- Email: [email protected]
Ptosis in migraine headache: a partial Horner syndrome or a pseudo-Horner syndrome?
Society Proceedings
49P
generalization, entered the study. Fourty-two patients were diagnosed as having complex partial and 17 as having simplex partial seizures. Epilepsy was symptomatic in 22 subjects and cryptogenic in 37. Seizure frequency varied from more than l/day to at least l/month. Routine EEGs showed focal epileptic abnormalities in 33 cases, generalized epileptic abnormalities in 4, and slow focal or generalized activities in 26 and 7 patients, respectively. Vigabatrin (GVG) was supplied as the only drug at a mean dose of 31.28 mg/kg per day. Patients were checked after 3, 6, 9 and 12 months of treatment. GVG administration induced a total disappearance or a significant ( > 50%) reduction of seizure frequency in 86-81% of the patients, depending on the time of the evaluation. In the remaining 14-19% a reduction < 50% or no modification of the seizure frequency was observed. NO substantial differences were found between cryptogenic and symptomatic epilepsies.
electroretinogram (PERG) and occipital visual evoked potentials (VEP). The electrophysiological alterations are abolished by L-DOPA therapy; however, a strict relation between PERG and VEP alterations and retina1 dopaminergic deficit is not yet clear. Few histochemical and biochemical data conceming dopaminergic deficit in PD are available. Therefore we compared the steady-state PERG responses at 2 different contrast levels (80% and 50%) and 4 spatial frequenties (SF) (0.6, 2, 2.7 and 4 c/d) reversed at 8.55 Hz in 15 post-traumatic patients showing extrapyramidal symptoms and 20 patients affected by idiopathic PD. The patients were compared with 2 age-matched control groups of 15 young and 14 aged subjects respectively. The amplitude and phase of the second harmonie (17.1 Hz) in the PERG responses were obtained by Fast Fourier Transformation. The data were analyzed by 2-way ANOVA: group as a “between” factor and SF as a “ within” factor.
35. Anti-epileptiform properties of B-endorphin fragments. - A. Di Giannuario, M. Luzi, S. Pieretti, A. Capasso, L. Sorrentino and A. Loizzo (Istituto Superiore di Sanità, Rome, and Università di Salerno, Salerno)
38. Partial epilepsy resistant to carbamazepine: usefulness of vigabatrin as fitst add-on treatment. - R. Galli, R. Massetani, S. Milani”, A. Iudice and L. Murri (Department of Clinical Neurophysiology, Institute of Neurology, and ’ Medical Statistics and Biometry, University of Pisa, Pisa)
We show that the non-opioid cleavage fragments of P-endorphin (BE), i.e., des-tyr-y-endorphin (DTyE) and des-enkephalin-y-endorphin (DEyE), have important anti-epileptiform properties in a few animal models of convulsive disorders, some of which are relatively insensitive to treatment with conventional anti-epileptic drugs. (1) Aher 4 days’ treatment DTyE and DEyE prevent EEG and behavioral disorders induced by BE injected into the cerebral ventricles (i.c.v.) of rabbits. (2) After 4 days’ treatment DTyE and DEyE prevent EEG and behavioral disorders induced by i.c.v. injected corticotropin releasing factor. (3) DTyE dose-dependently blocks inherited spike-and-wave neocortical bursts recorded in DBA/2J mice. Both drugs exert a strong influence on the dopaminergic system and do not interfere directly with any of the opioid receptors. although they can prevent morphine analgesia. 