Luana Benedetti

Sant´Andrea Hospital, Roma, Latium, Italy

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Publications (31)128.77 Total impact

  • XLV CONGRESSO SOCIETÀ ITALIANA DI NEUROLOGIA, Cagliari - Italia; 10/2014
  • Atti del IV Meeting dell'associazione Italiana per lo studio del sistema periferico, S2-S2; 04/2014
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    ABSTRACT: Fifteen days after a respiratory infection, a 45-year-old woman presented with paresthesias in the hands and feet, bilateral loss of vision, fever, headache, and impairment of consciousness. Magnetic resonance imaging (MRI) showed predominant lesions in the optic tracts, optic chiasm, and hypothalamus. Cerebrospinal fluid analysis revealed elevated protein level, and lymphocytic pleocytosis. Neurophysiological studies disclosed a demyelinating sensorimotor polyneuropathy. Serum anti-Mycoplasma pneumoniae immunoglobulin (Ig)M, anti-GM1 IgG, and anti-AQP4 IgG were positive. This case, which is remarkable for post-infectious meningoencephalitis-like onset, MRI picture, and dysimmunity to central and peripheral nervous system autoantigens, underpins the pivotal diagnostic role of anti-AQP4-IgG, and expands the list of clinico-pathological findings that can associate with neuromyelitis optica spectrum disorders.
    Multiple Sclerosis 02/2014; DOI:10.1177/1352458514524294 · 4.86 Impact Factor
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    ABSTRACT: Mycosis Fungoides (MF) is a rare malignant T-cell lymphoma, involving mainly the skin. In 50%-75% of cases, it can involve organs other than skin, with a 11%-14% Central Nervous System involvement (CNS). A 82-year-old woman presented to our Department with a 15-years history of MF with skin lesions. Neurological examination showed dysarthria and a left facio-brachial-crural hemiparesis. A CT scan showed a right fronto-rolandic lesion. A MRI, including DWI, confirmed the presence of the "neoplastic" lesion with slight hemorrhagic component and leptomeningeal contrast enhancement. Molecular TCR rearrangement test by PCR analysis was performed on skin biopsy, showed the presence of a single peak which fits with a monoclonal TCRG gene rearrangement (size 67). Molecular TCR test was also performed on the cerebrospinal fluid (CSF), which confirmed the presence of lymphocyte clone T g/ more expressed with the same size of that observed in the skin biopsy A total body CT scan did not show any lymphnodal or extranodal disease. The patient died after ten days. MF usually occurs in the context of advanced and often histologically transformed cutaneous disease. Isolated CNS involvement is remarkably rare. This case highlights the need for regular neurologic follow-up after the diagnosis of MF, in particular when features that suggest risk of disease progression are present. Furthermore, the analysis of the skin biopsy and above all of CSF by PCR technique, based on our experience, should always be executed in MF patients with signs or symptoms suggesting CNS involvement.
    SpringerPlus 01/2014; 3:29. DOI:10.1186/2193-1801-3-29
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    ABSTRACT: BACKGROUND: Natural killer (NK) cells can bridge innate and acquired immunity, and play a role in autoimmunity. A few studies evaluated the distribution of NK cells and the expression of their receptors in chronic immune-mediated demyelinating polyneuropathies. We investigated NK cell distribution and NK cell receptor expression in 20 naïve patients with anti-MAG polyneuropathy (MAG-PN). METHODS: Using flow cytometry, we analysed NK cells and a series of NK cell receptors in the peripheral blood of patients with MAG-PN, and, as controls, in patients with chronic inflammatory demyelinating peripheral polyradiculoneuropathy (CIDP) and in healthy subjects. Six MAG-PN patients were also tested after rituximab treatment. RESULTS: At baseline the percentage of NK cells did not differ among the groups. KIR2DL2 receptor expression in MAG-PN patients was higher, andCD94/NKG2A receptor expression in both MAG-PN and CIDP patients was lower than in healthy controls. These abnormalities did not correlate with any clinical or demographic variable. No modification was found after rituximab therapy. CONCLUSIONS: The data suggest that MAG-PN shows abnormalities in NK cell receptors that characterise other autoimmune diseases, and cannot help in differential diagnosis with CIDP. The impairment of the relevant CD94/NKG2A inhibitory pathway, which might play a central role in the development and perpetuation of MAG-PN, warrants further functional investigations.
