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Objective: The association between restless legs syndrome (RLS) and obstructive sleep apnea syndrome (OSAS) has seldom been reported. There is one study reporting improvement of RLS symptoms severity in patients naive of treatment after initiation of continuous positive airway pressure (CPAP) for OSAS. The aim of this study was to evaluate the impact of the OSAS treatment in patients with previously diagnosed, clinically significant RLS in a usual clinical setting., Methods: This was a retrospective study of RLS patients from a sleep clinic with a concomitant or subsequent diagnosis of OSAS. All patients who started treatment for OSAS and had a follow-up of ≥3 months were selected. Exclusion criteria included noncompliance, absence of sufficient information on clinical records, and RLS improvement following treatment of secondary causes. The primary outcome variable was clinical status of RLS symptoms following OSA treatment. Other variables included demographics, RLS treatment and outcome, OSA diagnosis, treatment, compliance, outcome, and PSG characteristics., Results: From a database of 97 RLS patients, 56 patients had both OSA and RLS. Of these patients, 28 met the criteria for the study. In all, 17 patients (60.9%) were female, with a mean age 60 years. A total of 16 patients (57.1%) were diagnosed with idiopathic RLS. OSAS was diagnosed, on average, 21 months after the RLS diagnosis. A total of 19 (70.4%) patients were overweight or obese, 26 (92.9%) reported snoring, and 10 (35.7%) witnessed apneas. A total of 16 patients (57.1%) had excessive daytime sleepiness and 23 (84.1%) had insomnia. The mean apnea-hypopnea index was 19. Patients were mostly treated with dopamine agonists (19 patients, 67.9%) for the RLS, and nine patients were treated with more than two drugs for RLS. A total of 20 patients (71.4%) had improvement of symptoms of RLS after therapy for OSAS, of whom nine stopped drug therapy and eight reduced the dose., Conclusions: In patients with clinically significant RLS, treatment of concomitant OSAS significantly improved RLS symptoms, enabling drug therapy reduction in more than half of the patients. These data reinforce the need to actively diagnose OSAS in RLS patients., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Cerebrovascular disease is the leading cause of epilepsy in adults, although post-stroke seizures reported frequency is variable and few studies used EEG in their identification. To describe and compare EEG and clinical epileptic manifestations frequency in patients with an anterior circulation ischaemic stroke. Prospective study of acute anterior circulation ischaemic stroke patients, consecutively admitted to a Stroke Unit over 24 months and followed-up for 1 year. All patients underwent standardized clinical and diagnostic assessment. Seizure occurrence was clinically evaluated during hospitalization and by a telephone interview at 6 months and a clinical appointment at 12 months after stroke. Video-EEG was performed in the first 72 h (1st EEG), daily after the 1st EEG for the first 7 days after the stroke, or later if neurological worsening, at discharge, and at 12 months. 151 patients were included (112 men) with a mean age of 67.4 (11.9) years. In the 1st year after stroke, 38 patients (25.2%) had an epileptic seizure. During hospitalization, 27 patients (17.9%) had epileptiform activity (interictal or ictal) in the EEG, 7 (25.9%) of them electrographic seizures. During the first week after stroke, 22 (14.6%) patients had a seizure and 4 (2.6%) non-convulsive status epilepticus criteria. Five (22.7%) acute symptomatic seizures were exclusively electrographic. At least one remote symptomatic seizure occurred in 23 (16%) patients. In the first 7 days after stroke, more than one-fifth of patients with seizures had exclusively electrographic seizures. Without a systematic neurophysiological evaluation the frequency of post-stroke seizures are clinically underestimated.