36. Magnetic recording of flash-evoked cortical oscillatory potentials and effect of novocainic blocking in man. - W.G. Sannita, L. Lopez, A. Pasquarelli and G.L. Romani (Neurophysiopathology, DISM, University, Genoa, ITAB, University, Chieti, and Institute of Solid State Electronics, CNR, Rome) A cortical origin is suggested for scalp-recorded responses based on existing differences from retina1 oscillations, e.g., in latency (15-18 and 35-45 msec, respectively), mean frequency (120 and 100 Hz) and spectra1 sensitivity, and distinct effects of adaptation. Oscillatory potentials superimposable to those obtained by conventional electrophysiological methods and time- and phase-locked to retina1 oscillations were recorded biomagnetically at occipital locations in healthy volunteers by a 28-Channel system in a shielded room (full-field flash at 3.1 cps/m’; acquisition at 0.16-250 Hz with high-pass filtering at 80-160 Hz) Topographic distribution was similar to broad-band VEPs, with dipolar organization of main wavelets. Acute scopolamine at 0.50 mg virtually abolished scalp-recorded oscillatory potentials and reduced the amplitude of long-latency waves (~2, n3) of broad-band VEPs, while leaving retina1 potentials unaffected. Theoretical implications and experimental data indicate that biomagnetic signals originate from primary currents related to intracellular activities, with negligible volume conduction. It is suggested that high frequency oscillatory responses to luminance stimuli generate in human cortex in parallel to broad-band VEPs and are modulated by cholinergic mechanisms. 37. Pattern PERG in Parkinson’s disease patients and in post-traumatic parkinsonian patients confirms retina1 dopaminergic deficit in Parkinson’s disease. - A. Peppe, R. Traversa, M. Pierantozzi, R Semprini, M. Loberti, A. Santilli and P. Stanzione (IRCCS, Clinica S. Lucia, Rome) Parkinson’s disease (PD) patients have been reported to show a deficit in visual function. Electrophysiological studies showed altered pattem
Numerous clinical reports and several controlled clinical trials have confirmed that vigabatrin (VGB) has an adequate therapeutic index in the add-on treatment of drug-resistant epilepsy. We report the results from 40 patients suffering from partial symptomatic epilepsy, refractory to carbamazepine (CBZ) monotherapy at the highest tolerated individual dose. In these patients, VGB was the first drug added on, so that clinical responses could be assessed before other antiepileptic drugs were considered. Forty patients, 22 females and 18 males, aged 19-60 years, with partial seizures (with or without secondary generalization) were given VGB 2-3 g/day in addition to CBZ 600-1800 mg/day for an average of 6 months. Their seizure frequency decreased significantly from a median of 13 seizures/month at baseline to 3 seizures/month during the last month on VGB (P < 0.01). Seven patients became seizure free. Drowsiness, diplopia-blurred vision stil1 present at baseline were the most common adverse events experienced during the study. In conclusion, VGB is effective in partial epilepsy refractory to CBZ monotherapy, and appears to be a worthy clinical alternative to other drug combinations. ambulatory monitoring of sleep and diurnal 39. Polysomnographic sleepiness in the healthy elderly. - F. Placidi, M. Diomedi, F. Baldinetti, L. Ciocari, M.G. Marciani and G.L. Gigli (Sleep Disorder Centre, Dept. of Neurology, University of Rome “Tor Verga&” Rome, and Associazione “Anni Verdi,” Rome) In order to better define sleep patterns of elderly individuals and to recognize possible episodes of daytime somnolence, we performed 24 h polysomnographic ambulatory monitoring. The study included 8 healthy subjects with a minimum age of 70 years, who were stil1 socially active. In comparison with a sample of younger subjects (age range 22-40 years) recorded in our laboratory, the elderly showed an increase in TIB, SL, IW and SI and a decrease in SE1 and the percentage of REM sleep and SWS (calculated as to SPT). The reduction of REM sleep was not so marked if compared with TST. Only 2 subjects showed relevant daytime somnolente. The majority of subjects did not show diumal sleepiness at ah, although 3 of them used to nap after the main meal (siesta). In conclusion, we observed a more superficial and fragmentary sleep but a preservation of cyclicity. Sleep of elderly people is not polyphasic; it is stil1 prevalent as a nocturnal phenomenon with a fairly good representation of stages. Daytime somnolence does not constitute a medico-social prohlem. 40. Ptosis in migraine headache: a partial Horner syndrome or a pseudo-Horner syndrome? - V. Tugnoli, R Eleopra, M. Artioli, L. Caniatti and D. De Grandis (Neurological Department, S. Anna Hospital, Ferrara) Sympathetic dysfunction and dissociation between ptosis and miosis have been described in cluster headache. The aim of the present work was
