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Morvan's syndrome (MoS) is a rare, complex neurological disorder characterized by neuromyotonia, neuropsychiatric features, dysautonomia and neuropathic pain. The majority of MoS cases have a paraneoplastic aetiology, usually occurring prior to the diagnosis of the underlying tumour and showing improvement following its treatment. The present study reports the case of a 35-year-old Caucasian male patient who was diagnosed with stage IVA thymoma. Thymectomy, lung resection, diaphragmatic pleurectomy and pericardio-phrenectomy were performed 6 months after neoadjuvant chemotherapy. The pathological evaluation revealed a type B2-B3 thymoma with focal squamous differentiation. Two years later, the patient underwent new surgical treatment for a local recurrence of the same histological type, and 4 weeks later, the patient presented with complex neurological symptoms compatible with MoS, including neuromyotonia, neuropsychiatric features, dysautonomia and neuropathic pain. Electromyography was compatible with a diagnosis of neuromyotonia. Brain magnetic resonance imaging scan and tests for serum anti-acetylcholine receptor, anti-striated muscle antibodies and anti-30-kDa titin fragment antibodies were all negative, whereas tests for anti-voltage-gated potassium channel (VGKC)-complex antibodies (333.3 pmol/l), anti-leucine-rich glioma inactivated protein 1 and anti-contactin-associated protein-like 2 antibodies were positive. The patient underwent 3 cycles of intravenous administration of immunoglobulins (0.4 g/kg/day for 5 days every 4 weeks) with little clinical and electrophysiological improvement. We speculated that the late onset of the symptoms in the present patient may have been triggered by an increase in the serum level of anti-VGKC antibody, which was caused by the surgery performed for the treatment of recurrent thymoma. To the best of our knowledge, the present report is the first case of MoS associated with this histological type of thymoma uncommonly occurring upon surgical treatment of recurrent thymoma.

Patient involvement in the improvement of health care is important for the best long-term treatment outcomes. Our objective is to assess patient satisfaction with offered care service and to identify parameters which influence the adherence to long-term therapy. A prospective single-center study based on the administration of a structured interview to multiple sclerosis (MS) patients attending our MS Centre with mild and moderate disabilities. The interview regarding clinical parameters, quality of life and satisfaction of care service was structured in three parts with multiple-choice answers to close- or open-ended questions. Patient satisfaction was evaluated by the concordance between the level of patient attention and judgment regarding the services offered. The impact of all variables on the adherence to therapy and on the perceived utility of treatment was evaluated. The concordance between patient attention and judgment on health care services resulted statistically significant for almost all parameters. The perceived utility of treatment was significantly correlated to patients feeling confident in the clinical staff, to their perception of being involved in therapeutic decision (p<0.05), and associated to therapy adherence (p=0.0001). In a multivariate model, the adherence to therapy resulted associated to possibility of choosing the physician (p=0.037) and inversely to therapy duration (p=0.001). Our conclusion reveals that, to improve the adherence to long-term therapy and the perceived utility of treatment, a particular attention should be devoted to physician-patient relationship.

Introduction: Literature data do not report any cases of erectile dysfunction (ED) during treatment with new antiepileptic drugs in patients with brain tumors., Aim: Concerning zonisamide (ZNS) therapy, data on sexual dysfunction are not available either in patients with epilepsy or in patients with brain tumor-related epilepsy., Methods: Our case study concerns one patient with partial epilepsy associated with oligoastrocytoma in whom reversible ED developed while taking ZNS. He came to our center already having undergone therapy with phenobarbital, oxcarbazepine, phenitoin, and clobazam. Due to the presence of psychomotor slowness and uncontrolled seizures, we substituted phenobarbital with ZNS 200 mg/day. Main Outcome Measures.  The main outcome measures were seizure frequency and side effects., Results: During ZNS therapy, we observed beneficial effects on seizure frequency with a notable reduction from 2-3 seizures per day to 2-3 per week. One month after starting therapy with ZNS, the patient complained about ED that disappeared when we suspended the drug., Conclusions: In the literature on patients with brain tumor-related epilepsy, sexuality is a subject that is often neglected by health-care providers who focus primarily on controlling systemic diseases and maintaining physical comfort. For this reason, the possible impact of antiepileptic therapies on sexuality should be taken into consideration., (© 2009 International Society for Sexual Medicine.)