    Journal of the neurological sciences 06/2013; DOI:10.1016/j.jns.2013.05.015 · 2.26 Impact Factor
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    ABSTRACT: Pain is a subjective condition that cannot be objectively measured; for this reason, self patient-perspective is crucial. Recently, several screening tools to discriminate between nociceptive and neuropathic pain have been developed. We aimed at assessing the consistence and discrepancy of two widely used screening tools, The Douleur Neuropathique 4 (DN4) and the 6-item questionnaire (ID-Pain), by comparing their ability in discriminating neuropathic from nociceptive pain. DN4 and ID-Pain were administered to 392 Italian patients attending 16 outpatient services for peripheral nerve diseases. Based on medical history, clinical findings and diagnostic tools, patients were divided into two groups (neuropathic and nociceptive). Globally, ID-Pain identified neuropathic pain in 60 % of patients (38 % probable, 22 % likely). Interestingly also DN4 diagnosed neuropathic pain in 60 % of cases. A discrepancy was observed in 16 % of cases. DN4 and ID-Pain resulted to be highly interrelated in the identification of neuropathic pain. Sensitivity of DN4 was 82 % and specificity was 81 %, while ID-Pain (considering both probable and likely groups) showed sensitivity 78 % and specificity 74 %. Reliable screening tools for neuropathic pain are well related between them; hence, they are available for researchers and clinicians who may choose the most appropriate for their activity. Since the gold standard for the diagnosis and treatment of neuropathic pain cannot do without a neurological evaluation, perhaps DN4, that includes physician objective measures, may help reducing the percentage of dubious cases. Conversely, when needing a more agile tool (not needing a physician) ID-Pain may be adopted.
    Neurological Sciences 03/2012; 34(3). DOI:10.1007/s10072-012-1011-3 · 1.50 Impact Factor
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    ABSTRACT: There are other options open to patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) who are non-responders to conventional treatment, including immunosuppressive and immunomodulatory agents (IA). The aim of this study was to assess whether the use of IA is able to increase the number of responders. Clinical and electrophysiological data of patients with refractory CIDP, followed at 10 Italian centres, were collected, and the clinical outcome (Rankin Scale) and drug side effects (SE) for the different therapies were analysed. A total of 110 patients were included. These patients underwent 158 different therapeutic procedures with IA. Seventy-seven patients were treated with azathioprine, 18 rituximab, 13 cyclophosphamide, 12 mycophenolate mofetil, 12 cyclosporine, 12 methotrexate, 11 interferon-alpha and three interferon beta-1a. The percentage of patients who responded to azathioprine (27%) was comparable to the percentage of responders to other therapies, after the exclusion of interferon beta-1a that was not effective in any of the three patients treated. The percentage of SE ranges from 8% (methotrexate) to 50% (cyclosporine). One-fourth of patients, refractory to conventional treatment, showed an improvement in their disability with IA. Methotrexate had the lowest SE; cyclosporine was associated with severe SE and often led to drug discontinuation.