5OP
Society Proceedings
to verify whether these signs are always related to sympathetic ocular impairment exclusively or whether other systems may be involved as wel]. Five migraine patients (MP) with ptosis only during headache attacks were compared with a control group of 10 normal subjects and 2 patients with Claude Bernard Horner syndrome (CBH). Sympathetic skin responses @SR) from hands and forehead, electromyographic examination from orbicularis oculi muscles, pretarsal and orbital portions, blink reflex (BR) and BR recovery cycle were recorded. The relevant findings were: presence of SSR in MPs and its absente in CBH at the affected side; involuntary muscle activation, particularly in orbicularis oculi pretarsal portion, and an early BR recovery cycle only in MPs, at the symptomatic side, during headache. The role of abnormal excitability in the peripheral and centra1 nerveus system in MPs is discussed. 41. Lamotrigine use in partial and generalized epilepsy: preliminary clinical and electrographic results. - 0. Mecarelli, M.R. de Feo, D. Del Priore, T. Cantelmi and G.F. Ricci (1 Neurolkiopatologia, Dip. Scienze Neurologiche, Università “La Sapienza,” Rome) Lamotrigine (LTD) is an interesting new anticonvulsant drug with few side effects, used as add-on therapy for the treatment of partial and generalized seizures as wel1 as of Lennox-Gastaut syndrome. We report preliminary results obtained using LTG as add-on therapy in 16 patients, aged 6-73 years (mean age = 28.9 years), affected by epileptic partial (6 cases) or generalized (10 cases) manifestations refractory to other antiepileptic drugs. In the pretrial period the seizure frequency was of at least 2 episodes/month. The LTG dose varied from 50 to 400 mg/kg. Minimum follow-up was 3 months, maximum 12 months. One patient was withdrawn after 15 days of therapy because of the appearance of rash. LTG therapy caused complete disappearance of seizures in 4 patients (27%); a reduction > 50% in 5 (33%) and no substantial modifications in 6 (40%). A good correlation between clinical and EEG features was observed in the majority of the patients: generally, the reduction or the disappearance of the seizures paralleled with a reduction or disappearance of the electrographic abnormalities, while no remarkable EEG variations were noticed in patients without clinical modifications. The incidence of side effects was very low, consisting of rash (2 cases), dizziness (1 case) and hyperexcitability (1 case). study 42. Monomelic atrophy (MA): clinical and electrophysiological of 6 cases. - R. Nucciotti ‘, V. Palma b, L. Santoro b and G. Caruso b (‘Department of Neurology, “Clinica del Lavoro” Foundation, Campoli (BN), and b Department of Clinical Neurophysiology, Naples) We describe 6 patients with monomelic atrophy (MA): in 3 patients muscular atrophy was confined to one hand and in 3 to one lower limb. Age onset was 24-42 years. In 4 patients muscle atrophy progressed for 1-2 years, then remained stationary for 3-9 years. In the other 2 patients muscle atrophy extended to the contralateral hand, and then slowly worsened, without involving other muscles, during the following 10-11 years. MRI, SSEPs, motor cortex stimulation, H reflex, F wave and sensory and motor conduction velocities along the median, ulnar, peroneal and tibial nerves were normal. Evoked potential amplitude was reduced in atrophic muscles. Standard EMG showed signs of diffuse chronic denervation, even in clinically unaffected muscles. The titer of serum anti-GMl antibodies was normal. In conclusion, we suggest sequentia] clinical and electrophysiological tests to reach a correct diagnosis of MA. A remarkable diagnostic criterion is the absente of focal conduction blocks.