Herpes simplex encephalitis (HSE) is a life-threatening condition with high mortality. The pathogenesis underlying the reactivation of latent herpes simplex virus (HSV) remains undefined. We present the case of a 55-year-old female who developed HSE type 1 during brain irradiation and antioedematous dexamethasone treatment for leptomeningeal metastasized breast tumor with epileptic seizures. During the radiotherapy (RT), after a total of 32 Gray administrated in 16 fractions, our patient developed cognitive impairment and partial epileptic status without fever. Two days later the patient's clinical conditions had deteriorated and high fever manifested. A diagnosis of HSE type 1 was made by a positive cerebrospinal fluid polymerase chain reaction. Antiviral therapy with high doses of acyclovir was practiced for four weeks but the comatose state persisted. The patient died 59 days after the last RT fraction. The temporal relationship of RT to the occurrence of HSE suggests that cranial irradiation may play a role in the reactivation of latent HSV. Although antiviral therapy resistance is infrequent in immunocompetent patients, it is one of the main problems in immunocompromized patients.

A case of avascular necrosis of whirlbone in a young man affected by multiple sclerosis and treated with high doses of corticosteroids is described. The authors discuss the causes of this collateral effect underlining the risks of underevaluating the symptoms.

Background: Sexual dysfunction following surgery for rectal cancer may be frequent and often severe. The aim of the present study is to evaluate the occurrence of this complication from both a clinical point of view and by means of neurophysiological tests., Methods: We studied a group of 57 patients submitted to rectal resection for adenocarcinoma. All the patients underwent neurological, psychological and the following neurophysiological tests: sacral reflex (SR), pudendal somatosensory evoked potentials (PEPs), motor evoked potential (MEPs) and sympathetic skin responses (SSRs). The results were compared with a control group of 67 rectal cancer patients studied before surgery. Only 10 of these patients could be studied both pre- and postoperatively. 10 patients submitted to high dose preoperative chemoradiation were studied to evaluate the effect of this treatment on sexual function. Statistical analysis was performed by means of the two-tailed Student's t test for paired observations and k concordance test., Results: 59.6% of patients operated reported sexual dysfunction, while this symptom occurred in 16.4% in the control group. Moreover, a significantly higher rate of alterations of the neurophysiological tests and longer mean latencies of the SR, PEPs, MEPs and SSRs were observed in the patients who had undergone resection. In the 10 patients studied both pre and post-surgery impotence occurred in 6 of them and the mean latencies of SSRs were longer after operation. In the 10 patients studied pre and post chemoradiation impotence occurred in 1 patient only, showing the mild effect of these treatments on sexual function., Conclusion: Patients operated showed severe sexual dysfunctions. The neurophysiological test may be a useful tool to investigate this complication. The neurological damage could be monitored to decide the rehabilitation strategy.

The aim of this pilot study was to investigate the effects of levetiracetam monotherapy on seizure control, quality of life and neurocognitive performance in patients with brain tumor-related epilepsy. We present here preliminary data from 18 patients with follow-up of 6 months. We evaluated seizure frequency at baseline. We used administered Mini Mental State Examination (MMSE), Karnofsky Performance Status (KPS), Barthel index (BI), QOLIE 31P (V2), EORTC QLQ-C30 and Adverse Events Profile. After 6 months, 16 patients were seizure free (88.9%), 2 (11.1%) had reduction in seizure frequency >50%. Compared to baseline, we observed a worsening of performance (KPS p = 0.011; BI = 0.008) and global lower cognitive performance (MMSE p = 0.011); distress related to seizure frequency (p = 0.003) and medication effects (p = 0.046) were significantly lower. Levetiracetam caused mild and reversible side effects. These preliminary data on LEV monotherapy in patients with brain tumor-related epilepsy show that this antiepileptic drug is efficacious and well tolerated.

Background: Seizure control doesn't represent the only challenging goal in patients with brain tumor-related epilepsy. Side effects have often taken precedence for patients' quality of life., Methods: We performed an observational retrospective study on patients with brain tumor-related epilepsy: 35 who had assumed oxcarbazepine monotherapy and 35 patients who had undergone treatment with traditional antiepileptic drugs. Primary variable of efficacy was the mean seizure frequency per month and safety variables were the drop-out for side effects and total incidence of side effects. We applied the Propensity Score technique to minimize selection bias., Results: Our results showed a similar efficacy of oxcarbazepine and traditional antiepileptic drugs over time, but the difference in safety and tolerability between the two groups was significant: traditional AEDs caused more side effects, both serious and non serious., Conclusion: This study highlights the importance of taking into consideration not only seizure control but also the appearance of side effects when choosing antiepileptic drugs in this patients population.

