7/29/2019 Familial Spastic Paraplegia- Clinical Analysis

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S326 Neuroepidemiology

5-35-07 Laser induced neuronal regenerationVladim ir Randjelovic , Dragan Vukic*. Laser Research Laborabory, Ei BullHN, Nis, Yugoslavia, 2 Special Psychiatric Hospital, Gomja Top onica, NiS,Yugoslavia

According to our experiences and different reports on biologi cal effects of lowpowar lasers published during the last 20 years, the mode l of laser lig ht andnerve tissue interactions is presented in this work. The mode l discusses dif-ferent neurologic effects, induced by laser irradiation, on molecular bases withspecial respect to nerve cell regeneration. Neuronal regeneration is suggestedto be the result of combined activity o f many different biochemica l processeswithin the nerve cell. These processes are primarily related to integral pro-teins of plasma and intracellular membranes, increased influx of glucose,intercation of laser with mitochond rial matrix soluble molecules, increase indATP production and mitochon drial DNA synthesis, increased energetic po-tentials, suppressed arachinodic acid metabo lism indicated by normalizationof prostaglandin E2 and leukotriene 84 production. Increase in concentrationof recative oxygen forms is a negative result necessarily followi ng laser irradi-ation on certain waveleng ths. As a result of these biochemica l processes thereappear numerous helin g effects of low recative level laser therpy (LLLT) ondamag ed peripheral nerves. The most evident are increase in action poten-tial of damag ed nerves above the normal value follow ed by its normalizatio n,regeneration of damag ed nerves, evidently decreased formation of scar tis-sue and suppression of inflamm ation. These prccesses are recognized onlyafter laser irradiation applying laser intensities and dosages within the certainrange: that is, positive effects seem to disappear above and below the giventhresholds.

36 Neuroepidemiology

L-l36 01 Neuroepidemiological survey about epi lepsy andpossible r isk factors of an elementary schoolpopulat ion from the northwest of Argent inaL.M. Bono mi, DR. Ferrero, L.H. Parodi, F. Martin. Dept. ofNeurologicalSciences, Hospital N ational de Clinicas, School of Medicine , Nationa lUniversity of CdrdoLw, ArgentinaA detaile d study about the whole school p opulatio n (5-16 years o ld) of the Con-cepcion City (Tucuman Province, Argentina) was carried out with the purposeof: 1) detecting some variables and/or events that could be considered as riskfactors for epilepsy. 2) Analizin g the feasibility of future analytic researches.-In order to get those objectives, the whole popula tion of children attending tothe different schools of the Conception City in the north west of Argentina wasstudied through a neuroep idemiolo gical survey previously proved in Bs. Aires.Analysis of frequencies, ratios, association coefficients and risk magnitu de(relative risks) were ma de using the procedures of cases and controls (oddratios). By this work we have reached some critical ideas about the validity,desirability and admissibility of this survey in certain popu lations.

15-36-02 1 Famil ial spast ic paraplegia: Clinical analysis ofPortuguese dominant and recessive famil iesJ. Barros, C. AIves*, J. Guimaties3, J.L. Loureiro4, E. Lourenpo5,P. Ribeiro, Ft. Chorgo, J.V. Santos3, M.C. Silvas, P. Coutinh o. Dept. ofNeurokwy; Hospital Santa Antdn io, Po rte, Porfugal. *Dept. of Neuro/owHospitai&nro Tirso, Porte, Portugal, 3 Dept. of ~eurolo& Hospital E$ sMonk Lisbon, Pcrfugal, 4 Dept. of Neurolcgx Hospital S. Pedro , V i /a Rea l ,Portugal, 5Dept of Neurology; Hospital S. Matws, Braga, P ortugal, 6 Dept.Popul ation Studies, I CBAS , University of Porte, PortugalFami lial spastic paraplegias (FSP) are a heterogeneous group of genetic dis-eases, characterized clinically by progressive spatiicity of lower limbs. Cor-tico-spinal signs may be isolated (pure forms), or associated with other neu-rological abnormalities (complex forms). The mode of transmission may bedomina nt, recessive or X-linked. Recently, pure forms of autosomal domina ntFSP have been linked to chr. 2 (40%) and familie s with a very earty age-at-onset to ch. 14, while a few pure recessive kindreds were linked to chr. 6. Theaim of this review is to define homo geneous diagnostic groups based on theanalysis of clinical data of 107 patients belong ing to 46 Portuguese families.Part of these pa tients were i dentifie d in a prevalence survey carried out inPortugal and others in hospital registers.In 47 patients (17families) transmission wasdomina nt. Age-at-onset ranged

