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Edoardo Ferlazzo

Publications and source records attributed to Edoardo Ferlazzo.

9 recordsLinked to original sources

Long-term evolution of EEG in Unverricht-Lundborg disease.

PURPOSE: Unverricht-Lundborg disease (ULD) is a progressive myoclonus epilepsy characterized by myoclonus, epilepsy, and ataxia, without major cognitive decline. There is no systematic study on the long-term evolution of EEG in this condition. PATIENTS AND METHODS: Twenty-five patients with ULD who came to our observation before 1995 and periodically followed in our Epilepsy Centres were included. All waking EEG traces were visually reviewed for the characterization background activity, with particular regard to the frequency of the posterior dominant rhythm (PR), and for the occurrence of spontaneous generalized spike or polyspike and wave discharges (GSWD) and photoparoxysmal response (PPR). Sleep recordings were analyzed with particular regard to the preservation of the physiological sleep patterns and the occurrence of GSWD and other epileptic abnormalities. RESULTS: PR was normal in 68% of patients at the beginning of the disease and kept stable over the years. GSWD were present in 92% of patients at the onset of the disease and gradually disappeared during the follow-up with a significant difference (p<0.001) after the 15th year of disease. PPR was present in 88% of patients at the disease onset and gradually disappeared with a significant difference (p<0.001) after the 10th year of disease. A gradual reduction of GSWD and a progressive disappearance of physiological sleep patterns were observed in sleep EEGs. CONCLUSION: In patients with ULD followed for an extended period of time, EEG shows no relevant deterioration of BA while a gradual reduction of GSWD and PPR is observed over time, well correlating with the good seizure outcome in this condition.

Adult↗

Unverricht-Lundborg disease, a condition with self-limited progression: long-term follow-up of 20 patients.

PURPOSE: To assess the long-term evolution of Unverricht-Lundborg disease (ULD), especially concerning myoclonus, seizures, and EEG characteristics. METHODS: We retrospectively evaluated 20 patients (six women, 14 men; mean age, 37.9 years; range, 26-53 years) with ULD who had been closely followed up since the onset of the disease (mean age, 12.3 years; range, 6-17 years) for an average of 25.6 years (range, 13-41 years). ULD was confirmed by genetic tests in all. We used simplified myoclonus and seizure rating scales. RESULTS: The geographic origin of the patients was Northern Africa in nine, France in two, Italy in six, and mixed European in three. Three patients were severely handicapped, six led fully autonomous lives, and 11 required various degrees of social support. Myoclonus progressed only during the first 5 years of disease. Major seizures occurred in 19. Three patients had a single seizure, and eight became seizure free, whereas six had rare seizures, and two had frequent attacks. Overall, seizures became much less frequent after 10 years of evolution. EEG changes abated during follow-up: background activity remained stable or improved, spontaneous discharges disappeared, and photoparoxysmal responses were abolished in all patients but two. CONCLUSIONS: This study shows that ULD progresses only over a limited period and stabilizes thereafter. This self-limited progression may be the consequence of age-related apoptosis of selected neuronal populations.

Adaptation, Physiological↗

Recurrent status epilepticus as the main feature of Hashimoto's encephalopathy.

Hashimoto's encephalopathy (HE) is a severe but treatable condition that rarely complicates Hashimoto's thyroiditis. Clinically it is characterized by progressive or relapsing symptoms, including tremor, myoclonus, stroke-like episodes, seizures, impairment of consciousness, and dementia. We describe a patient presenting with recurrent generalized convulsive status epilepticus (GCSE), despite antiepileptic medications, who was successfully treated with methylprednisolone. Our observation confirms that the clinical spectrum of HE at presentation is heterogeneous and diagnosis is often difficult. This case highlights the crucial importance of antithyroid antibody measurement in patients presenting with otherwise unexplained episodes of GCSE with or without adjunctive signs of encephalopathy or thyroiditis.

Adult↗

Cortical triggers in generalized reflex seizures and epilepsies.

