Sturge-Weber syndrome is a sporadic congenital neurocutaneous disorder often related to varying degrees of motor impairment. The phenomenon of prolonged ictal paresis is a rare seizure sign that can ...be due to lesions affecting the centro-parietal lobe. Focal inhibitory motor seizures can be difficult to differentiate from other clinical entities such as stroke, migraine or postictal paresis. We describe the case of a 40-year-old patient suffering from Sturge-Weber syndrome, admitted due to prolonged right-sided hemiparesis following a usual seizure. Repeated EEGs during the prolonged deficit showed only intermittent left fronto-parietal sharp waves. (99m)Tc HMPAO-brain SPECT performed seven days after the last seizure showed a vast area of parieto-occipital hyperperfusion in the left hemisphere. Aggressive antiepileptic therapy dramatically improved the clinical symptoms and scintigraphic images, which corroborated the diagnosis of ictal paresis. This case highlights the role of SPECT in the evaluation of Sturge-Weber syndrome, not only to investigate progressive neurological deterioration, but also exacerbation of seizures or prolonged neurological deficits. In fact, it may be possible to document ongoing epileptic activity using SPECT, despite a non-contributory EEG, which may be of help in adapting a therapeutic strategy.
Radiofrequency (RF) ablation of paroxysmal supraventricular tachycardia (SVT) that could be an atrioventricular node re-entrant tachycardia (AVNRT) or AV reentrant tachycardia (AVRT) through a ...concealed accessory pathway (AP) is widely used in adults, but the indications remain controversed in children and teenagers. The purpose of the study was to evaluate the clinical and electrophysiological factors of indications of AVNRT and AVRT ablation in the youth and the results of the procedure.
66 children or teenagers aged from 6 to 18 years (15 ± 2) among 1099 patients consecutively recruited for SVT; they had a normal ECG in sinus rhythm; AVNRT or AVRT was confirmed at electrophysiological study (EPS). Patients with anterograde conduction through an AP were excluded.
RF ablation was indicated in 26 children or teenagers (39%) (group I) significantly less frequently than in adults (668/1033; 65%) (p < 0.0000). 40 other children were not treated or received beta blockers (group II). Group I and II have the same age (15 ± 2.6 vs 15 ± 2.8). Male gender tended to be less frequent in group I (9/26; 35%) than in group II (20/40; 50%) (NS, 0.2). At EPS, group I had more frequently a concealed AP (13/26; 50%) than group II (8/40; 20%) (p < 0.01). The incidence of concealed AP in group I is higher than in adults (102 of 668 procedures;15%) (p < 000000). In remaining children, ablation of slow pathway was performed in 13 children. There were no complications, but failures or reappearances of SVT after ablation were frequent (6/26; 23%), more frequent in AVRT (4/13) than in AVNRT (2/13) (NS) and were related to the age (12.7 ± 3 vs 16 ± 2; p < 0.002). They tended to be more frequent than in adults (77/668; 11.5%) (p < 0.07). In group II, 3 children continue the beta blockers and other children are not treated.
The indications of slow pathway remain rare in children. Most of the indications of SVT ablation in the youth which are rare, concern generally the children and teenagers with concealed AP. The results are almost as good as in adults, mainly for the slow pathway ablation and in teenagers.
Radiofrequency (RF) ablation of accessory pathway (AP) is widely used in Wolff-Parkinson-White syndrome (WPW), but the indications remain controversial in children and teenagers. The purpose of the ...study was to evaluate the clinical and electrophysiological factors associated with the indications of AP ablation in the youth.
133 children and teenagers aged from 5 to 18 years (13.5±3) were admitted for a WPW and electrophysiological study (EPS) either for tachycardia, syncope or they were asymptomatic. EPS was performed in control state and after isoproterenol. Then RF ablation was proposed in case of recurrent tachycardias or induction of rapid atrial fibrillation (AF).
AP ablation was indicated in 51 children (38%), several years after initial evaluation in 5 of them (group I), and was not performed in 82 children (group II). The indications did not differ significantly from the adult population (232/504, 46%). Clinical and electrophysiological data of groups I and II were compared. Group I was older than group II (15±2 vs 13±4) (p<0.001); male sex was more frequent in group I (72.5%) than in group II (56%) (p<0.05); group I was less frequently asymptomatic (27%) than group II (55%) (p< 0.01) and had more frequently spontaneous reentrant tachycardias than group II (49% vs 28%) (p< 0.05). Syncope was as frequent in group I (21.5%) as II (18%). At EPS, group I had a more rapid conduction through AP than group II, in control state (208±68 b/min vs 176±53, p< 0.001), but not after isoproterenol (245±74 vs 230±63, NS). Reentrant tachycardia was induced more frequently in group I (70.5%) than II (37.5%) (p< 0.001). AF was induced more frequently in group I 35%) than in group II (11%). Malignant forms (rapid AF) were more frequent in group I (31%) than in group II (5%) (p< 0.001). Failures of ablation were as frequent in children (8%) as in adults (2.5%) (NS).