Objective: Seizures and electroencephalographic (EEG) abnormalities have been associated with unfavorable stroke functional outcome. However, this association may depend on clinical and imaging stroke severity. We set out to analyze whether epileptic seizures and early EEG abnormalities are predictors of stroke outcome after adjustment for age and clinical/imaging infarct severity., Methods: A prospective study was made on consecutive and previously independent acute stroke patients with a National Institutes of Health Stroke Scale (NIHSS) score ≥ 4 on admission and an acute anterior circulation ischemic lesion on brain imaging. All patients underwent standardized clinical and diagnostic assessment during admission and after discharge, and were followed for 12 months. Video-EEG (<60 min) was performed in the first 72 h. The Alberta Stroke Program Early CT Score quantified middle cerebral artery infarct size. The outcomes in this study were an unfavorable functional outcome (modified Rankin Scale [mRS] ≥ 3) and death (mRS = 6) at discharge and 12 months after stroke., Results: Unfavorable outcome at discharge was independently associated with NIHSS score (p = 0.001), EEG background activity slowing (p < 0.001), and asymmetry (p < 0.001). Unfavorable outcome 1 year after stroke was independently associated with age (p = 0.001), NIHSS score (p < 0.001), remote symptomatic seizures (p = 0.046), EEG background activity slowing (p < 0.001), and asymmetry (p < 0.001). Death in the first year after stroke was independently associated with age (p = 0.028), NIHSS score (p = 0.001), acute symptomatic seizures (p = 0.015), and EEG suppression (p = 0.019)., Significance: Acute symptomatic seizures were independent predictors of vital outcome and remote symptomatic seizures of functional outcome in the first year after stroke. Therefore, their recognition and prevention strategies may be clinically relevant. Early EEG abnormalities were independent predictors and comparable to age and early clinical/imaging infarct severity in stroke functional outcome discrimination, reflecting the concept that EEG is a sensitive and robust method in the functional assessment of the brain.

Introduction: Patients undergoing carotid endarterectomy (CEA) require strict arterial blood pressure (BP) control to maintain adequate cerebral perfusion. Invasive blood pressure (IBP) is the gold standard, however artifacts may lead to erroneous readings., Methods: We report a case of CEA using IBP monitoring., Results: A 64-year-old man, American Society Anaesthesiology (ASA) physical status 3 (diffuse atheromathosis, dyslipidemia and non-medicated hypertension), was presented for an elective right CEA. ASA standard, neuromuscular block monitoring, anesthesia depth and cerebral oximetry were used as monitorization. On preanaesthetic assessment noninvasive BP (NIBP) had no significant difference between right and left arms (180/90 mmHg). IBP monitoring was placed in left radial artery after several attempts in both arms. Surgery was performed under balanced general anesthesia (GA). Intra-operatively the patient remained stable (140/86 mmHg) however the systolic carotid artery stump pressure (SP) was 210-220mmHg. This finding was confirmed by measuring NIBP in both legs. At this point NIBP was used to monitor and guide the BP target until the end of the procedure and during postoperative period (PO) in postanesthetic care unit (PACU). Surgery proceeded uneventfully. After discharge to the ward (48h stay at PACU), a hypertensive crisis lead to cervical neck haematoma which required emergent surgery under GA. Intraoperatively the BP was assessed with NIBP. After a new period of 48h at PACU the patient was discharged to the ward and subsequently from the hospital on the 8th postoperative day, without further complications., Conclusion: IBP allow beat-by-beat measures with optimization of BP in order to improve cerebral perfusion during CEA. IBP can be inaccurate in patients with diffuse atheromatosis. NIBP may be an alternative, however is not continuous and is expected to be less accurate than the IBP.1 The high IBP-NIBP difference (>40 mmHg) was clinically relevant and in this patient might be explained by diffuse atheromatosis. NIBP was compatible with carotid SP, indicating that, in this case was a reliable and accurate method of monitoring.