Background: In recurrent malignant gliomas (MGs), a high rate of haematological toxicity is observed with the use of fotemustine at the conventional schedule (100 mg/m(2) weekly for 3 consecutive weeks followed by triweekly administration after a 5-week rest period). Also, the impact of O6-methylguanine-DNA methyltransferase (MGMT) promoter methylation status on fotemustine activity has never been explored in the clinical setting., Methods: 40 patients with recurrent pretreated MG were identified as being treated with fotemustine at doses ranging from 65 mg/m(2) to 100 mg/m(2). Patients were classified into 3 groups according to the dose of fotemustine received, from the lowest dosage received in group A, to the highest in group C. Analysis of MGMT promoter methylation in tumor tissue was successfully performed in 19 patients., Results: Overall, 20% of patients responded to treatment, for a disease control rate (DCR, responses plus stabilizations) of 47.5%. Groups A and B experienced a response rate of 40% and 26.5% respectively, while the corresponding value for group C was 10%. Out of 19 patients, MGMT promoter was found methylated in 12 cases among which a DCR of 66.5% was observed. All 7 patients with unmethylated MGMT promoter were progressive to fotemustine., Conclusion: Low-dose fotemustine at 65-75 mg/m(2) (induction phase) followed by 75-85 mg/m(2) (maintenance phase) has an activity comparable to that of the conventional schedule. By determination of the MGMT promoter methylation status patients might be identified who are more likely to benefit from fotemustine chemotherapy.

Despite aggressive antitumor treatment, the prognosis of brain tumor (BT) patients remains poor. In the last stage of disease, BT patients present severe symptoms due to the growing tumor or to treatment side-effects, which require adequate palliative management and supportive therapy. However, studies specifically addressing palliative care and end-of-life (EoL) issues in BT patients are lacking. This study explores symptoms experienced by BT patients in the last weeks of disease and EoL issues observed in a population of brain tumor patients followed at home until death by a neuro-oncological home care palliative unit set up in our Institution in 2000. From October 2000 to December 2005, 324 patients affected by brain tumor and discharged from our Institution were enrolled in a comprehensive program of neuro-oncological home care supported by the Regional Health System. Out of 324 patients enrolled in the home care program, 260 patients died of which 169 (65%) were assisted at home until the end of life and have been included in this study. Clinical symptoms, palliative treatments and EoL treatment decisions were collected from home clinical records. Among the 169 patients assisted at home until death, the most frequent symptoms observed in the last four weeks of life were: epilepsy 30%, headache 36%, drowsiness 85%, dysphagia 85%, death rattle 12%, agitation and delirium 15%. Palliative management of brain tumor patients requires a multidisciplinary approach performed by a well trained neuro-oncology team. Development of home care models of assistance may represent an alternative to in-hospital care for the management of patients dying of brain tumor and may improve the quality of end-of-life care.

Objective: Medical management of brain tumor-related epilepsy is complicated by interactions between antiepileptic and chemotherapeutic drugs. We studied the effect of temozolomide therapy on the disposition of the new antiepileptic drugs topiramate (TPM) or oxcarbazepine (OXC)., Methods: Fifteen patients chronically treated with TPM or OXC in monotherapy starting a chemotherapeutic treatment with temozolomide were enrolled in the study, of which ten were available for the final analyses. Blood samples were collected before temozolomide treatment (T(0)), at its end (T(7)) and after further 1-3 weeks (T(14)-T(28)). For each patient, more than one treatment cycle was studied. Topiramate and OXC mono-10-hydroxy derivative (MHD) plasma concentrations were determined by hplc coupled with ion spray mass spectrometer (TPM) or ultraviolet (MHD) detection., Results: Mean TPM concentrations were 5.4 +/- 2.4 microg/ml at T(0) vs. 5.5 +/- 2.4 microg/ml at T(7) (n = 14), and 5.4 +/- 2.4 microg/ml at T(0) vs. 5.6 +/- 2.8 microg/ml at T(14)-T(28) (n = 14). Mean MHD concentrations were 16.4 +/- 7.6 microg at T(0) vs. 18.5 +/- 9.0 microg/ml at T(7) (n = 5), and 16.8 +/- 7.0 microg/ml at T(0) vs. 18.0 +/- 8.7 microg/ml at T(14)-T(28) (n = 8) (all comparisons not statistically significant; Student's t-test for paired samples)., Conclusion: Temozolomide treatment did not affect TPM plasma concentrations in chronically treated patients. Data for MHD in OXC-treated patients were similar, but, due to the small sample size, results should be interpreted cautiously.These findings confirm that TPM (and possibly OXC) are a reasonable choice of antiepileptic drug in patients with brain tumor-related epilepsy.

