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Seizures in fetal alcohol spectrum disorders: Evaluation of clinical, electroencephalographic, and neuroradiologic features in a pediatric case series
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Epilepsia
Volume 55, Issue 6, June 2014, Pages e60-e66
Seizures in fetal alcohol spectrum disorders: Evaluation of clinical, electroencephalographic, and neuroradiologic features in a pediatric case series (Article)
Nicita, F.a , Verrotti, A.b, Pruna, D.c, Striano, P.d, Capovilla, G.e, Savasta, S.f, Spartà, M.V.f, Parisi, P.g, Parlapiano, G.h, Tarani, L.h, Spalice, A.a
a Child Neurology Division, Department of Pediatrics, Sapienza University of Roma, Viale Regina Elena, Rome-324-00161, Italy
b Department of Pediatrics, University of Perugia, Perugia, Italy
c Division of Child Neurology and Psychiatry, Azienda University Hospital of Cagliari, Cagliari, Italy
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Abstract
Seizures are observed with a frequency of 3-21% in children with fetal alcohol spectrum disorders (FASD). However, clinical, neuroradiologic, and electroencephalography (EEG) features are poorly described. In this study, 13 patients with FASD and epilepsy or seizures were identified retrospectively from the databases of seven Italian pediatric neurology divisions. Eleven children were affected by epilepsy, and two had at least one documented seizure. Both generalized and focal seizures were observed. EEG showed diffuse or focal epileptic activity; two children developed electric status epilepticus during sleep (ESES). Structural brain anomalies, including polymicrogyria, nodular heterotopia, atrophy, and Arnold-Chiari type 1 malformation, were discovered in almost 50% of patients. Control of seizures was not difficult to obtain in 11 cases; one patient showed pharmacoresistant epilepsy. EEG and clinical follow-up are recommended in children with FASD and epilepsy, since severe conditions requiring aggressive treatment, such as in ESES, may develop. Neuroradiological evaluation is warranted because several types of brain anomalies could be associated with maternal alcohol consumption during pregnancy
Clinical Features in Patients With PANDAS/PANS and Therapeutic Approaches: A Retrospective Study
No full textObjective: The clinical characteristics of patients with PANDAS (pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection) and PANS (pediatric acute-onset neuropsychiatric syndrome) and the efficacy of antibiotic therapy with psychotherapy and antipsychotics were investigated to improve neurological symptoms as well as obsessive compulsive disorder (OCD). Methods: We retrospectively analyzed 62 patients with a clinical diagnosis of PANDAS/PANS enrolled from May 14, 2013 to September 15, 2020 in the Neurology Childhood Division, Department of Pediatrics at Sapienza, Rome. Clinical manifestations, neurological and psychiatric, laboratory investigations, and familiar history were collected to evaluate the differences between the two groups. The effects of various therapeutic approaches were examined. Descriptive and comparative statistical analyses were performed. Results: The mean age at onset of PANDAS/PANS symptoms was 6.2 ± 1.2 years. The most common diagnosis was PANDAS, followed by PANS. Neurological and psychiatric symptoms were mostly evident in both groups (>70% of the population), with no significant difference between them (P = 0.52 and P = 0.15, respectively). Irritability, aggressivity, and food restriction were more prevalent in children with PANS than in those with PANDAS (P = 0.024 and P = 0.0023, respectively). The levels of anti-streptolysin O and anti-DNAse B 10-fold higher in PANDAS than those in PANS (P < 0.0001). Antibiotics or psychotherapy were administered in most cases (90.3 and 53.2%, respectively), followed by antipsychotic treatments (24.2%). In the multivariate analysis, among the therapies used, psychotherapy significantly resulted in the most efficacious relief of OCD, reducing stress in patients and their parents (P = 0.042). Conclusion: Our findings confirm a clear clinical difference between the two groups, PANDAS and PANS, using different approaches. In fact, irritability, aggressivity, and food restriction were significantly more frequent in children with PANS and the levels of anti-streptolysin O and anti-DNAse B were higher in PANDAS. Another relevant finding is the efficacy of psychotherapy, especially for obsessive-compulsive disorder, and of antibiotic prophylaxis in managing acute neurological symptoms
Eye movement desensitisation and reprocessing (EMDR) treatment associated with parent management training (PMT) for the acute symptoms in a patient with PANDAS syndrome: a case report
No full textBACKGROUND: The purpose of this report was to present the results of eye movement desensitisation and reprocessing (EMDR) therapy associated with parent management training (PMT) in a child with paediatric autoimmune neuropsychiatric disorder associated with streptococcus (PANDAS), who had previously only been treated with pharmacological treatment. CASE PRESENTATION: The case concerns an 11-year-old boy who presented with simple and complex vocal tics, motor tics, obsessive-compulsive traits and irritability from the age of 6 years, in addition to a positive result for streptococcal infection. The course of symptoms followed a relapsing-remitting trend with acute phases that were contingent on the infectious episodes. CONCLUSIONS: Following eight sessions of EMDR, preceded by training sessions with the parents, the child showed a significant reduction in symptoms and disappearance of the exacerbation. These results indicate the possibility of improving the treatment outcomes of patients with PANDAS by a combined approach using both antibiotic and EMDR therapies
Lamotrigine treatment in Tuberous Sclerosis: EEG aspects.