Subcortical band heterotopia is a neuronal migration disorder that may cause refractory epilepsy. In these patients, resective surgery has yielded inadequate results. Deep brain stimulation of the anterior nuclei of the thalamus has been used for the treatment of refractory epilepsy with good results. We describe the first two patients with subcortical band heterotopia who were submitted to deep brain stimulation of the anterior nuclei of the thalamus, with evaluation of seizure outcome after 12 and 18 months of follow-up. At these times, both showed a >50% decrease in seizure frequency and an increase in seizure freedom. Both patients had a depressive syndrome after surgery that responded fully to anti-depressive medication in one patient and partly in the other. In both, deep brain stimulation of the anterior nuclei of the thalamus was associated with good seizure outcome. This procedure can therefore be considered in the treatment of patients with subcortical band heterotopia and refractory epilepsy. Depression may be a transient adverse event of the surgery or stimulation, however, its aetiology is probably multifactorial.

The occurrence of seizures in specific types of epilepsies can follow a 24-hour nonuniform or nonrandom pattern. We described the 24-hour pattern of clinical seizures in patients with focal refractory epilepsy who underwent video-electroencephalography monitoring. Only patients who were candidates for epilepsy surgery with an unequivocal seizure focus were included in the study. A total of 544 seizures from 123 consecutive patients were analyzed. Specific time of seizures were distributed along 3- or 4-hour time blocks or bins throughout the 24-hour period. The mean age of the subjects was 37.7 years, with standard deviation of 11.5 years, median of 37. The majority were females (70/56%). The majority of patients had a seizure focus located in the mesial temporal lobe (102/83%) and in the neocortical temporal lobe (13/11%). The remaining patients had a seizure focus located in the extratemporal lobe (8/6%). The most common etiology was mesial temporal sclerosis (86/69.9%). Nonuniform seizure distribution was observed in seizures arising from the temporal lobe (mesial temporal lobe and neocortical temporal lobe), with two peaks found in both 3- and 4-hour bins: 10:00-13:00/16:00-19:00 and 08:00-12:00/16:00-20:00 respectively (p=0.004). No specific 24-hour pattern was identified in seizures from extratemporal location. The 24-hour rhythmicity of seizure distribution is recognized in certain types of epilepsy, but studies on the topic are scarce. Their replication and validation is therefore needed. Our study confirms the bimodal pattern of temporal lobe epilepsy independently of the nature of the lesion. However, peak times differ between different studies, suggesting that the ambient, rhythmic exogenous factors or environmental/social zeitgebers, may modulate the 24-hour rhythmicity of seizures. Characterization of these 24-hour patterns of seizure occurrence can influence diagnosis and treatment in selected types of epilepsy, such as the case of temporal lobe epilepsy, the most common drug-resistant epilepsy., (Copyright © 2015 Elsevier Inc. All rights reserved.)

The aim of the present study was to evaluate the correlation between the end of an Oxford sleep resistance (OSLER) test session and a neurophysiological marker of sleep onset in Parkinson's disease (PD) patients. Single center study was conducted in PD patients with excessive daytime sleepiness [Epworth sleepiness scale (ESS) >9]. The OSLER test was conducted with a concomitant electroencephalography (EEG), electromyography (mentalis), right and left electroculogram, and video monitoring. Neurophysiological (NP) sleep onset was defined according to AASM criteria (2005). Five PD patients with mean ESS of 14 (10-16) were included. OSLER test duration was shorter than 40 min in all patients (mean duration 20 min and 39 s). No patient fulfilled neurophysiological criteria to sleep onset at the time of OSLER test termination. In 13 OSLER sessions that ended before 40 min, eight had microsleeps in the last 30 s before the end of the test. NP monitoring showed signs of sleepiness in all patients. In PD patients, the early termination of an OSLER test session may not correspond to NP criteria of sleep onset. However, in all PD patients with abnormal OSLER results, there were EEG signs of sleepiness, which do not exclude the potential utility of OSLER test to evaluate the risk of falling asleep.

