Simultaneous occurrence of linear scleroderma and homolateral segmental vitiligo.

Localized scleroderma and vitiligo only rarely have been reported to occur simultaneously. Here we report a case of a 21 year old man affected with both linear scleroderma of the left upper limb and homolateral segmental vitiligo of the trunk. Since the two diseases appeared during the same period, involved the same side of the body and their progression paralleled, a possible non-coincidental association between these two diseases is discussed.

Demyelinating disease in monoclonal gammopathy of undetermined significance.

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.

Nerve-sparing surgery in rectal cancer: feasibility and functional results.

Traditional rectal cancer surgery has been burdened with a high rate of sexual and urinary dysfunctions due to intraoperative injury or the cutting of the sympathetic and/or parasympathetic nerves. The experience acquired in the last ten years with total mesorectal excisions has permitted the use of the "nerve-sparing" technique. The present study regards 239 patients from two surgical centres, most of whom underwent sphincter-saving radical surgery between 1994 and 1998 with the above mentioned technique for resectable colon cancer. Details regarding the technique were recorded in the last 58 patients, in order to examine the severity of the surgical damage. The subgroup with the longest follow-up, which included 36 patients, was diagnostically evaluated by a surgeon, psychologist, urologist and neurologist to analyze the risk of sexual and urinary dysfunctions. A complete nerve-sparing was performed in 86.3% of the cases. The parasympathetic nerve trunks were those most often damaged because of perineural tumor spreading. Partial to complete sexual impotence was observed in 44% of the patients and surprisingly, preoperative dysfunctions were detected by means of the multidisciplinary approach in one third of these patients. Therefore, only 30.5% of the patients presented with strictly postoperative sexual impotency, above all, those who had undergone high-dose preoperative chemoradiation for T3 or T4 middle to low rectal cancer. A prospective study was initiated to evaluate the genitourinary dysfunctions after rectal cancer surgery in all of the clinical phases by means of a multidisciplinary approach aimed at functional recovery and improved quality of life.

Brachial plexus neuropathy as unusual onset of diffuse neurolymphomatosis.

We present a patient with a large B cell gastric lymphoma in total remission who, after 4 months, developed a fatal progressive peripheral neuropathy with an unusual early involvement of the right brachial plexus. No evidence of lymphoma was found at whole body computed tomography, magnetic resonance imaging of the head, cervical spine and right brachial plexus, bone marrow biopsy or repeated lumbar punctures. The diagnosis of neurolymphomatosis was made only at postmortem examination.

Neurophysiological evaluation of sexual dysfunction in patients operated for colorectal cancer.

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.

Epilepsy and gliomas: incidence and treatment in 119 patients.

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.

Paclitaxel neurotoxicity: clinical and neurophysiological study of 23 patients.

Paclitaxel is the prototype of a new class of chemotherapeutic agents with an antimitotic effect that is related to its ability to interfere with the microtubule system. It causes peripheral neurological toxicity by means of its activity on the axonal microtubules. To define the clinical and neurophysiological characteristics of paclitaxel neuropathy 23 patients undergoing paclitaxel therapy at a dose of 175 mg/m2 were studied. The patients were divided into two groups, with only one group receiving pretreatment with potentially neurotoxic drugs such as cisplatin and carboplatin. The results showed a high incidence of mild neurotoxicity in both groups. Treatment was discontinued due to severe neurotoxicity in only one patient pretreated with platinum-compounds. The clinical and neurophysiological data make it possible to define paclitaxel neurotoxicity as a distal axonal neuropathy with a summatory effect in patients pretreated with cisplatin; the possible reversibility of paclitaxel neurotoxicity requires further confirmation.

Vinorelbine neurotoxicity: clinical and neurophysiological findings in 23 patients.

Vinorelbine (5'-noranhydrovinblastine) is a new semisynthetic antineoplastic vinca alkaloid which interfers with axonal transport, inducing spiralisation of axonal microtubules and resulting in peripheral neurotoxicity. A prospective detailed neurological and electrophysiological evaluation was performed in 23 patients treated with 25 mg vinorelbine a week. All patients developed a sensory-motor distal symmetric axonal neuropathy. The neurotoxicity increased with cumulative vinorelbine doses and peripheral neuropathy was mild or moderate in most patients. After discontinuation of vinorelbine treatment, neuropathic signs and symptoms were partially reversible.

Neurophysiological examination of end-plate function in patients with lung cancer.

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.

Neurotoxicity of antitumoral IL-2 therapy: evoked cognitive potentials and brain mapping.

Antitumoral interleukin-2 (IL-2) therapy is frequently associated with a neurotoxicity that manifests itself in neuropsychiatric disturbances and, more rarely, with neurological focal signs. With the aim of documenting the involvement of cognitive functions after IL-2 treatment, we studied 20 patients using evoked cognitive potentials (P300) and computer analysis of the EEG signal. A comparison of the tests performed before and after the subcutaneous infusion of IL-2 showed a significant lengthening in mean P300 latencies and a percentage increase in the EEG frequencies contained with the delta and theta bands, especially in the frontal regions.

Brain-stem auditory evoked potentials in supratentorial brain tumors.

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.

The VEP in evaluation of pituitary tumors.

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.

The effect of brain radiotherapy on the P3 component of ERP's.

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.

Silent period in spinal cord pathology.

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.

Anterior interosseous nerve syndrome.

Weakness of the flexor pollicis longus, flexor digitorum profundus and pronator quadratus is usually related to an isolated paralysis of the anterior interosseous nerve in the volar aspect of the forearm. The same syndrome may be caused by lesions of the median nerve in the supracondylar region and by involvement of the fibers assigned to the abovementioned nerve in the medial and lateral cords of the brachial plexus. We report 4 cases of the anterior interosseous nerve syndrome (3 spontaneous and one traumatic), review and discuss etiological factors, clinical features and electromyographic findings in the syndrome.

Segmental abdominal zoster paresis.

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.

Spasmophilia: a clinical neurophysiopathological and biochemical study.

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 pronator teres syndrome. Clinical and electrophysiological features in six surgically verified cases.

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.