from 3 to 70 years (mean 37.1, sd = 15.9). Fifteen families had pure forms andthe other two presented mild cerebellar and extrapyramidal signs. Amo ng thoseat risk examined , 33 had no complains, whilst presenting clear cortico-spinalsigns (lower limb hyperreflexia, sometimes associated with clonus and mildgait spasticity).In 60 patients (29 families, 16 of which with variable inbreedin g degree)transmission was recessive. Several sub-groups could be advanced based onthe clinical variability observed: (1) Pure forms: 2 1 patients (11 families), meanage-at-onset of 11.2 years, sd = 12.1; (2) Forms with menta l retardation andperipheral neuropathy: 23 patients (10 families), mean age-at-onset of 13.7years, sd = 8.4; (3) Forms with menta l retardation and anterior horn cell signs:9 patients (5 families), mean ag e-at-onset of 19.0 years, sd = 4.3; (4) Otherforms: 7 patients belong ing to three families, one with dystonia, another withoptic atrophy and the third one with posterior columns signs.No X-linked families were identifi ed so far.

5-36-03 Validity of quest ionnaire information on previousstroke in the elderlyA. Di Carlo, D. Inzitari, L. Candelise , C. Gandolfo, F. Grigoletto,M. Baldereschi, N. Minicuci, G. Scarlato, N. Canal, L. Bat&t in, C. Loeb,R. Vergassola, S. Bonaiu to, F. Rengo, P. Livrea, G. Camazzo, L. Amaducci.Targeted Project on Aging , National Research Council, Rome, k/y

The use of non-comparable case ascertainment strategies may partially explaindifferences in stroke prevalence figures. We evaluated the possible impact offalse positive screening on prevalence of stroke in the Italian Longi tudina lStudy on Aging , a population-ba sed study involving a random sample of 5632individuals aged 65-64, identif ied o n the demographic lists of eight Italianmunicipa lities. The screening for stroke included: i) a simple question: haveyou ever had a diagnosis of stroke? ii) questions about previous symptoms(speech disturbances, hemifa cial palsy, limb weakness or paralysis) and iii) asimple neurologica l examin ation performed by general physicians. Overall,652 subjects screened as positive for stroke and only 306 (46.9%) wereconfined by a structured neurologic al evaluation. More than half of the falsepositives were accounted for by peripheral paralysis of the facial newe (19.6%),transient ischemic attacks (17.6%), dizziness (10.1%) and syncopal attacks(6.7%). Age, sex and education did not significantly differentiate confirmed fromfalse positive subjects. The high number of false positive respondents pointout the problems related to the use of questionnaires to gather informatio nabout previous stroke in the elderly, and the need for a subsequent evaluationperformed by neurologists to definitely assess prevalence.

Supported by the CNR (Grant 95.00994.PF40), Targeted Project on Aging ,Italy.

5-36-04 Case-control study of seroposit iv i ty forcyst icercosls in Cuenca, EcuadorKaryn Goodman , Arturo Carpio *, Susan A . Balla gh l, Marco Carrion *,Geovany Abril *, David Ac hig 2. Stanford University School of Medicine ,Stanford CA, USA, 2 nstituto de lnvestigaciones de /a Universidad de Cuenca,EcuadorNeurocysticercosis (NC) is considered the most common parasitic disease ofthe CNS and is widely prevalent in developin g countries. There are no data foridentifying popula tion groups w ith a higher risk of acquiring cysticercosis, orrisk factors which influence the manifestatio n of the disease.Objective: To determine the rate of seropositivity among fam ily membersof patients diagnosed with NC, compared to those of the general popula tion.

Methods: We used the enzyme-linked immunoelectrotransfer blot assay(EITB) to diagnose cysticercosis among fam ily members of index cases of NCand amon g controls in the city of Cuenca, Ecuador. The case group consistedof family members of 33 patients who were diagnosed with parenchymal neu-rocysticercosis by computed tomography (CT). Control were family membersof 17 patients with trauma or migraine who had normal CT.Results: The EITB test was performed on 173 individuals (72%) from thecase group, and 66 (26%) from the control group. The EITB test was positive12.7% of the case group, and 2.9% o f the control group (p -Z 0.02) The oddsratio was 4.6 (95% confidence interval: 1.5 to 30.4. Approximately 25% ofindividuals from both groups who had a positive EITB test had NC on the CTscan.

Discussion: This study confirms the high rate of seropositivity of cysticer-cosis among individuals from a developin g country. Household contacts ofpatients with NC have a four fold higher risk of acquiring cysticercosis, incomparison with the general population.