Purpose: The rationale for intensification strategies is that more frequent exposure to chemotherapeutics could enhance antitumor activity. Several trials investigated weekly paclitaxel administration, but there are no clear data concerning peripheral neurotoxicity. The aim of this study was to assess the incidence of peripheral neurotoxicity in patients affected by advanced breast cancer treated with weekly paclitaxel., Patients and Methods: Neurotoxicity was assessed with neurologic and neurophysiologic evaluation before treatment and after 12 weeks and 24 weeks. A total neurotoxicity score was assigned to each patient on the basis of neurophysiologic and neuropathic signs and symptom changes. Seventeen patients entered the study., Results: After 12 weeks of treatment, 71% showed moderate clinical and/or neurophysiologic signs of neurotoxicity; after 24 weeks, the incidence of neurotoxicity increased to 96%. Sural amplitude at the 24-weeks examination significantly decreased from basal mean value (13.5 microv, standard deviation [SD] 6 microv vs. 7 microv, SD 5.9 microv, respectively; P = 0.01), whereas median sensory amplitude decreased after 24 weeks from 10.3 microv, SD 6.2 microv to 4.9 microv, SD 3.8 microv (P = 0.001). In a subset of 11 patients, we obtained a follow-up examination after 6 months from the end of treatment. In all patients, examined signs and symptoms of neurotoxicity improved with recovery of subjective neuropathic symptoms and neurophysiologic findings., Conclusion: Our results demonstrate, in a little population of patients evaluated with a comprehensive neurologic assessment, that weekly paclitaxel is related to a very high incidence of peripheral neurotoxicity. Follow-up data obtained in a subset of patients indicate that peripheral neurotoxicity is reversible.

Epilepsy is a common clinical problem in patients with brain tumours, strongly affecting patients' quality of life. Tumour-related seizures are often difficult to control, and the clinical picture is complicated by frequent interactions between antiepileptic drugs (AEDs) and antineoplastic agents. We studied the safety and efficacy of levetiracetam (LEV), a new AED with a different pharmacological profile from traditional anticonvulsants, in 19 patients (6 females; age range 28-70 years, mean 48 years) with supratentorial gliomas and epilepsy. Seizure types were simple partial in four patients, complex partial in 4, complex partial with secondary generalization in 7, and generalized tonic-clonic in 4. LEV was added to the existing AED treatment on account of persisting seizures, and titrated at dosages of 1,000-3,000 mg/day. Patients were seen at the Outpatient's Centre every 1-3 months, and followed-up for 7-50 months (mean 25 months, median 20 months). At the end of the observation period, nine patients were seizure free (seizure free period ranging from 7 to 33 months, mean 16, median 12) and five patients reported an improvement in seizure-frequency from daily to weekly (n=1) or from weekly to monthly (n=3). Seizure frequency was unmodified in four patients and increased (from monthly to weekly) in one. No LEV-related adverse effects were observed. LEV plasma concentrations monitored in 12 subjects ranged from 11.9 to 82.1 microg/ml. Our preliminary open data indicate that add-on treatment with LEV in patients with brain tumours is safe and appears to be effective in reducing seizure frequency. Controlled studies on larger populations are warranted to confirm these open observations.

Forty-seven patients with Glioblastoma (42) and Anaplastic Astrocytoma (5) were studied with MR 24 hrs after surgery. In order to evaluate the role of early MR in defining the extent of surgical resection and its relation with the prognosis of malignant glioma patients, three categories of surgical resection were considered: gross total, sub-total and partial resection. The results were correlated with progression-free survival (PFS) and overall survival (ST). As demonstrated by early-MR, gross total resection was performed in 17 patients, sub-total and partial resection in 19 and 11 patients, respectively. The PFS was 6 months in gross total resection, 6 and 3 months in sub-total and in partial resection, respectively. The median survival time was 16 months in total resection patients, 13 months and 7 months in sub-total resection and partial resection patients, respectively. The study confirms that early-MR has to be considered an accurate technique for monitoring the extension of malignant glioma surgical resection and shows a good correlation between early-MR findings, PFS and ST.