Activation of specific cortical territories by sensory stimuli or of less restricted areas of the brain by cognitive stimuli is known to induce apparently generalized seizures in predisposed patients; this is clinically and electroencephalographically distinct from reflex triggering of partial seizures. Photosensitive patients may have seizures when exposed to environmental stimuli producing appropriate flickering light or geometric patterns. Some children with benign myoclonic epilepsy in infancy have seizures triggered by unexpected touch or noise. Seizures induced by thinking have been reported in response to non-verbal higher mental activity such as mental arithmetic. Praxis-induced seizures are triggered by similar mental activities accompanied by the use of the hands. Language-induced seizures are usually triggered by verbal higher mental activity. Functional imaging and other methods have contributed to understanding how these seizures arise. Patients with these generalized reflex seizures appear to have regions of cortical hyperexcitability overlapping or coinciding with areas physiologically activated during specific sensory stimulations and cognitive or motor activities. When these areas receive appropriate afferent volleys and a critical mass of cortex is activated, an epileptic activity is produced that ultimately involves cortico-reticular or cortico-cortical pathways resulting in a generalized or bilateral epileptic event.

Cerebral Cortex↗

Transient edematous lesions of the splenium in epileptic patients.

BACKGROUND: Transient focal lesions in the splenium of the corpus callosum (SCC) have been previously described in patients with epilepsy or without epilepsy but receiving antiepileptic drugs (AED). CASE REPORTS: Two epileptic patients were admitted to our long-term monitoring unit. Antiepileptic drugs were completely discontinued a few days later. One patient had no seizures. The other had three attacks, the last of which occurred two days before a brain magnetic resonance imaging (MRI) was performed. In both cases brain MRI showed a lesion in the SCC characterized by high signal on T2-weighted images and no enhancement after Gadolinium infusion. The patients were discharged with their pre-admission medications. A follow-up MRI five weeks later showed resolution of the SCC lesions. CONCLUSIONS: The pathogenesis of transient SCC lesions in epileptic patients is still unclear. In our patients, either the sudden AED withdrawal or the seizures activity may be presumed to be the cause, though an individual susceptibility must also be considered.

Adult↗

Montelukast-induced generalized urticaria.

OBJECTIVE: To report a case of generalized urticaria induced by montelukast treatment. CASE SUMMARY: A 28-year-old man with allergic rhinitis and moderate persistent asthma developed generalized urticaria 5 days after the initiation of montelukast and inhaled fluticasone. Symptoms disappeared within one day after suspension of both drugs. Two months later, after the resumption of montelukast and fluticasone, the patient developed generalized urticaria and eyelid angioedema, which were successfully treated with intravenous betamethasone, achieving complete remission within hours. After 2 days, the patient resumed inhaled fluticasone only and continued this therapy for several months without any adverse reaction. DISCUSSION: We attributed the adverse reaction to montelukast because of the temporal relationship between use of montelukast and urticaria, the absence of other identified causative factors and other explanations for allergic reactions, and the positive dechallenge and rechallenge. The Naranjo probability scale showed a probable relationship between skin manifestations and montelukast treatment. CONCLUSIONS: The use of antileukotrienes is increasing in asthma therapy. In cases of generalized urticaria in asthmatic patients undergoing montelukast therapy, physicians should be aware of a potential adverse reaction to this drug.

Acetates↗

Involvement of fractalkine and macrophage inflammatory protein-1 alpha in moderate-severe depression.

Moderate-severe depression (MSD) is linked to overexpression of proinflammatory cytokines and chemokines. Fractalkine (FKN) and macrophage inflammatory protein-1 alpha (MIP-1alpha) are, respectively, members of CX3C and C-C chemokines, and both are involved in recruiting and activating mononuclear phagocytes in the central nervous system. We analysed the presence of FKN and MIP-1alpha in sera of untreated MSD patients and healthy donors. High FKN levels were observed in all MSD patients as compared with values only detectable in 26% of healthy donors. MIP-1alpha was measurable in 20% of patients, while no healthy donors showed detectable chemokine levels. In conclusion, we describe a previously unknown involvement of FKN in the pathogenesis of MSD, suggesting that FKN may represent a target for a specific immune therapy of this disease.

Chemokine CCL3↗