The indications of AP ablation in the young population are based on recommended data and are similar to those of adults, with a similar risk of failure; they concern symptomatic or high risk children. The boys are referred more frequently than girls for the ablation.
The rate of supraventricular tachycardias (SVT) depends on the atrioventricular (AV) nodal conduction. Classicaly the maximal rate of AV nodal conduction decreases with the age. The purpose of the ...study was to look for the rate of induced SVT according to the age of the patient.
Electrophysiological study was performed in 1003 patients without WPW in sinus rhythm, but presenting with SVT; 68 were aged from 5 to 20 years (mean 15±3),102 from 21 to 30 (25±3), 134 from 31 to 40 (37±3), 188 from 41 to 50 (46±3), 187 from 51 to 60 (55±3), 155 from 61 to 70 (66±3), 155 aged from 71 to 80 (75±3), and 51 aged more than 80 years. SVT was induced by programmed atrial.
The rate in SVT was similar in teenagers and adults until 50 years (mean 196±37 b/min) (respectively 197±29 b/min before 21 years, 217±44 b/min from 21 to 30 years, 193±40 b/min from 31 to 40 years and 192±33 b/min from 41 to 50 years) (NS). The rate in SVT was similar from 51 to 80 years (mean 172±33 b/min), but was significantly slower than before 51 years (p < 0.01); the rates were respectively 178±36 from 51 to 60 years, 167±34 b/min from 61 to 70 years, 172±26 b/min from 71 to 80 years (NS). The rate was significantly slower in patients older than 80 years compared to all ranges of age (138±32 b/min). Patients with syncope associated to SVT had a higher rate of SVT (199±41 b/min) than patients without syncope (182.5±36.5 b/min) (p<0003) However, the incidence of syncope was similar in teenagers and adults < 51 years (13% in all ranges of age), decreased between 51 and 70 years (5 and 8%) and increased from 71 to 93 years (15 and 16%).
The rate of SVT seems depend on the autonomic nervous tone. The rate was relatively high in teenagers but also in all adults < 51 years. It is slower from 51 until 80 years but it is more markedly slower after 80 years. Similar correlations were found with the incidence of syncope associated with SVT.
A rare case of lymphoplasmacyte-rich meningioma observed in a young girl is reported. The first clinical manifestations of the disease were seizures. Neuroradiological images favored the existence of ...a meningioma. Abnormalities in the patient's blood chemistry, principally including hypergammaglobulinemia and inflammatory syndrome, were associated with the disease. The tumor was histologically confirmed as meningioma with massive infiltrates of type B lymphocytes. The pathophysiology of the conspicuous lymphoplasmacyte infiltrates, responsible for peripheral blood abnormalities, has remained poorly understood. Alternative diagnostic hypotheses of masses that mimic this type of meningioma are discussed.
Aims
Symptoms in children are often difficult to interpret. The purpose of this study was to report the results of transoesophageal electrophysiological study (EPS) performed in children complaining ...of sudden onset tachycardia with normal non-invasive studies.
Methods and results
Eighty-two children and teenagers (mean age 15 ± 3 years) presented with suspected but no documented paroxysmal supraventricular tachycardia (SVT). ECG was normal. Non-invasive studies were negative; 23 children had syncope with tachycardias. They underwent transoesophageal EPS in our out-patient clinic. The mean duration of transoesophageal EPS was 11 ± 5 min. Electrophysiological study was negative in 25 children. AV nodal re-entrant tachycardia could be induced in 37 children, 11 of them associated with syncope. Wolff-Parkinson-White syndrome (WPW) was diagnosed in five children in which atrioventricular re-entrant tachycardia was inducible. Atrioventricular re-entrant tachycardia due to a concealed AP was induced in 14 children. Verapamil-sensitive ventricular tachycardia was induced in one patient.
Factors associated with tachycardia inducibility were an older age (15.5 ± 2 vs. 14 ± 4 years) (P < 0.05) and the absence of syncope (81 vs. 52%) (P < 0.05). During a mean follow-up of 3 ± 1 year, no patient with negative EPS developed documented tachycardia. In 17 children with inducible SVT, radiofrequency ablation of the re-entrant circuit was subsequently performed.
Conclusion
Transoesophageal EPS is a fast method for proving the nature of paroxysmal tachycardia in children and teenagers presenting with normal ECG and for demonstrating WPW syndrome not visible on standard ECG. The negative predictive value of transoesophageal EPS for the diagnosis of SVT was 100%.
Massive occurrence of polyglucosan bodies (PBs) was found within the surgically removed temporal lobe of a 34 year old woman with complex partial seizures. This peculiar feature is very unusual in ...neuropathological examinations of epileptogenic foci. This patient could not be included in any of the classic diseases in which PBs are found. She exhibited a localised form of glycogen storage disease.
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