Oxaliplatin (L-OHP) has become a standard treatment for advanced colorectal cancer and a valid option for patients in the adjuvant setting. Compared with cisplatin, L-OHP has no renal toxicity, only mild hematological and gastrointestinal toxicity, while neurotoxicity is the limiting toxicity. This side effect has been described as a transient distal dysesthesia, enhanced by exposure to cold, and as a dose-related cumulative mild sensitive neuropathy. We studied two groups of patients (18 and 13) with advanced colorectal cancer, treated with median cumulative doses of L-OHP 862 mg/m2 and 1,033.5 mg/m2. All the patients had been evaluated previously, during treatment, after discontinuation and after a long follow-up of 5 years to verify the incidence and the characteristics of the neuropathy induced by this antineoplastic agent. The clinical and neurophysiological examinations showed an acute and transient neurotoxicity and a cumulative dose-related sensory neuropathy in nearly all the patients. The reversibility of these effects was studied. Five patients continue to manifest symptoms and signs of neurotoxicity after a long follow-up, indicating persistence of this peculiar type of neuropathy., (Copyright 2006 S. Karger AG, Basel.)

The patient affected by epilepsy related to brain tumours presents certain features linked to the summation of his cancer-related problems and his epilepsy-related problems. Furthermore, epilepsy in brain tumour patients is often refractory to pharmacological treatments and can complicate the therapeutic management of these patients due to the increased incidence of pharmacological interactions and adverse effects. Analysis of the data in the literature suggests that it is opportune, when planning antiepileptic therapy in these cases, to choose the new-generation drugs, as these show a lower incidence of pharmacological interactions with the therapies used in brain tumour patients (chemotherapies, radiotherapy and support therapies), have fewer adverse effects, and have less impact on neuropsychological functions, all factors that strongly influence the patient's quality of life. Of the new antiepileptic drugs, the following seem to be promising in the treatment of cancer-related epilepsy: oxcarbazepine, topiramate and levetiracetam (the latter as an add-on therapy). The pharmacokinetic features of these drugs, their effectiveness in controlling seizures, and the reduced incidence of adverse effects make them useful in this particular group of patients.

Central Nervous System involvement in Monoclonal gammopathies of undetermined significance has seldomly been reported and in all the cases a demyelinating disease was found. We report the case of a young man who had been suffering for five years of progressive cerebellar syndrome. MRI showed marked cerebellar vermis atrophy. An IgG lambda monoclonal gammopathy was revealed in the serum. Cerebrospinal fluid examination showed oligoclonal bands and elevated Link-Index. Serologic research for HBV, HCV, HIV, Lues, Rubella, Measles was negative, as also genetic analysis for SCA1, SCA2, SCA3, SCA7 and Friederich's ataxia. Nerve conduction studies were normal. Plasmatic vit.E was low, but treatment with high doses of tocopherol was ineffective. i.v. immunoglobulins and steroids obtained only transient clinical benefits. In conclusion, we hypothesize a pathogenetic role of the IgG in this cerebellar atrophy.

Patients with cerebral neoplasms often report pain or other kinds of symptoms which, even though not directly connected to the disease itself, can cause complications in the approach to therapy and worsen the quality of life of the patients. The therapies aimed at controlling these kinds of disturbances are referred to as supportive therapies as they do not cure the underlying disease. However, these therapies should not be underestimated, because, by controlling these disturbances, they are able not only to greatly improve the quality of life of the neuro-oncologic patients but also increase their survival. Based on this hypothesis, we will discuss and examine the drugs more frequently used for supportive therapy in neuro-oncologic patients. We assert, in fact, that the desire to offer neuro-oncologic patients better assistance guarantees not only adequate specialized input from the health operators involved, concerning the quality of life and the uniqueness of the 'person-patient', but also the ability to be able to listen to the patients, understand their choices and allow them to express their priorities.

