Objective: Despite the high prevalence of epilepsy in brain tumors, long-term follow-up studies on tumoral epilepsy are lacking. Here we report the longterm follow-up results of the PERNO study (Project of Emilia-Romagna Region on NeuroOncology). Methods: PERNO study enrolled for a three-year period every person living in Emilia-Romagna Region with a new diagnosis of primary brain tumor (PBT). We previously described the short-term results of epilepsy evolution after the first surgical procedure in 100 patients; for 52 patients of this group, the long-term results of seizure outcome after a mean follow-up of 24 months are now available. Results: Out of these 52 patients, 41 presented with a high-grade glioma (HGG), whereas 11 had a lowgrade glioma (LGG) at the onset of the disease (HGG/LGG ratio=3.7) and were followed-up for a median period of 548 and 1032 days, respectively. The HGG group showed a seizure-freedom rate of more than 60%, with a better seizure control (up to 87%) being achieved in patients without evidence of tumor progression. Conversely, in the LGG group complete seizure control occurred only in one case (less than 10% of patients), with most patients requiring surgical revision due to the extension of the tumor. Conclusions: Our data show that epilepsy associated with HGG seems to have a relatively good outcome, especially if the tumor is stable. In contrast, seizure control in LGG was more difficult to be achieved, suggesting the need of detailed epileptological study (including long-term video-EEG monitoring) before surgery to improve seizure control.

Takotsubo cardiomyopathy (TTS) is a reversible dysfunction of the left ventricle without coronary disease, probably related to excessive catecholamine release induced by stressful factors in predisposed people. ECG may present with ST segment elevation or T wave inversion.1 Several neurological conditions predispose to TTS, included epilepsy.2 We describe a patient who developed TTS after recurrent seizures. A 78 years old man with parossistic atrial fibrillation and epilepsy symptomatic of previous ischemic stroke was hospitalized for relapsing of repeated partial seizures. In Emergency Room, cerebral CT, blood exams and ECG excluded urgent abnormalities. Three hours after hospitalization the patient appeared sweaty with hypotension (90/50 mmHg). No other symptoms. ECG showed T wave inversion. Echocardiography demonstrated a hypokinesia apex with EF 35% and modest rise of troponin. Coronary angiography showed apical ballooning and vessels unharmed. The patient slowly recovered ventricular dysfunction. This case seems worthy of mention to underline that shared cardiovascular risk factors can account for the relationship between epilepsy and heart disease, in addition to genetics and etiological factors. Therefore, we think that ECG recordings after seizures could be indicated. Timely recognition of this syndrome is important to provide adequate supportive care and prevent complications.

Leucine-rich glioma inactivated 1(LGI1) antibodies are frequently associated with a common form of limbic encephalitis (LE) presenting with cognitive impairment, tonic-clonic and distinctive facio-brachial dystonic seizures (FBDS), not related with a specific EEG pattern. We describe clinical-EEG features of three patients with LGl1-LE with different response to immunotherapy. Three pts, admitted to our Neurology department for LE with positive test for LGI1 antibodies, performed video-EEG monitoring and immunotherapy. Video and still images showed frequently (3–40 episodes/day) paroxysmal brief events characterized by facial grimacing and sometimes dystonic arm posturing, with alternating side, suggestive for FBDS. We observed electrodecremental events (EDEs) or a contralateral slow-wave before “dystonic seizures” but not a clear ictal-EEG pattern. In all of the patients interictal EEG was normal. Furthermore, Video-EEG monitoring revealed multiple daily electrographic temporal seizures. Immunotherapy were associated with control of FBDS in two of three presented patients, with different latency. Video-EEG monitoring of our LGI1-LE patients showed frequent FBDS and bilateral temporal seizures. The lack of a clear ictal-EEG pattern for FBDS and the positive response to immunotherapy, support the hypothesis that dystonic episodes does not have a cortical origin, but are rather related to a possible basal ganglionic dysfunction.