symptomatic epilepsy (mainly the Lennox-Gastaut syndrome and myoclonic epilepsies) has been reported in a number of international studies. Several infantile cases were studied but the data available at present do not yet allow a precise identification of the type of infantile epilepsy in which lamotrigine is most effective. Therefore, we started an open study (follow-up 6 months) in which lamotrigine was given as add-on to a group of 7 children (1 female, 6 malesl with drug-resistant symptomatic myoclonic epilepsy. Only subjects with grade 5 resistance according to Schmidt’s criteria entered the study. Their ages ranged from 2.6 to 16.9 years (mean age 8.61. The 2 youngest children were immediately withdrawn from the trial because of the onset of widespread maculopapular rash. In another patient lamotrigine was discontinued because of lack of efficacy. The other 4 children (mean age 10.8) had only 1 seizure per day, al1 other seizures being completely suppressed with a dose of 75-300 mg/day. In al1 cases LTG was combined with sodium valproate; myoclonic relapses arose while trying to slowly discontinue the latter. This led to the administration of the initial dose of sodium valproate. 44. Atypical benign partial epilepsy of childhood (ABPEC): a novel therapeutic strategy. - A. Pelliccia, 1. Lacapra, E. Piazza and F. Pisani (Sezione Studio e Terapia della Convulsività ed Epilessia in Età Evolutiva del CIPSMS) After the original description of Aicardi and Chevrie, only Deonna and Ziegler have reported a series of children with myoclonic-astatic seizures and electroclinical patterns consistent with atypical benign partial epilepsy of childhood (ABPEC). In spite of the consistency of its clinical features ABPEC has not yet been widely recognized and therefore not yet included in the 1985 and 1989 ILAE Classifications. In the last 4 years, out of a group of 253 patients with benign partial epilepsy we were able to identify a group of 10 cases of ABPEC (6 males and 4 females). In 5 cases a family history of seizure disorder was reported. The age of seizure onset was 2.4-7 years and in 4 cases epilepsy was preceded by simple febrile convulsions. In al1 cases a significant improvement in clinical symptomatology was obtained with the administration of sodium valproate or clobazam, whereas only the combination with acetazolantide resulted in a full remission of EEG abnormalities. 45. Quantified EEG in the sleep evaluation of insomniacs. - G. Schiavi, L. Nobili, M. Gabarra, V. Balestra ’ and F. Ferrillo (Sleep Disorder Centre, Department of Motor Science, University of Genoa, Genoa, and ’ Department of Endocrinology and Science of Metabolism, University of Genoa, Genoa) The interplay between homeostatic and ultradian processes determines the cyclic structure of sleep. EEG spectra1 analysis techniques allow the study of these features, The effect of the homeostatic process is rejected by the exponential declining trend, as a function of time, of the delta band power density. The ultradian process causes EEG desynchronization and it can be detected as the periodic gathering of theta and slow alpha band power density. The neural mechanisms underlying insomnia are stil1 unknown. However, a derangement of homeostatic and ultradian processes has been hypothesized. We studied 50 insomniac patients. The distribution curves of delta band power density in each NREM sleep cycle during the whole night have been determined and a procedure of cluster analysis was applied. Four different groups, characterized by different tempora1 distribution of delta activity curves, were identified. The differences between the patterns of the distribution curves led to suppose different underlying physiopathological mechanisms for each group.