We describe herein the case of a 57 year old man who, over the last five years, has presented ataxic and spastic gait on the right side, a reduction in fine motor movement of the fingers mainly on the right side, superficial right side brachiocrural hypoesthesia and a marked dysarthria associated with internuclear ophthalmoplegia. The neurological picture, after an initial progressive worsening which lasted some months, remained relatively stable over the years. Repeated magnetic resonance imaging (MRI) of the brain and spinal cord documented the presence of demyelinating plaques spread in the white matter of the periventricular region and the semioval centres, and a right side paramedian plaque at the C4-C5 level, none of which were in the active phase. Oligoclonal bands were revealed in the cerebrospinal fluid (CSF). Monoclonal IgM/lambda gammopathy with anti-myelin and anti-nucleo reactivity, found with serum immunofixation, were confirmed several times in successive annual controls, not associated to myeloproliferative pathology. The lack of progression in the clinical picture would seem to contradict the diagnosis of late Multiple Sclerosis. The presence of antibody activity against the myelin might support the hypothesis of a pathogenetic role of the immunoglobulins at the onset of the demyelinating disease in this patient. However, in the end, there is the possibility of casual association with a poorly functioning immune system connected to age.

Purpose: The aim of this study is to evaluate the neuroprotective effect of antioxidant supplementation with vitamin E in patients treated with cisplatin chemotherapy., Methods: Between April 1999 and October 2000, forty-seven patients were randomly assigned to either group one, which received vitamin E supplementation during cisplatin chemotherapy, or to group two, which received cisplatin chemotherapy alone. Alpha-tocopherol (vitamin E; 300 mg/d) was administered orally before cisplatin chemotherapy and continued for 3 months after the suspension of treatment. For preclinical studies, nude mice carrying the human melanoma tumor were treated with cisplatin alone or in combination with vitamin E., Results: Twenty-seven patients completed six cycles of cisplatin chemotherapy: 13 patients in group one and 14 patients in group two. The incidence of neurotoxicity was significantly lower in group one (30.7%) than it was in group two (85.7%; P <.01). The severity of neurotoxicity, measured with a comprehensive neurotoxicity score based on clinical and neurophysiological parameters, was significantly lower in patients who were supplemented with vitamin E than in patients who were not supplemented with vitamin E (2 v 4.7, P <.01). The results of the preclinical studies showed that when cisplatin was combined with vitamin E, no differences were observed in tumor weight inhibition, tumor growth delay, or life span as compared with treatment with cisplatin alone., Conclusion: Supplementation of patients receiving cisplatin chemotherapy with vitamin E decreases the incidence and severity of peripheral neurotoxicity.

Object: After radical surgery for childhood cerebellar astrocytomas, patients are considered to be cured. Long-term follow up demonstrates that these patients survive, with most of them leading a normal life. The study reported here was aimed at assessing the quality of life (QOL) of these adults, which is defined as a person's sense of well-being, as derived from his or her current experience of life as a whole., Methods: Twenty patients who had undergone surgery between 1970 and 1985 were enrolled in the study. In four patients ventriculoperitoneal shunts were in place; two of these patients had required more than six shunt revisions. At present, all patients have clear neuroimaging studies and their Karnofsky Performance Scale (KPS) scores are as follows: 70 in three, 80 in seven, 90 in six, and 100 in four. A QOL questionnaire was administered to the patients and to a control group consisting of 20 healthy volunteers of matching age and sex. The chi-square test was applied to compare patients and controls. Traditional questions on the level of education, work, whether the patients have their own families, and whether they possessed a driver's license were asked at the end of the questionnaire. In all the dimensions assessed except one (sex life), the difference between patients and control volunteers was significant, socializing and adolescence being the most striking ones. This was also true when the three patients with the lowest KPS scores and the worst QOL results were excluded., Conclusions: By traditional standards, these patients appear to fare quite well. Nevertheless, their self-reported life experience is unsatisfying when compared with the control group. The authors conclude that psychosocial factors are critical to complete recovery and the QOL of children who undergo successful operations for benign cerebellar astrocytoma.

Peripheral sensory neuropathy is the main non-hematological side-effect related to cisplatin chemotherapy. The strong similarity between clinical and neuropathological aspects in peripheral neuropathy induced by cisplatin and neurologic syndromes due to vitamin E deficiency, prompted us to investigate the relationship between cisplatin neuropathy and plasmatic level of vitamin E (alpha-tocopherol). We measured vitamin E in the plasma of 5 patients (Group 1) which developed severe neurotoxicity after cisplatin treatment and in another group of 5 patients (Group 2) we analyzed the plasmatic level of vitamin E before and after 2 or 4 cycles of cisplatin treatment. The results showed that the patients of group 1 presented low plasmatic levels of vitamin E and that the patients of group 2 presented significantly lower levels of vitamin E after 2 or 4 cycles of cisplatin than before treatment. Our preliminary data suggest that an inadequate amount of the antioxidant vitamin E due to cisplatin treatment could be responsible of the peripheral nerve damage induced by free-radicals. Given the lack of toxicity of vitamin E, we need to systematically assess the possible neuroprotective role of vitamin E supplementation in patients treated with cisplatin chemotherapy.