No full textLamotrigina nelle epilessie in bambini con Slcerosi Tuberosa
Paroxysmal tonic upgaze. physiopathological considerations in three additional cases
No full textIn questo articolo vengono discussi tre casi clinici sottolineando gli elementi per la differenziazione elettro-clinica di movimenti parossistici oculari con alcune epilessie idiopatiche e lesionali della età evolutiva.Paroxysmal tonic upgaze of childhood has been described as a benign distinctive syndrome of abnormal ocular movement, with or:without concomitant ataxia. After the first observation of four children, a further 29 patients have been reported with a wide spectrum of neurologic abnormalities such as ataxia, unsteady of gait, learning disabilities and mental retardation at follow-up. Electroencephalograms were normal in all the subjects and magnetic resonance imaging showed deficient myelination in only one patient. Recently it has been suggested that paroxysmal tonic upgaze could be a heterogeneous syndrome, ranging from a simply age-dependent manifestation to a clinical appearance of a variety of disorders affecting the corticomesencephalic loop of vertical eye movement, Moreover, it also could be an early sign of more widespread neurologic dysfunction. We describe three patients who presented paroxysmal tonic upgaze; in one, ataxia was present; in the second child, ata;da and language disorder also-were observed; and in the third patient paroxysmal tonic upgaze was associated with loss of muscle tone (drop-attack-like events). On magnetic resonance imaging, a pinealoma compressing the dorsal mesencephalic region was detected. On the basis of our observations, we suggest that any insult with periaqueductal mesencephalic gray-matter involvement could be considered the basic condition for this peculiar clinical manifestation
Ipotonia nel primo anno di vita.
No full textDiagnosi diferrenziale delle ipotonie nel primo anno di vita
Residual and persistent Adie’s pupil after pediatric ophthalmoplegic migraine
No full textWe report on a 9-year-old girl diagnosed with ophthalmoplegic migraines who had been previously diagnosed, at age 7 years, with typical migraines with aura. After resolution of the third ophthalmoplegic migraine attack, the only evident residual clinical sign was Adie’s pupil. During 24-month follow-up, at age 11 years, a neurologic examination produced completely normal results. However, Adie’s pupil persisted. Adie’s tonic pupil can be associated with extraocular diseases, which were all excluded in this patient. The mechanisms underlying tonic pupil are not fully understood. This is the first
report, to the best of our knowledge, of an ophthalmoplegic migraine followed by persistent Adie’s pupil. Possible
pathogenic mechanisms are discussed
Diffuse onset of ictal electroencephalography in a typical case of Panayiotopoulos syndrome and review of the literature.
No full textPanayiotopoulos syndrome is a common and benign childhood autonomic epilepsy of debated localization. Although officially considered as occipital epilepsy, this is most likely of multifocal origin. Ictal electroencephalography is the gold standard of seizure localization, but in Panayiotopoulos syndrome, because patients have single or rare seizures, only 7 cases with ictal electroencephalography have been reported. Ictal onsets show variable anterior and more often posterior locations. We describe an 8-year-old girl with 5 nocturnal autonomic seizures typical of Panayiotopoulos syndrome from age 4. The last seizure was captured with electroencephalography and showed a diffuse onset of the ictal discharge, whereas various interictal electroencephalography had infrequent multifocal spikes. This case contributes to the understanding of the pathophysiology of Panayiotopoulos syndrome in favor of a diffuse and multifocal cortical epileptogenicity that triggers an unstable central autonomic nervous system solely or prior to the focal cortical symptoms
Positron emission tomography in neuronal ceroid lipofuscinosis (Jansky-Bielschowsky disease): a case report
No full textWe report on a 13-year-old girl with late infantile neuronal ceroid lipofuscinosis (NCL) in whom PET scanning with [18F]-2-fluoro-2-deoxy-d-glucose ([18F]/FDG) was performed. Early psychomotor development was normal. At the age of 2 years, neurological signs such as hypotonia and incoordination appeared, followed by visual failure and ataxia. At the age of 4, funduscopic examination showed macular degeneration and papillary atrophy. At the age of 9, myoclonic jerks were observed; subsequently, generalized seizures together with failing vision, mental deterioration, and visual and auditory hallucinations appeared. Brain MRI showed severe cortical and subcortical atrophy. A skin biopsy detected the presence of 'finger-print' inclusions in the cytoplasm of smooth muscle fibers. Late infantile NCL (Jansky-Bielschowsky disease) was diagnosed. FDG/PET revealed a severe reduction of metabolism in all the cortical and subcortical structures. A regional analysis of the distribution of the tracer revealed marked bilateral hypometabolism, particularly in calcarine, lateral, occipital, and temporal cortices and in the thalamus
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