Pure alexia (PA) has been associated with visual deficits or a failure to activate the visual word form area (VWFA). We report a patient with pure alexia due to posterior cortical atrophy, in whom event-related potentials revealed a delay in the P100 component and an absent N170 compared with controls. Furthermore, there was a tendency for a larger delay in P100 latencies associated with incorrectly read words. This suggests that some cases of PA might result from deficits in visual perception, signaled by the P100 early potential which could lead to an inability to consistently activate the VWFA, marked by the absent N170.

We describe a patient with an acute middle cerebral artery ischemic stroke developing subtle involuntary movements of the paretic upper limb with cortical origin during rt-PA perfusion. Despite the multiple potential pathophysiological mechanisms for the relationship between thrombolysis and epileptic activity, seizures during this procedure are scarcely reported. Our hypothesis is that subtle and transient clinical seizures, like those described in our patient, may not be detected or are misdiagnosed as nonepileptic involuntary movements. We aimed to draw attention to the recognition challenge of this paroxysmal motor behavior, highlighting this clinical and neurophysiological identification using video recording and back-average analysis of the EEG.

Object: The removal of mesial temporal structures, namely amygdalohippocampectomy, is the most efficient surgical procedure for the treatment of epilepsy. However, disconnection of the epileptogenic zones, as in temporal lobotomy or, for different purposes, hemispherotomy, have shown equivalent results with less morbidity. Thus, authors of the present study began performing selective amygdalohippocampotomy in cases of refractory mesial temporal lobe epilepsy (TLE) to treat mesial temporal lobe sclerosis (MTLS)., Method: The authors conducted a retrospective analysis of all cases of amygdalohippocampotomy collected in a database between November 2007 and March 2011., Results: Since 2007, 21 patients (14 males and 7 females), ages 20-58 years (mean 41 years), all with TLE due to MTLS, were treated with selective ablation of the lateral amygdala plus perihippocampal disconnection (anterior one-half to two-thirds in dominant hemisphere), the left side in 11 cases and the right in 10. In 20 patients the follow-up was 2 or more years (range 24-44 months, average 32 months). Clinical outcome for epilepsy 2 years after surgery (20 patients) was good/very good in 19 patients (95%) with an Engel Class I (15 patients [75%]) or II outcome (4 patients [20%]) and bad in 1 patient (5%) with an Engel Class IV outcome (extratemporal focus and later reoperation). Surgical morbidity included hemiparesis (capsular hypertensive hemorrhage 24 hours after surgery, 1 patient), verbal memory worsening (2 patients), and quadrantanopia (permanent in 2 patients, transient in 1). Late psychiatric depression developed in 3 cases. Operative time was reduced by about 30 minutes (15%) on average with this technique., Conclusions: Amygdalohippocampotomy is as effective as amygdalohippocampectomy to treat MTLS and is a potentially safer, time-saving procedure.

Sleep-related eating disorder (SRED) is characterised by eating episodes during the first period of the night sleep with partial loss consciousness, and amnesia. It can rarely be induced by some drugs, including zolpidem. We present a video report of a patient with a 1-year history of SRED caused by zolpidem causing important repercussions in the sleep structure and life quality. The night eating episodes ceased promptly with discontinuation of zolpidem. Upon the follow-up, the sleep structure improved and the daily consequences disappeared. As in few reported cases of zolpidem-induced SRED, our patient was suffering from the parasomnia for a long time before the diagnosis. Active exclusion of symptoms suggestive of SRED in patients under zolpidem treatment can avoid the deleterious effect of the sleep disorder.

Limbic encephalitis with LGI1 antibodies may cause drug-resistant temporal lobe epilepsy. We report a case of a young man with progressive drug-resistant focal epilepsy, hyperhidrosis, and memory impairment associated with a left mesial temporal lesion. Epilepsy surgery was performed with the provisional diagnosis of cortical dysplasia or tumour. A neuropathological study following amygdalohippocampectomy revealed limbic encephalitis and LGI1 antibodies were identified in the serum. Two and a half years after surgery, the patient remains seizure-free without medication, with normal memory and without hyperhidrosis. Although immunosuppression is the first-line therapy for autoimmune limbic encephalitis, this case suggests that, in selected cases, a lasting response can be achieved with surgery.