    European Journal of Neurology 08/2011; 18(12):1417-21. DOI:10.1111/j.1468-1331.2011.03495.x · 3.85 Impact Factor
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    ABSTRACT: A few case reports have shown controversial results of rituximab efficacy in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). To analyse the efficacy of rituximab in a large CIDP cohort. A retrospective, observational and multicentre study on the use of rituximab in CIDP. 13 Italian CIDP patients were treated with rituximab after the partial or complete lack of efficacy of conventional therapies. Eight patients had co-occurring haematological diseases. Patients who improved by at least two points in standard clinical scales, or who reduced or discontinued the pre-rituximab therapies, were considered as responders. Nine patients (seven with haematological diseases) responded to rituximab: six of them, who were non-responders to conventional therapies, improved clinically, and the other three maintained the improvement that they usually achieved with intravenous immunoglobulin or plasma exchange. Significantly associated with shorter disease duration, rituximab responses started after a median period of 2.0 months (range, 1-6) and lasted for a median period of 1 year (range, 1-5). Rituximab seems to be a promising therapeutic choice when it targets both CIDP and co-occurring haematological diseases. Timely post-onset administration of rituximab seems to be associated with better responses.
    Journal of neurology, neurosurgery, and psychiatry 03/2011; 82(3):306-8. DOI:10.1136/jnnp.2009.188912 · 5.58 Impact Factor
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    ABSTRACT: Antimyelin-associated glycoprotein (MAG) polyneuropathy is a slowly progressive distal form of mixed motor-sensory polyneuropathy that is scarcely responsive to conventional immunosuppressive therapy. Rituximab, a B-cell depleting antibody, is a promising therapeutic choice for anti-MAG polyneuropathy, and the evaluation of factors, such as B-cell-activating factor (BAFF), that control B-cell homeostasis is important to understand how this drug works. Using an ELISA method, the authors measured serum BAFF concentrations in 23 patients with anti-MAG polyneuropathy, before and after rituximab therapy, in 20 neurological controls and in 14 healthy subjects. The patients were followed up over a mean period of 38±12 months and categorised as responders/non-responders, and, between the responders, as relapsing/non-relapsing. Pretherapy serum BAFF concentrations in non-responders were higher than in responders (cut-off 1665 pg/ml; sensitivity 71.4%; specificity 93.7%; likelihood ratio 11.4), with the highest post-therapy increases in responders. In the responders who relapsed, relapses occurred when serum BAFF concentrations returned to baseline values, 1-2 years after blood B-cell reappearance. Before and during therapy, measurements of serum BAFF in rituximab-treated patients with anti-MAG polyneuropathy may help predict the response to the therapy. The findings in this study also provide information about rituximab-induced modifications on B-cell homeostatic regulation.
    Journal of neurology, neurosurgery, and psychiatry 12/2010; 82(11):1291-4. DOI:10.1136/jnnp.2010.222216 · 5.58 Impact Factor
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    ABSTRACT: Hereditary inclusion body myopathy (IBM2) was mainly reported in Middle Eastern Jewish patients. Distal myopathy with rimmed vacuoles has been described as a worldwide distributed distal myopathy. Both diseases are caused by mutations of the UDP-N-acetylglucosamine 2-epimerase/N-acetylmannosamine kinase (GNE) gene. Herein we report two patients: an Egyptian Muslim patient with the "common" Middle Eastern mutation (M712T), rarely described in non-Jewish patients; and an Italian patient carrying a novel GNE mutation (L179F) in the epimerase domain. Our patients share common clinical and histopathological features, with some interesting aspects. The first patient presented a clinical deterioration during her first pregnancy confirming that an increased requirement of sialic acid during pregnancy may trigger a clinical worsening. The second patient showed a slowly progressive deterioration, different from other patients carrying mutations in the epimerase domain, who had a severe and rapid progression.
    Neurological Sciences 03/2010; 31(3):377-80. DOI:10.1007/s10072-010-0248-y · 1.50 Impact Factor
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    ABSTRACT: The guidelines for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) therapy suggest to use immunoglobulins (IVIg) and steroid as first-line therapies. Patients who do not respond to one of the two drugs should be switched to the other drug. We collected therapeutic outcome data in patients followed at 11 centres in order to document the clinical practice in Italy. Clinical and electrophysiological data of patients with CIDP were entered into a central database. The clinical outcome (Rankin Scale) and drug side effects (SE) for first- and second-line therapies were recorded. A total of 267 patients were included. The percentage of responders (R) to first-line therapy [steroid or IVIg or plasma exchange (PE)] was 69%; this number increased to 81% when patients who switched to different therapies were included. Overall, the percentage of R to IVIg was similar to R to steroids (P = 0.07) and higher than R to PE (P < 0.001). Of the main therapies, PE frequently caused SE (19%), followed by steroids (12.5%) and IVIg (4%). Switching between traditional therapies increases the number of responder patients. IVIg was confirmed to be a therapy with low SE.