Previous epidemiologicalsurveys, both analytic anddescriptive, in the Local HealthDistrict (LHD) of Ferrara, northernItaly, have indicated that rural residenceand agricultural work mightconstitute risk factors for AmyotrophicLateral Sclerosis (ALS).The present investigation is ademographic survey in the LHD ofFerrara in the years 1964–1998which aimed to verify whether thelevel of urbanization and agriculturalactivities might influence therisk of ALS. Based on the data obtainedin a recent incidence studyin the LHD of Ferrara which reporteda mean annual crude incidencerate of ALS in the years1964–1998 of 1.63 per 100,000 population(95 % CI 1.31–2.00), it waspossible to compare the number ofobserved ALS cases and the numberof expected ALS cases accordingto the level of urbanization andusual occupation on the basis ofthe residential and occupationalpattern identified in the populationof the LHD of Ferrara in the studyperiod under the assumption of ahomogeneous distribution of ALS.The present survey identified fourdifferent levels of urbanization inthe LHD of Ferrara in the studyperiod and for none of them was adifference between the number ofobserved and expected ALS casesfound. Also in the most rural of thefour identified levels of urbanization(small villages with an averagepopulation in the study periodlower than 1,000 inhabitants andscattered houses in the countryside)no difference was found betweenobserved and expectednumber of ALS cases (observedALS cases 16, 95% Poisson CI9.1–25.9, expected ALS cases 18.3).Based on the occupational patternidentified in the population of theLHD of Ferrara in the study periodthe number of incident cases ofALS whose usual occupation was inagricultural work exceeded theexpected number (observed ALScases 22, 95% Poisson CI 13.8–32.3,expected ALS cases 6.0). The presentfindings indicate that ruralresidence itself does not influencethe risk of ALS while agriculturalactivities could influence the riskof ALS, with occupational exposureto agricultural chemicals playing apossible role.

Background: The reported annual incidence of myasthenia gravis (MG) ranges from 0.25 to 15 per million. The sex- and age-related pattern of disease incidence is still debated. Methods: An intensive descriptive study was performed in the province of Ferrara (mean population 360,950 people) over the period 1985 through 2000. Results: The average crude annual incidence rate was 2 per 100,000. We confirm a female preponderance in the total population, particularly in the youngest age groups. Conclusions: We observed an early increase in incidence in females, partly due to thymoma-associated MG, while MG without thymoma showed increasing incidence with age nonsignificantly different in the two sexes.

BACKGROUNDStudies have reported circadian variation in the onset of ischemic stroke, which may carry important pathophysiological implications. However, there is no detailed information about circadian variations among the subtypes of stroke.OBJECTIVETo determine whether subgroups of patients with ischemic stroke with specific clinical characteristics would exhibit different circadian patterns, to more systematically examine the role of possible triggering or precipitating factors.DESIGN AND SETTINGAnalysis of the effects of demographic, medical, and pathophysiological factors on the circadian pattern of an unselected series of patients with ischemic stroke consecutively admitted to our hospital.RESULTSThe study included 1656 patients. As in other studies, the peak of stroke onset occurred in the morning, with a second peak in the evening. Circadian variation in ischemic stroke onset was shown to be independent of clinical variables considered.CONCLUSIONSOur study confirms the circadian rhythm of stroke reported in previous studies. There is a chronological pattern of ischemic stroke in the morning, which appears to be independent of the presence of risk factors and of clinical stroke subtypes. The role of circadian variability of blood pressure (present in patients with and without hypertension) and a concurrent morning hypercoagulability are suggested as possible determinants of this pattern. Preventive pharmacological interventions aimed at specifically targeting the morning rise in risk factors could be advantageous in reducing the overall risk of ischemic stroke.

The onset of stroke has a specific temporal pattern ( 1 Manfredini R Gallerani M Portaluppi F et al. Chronobiological patterns of onset of acute cerebrovascular diseases. Thromb Res. 1997; 88: 451-463 Abstract Full Text Full Text PDF PubMed Scopus (122) Google Scholar ), characterized by a higher frequency in winter and in the mornings. According to a meta-analysis of more than 11,000 patients, an estimated 37% of strokes occurred during morning hours ( 2 Elliott W.J Circadian variation in the timing of stroke A meta-analysis. Stroke. 1998; 29: 992-996 Crossref PubMed Scopus (548) Google Scholar ). Variability of the day of week has not been extensively investigated, however. To verify a temporal weekly pattern, we reviewed all cases of stroke admitted to our hospital during a 4-year period.