Surgery is the treatment of choice for refractory temporal lobe epilepsies, but unexpected seizure recurrences occur and the AEDs management strategy may be an implicated factor. We evaluated the AEDs management's role in the outcome of post surgical epilepsy patients with hippocampal sclerosis (HS). Epileptic patients submitted to amigdalohippocampectomy due to HS in Engel class IA 12 months after surgery were selected. The following variables were studied: age, gender, time of post-surgical follow-up, present Engel class, number of antiepileptic AEDs before surgery and at the time of the interview, AED changes after surgery (stopped, increased, decreased, maintained), timing for AED changes after surgery and seizure recurrences. Sixty-seven consecutive patients were studied (mean time of follow-up of 4.9 ± 2.8 years). Among these, 46.3% were tapering AEDs, 38.8% had not changed and 14.9% had increased AEDs. The global recurrence rate was 32.8%. Recurrence rates for patients tapering and not tapering AEDs were similar (34.2% and 31%, respectively). Fifteen patients tapered AEDs before 2 years and 20 at or 2 years after surgery, with similar recurrence rates (33% and 30%, respectively). All patients who recurred due to AED tapering and 66.7% of the patients who recurred with no AED reduction resumed the Engel class I. This study suggests that in HS patients submitted to AHE who are seizure free during the first postsurgical year, AEDs tapering is achieved in a substantial percentage of patients. Tapering AEDs, independently of its timing, will induce seizure recurrence in about a third of patients. However, patients relapsing after tapering AEDs regain control after resuming therapy., (Copyright © 2012 Elsevier B.V. All rights reserved.)

Individuals with Parkinson's disease (PD) and temporal lobe epilepsy (TLE) have hallucinations and mild cognitive dysfunction. The objective of this work was to study dreams in PD and TLE patients using a common functional model of dream production involving the limbic and paralimbic structures. Dreams were characterised in early-stage PD (19 males) and TLE patients (52) with dream diaries classified by the Hall van de Castle system and were compared with matched controls. In PD, there were significant differences between patients' dreams and those of controls: animals, physical aggression, and a befriender were more common in patients, and aggressor and bodily misfortunes were less common. The dreams of patients with frontal dysfunction showed more aggressive features. TLE patients had lower recall than PD patients and a higher proportion of dreams involving family and familiar settings, lower proportions involving success, and a higher incidence of frontal dysfunction. The dreams of PD and TLE patients share important features., (Copyright © 2011. Published by Elsevier Inc.)

Normal sleep architecture and arousal require an intact thalamus. Thalamic vascular lesions, particularly in the paramedian region may cause arousal disturbances and hypersomnolence. Although hypersomnolence is one of the main characteristics of acute bilateral paramedian thalamic infarcts, there are only scarce reports in literature concerning polysomnographic follow-up of these patients. The few reported cases in literature show that sleep stages do not significantly change from the acute to chronic phase. We present a case report of a patient with a bilateral paramedian thalamic infarct in which a polysomnographic evaluation of sleep was performed four days and five months after stroke. In the acute phase, polysomnography showed an impairment of phase 2 NREM and absence of phase 3 and 4 NREM with absent sleep spindles. After the acute stroke phase, hypersomnolence improved and sleep spindles reappeared as well as phase 3 and 4 of NREM sleep. Our patient clear clinical and polysomnographic improvement makes us suppose that in this case the initial impairment could have been essentially due to a functional transitory impairment of the thalamocortical and corticothalamic connections. This case report is peculiar because it discloses a marked improvement of sleep architecture which to the best of our knowledge has not been clearly described before., (Copyright © 2011 Elsevier B.V. All rights reserved.)