The aim of our study was to explore whether nerve growth factor (NGF) plays any role in the development of peripheral neuropathy induced by anticancer treatment. We measured the circulating NGF levels in 23 cancer patients before and after chemotherapy. We evaluated whether the development of peripheral neurotoxicity was associated with changes in basal NGF concentrations in patients studied with a comprehensive neurological and neurophysiological examination. The results of these studies showed that the circulating levels of NGF, which are about 20 pg/ml in plasma of controls, decrease during chemotherapy and in some cases completely disappeared after prolonged treatment with antitumor agents. The decrease in NGF levels seems to be correlated with the severity of neurotoxicity. These results clearly suggest that NGF might become a useful agent to prevent neuropathies induced by antineoplastic drugs and restore peripheral nerve dysfunction induced by these pharmacological compounds.

Sexual dysfunction after colorectal cancer surgery may be severe and occurs in 25% to 100% of cases. Thirty-eight patients underwent colorectal resection; eight (21%) who were totally impotent and two (5%) who had ejaculatory failure were therefore studied to better understand the neurophysiological alterations related to this type of surgery. The patients were evaluated after surgery with electrophysiological testing, including examination of the sacral reflex (SR), pudendal somatosensory evoked potential (PEP), and motor evoked potential (MEP) responses. Sudomotor skin response (SSR) was also studied in a group of patients. Of the 38 patients studied, 29 showed abnormalities: six of SR, three of PEP, six of MEP, and fourteen of SSR. Only a combination of all these tests permits correct evaluation of the sexual dysfunction.

One hundred and nineteen consecutive patients affected by supratentorial gliomas were studied in order to determine the incidence of seizure at diagnosis, the occurrence of subsequent seizures and the efficacy of anticonvulsant treatment. The overall incidence of seizures as presentation symptom was 52% (62 patients). Preoperative seizures were present in 83% of patients affected by low-grade astrocytoma, in 46% of patients affected by anaplastic astrocytoma and in 36% of patients affected by glioblastoma. Postoperative epilepsy refractory to anticonvulsant treatment significantly related to low-grade histology and presence of preoperative seizures occurred in 48% of patients. Adverse effects associated with anticonvulsants were observed in 33.8% of patients treated with phenobarbital, 14.3% in the group treated with carbamazepine and 12% of patients treated with vigabatrin. We conclude that anticonvulsant treatment in patients affected by gliomas is often ineffective and prophylactic treatment should be discontinued after 6 months in patients preoperative seizures free. In patients with high risk of seizures antiepileptic drugs with good efficacy and lower incidence of adverse effects than phenobarbital are indicated.

Twenty-eight patients affected by lung cancer were studied with neurophysiological and clinical examinations to show the prevalence of alteration of neuromuscular transmission. During repetitive stimulation at 20 Hz we found in 6 cases (22%) an increment (> 20%) and in 4 cases a decrement ( > 10%) of the size of muscle action potentials. In 15 patients (57%) we found low sensory action potential amplitude (< 11 uv). In 3 (11%) of the 6 cases with incremental response the overall examination suggested a disorder of neuromuscular junction such as Eaton-Lambert syndrome. Our results suggest that the peripheral nervous system involvement in patients affected by lung cancer is higher than usually estimated.

A group of 42 adult patients with supratentorial brain tumors were studied with computed tomography (CT or/and NMR) and brain-stem evoked potentials (BAEPs). 12 patients had meningiomas and 30 had malignant tumors (gliomas and metastases). The latencies of wave V and of the interpeak latency I-III, III-V and I-V were evaluated both on the lesion side and the opposite side. In meningiomas mean latencies did not show significant differences from normal, while mean latencies in malignant tumors were significantly prolonged, except for the I-III interpeak latency. The results of this study demonstrate that BAEPs may be altered by supratentorial brain tumors. This examination appears to be useful in the monitoring of these lesions after treatment.

12 patients with pituitary tumors were studied with VEP, to demonstrate involvement of the optic chiasma. In 3 patients VEP was recorded pre- and post-surgery to evaluate a modification in the shape and latencies of the potentials. 2 patients were studied only pre-surgery, the other 7 post-surgery. The utility of this test is discussed.