    European Journal of Neurology 10/2009; 17(2):289-94. DOI:10.1111/j.1468-1331.2009.02802.x · 3.85 Impact Factor
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    ABSTRACT: The aim of this study was to validate the Italian version of the Neuropathic Pain Symptom Inventory (NPSI) in patients with neuropathic pain due to peripheral nerve diseases, and also to evaluate the validity of a new NPSI score: a frequency weighted NPSI score (NPSI-FW). First, the original version of the NPSI was translated into Italian. Then the validity and reliability of the Italian NPSI (I-NPSI) were tested in 392 Italian patients consecutively referred to 16 Italian outpatient services for peripheral nerve diseases, by correlating the I-NPSI scores with other pain scales. The repeatability and responsiveness were assessed. A significant correlation between the I-NPSI scores and all the other pain measures was seen. Reproducibility and responsiveness were good. Our study shows the validity of the I-NPSI and demonstrates its reliability for assessing neuropathic pain in patients with peripheral nerve diseases. The I-NPSI scores represent reliable measurements to assess neuropathic symptoms and effectiveness of treatment on them.
    Neurological Sciences 03/2009; 30(2):99-106. DOI:10.1007/s10072-009-0025-y · 1.50 Impact Factor
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    ABSTRACT: BACKGROUND: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) responds to treatment with corticosteroids, intravenous immunoglobulin, and plasma exchange. We aimed to test whether the standard immunosuppressive drug methotrexate was of use in treatment of CIDP. METHODS: In a pilot, multicentre, randomised, double-blind, controlled trial we compared oral methotrexate 7.5 mg weekly for 4 weeks, then 10 mg weekly for 4 weeks, and finally 15 mg weekly for 32 weeks (40 weeks' total treatment) with placebo in patients with CIDP requiring intravenous immunoglobulin or corticosteroids. After about 16 weeks, the dose of corticosteroids or intravenous immunoglobulin was decreased by 20% every 4 weeks if participants did not deteriorate. Primary outcome was a greater than 20% reduction in mean weekly dose in the last 4 weeks of the trial compared with the first 4 weeks. Secondary outcomes analysed separately at the mid-trial and final visits measured activity limitations and strength. Analyses were done by intention to treat. This study is registered as an International Standard Randomised Controlled Trial, number ISRCTN73774524. FINDINGS: 59 of the 60 enrolled participants completed the trial. 14 (52%) of 27 taking methotrexate and 14 (44%) of 32 taking placebo had a greater than 20% reduction in mean weekly dose of corticosteroids or intravenous immunoglobulin (adjusted odds ratio 1.21, 95% CI 0.40-3.70). There were no clinically and statistically significant differences in secondary outcomes. The one serious adverse event in the placebo group and the three in the methotrexate group were not thought to be related to treatment. INTERPRETATION: Oral methotrexate 15 mg weekly showed no significant benefit, but limitations in the trial design and the high rate of response in the placebo group meant that a treatment effect could not be excluded. This study can inform design of future trials in CIDP. FUNDING: The GBS/CIDP Foundation International.