Purpose: To evaluate morning dream recall frequency and content in patients with temporal lobe epilepsy (TLE)., Methods: Fifty-two patients with pharmacoresistant TLE submitted to a written dream diary during five consecutive days and continuous video-electroencephalographic (video-EEG) monitoring. A matched control group of 41 healthy subjects completed the same diary at home. The number of recalled dreams (including long dreams) and nonrecalled dream mentation were collected, and the Dream Recall Rate (DRR) was calculated. Hall and Van de Castle dream content analysis was performed., Key Findings: Greater than 70% of patients with TLE (37 of 52) recall their dreams, but DRR rate in these patients is lower than in controls (p ≤ 0.001). Dream recall does not appear to be influenced by the presence of neuropsychological deficits nor seizure frequency. In dreams descriptions, TLE patients (vs. controls) have a higher percentage of familiarity in settings and fewer dreams with at least one success., Significance: Onirical activity of patients with TLE is different from that of healthy subjects. Our results support the role of mesial and neocortical temporal structures in dream experience. The selective activation of dysfunctional mesial structures may be responsible for some of the observed variability. However, dream content changes can also mirror social and psychological comorbidities of patients with epilepsy., (Wiley Periodicals, Inc. © 2011 International League Against Epilepsy.)

Purpose: To gain new insights into the clinical presentation, causes, treatment and prognosis of epilepsia partialis continua (EPC), and to develop hypotheses to be tested in a prospective investigation., Methods: In this retrospective multicenter study, all cases were included that fulfilled these criteria: constantly repeated fragments of epileptic seizures, with preserved consciousness, lasting ≥ 1 h and representing locally restricted motor or sensory epileptic activity. Single episodes were included when they lasted for a minimum of 1 day. EPC with Rasmussen syndrome and acute stroke were excluded., Key Findings: Three time courses with two subtypes each were distinguished, that is, EPC as a solitary event (de novo or in preexistent epilepsy); chronic repetitive nonprogressive EPC (with frequent or rare episodes); and chronic persistent nonprogressive EPC (primarily or evolving out of an episodic course). These were unrelated to etiologies (morphologic lesions 34%, inflammatory 29%, systemic disorders 9%, idiopathic 5%, unknown 23%). Precipitation and inhibition of seizures is a frequent feature of EPC. Levetiracetam and topiramate have improved the possibilities for pharmacotherapy. Topiramate seems to be particularly effective with dysontogenetic etiologies., Significance: The existence of several clearly distinct courses of nonprogressive EPC is a new finding. These distinctions will be further investigated in a prospective study with precise protocols for electroencephalography (EEG), imaging, and other studies. This should better establish the relation of motor and somatosensory EPC; further clarify the relations, pathogenesis, and significance of the different types and their etiologies; and possibly identify more semiologic variants. It should also provide more precise knowledge about therapy and modification of ictogenesis by external stimuli., (Wiley Periodicals, Inc. © 2011 International League Against Epilepsy.)

Aim: Although surgery for temporal lobe epilepsy (TLE) harbours a good prognosis, post-operative seizures may occur. Long-term, post-operative seizure follow-ups are rare but necessary to properly define outcome., Methods: Longitudinal, long-term, post-operative seizure follow-up in TLE patients with outcome analysed using Engel's classification. Three groups were considered according to the type of resection: isolated amygdalohippocampectomy (IAH), further divided into anterior and complete, AH plus focal neocortical resections (AH + FR) and focal neocortical resections (FR)., Results: Eighty-nine patients were enrolled (61 in the IAH group, 24 in the AH + FR group, and four in the FR group), with a mean follow-up time of 46.7 months. For the three groups together, 90.9% and 86.7% of the patients were in Engel class I for six months and five years, respectively. Kaplan-Meir analysis of the IAH and AH + FR groups showed that, while 82.2% of patients of the IAH group tended to remain in class I within 84 months after surgery, 86.7% of the AH + FR group tended to remain in class I within 12 months. Kaplan-Meier analysis of the IAH sub-groups showed that more patients (91.0%) with anterior resection tended to remain in class I, although for a longer period of time (36 months), compared to those with complete resection (84.0% of patients and 12 months, respectively). For the IFR group, only three patients were in Engel class I for long-term follow-up., Conclusions: High rates of seizure freedom were obtained and stably maintained for years. The reasons for better long-term prognosis of the anterior IAH group are so far unclear, the IFR group was too small to draw any conclusive data.