43. Benefit of sodhtm valproate-lamotrigine combination in the treatment of drug-resistant infantile myoclonic epilepsies. - A. Pelliccia and M.C. Tini (Sez. Epilessia, CIPSMS, Univ. “La Sapienza,” Rome)
46. Arousal oscillations in NREM parasomnias: role of cyclic alternating pattern (CAP). - M. Zucconi, A. Oldani, L. FeriniStrambi, M. Zamboni, V. Castronovo, F. Milani and S. Smirne (Sleep Disorder Center, Dept. of Neurology, State University and IRCCS, Hosp. San Raffaele, Milan)
Abhough lamotrigine (LTG) has not yet been included in the Italian pharmaceutical panel, the benefit of its administration in generalized
Some of NREM parasomnias, such as sleepwalking (SW), sleep terror (ST) and, in some aspects, sleep enuresis (SE), are considered “arousal
49P
generalization, entered the study. Fourty-two patients were diagnosed as having complex partial and 17 as having simplex partial seizures. Epilepsy was symptomatic in 22 subjects and cryptogenic in 37. Seizure frequency varied from more than l/day to at least l/month. Routine EEGs showed focal epileptic abnormalities in 33 cases, generalized epileptic abnormalities in 4, and slow focal or generalized activities in 26 and 7 patients, respectively. Vigabatrin (GVG) was supplied as the only drug at a mean dose of 31.28 mg/kg per day. Patients were checked after 3, 6, 9 and 12 months of treatment. GVG administration induced a total disappearance or a significant ( > 50%) reduction of seizure frequency in 86-81% of the patients, depending on the time of the evaluation. In the remaining 14-19% a reduction < 50% or no modification of the seizure frequency was observed. NO substantial differences were found between cryptogenic and symptomatic epilepsies.
electroretinogram (PERG) and occipital visual evoked potentials (VEP). The electrophysiological alterations are abolished by L-DOPA therapy; however, a strict relation between PERG and VEP alterations and retina1 dopaminergic deficit is not yet clear. Few histochemical and biochemical data conceming dopaminergic deficit in PD are available. Therefore we compared the steady-state PERG responses at 2 different contrast levels (80% and 50%) and 4 spatial frequenties (SF) (0.6, 2, 2.7 and 4 c/d) reversed at 8.55 Hz in 15 post-traumatic patients showing extrapyramidal symptoms and 20 patients affected by idiopathic PD. The patients were compared with 2 age-matched control groups of 15 young and 14 aged subjects respectively. The amplitude and phase of the second harmonie (17.1 Hz) in the PERG responses were obtained by Fast Fourier Transformation. The data were analyzed by 2-way ANOVA: group as a “between” factor and SF as a “ within” factor.
35. Anti-epileptiform properties of B-endorphin fragments. - A. Di Giannuario, M. Luzi, S. Pieretti, A. Capasso, L. Sorrentino and A. Loizzo (Istituto Superiore di Sanità, Rome, and Università di Salerno, Salerno)
38. Partial epilepsy resistant to carbamazepine: usefulness of vigabatrin as fitst add-on treatment. - R. Galli, R. Massetani, S. Milani”, A. Iudice and L. Murri (Department of Clinical Neurophysiology, Institute of Neurology, and ’ Medical Statistics and Biometry, University of Pisa, Pisa)
We show that the non-opioid cleavage fragments of P-endorphin (BE), i.e., des-tyr-y-endorphin (DTyE) and des-enkephalin-y-endorphin (DEyE), have important anti-epileptiform properties in a few animal models of convulsive disorders, some of which are relatively insensitive to treatment with conventional anti-epileptic drugs. (1) Aher 4 days’ treatment DTyE and DEyE prevent EEG and behavioral disorders induced by BE injected into the cerebral ventricles (i.c.v.) of rabbits. (2) After 4 days’ treatment DTyE and DEyE prevent EEG and behavioral disorders induced by i.c.v. injected corticotropin releasing factor. (3) DTyE dose-dependently blocks inherited spike-and-wave neocortical bursts recorded in DBA/2J mice. Both drugs exert a strong influence on the dopaminergic system and do not interfere directly with any of the opioid receptors. although they can prevent morphine analgesia. 