    The Lancet Neurology 02/2009; · 21.82 Impact Factor
  • Neurology 12/2008; 71(21):1742-4. DOI:10.1212/01.wnl.0000335268.70325.33 · 8.30 Impact Factor
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    ABSTRACT: Reactivation of the varicella-zoster virus (VZV) along the distribution of the sensory nerves innervating the ear, which usually includes the geniculate ganglion, is responsible for herpes zoster (HZ) oticus. When associated with facial paralysis, the infection is called Ramsay Hunt syndrome (RHS) [1]. Typically, RHS involves facial nerve unilaterally. Rarely other cranial nerves may be affected [2, 3]. Here we report an 82-year-old man who developed a hoarse voice, decreased hearing, difficulties in swallowing, nasal reflux and left facial droopiness five days after the onset of ear pain and fever. Crusted scars and an erythematous base on the external left ear were visible on examination. Neurological examination revealed hypoaesthesia of the 2nd branch of the left trigeminal nerve, together with the involvement of left VIIth (facial weakness), VIIIth (decreased hearing), IX and Xth (palatal and laryngeal paresis with dysphonia and dysphagia), XIth (weakness of the sternocleidomastoid muscle) and XIIth (leftward tongue protrusion) nerves. The clinical picture developed gradually over a few days. The first symptoms were dysphagia and facial weakness. Laryngoscopy revealed multiple ruptured vesicles and oedema of left hemi-pharynx and of the tongue. Cerebrospinal fluid (CSF) analysis showed increased protein levels, with normal cell count. Polymerase chain reaction for VZV was positive. CSF cytology was normal. MRI of the brain showed enhancement of left cranial nerves V, VII and VIII, and of the anterior condyloid canal (Fig. 1), as well as hyperintensity of the left hemipharynx (STIR T2-weighted image). Considered together the data suggested RHS with multiple cranial nerve involvement and intravenous treatment with acyclovir (10 mg/kg every 8 h daily for two weeks) and prednisolone (500 mg/day for 3 days) was therefore started. After 6 weeks, improvement of the facial weakness and dysphagia was observed, as well as recovery of sensitivity in the trigeminal nerve zone. However, the dysphonia and the sternocleidomastoid weakness remained unchanged. This is a quite atypical case in which the HZ oticus infection was complicated by a multiple unilateral palsy of the lower cranial nerves. From a clinical point of view the patient had involvement of the V, VII/VIII, IX, X, XI and XII left cranial nerves consistent with multicranial neuritis. An interesting aspect of our case report is the documentation of the inflammatory involvement of cranial nerves upon neuroimaging. In fact, the contrast-enhanced MRI demonstrates enhancement of some of these nerves: V, VII and VIII. Enhancement of the anterior condyloid canal could be related to inflammatory involvement of the XII nerve,
    Neurological Sciences 12/2008; 29(6):497-8. DOI:10.1007/s10072-008-1022-2 · 1.50 Impact Factor
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    ABSTRACT: Leptomeningeal (LM) carcinomatosis is an increasing clinical complication in patients with advanced breast cancer (BC). The LM carcinomatosis diagnostic procedures rely mainly on cerebrospinal fluid (CSF) cytology, although both the amount of CSF and the number of malignant cells remain limiting factors. Therefore, efforts should be made to design new highly sensitive diagnostic tools to detect malignant cells in CSF of BC patients with LM carcinomatosis. In this study, the human Mammaglobin (hMAM) mRNA amplification by RT-PCR was employed to detect metastatic cells in CSF and thus, to diagnose LM carcinomatosis in a BC patient. Our data demonstrate that hMAM transcripts are expressed in the CSF of a BC patient with LM carcinomatosis, hence making RT-PCR for hMAM a potentially suitable test to identify occult BC cells in the brain.
    Journal of Neuro-Oncology 11/2008; 91(3):295-8. DOI:10.1007/s11060-008-9711-5 · 2.79 Impact Factor
  • Muscle & Nerve 08/2008; 38(2):1076-7. DOI:10.1002/mus.21073 · 2.31 Impact Factor
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    ABSTRACT: To describe the unique case of a patient with multiple sclerosis (MS) and anti-myelin-associated glycoprotein (MAG) polyneuropathy who developed MS relapses after treatment with rituximab. Case report. Department of Neurosciences, Ophthalmology, and Genetics, University of Genova, Genova, Italy. Patient A 59-year-old man with an 18-year history of MS presented with an unusually rapid progression of paraparesis with hypopallesthesia and areflexia in 4 limbs. Neurophysiological and serological studies led to the diagnosis of anti-MAG polyneuropathy. Cerebrospinal fluid analysis disclosed the loss of oligoclonal IgG bands that were previously detected at MS onset. Intervention Rituximab was administered at a dosage of 375 mg/m(2)/wk for 4 weeks. Result The patient developed 2 corticosteroid-responsive MS relapses with improvement of the polyneuropathy. Rituximab can be effective in anti-MAG polyneuropathy but can possibly lead to unexpected consequences in individuals with MS.
