly on surgical parameters and brain conditions before the operation.

Callosotomy for the treatment of drug resistant generalized seizures.

Fifteen patients have been followed for more than one year following callosotomy having presented with long standing epilepsy, no well defined focus amenable to radical excision, and severely incapacitating atonic seizures that were refractory to anticonvulsant therapy. Atonic fits have been reduced by more than 80% in thirteen patients, with two patients suffering long term sequelae (slight dysarthria in one, and dyslexia with mild visuo-spatial disturbances in another). Anticonvulsant therapy was still required post-operatively.

Quantitative evaluation of recurrent meningiomas.

Clinical, histological and karyometric parameters, nuclear DNA content and the number of nucleolar organizer regions were investigated in 9 recurrent meningiomas and 10 meningiomas which had not recurred within a 10-year period. There were no significant differences between the two groups as to age, sex, site of the tumours and most of the histological parameters scored. Recurrent tumours showed a higher number of mitotic figures and the nucleolus was visible in most of the cells. Cell density, nuclear area, perimeter and nuclear DNA content had values with no statistically significant differences between the two groups. However, significant differences were found in the distribution of the nuclei in the different ploidy regions. Most of the nuclei in the non recurrent cases were in the diploid range, whereas in recurrent tumours there was a reduction in the number of diploid cells associated with an increase in 2c--4c and 4c components. Recurrent tumours also showed a higher number of nucleolar organizer regions positively stained using an argyrophil method. The mitotic count and the nucleolar organizer regions appeared to be the best predictors for recurrence.

[Remarks on callosotomy in the treatment of drug-resistant epilepsy]. / Remarques sur la callosotomie dans le traitement de l'épilepsie pharmaco-résistante.

In this report 17 patients with long-standing non-focal epilepsy underwent callosotomy (this was total in two patients and performed in two stages, and anterior-subtotal in the remaining patients). In all patients the atonic-hypertonic seizures with sudden falls were the most disabling epileptic fits. Callosotomy proved efficient in controlling atonic fits in 10 out of 15 patients in whom surgical results are evaluated. In 3 additional patients the frequency of atonic fits was reduced by more than 50%. In the remaining two patients, no therapeutic effect was observed. Callosotomy was less effective on seizures which were not atonic. Therefore, this procedure appears to be indicated in patients in whom atonic fits are predominant. The main effect of callosotomy is to transform drug-resistant seizures into drug-sensitive ones. Neuropsychological sequels are insignificant unless the splenium is severed. However, considerable psychic and behavioral improvement was nearly always observed after surgery. Despite the fact that on a therapeutic level results were often satisfactory, a number of practical problems still remain. These concern the full spectrum of indications for callosotomy, the extent of corpus callosum section, choice of methods in severely mentally retarded patients and, finally, the age at which the operation should be carried out.