36. Magnetic recording of flash-evoked cortical oscillatory potentials and effect of novocainic blocking in man. - W.G. Sannita, L. Lopez, A. Pasquarelli and G.L. Romani (Neurophysiopathology, DISM, University, Genoa, ITAB, University, Chieti, and Institute of Solid State Electronics, CNR, Rome) A cortical origin is suggested for scalp-recorded responses based on existing differences from retina1 oscillations, e.g., in latency (15-18 and 35-45 msec, respectively), mean frequency (120 and 100 Hz) and spectra1 sensitivity, and distinct effects of adaptation. Oscillatory potentials superimposable to those obtained by conventional electrophysiological methods and time- and phase-locked to retina1 oscillations were recorded biomagnetically at occipital locations in healthy volunteers by a 28-Channel system in a shielded room (full-field flash at 3.1 cps/m’; acquisition at 0.16-250 Hz with high-pass filtering at 80-160 Hz) Topographic distribution was similar to broad-band VEPs, with dipolar organization of main wavelets. Acute scopolamine at 0.50 mg virtually abolished scalp-recorded oscillatory potentials and reduced the amplitude of long-latency waves (~2, n3) of broad-band VEPs, while leaving retina1 potentials unaffected. Theoretical implications and experimental data indicate that biomagnetic signals originate from primary currents related to intracellular activities, with negligible volume conduction. It is suggested that high frequency oscillatory responses to luminance stimuli generate in human cortex in parallel to broad-band VEPs and are modulated by cholinergic mechanisms. 37. Pattern PERG in Parkinson’s disease patients and in post-traumatic parkinsonian patients confirms retina1 dopaminergic deficit in Parkinson’s disease. - A. Peppe, R. Traversa, M. Pierantozzi, R Semprini, M. Loberti, A. Santilli and P. Stanzione (IRCCS, Clinica S. Lucia, Rome) Parkinson’s disease (PD) patients have been reported to show a deficit in visual function. Electrophysiological studies showed altered pattem
Numerous clinical reports and several controlled clinical trials have confirmed that vigabatrin (VGB) has an adequate therapeutic index in the add-on treatment of drug-resistant epilepsy. We report the results from 40 patients suffering from partial symptomatic epilepsy, refractory to carbamazepine (CBZ) monotherapy at the highest tolerated individual dose. In these patients, VGB was the first drug added on, so that clinical responses could be assessed before other antiepileptic drugs were considered. Forty patients, 22 females and 18 males, aged 19-60 years, with partial seizures (with or without secondary generalization) were given VGB 2-3 g/day in addition to CBZ 600-1800 mg/day for an average of 6 months. Their seizure frequency decreased significantly from a median of 13 seizures/month at baseline to 3 seizures/month during the last month on VGB (P < 0.01). Seven patients became seizure free. Drowsiness, diplopia-blurred vision stil1 present at baseline were the most common adverse events experienced during the study. In conclusion, VGB is effective in partial epilepsy refractory to CBZ monotherapy, and appears to be a worthy clinical alternative to other drug combinations. ambulatory monitoring of sleep and diurnal 39. Polysomnographic sleepiness in the healthy elderly. - F. Placidi, M. Diomedi, F. Baldinetti, L. Ciocari, M.G. Marciani and G.L. Gigli (Sleep Disorder Centre, Dept. of Neurology, University of Rome “Tor Verga&” Rome, and Associazione “Anni Verdi,” Rome) In order to better define sleep patterns of elderly individuals and to recognize possible episodes of daytime somnolence, we performed 24 h polysomnographic ambulatory monitoring. The study included 8 healthy subjects with a minimum age of 70 years, who were stil1 socially active. In comparison with a sample of younger subjects (age range 22-40 years) recorded in our laboratory, the elderly showed an increase in TIB, SL, IW and SI and a decrease in SE1 and the percentage of REM sleep and SWS (calculated as to SPT). The reduction of REM sleep was not so marked if compared with TST. Only 2 subjects showed relevant daytime somnolente. The majority of subjects did not show diumal sleepiness at ah, although 3 of them used to nap after the main meal (siesta). In conclusion, we observed a more superficial and fragmentary sleep but a preservation of cyclicity. Sleep of elderly people is not polyphasic; it is stil1 prevalent as a nocturnal phenomenon with a fairly good representation of stages. Daytime somnolence does not constitute a medico-social prohlem. 40. Ptosis in migraine headache: a partial Horner syndrome or a pseudo-Horner syndrome? - V. Tugnoli, R Eleopra, M. Artioli, L. Caniatti and D. De Grandis (Neurological Department, S. Anna Hospital, Ferrara) Sympathetic dysfunction and dissociation between ptosis and miosis have been described in cluster headache. The aim of the present work was