Singing is a rare ictal symptom of focal epilepsy. We report a case of a left-handed woman, with no musical education, experiencing refractory seizures consisting of the singing of a popular Portuguese folksong, with simultaneous, rhythmic hand clapping. A left mesial temporal sclerosis was successfully treated by selective amygdalohippocampectomy. As in most previously reported cases, the epileptogenic zone was located in the left hemisphere, although the ictal onset zone could not be lateralized on scalp EEG. This clinical case supports the evidence that cerebral music production seems to require both hemispheres, in accordance to the concept that brain specializations for music is associated with a dynamic, well-orchestrated interaction of music-specific neural networks. [Published with video sequences].

Gastrointestinal symptoms are considered manifestations of ictal phenomena. Eructations are only rarely described. The present case illustrates eructation as an ictal sign in a patient with symptomatic refractory epilepsy and a left temporal lesion submitted to video-EEG monitoring. The ictal EEG reveals a generalized attenuation of background activity followed by a lateralised right anterior temporal theta rhythmic activity. Eructations take place during the initial generalized ictal EEG changes. The rarity of the ictal eructation is emphasised, and the potential localizing and lateralizing value are discussed in the present case.

Familial amyloid polyneuropathy (FAP) is associated with massive endoneurial and extracellular deposition of amyloid, which isformed from a mutated transthyretin (TTR) protein. Ninety percent of TTR protein is produced in liver. Liver transplantation (LT) is the only treatment that can halt FAP clinical progression. We studied 35 LT patients. The mean age of the first symptoms was 36.6 years (ranging from 27 to 56), 19 were males, and 16 females, they underwent LT after a mean time of 5 years of symptomatic disease. Fifteen patients followed for more than 24 months after LT had periodic evaluations with clinical and neurophysiological scores (CS and NS). Ten were first evaluated before LT (mean follow-up time of 44 months after LT), and 5 were evaluated only after LT (or a mean time of 41 months). Five patients were followed periodically before LT (mean time of 44 months) to study the natural course of this condition. The mortality rate was of 14% in the first 6 months and was related to known complications of the surgery. No deaths occurred in the period 6 months to 1 year after LT. Five patients (14%) died 1-2 years after LT, 4 of whom were transplanted in advance stages. In the survival group, CS tended to stabilize shortly after LT and to remain invariable later on. The NSprogressed in the first year following LT, and subsequently it did not increase significantly. LT changed the natural course of FAP-I.

Ependymomas account for 3 to 9% of all neuroepithelial tumors and, although occurring most often within the ventricular system, they may arise from the extraventricular parenchyma as well. Several histologic patterns of these neoplasm are well known, but little attention has been devoted to a variant composed of giant elements. We describe the case of a 13-year-old girl experiencing a 3-month history of partial seizures in whom cranial magnetic resonance imaging showed an extraventricular, right parietotemporal neoplasm, adherent to the overlying dura mater. Histologic, immunohistochemical and ultrastructural findings were consistent with those of a high-grade ependymoma. The tumor was characterized by the presence of a major component of pleomorphic giant cells, as also seen in pleomorphic xantoastrocytoma, subependymal giant cell astrocytoma and glioblastoma multiforme. Similar elements have been described in two filum terminale and one supratentorial, intraventricular ependymoma, respectively. Histologic and evolutional data of those and of our own case suggest that isolated giant cells are not necessarily linked to a bad prognosis in ependymomas.