Lonidamine (LND) is a drug that interferes with energy metabolism of cancer cells, principally inhibiting aerobic glycolytic activity, by its effect on mitochondrially-bound hexokinase (HK). In such way LND could impair energy-requiring processes, as recovery from potentially lethal damage, induced by radiation treatment and by some cytotoxic drugs. A randomized study started in November 1983, to evaluate the efficacy of LND in association with radiotherapy as first line treatment in malignant gliomas, after surgical procedure. LND was also used in association with Lomustine (CCNU) at the moment of documented clinical and neuroradiological recurrence. At the present time 60 patients entered the study, and 47 are evaluable. Present preliminary results are not statistically significant, however indicate that LND tends to prolong the median survival time and the rate of one year survivors.

Nine patients with lung cancer without brain metastasis were studied pre and post brain prophylactic radiotherapy (30 Gy) to verify the effect of radiation on the cognitive processes. The P3 component of event-related potentials was used in this study. The latencies pre and post-radiation were compared and a significant delay (p less than 0.01) was detected in patients after treatment. These results suggest that brain radiotherapy may produce a cognitive impairment, as reported by many Authors.

Spasmophilia is a problem much debated as regards its nosographic location. Its paroxysmal onset in a "neurotic" patient has confined spasmophilia within a psychiatric syndromic complex for many years. In the present study, strict clinical criteria used in selecting a population of spasmophilic patients included clinical, neurophysiological, biohumoral and psychological evaluations, providing further information about the real organic features of this syndrome. Moreover, on the basis of an organic pathogenetic hypothesis, all patients were administered medical therapy which resulted in clinical amelioration in a significant number of cases.

The present investigation evaluates those few patients of our series with basal ganglia and brain-stem tumor who refused either surgical decompression and biopsy or radiation therapy. Four patients were suffering from tumors of the basal ganglia and three from brain-stem tumors; all the tumors were diagnosed by classical neuroradiological investigations and computerized tomography. The patients were given CCNU by mouth, 13 mg/sq m every 6 weeks. No toxicity was recorded. Mean survival was 19 weeks for patients with basal ganglia tumors and 48 weeks for those with brain-stem tumors. All patients were evaluated with respect to the quality of survival. Results were compared with those obtained in a control group of patients who received methylprednisolone therapy only.

The authors report their experience on 36 cases of thoracic tunnel syndrome. They deal with the nosographic arrangement of neurovascular syndromes of the upper limbs. Within the framework of the casuistics 13 patients were operated: the authors present clinical and E.M. Graphic criteria on the ground of which it was decided to operate. Results appear to be positive as to subjective symptomatology; they are less positive as to the objective one.

The silent period evoked by the electrical stimulus of the abductor pollicis brevis and the triceps surae muscles was studied in both normal subjects and in patients affected by spinal compression, by multiple sclerosis and by familial degenerative myelopathy. In normal subjects, the duration of the silent period is shorter in the upper limbs than in the lower limbs. In patients with spinal compression and multiple sclerosis, the duration of the silent period in the lower limb is significantly greater. On the contrary, the duration in familial degenerative myelopathy is significantly longer, recording from the abductor pollicis brevis. The authors conclude that this technique is useful in the diagnosis of spinal lesions from compression and demyelination.

A case of uncommon feature of herpes zoster, a segmental abdominal paresis, is described. The importance of searching a motor defect in the thoracoabdominal segments and the utility of the electromyographic examination are stressed.

Six surgically verified cases of pronator teres syndrome are reported. The anatomy of the median nerve at the upper third of the forearm, the relationships between the nerve and the pronator teres muscle and the serial innervation of the muscles of the forearm supplied by the n. medians are described. Clinical and electrophysiological features are reported in detail and their contribution in diagnosing the lesion is discussed.

The Authors describe a case of "stiff-man syndrome" accompanied by all clinical and laboratory examinations; they point out the unusual implication of facial muscles and briefly report pathogenic theories and diagnostical criteria of this syndrome.

The Authors describe two cases of "Anterior interosseus nerve syndrome" with spontaneous recovery. The etiopathogenesis of the syndrome is discussed after reviewing the anatomy of the nerve and muscles, as well as diagnostic procedures. The main clinical features are: 1) no sensory troubles, 2) impairment of muscles innervated by interosseus nerve (flexor pollicis longus, external portion of flexor digitorum profundus, pronator quadratus). The electrophysiological confirmation of the diagnosis is based on EMG findings, while conduction velocity appears to be complementary but not conclusive. The therapeutic aspects are discussed.