5OP
Society Proceedings
to verify whether these signs are always related to sympathetic ocular impairment exclusively or whether other systems may be involved as wel]. Five migraine patients (MP) with ptosis only during headache attacks were compared with a control group of 10 normal subjects and 2 patients with Claude Bernard Horner syndrome (CBH). Sympathetic skin responses @SR) from hands and forehead, electromyographic examination from orbicularis oculi muscles, pretarsal and orbital portions, blink reflex (BR) and BR recovery cycle were recorded. The relevant findings were: presence of SSR in MPs and its absente in CBH at the affected side; involuntary muscle activation, particularly in orbicularis oculi pretarsal portion, and an early BR recovery cycle only in MPs, at the symptomatic side, during headache. The role of abnormal excitability in the peripheral and centra1 nerveus system in MPs is discussed. 41. Lamotrigine use in partial and generalized epilepsy: preliminary clinical and electrographic results. - 0. Mecarelli, M.R. de Feo, D. Del Priore, T. Cantelmi and G.F. Ricci (1 Neurolkiopatologia, Dip. Scienze Neurologiche, Università “La Sapienza,” Rome) Lamotrigine (LTD) is an interesting new anticonvulsant drug with few side effects, used as add-on therapy for the treatment of partial and generalized seizures as wel1 as of Lennox-Gastaut syndrome. We report preliminary results obtained using LTG as add-on therapy in 16 patients, aged 6-73 years (mean age = 28.9 years), affected by epileptic partial (6 cases) or generalized (10 cases) manifestations refractory to other antiepileptic drugs. In the pretrial period the seizure frequency was of at least 2 episodes/month. The LTG dose varied from 50 to 400 mg/kg. Minimum follow-up was 3 months, maximum 12 months. One patient was withdrawn after 15 days of therapy because of the appearance of rash. LTG therapy caused complete disappearance of seizures in 4 patients (27%); a reduction > 50% in 5 (33%) and no substantial modifications in 6 (40%). A good correlation between clinical and EEG features was observed in the majority of the patients: generally, the reduction or the disappearance of the seizures paralleled with a reduction or disappearance of the electrographic abnormalities, while no remarkable EEG variations were noticed in patients without clinical modifications. The incidence of side effects was very low, consisting of rash (2 cases), dizziness (1 case) and hyperexcitability (1 case). study 42. Monomelic atrophy (MA): clinical and electrophysiological of 6 cases. - R. Nucciotti ‘, V. Palma b, L. Santoro b and G. Caruso b (‘Department of Neurology, “Clinica del Lavoro” Foundation, Campoli (BN), and b Department of Clinical Neurophysiology, Naples) We describe 6 patients with monomelic atrophy (MA): in 3 patients muscular atrophy was confined to one hand and in 3 to one lower limb. Age onset was 24-42 years. In 4 patients muscle atrophy progressed for 1-2 years, then remained stationary for 3-9 years. In the other 2 patients muscle atrophy extended to the contralateral hand, and then slowly worsened, without involving other muscles, during the following 10-11 years. MRI, SSEPs, motor cortex stimulation, H reflex, F wave and sensory and motor conduction velocities along the median, ulnar, peroneal and tibial nerves were normal. Evoked potential amplitude was reduced in atrophic muscles. Standard EMG showed signs of diffuse chronic denervation, even in clinically unaffected muscles. The titer of serum anti-GMl antibodies was normal. In conclusion, we suggest sequentia] clinical and electrophysiological tests to reach a correct diagnosis of MA. A remarkable diagnostic criterion is the absente of focal conduction blocks.