Stroke is one of the most frequent causes of seizures in adulthood. Subcortical infarcts have been associated with post-stroke epileptic seizures, although less frequently than cortical ischaemia. We studied 113 patients from a hospital stroke registry. The patients had subcortical non-lacunar infarcts (64 striatocapsular infarcts, 13 thalamic, 8 choroidal artery territory, 28 internal border zone and white matter medullary infarcts) and at least 1 year of follow-up. Only 4 patients (3.5%) with striatocapsular infarcts suffered an epileptic seizure. Two seizures occurred within the first 24 h, 1 within the first month and 1 within the first year of stroke onset. Emboligenic cardiac conditions were significantly more common in patients with seizures (difference 37%; 95% confidence intervals 2-72%). Subsequently to subcortical infarct, epileptic seizures are infrequent, tend to occur early after stroke and have a very low 1-year recurrence risk., (Copyright 2001 S. Karger AG, Basel)

Neuropathy of Common Peroneal Nerve (CPN) is a frequent clinical condition, generally caused by compression at the fibula head. Three neurophysiological patterns were described: 1) segmental demyelination with conduction block; 2) axonal damage with loss of motor units and sensory potential; 3) a mixed pattern. We report 5 patients with foot drop in whom CPN neuropathy was identified. In 3 in spite of impressive abnormalities in various motor branches and fascicles of the nerve, the peroneus nerve sensory potential remained well preserved. Focal neuropathies can be remarkably selective in terms of motor and sensory deficits, the reason can rely on a different location of the fibres or be related to a distinct histological-biochemical constitution. A preserved SPSP should not exclude a CPN neuropathy.

Background: Fibrillation/sharp-waves (fibs-sw) are considered an essential neurophysiological marker in Amyotrophic Lateral Sclerosis (ALS). We investigated how frequently an initial electrophysiological (EMG) evaluation, performed in ALS patients, revealed no fibs-sw., Methods: The initial EMG studies from 68 ALS patients were analysed. The presence of fibs-sw in at least two muscles of each limb and two cranial-innervated muscles was assessed., Results: We observed no fibs-sw in cranial-innervated muscles, and 7/15 bulbar-onset patients had no fibs-sw in limb muscles. Two of 28 upper-limb-onset patients, who had a short history before EMG, showed no fibs-sw. All lower-limb-onset patients had fibs-sw. Generally patients with no fibs-sw had diffuse abundant fasciculations., Conclusions: The absence of fibs-sw should not necessarily preclude the neurophysiological diagnosis of ALS in bulbar-onset patients. Early affected upper-limb-onset patients might show no fibs-sw, but profuse fasciculations associated with mild to moderate denervation-reinnervation in weak muscles are found.

Introduction: Early diagnosis and therapeutic measures of frequent pathologies like hyperthyroidism made their neurological complications less frequent. In spite of well known, these neurological complications are some times forgotten., Clinical Cases: We describe two myopathy cases, which presentation was namely fatigue. The inespecificity of this complaint create aetiology and syndromatic diagnosis difficulties, and therapeutic decision. In first case, the symptoms were very gradual, what is usual in older patients, and became extremely incapacitate, depending on others for every daily activity. In the second case, a young patient with diplopia suggested myasthenia. Both cases were diagnosed with hyperthyroidism and a quick improvement occurred with proper medication. Based in these cases, a thyrotoxic myopathy brief comment is made, as in spite of being frequent, these cases presented diagnosis difficulties.

The association of central and peripheral demyelination was reported previously. Most of the cases refer to central chronic relapsing demyelination with clinical criteria for multiple sclerosis associated with later signs of peripheral nerve involvement. Other authors, described central lesions in patients with chronic inflammatory demyelinating polyneuropathy and in Guillain-Barré syndrome, as a seldom occurrence. We report a patient in which a chronic steadily progressive central and peripheral predominantly motor nervous system demyelination, involving the cranial nerves, was identified. The patient improved after intravenous immunoglobulin suggesting an immune-mediated mechanism. To our knowledge this presentation was not described before.