The objective of this study was to assess the long-term course and treatment of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). We evaluated, according to a predefined protocol, a series of 60 CIDP patients who received a long-term course of steroids and immunosuppressants. Eighteen of them also had monoclonal gammopathy of undetermined significance (MGUS). Mean follow-up was 4.4 years and was similar for CIDP and CIDP-MGUS patients. At the end of the follow-up, improvement was ascertained in 60% of patients (69% CIDP, 39% CIDP-MGUS). Complete remission was achieved in 13%. Out of 26 patients receiving steroids as a monotherapy, 19 improved (73%). The following variables were predictive of a better outcome: female gender, younger age at onset, relapsing-remitting course, and absence of axonal damage at neurophysiologic study. In the multivariate analysis, younger age at onset and demyelination without axonal damage still retained an independent positive value.

Annals of the New York Academy of SciencesVolume 883, Issue 1 p. 477-480 Heterozygous Null Mutation in the P0 Gene Associated with Mild Charcot-Marie-Tooth Disease D. PAREYSON, Corresponding Author D. PAREYSON Istituto Nazionale Neurologico "C. Besta," 20133 Milan, ItalyaAddress for correspondence: Davide Pareyson, Department of Neurology, Istituto Nazionale Neurologico "C. Besta," Via Celoria 11, 20133 Milan, Italy; +39-02-2394-293 (voice, fax); [email protected] (e-mail).Search for more papers by this authorD. MENICHELLA, D. MENICHELLA Department of Neurology, Wayne State University School of Medicine, Detroit, Michigan 48102, USASearch for more papers by this authorS. BOTTI, S. BOTTI Istituto Nazionale Neurologico "C. Besta," 20133 Milan, ItalySearch for more papers by this authorA. SGHIRLANZONI, A. SGHIRLANZONI Istituto Nazionale Neurologico "C. Besta," 20133 Milan, ItalySearch for more papers by this authorE. FALLICA, E. FALLICA Istituto Nazionale Neurologico "C. Besta," 20133 Milan, ItalySearch for more papers by this authorM. MORA, M. MORA Istituto Nazionale Neurologico "C. Besta," 20133 Milan, ItalySearch for more papers by this authorC. CIANO, C. CIANO Istituto Nazionale Neurologico "C. Besta," 20133 Milan, ItalySearch for more papers by this authorM. E. SHY, M. E. SHY Department of Neurology, Wayne State University School of Medicine, Detroit, Michigan 48102, USASearch for more papers by this authorF. TARONI, F. TARONI Istituto Nazionale Neurologico "C. Besta," 20133 Milan, ItalySearch for more papers by this author D. PAREYSON, Corresponding Author D. PAREYSON Istituto Nazionale Neurologico "C. Besta," 20133 Milan, ItalyaAddress for correspondence: Davide Pareyson, Department of Neurology, Istituto Nazionale Neurologico "C. Besta," Via Celoria 11, 20133 Milan, Italy; +39-02-2394-293 (voice, fax); [email protected] (e-mail).Search for more papers by this authorD. MENICHELLA, D. MENICHELLA Department of Neurology, Wayne State University School of Medicine, Detroit, Michigan 48102, USASearch for more papers by this authorS. BOTTI, S. BOTTI Istituto Nazionale Neurologico "C. Besta," 20133 Milan, ItalySearch for more papers by this authorA. SGHIRLANZONI, A. SGHIRLANZONI Istituto Nazionale Neurologico "C. Besta," 20133 Milan, ItalySearch for more papers by this authorE. FALLICA, E. FALLICA Istituto Nazionale Neurologico "C. Besta," 20133 Milan, ItalySearch for more papers by this authorM. MORA, M. MORA Istituto Nazionale Neurologico "C. Besta," 20133 Milan, ItalySearch for more papers by this authorC. CIANO, C. CIANO Istituto Nazionale Neurologico "C. Besta," 20133 Milan, ItalySearch for more papers by this authorM. E. SHY, M. E. SHY Department of Neurology, Wayne State University School of