symptomatic epilepsy (mainly the Lennox-Gastaut syndrome and myoclonic epilepsies) has been reported in a number of international studies. Several infantile cases were studied but the data available at present do not yet allow a precise identification of the type of infantile epilepsy in which lamotrigine is most effective. Therefore, we started an open study (follow-up 6 months) in which lamotrigine was given as add-on to a group of 7 children (1 female, 6 malesl with drug-resistant symptomatic myoclonic epilepsy. Only subjects with grade 5 resistance according to Schmidt’s criteria entered the study. Their ages ranged from 2.6 to 16.9 years (mean age 8.61. The 2 youngest children were immediately withdrawn from the trial because of the onset of widespread maculopapular rash. In another patient lamotrigine was discontinued because of lack of efficacy. The other 4 children (mean age 10.8) had only 1 seizure per day, al1 other seizures being completely suppressed with a dose of 75-300 mg/day. In al1 cases LTG was combined with sodium valproate; myoclonic relapses arose while trying to slowly discontinue the latter. This led to the administration of the initial dose of sodium valproate. 44. Atypical benign partial epilepsy of childhood (ABPEC): a novel therapeutic strategy. - A. Pelliccia, 1. Lacapra, E. Piazza and F. Pisani (Sezione Studio e Terapia della Convulsività ed Epilessia in Età Evolutiva del CIPSMS) After the original description of Aicardi and Chevrie, only Deonna and Ziegler have reported a series of children with myoclonic-astatic seizures and electroclinical patterns consistent with atypical benign partial epilepsy of childhood (ABPEC). In spite of the consistency of its clinical features ABPEC has not yet been widely recognized and therefore not yet included in the 1985 and 1989 ILAE Classifications. In the last 4 years, out of a group of 253 patients with benign partial epilepsy we were able to identify a group of 10 cases of ABPEC (6 males and 4 females). In 5 cases a family history of seizure disorder was reported. The age of seizure onset was 2.4-7 years and in 4 cases epilepsy was preceded by simple febrile convulsions. In al1 cases a significant improvement in clinical symptomatology was obtained with the administration of sodium valproate or clobazam, whereas only the combination with acetazolantide resulted in a full remission of EEG abnormalities. 45. Quantified EEG in the sleep evaluation of insomniacs. - G. Schiavi, L. Nobili, M. Gabarra, V. Balestra ’ and F. Ferrillo (Sleep Disorder Centre, Department of Motor Science, University of Genoa, Genoa, and ’ Department of Endocrinology and Science of Metabolism, University of Genoa, Genoa) The interplay between homeostatic and ultradian processes determines the cyclic structure of sleep. EEG spectra1 analysis techniques allow the study of these features, The effect of the homeostatic process is rejected by the exponential declining trend, as a function of time, of the delta band power density. The ultradian process causes EEG desynchronization and it can be detected as the periodic gathering of theta and slow alpha band power density. The neural mechanisms underlying insomnia are stil1 unknown. However, a derangement of homeostatic and ultradian processes has been hypothesized. We studied 50 insomniac patients. The distribution curves of delta band power density in each NREM sleep cycle during the whole night have been determined and a procedure of cluster analysis was applied. Four different groups, characterized by different tempora1 distribution of delta activity curves, were identified. The differences between the patterns of the distribution curves led to suppose different underlying physiopathological mechanisms for each group.
43. Benefit of sodhtm valproate-lamotrigine combination in the treatment of drug-resistant infantile myoclonic epilepsies. - A. Pelliccia and M.C. Tini (Sez. Epilessia, CIPSMS, Univ. “La Sapienza,” Rome)
46. Arousal oscillations in NREM parasomnias: role of cyclic alternating pattern (CAP). - M. Zucconi, A. Oldani, L. FeriniStrambi, M. Zamboni, V. Castronovo, F. Milani and S. Smirne (Sleep Disorder Center, Dept. of Neurology, State University and IRCCS, Hosp. San Raffaele, Milan)
Abhough lamotrigine (LTG) has not yet been included in the Italian pharmaceutical panel, the benefit of its administration in generalized
Some of NREM parasomnias, such as sleepwalking (SW), sleep terror (ST) and, in some aspects, sleep enuresis (SE), are considered “arousal