    JAMA Neurology 11/2007; 64(10):1531-3. DOI:10.1001/archneur.64.10.1531 · 7.01 Impact Factor
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    ABSTRACT: We evaluated the efficacy and safety of rituximab in an open-label, uncontrolled study of 13 patients with polyneuropathy associated with antibodies to myelin-associated glycoprotein (MAG) and correlated the response to therapy with clinical and laboratory features. One year after rituximab therapy, anti-MAG immunoglobulin M (IgM) titers were significantly reduced. At that time, eight patients (62%) had improved in both the inflammatory neuropathy cause and treatment (INCAT) sensory sumscore and the Medical Research Council sumscore for muscle strength and seven of them also in the INCAT disability score. The improvement in the mean INCAT sensory sumscore was significant at 12 months and correlated with lower anti-MAG antibody at entry and at follow-up. This study suggests that rituximab may be efficacious in patients with anti-MAG associated neuropathy and particularly on sensory impairment and in those with moderately elevated antibody titers. These findings suggest that antibody reduction below a critical level may be necessary to achieve clinical improvement.
    Journal of the Peripheral Nervous System 07/2007; 12(2):102-7. DOI:10.1111/j.1529-8027.2007.00129.x · 2.50 Impact Factor
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    ABSTRACT: To assess the prevalence of peripheral neuropathy (PN) and its correlation with cryoglobulinemia (CG) in an unselected, untreated referral hepatitis C virus (HCV) population. Two hundred and thirty four patients (120 women and 114 men) with untreated HCV infection were consecutively enrolled by seven Italian centres. Clinical neuropathy was diagnosed when symptoms and signs of peripheral sensory or motor involvement were present. Median, ulnar, peroneal, and sural nerves were explored in all patients and distal symmetric polyneuropathy was diagnosed when all explored nerves or both lower limb nerves were affected. Mononeuropathy and mononeuropathy multiplex were diagnosed when one nerve or two non-contiguous nerves with asymmetrical distribution were affected. Screening for CG was done in 191 unselected patients. Clinical signs of PN were observed in 25 of the 234 patients (10.6%). Electrophysiological PN was found in 36 (15.3%). CG was present in 56/191 patients (29.3%). The prevalence of CG increased significantly with age (p<0.001) and disease duration (p<0.05). PN was present in 12/56 (21%) patients with CG and 18/135 (13%) without CG (p=NS). PN increased significantly with age (p<0.001) and logistic regression analysis confirmed age as the only independent predictor of PN (OR 1.10 for each year; 95% CI 1.04 to 1.15; p<0.001). Electrophysiological examination detected subclinical neuropathy in 11 patients (4.7%). Statistical analysis showed that CG was not a risk factor for PN whereas PN prevalence increased significantly with age.
    Journal of Neurology Neurosurgery & Psychiatry 05/2006; 77(5):626-9. DOI:10.1136/jnnp.2005.081570 · 5.58 Impact Factor

Publication Stats

352 Citations
128.77 Total Impact Points

Institutions

  • 2008–2014
    • Sant´Andrea Hospital
      Roma, Latium, Italy
  • 2004–2010
    • Università degli Studi di Genova
      • Dipartimento di Medicina sperimentale (DIMES)
      Genova, Liguria, Italy
  • 2006
    • University of Naples Federico II
      Napoli, Campania, Italy