Medicine, Detroit, Michigan 48102, USASearch for more papers by this authorF. TARONI, F. TARONI Istituto Nazionale Neurologico "C. Besta," 20133 Milan, ItalySearch for more papers by this author First published: 06 February 2006 https://doi.org/10.1111/j.1749-6632.1999.tb08615.xCitations: 15Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onEmailFacebookTwitterLinkedInRedditWechat REFERENCES 1 Warner, L.E., M.J. Hilz, S.H. Appel, et al. 1996. Clinical phenotypes of different MPZ (P0) mutations may include Charcot-Marie-Tooth type 1B, Dejerine-Sottas, and congenital hypomyelination. Neuron 17: 451–460. 2 Marrosu, M.G., S. Vaccargiu, G. Marrosu, A. Vannelli, C. Cianchetti & F. Muntoni. 1998. Charcot-Marie-Tooth disease type 2 associated with mutation of the myelin protein zero gene. Neurology 50: 1397–1401. 3 Chapon, F., B. Lechevalier, S. Schaeffer, et al. 1997. A family presenting with electrophysiological 'CMT type II' and linked to P0 gene [abstract]. Neuromusc. Disord. 7: 472. 4 De Jonghe, P., V. Timmerman, C. Ceuterick, et al. 1998. A novel peripheral myelin zero (MPZ) mutation is associated with a clinically distinct Charcot-Marie-Tooth type 2 phenotype [abstract]. J. Neurol. 245: 352. 5 Sghirlanzoni, A., D. Pareyson, M.R. Balestrini, et al. 1992. HMSN III phenotype due to homozygous expression of a dominant HMSN II gene. Neurology 42: 2201–2203. 6 Taroni, F., S. Botti, A. Sghirlanzoni & D. Pareyson. 1996. PMP22 and MPZ point mutations in Italian families with hereditary neuropathy with liability to pressure palsies (HNPP) and Dejerine-Sottas disease (DSD) [abstract]. Am. J. Hum. Genet. 59: A288. 7 Pareyson, D., A. Sghirlanzoni, S. Botti, et al. 1999. Charcot-Marie-Tooth disease type 2 associated with mutation of the myelin protein zero gene [letter]. Neurology 52: 1110–1111. 8 Martini, R., J. Zielasek, K.V. Toyka, K.P. Giese & M. Schachner. 1995. Protein zero (P0)-deficient mice show myelin degeneration in peripheral nerves characteristic of inherited human neuropathies. Nat. Genet. 11: 281–286. 9 Shy, M.E., E. Arroyo, J. Sladky, et al. 1997. Heterozygous P0 knockout mice develop a peripheral neuropathy that resembles chronic inflammatory demyelinating polyneuropathy (CIDP). J. Neuropathol. Exp. Neurol. 56: 811–821. Citing Literature Volume883, Issue1CHARCOT‐MARIE‐TOOTH DISORDERSOctober 1999Pages 477-480 ReferencesRelatedInformation

Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal dominant disorder characterized by recurrent mononeuropathies or brachial plexopathies, commonly associated with a chromosome 17p11.2-12 deletion encompassing the peripheral myelin protein-22 (PMP22) gene. We tried to identify criteria distinguishing HNPP among patients with acute painless mononeuropathy/plexopathy. We investigated by pulsed-field gel electrophoresis the presence of the deletion in 27 patients with isolated or recurrent acute painless mononeuropathy or brachial plexopathy, and no obvious cause of neuropathy. Eight patients carried the deletion, whereas 19 had neither the deletion nor mutations in the PMP22 gene. Age at onset, presenting modality, precipitating events, and rate of recovery did not significantly differ in the two groups. Family history was informative for HNPP diagnosis in 3 cases only. HNPP patients more often showed recurrent episodes, brachial plexopathy, and clinical or electrophysiologic involvement of other nerves. Non-HNPP patients more frequently had peroneal palsy, recent weight loss, and normal electrophysiologic examination in other nerves. Signs of generalized neuropathy and evidence of disease in other family member are often subtle in HNPP and must be thoroughly investigated in patients with acute painless mononeuropathy/plexopathy.