Gelastic seizures not associated with hypothalamic hamartoma: A long-term follow-up study.

OBJECTIVE: The objective of the study was to describe the electroclinical features, seizure semiology, and the long-term evolution of gelastic seizures (GS) not associated with hypothalamic hamartoma (HH). METHODS: We reviewed video-electroencephalogram (video-EEG) recordings from pediatric patients with GS without HH admitted to 14 Italian epilepsy centers from 1994 to 2013. We collected information about age at onset, seizures semiology, EEG and magnetic resonance imaging (MRI) findings, treatment, and clinical outcome in terms of seizure control after a long-term follow-up. RESULTS: A total of 30 pediatric patients were stratified into two groups according to neuroimaging findings: group 1 including 19 children (63.3%) with unremarkable neuroimaging and group 2 including 11 children with structural brain abnormalities (36.7%). At the follow-up, patients of group 1 showed better clinical outcome both in terms of seizure control and use of AED polytherapy. Our patients showed remarkable clinical heterogeneity, including seizure semiology and epilepsy severity. Electroencephalogram recordings showed abnormalities mainly in the frontal, temporal, and frontotemporal regions without relevant differences between the two groups. Overall, carbamazepine showed good efficacy to control GS. CONCLUSIONS: Patients with nonlesional GS have a more favorable outcome with better drug response, less need of polytherapy, and good long-term prognosis, both in terms of seizure control and EEG findings.

Seizure burden and neurodevelopmental outcome in newborns with hypoxic-ischemic encephalopathy treated with therapeutic hypothermia: A single center observational study.

OBJECTIVE: To examine the relationship between electrographic seizures and developmental outcome at 18 and 24 months in neonates with moderate and severe hypoxic-ischemic encephalopathy [HIE] treated with therapeutic hypothermia [TH]. STUDY DESIGN: 30 term infants with moderate-severe HIE treated with TH were enrolled prospectively from June 2012 to May 2018. All had continuous single channel amplitude integrated EEG (aEEG) monitoring for a minimum of 72 h and brain MR within 4 weeks. The aEEG was classified by severity of background and seizure burden. MR images were graded by the severity of injury. Outcome (defined abnormal in case of death, dyskinetic or spastic quadriplegic cerebral palsy, epilepsy, or Bayley III score < 85 in all three subscales or < 70 in any individual subscale) was assessed at 18 and 24 months. RESULTS: Seizures were recorded in 24 out of 30 [80 %] neonates and an abnormal outcome was observed in 7 [23 %] of infants. Patients with poor outcome had a statistically significant correlation with: high seizure burden (p = 0.0004), need for more than one antiepileptic drugs (p = 0.006), a persistent abnormal aEEG trace at 48 h (p = 0.0001) and moderate-severe brain injury at MRI (p = 0.0001). Moreover, infants with status epilepticus or frequent seizures reported a significantly association with abnormal MR imaging and poor outcome than patients with sporadic seizures (p = 0.0009). CONCLUSION: The role of seizures in the pathogenesis of brain injury remains controversial. In our cohort the presence of seizures, per se, was not associated with abnormal outcome; however a high seizure burden as well as a persistent abnormal aEEG background pattern and MR lesions resulted significantly associated with poor prognosis.

Reliability of TMS-related measures of tibialis anterior muscle in patients with chronic stroke and healthy subjects.

A lack of normative data for transcranial magnetic stimulation (TMS)-related measures of the lower limb muscles in patients with stroke prevents us from understanding whether changes in TMS-related measures are induced by treatment or are due to their variability and/or the natural evolution of the disease. The purpose of this study was to determine the reliability of three TMS-related measures: motor threshold (MT), motor evoked potential latency (MEP Lat) and MEP amplitude (MEP Amp), linked to the corticospinal control of the tibialis anterior (TA) muscle in sixteen patients with chronic stroke and in sixteen aged-matched healthy subjects. Test-retest reliability was estimated using the intraclass correlation coefficient (ICC) with its 95% confidence interval (95% CI) and standard error of measurement (SEM). In healthy subjects the reliability of all the TMS-related measures yielded an ICC≥0.75. Similar reproducibility levels were found in patients with chronic stroke, with the exception of MEP Amp on the paretic side (ICC=0.38). These results suggest that the TMS-related measures investigated are reliable both in healthy subjects and, with the exception of MEP Amp on the paretic side, in patients with chronic stroke.

Reliability of transcranial magnetic stimulation-related measurements of tibialis anterior muscle in healthy subjects.

OBJECTIVE: The main purpose of this study was to determine the reliability of transcranial magnetic stimulation (TMS)-related measurements linked to the corticospinal control of the tibialis anterior (TA) muscle in healthy subjects. METHODS: Inter-investigator and intra-investigator (within-session with a 1.5-h interval; between-session with a 4-week interval) reliability of the motor threshold, recruitment curve with its slope and MEP area at the plateau, MEP latency, maximum MEP (MEPmax) area, and duration of the maximum and minimum silent period (SPmax and SPmin) were assessed in 50 (29 men and 21 women) healthy subjects (mean age 44.8 years, range 22-74 years) using intraclass correlation coefficients (ICC), standard error of measurement (SEM) and smallest real difference (SRD). RESULTS: The high ICC values and lower SEM and SRD values indicate a good intra- and inter-investigator reliability for motor threshold (ICC range 0.94-0.98), MEP latency (ICC range 0.79-0.93), SPmax (ICC range 0.89-0.95) and SPmin (ICC range 0.79-0.81) in healthy subjects. CONCLUSIONS: These results suggest that the TMS-related measurements investigated are reliable in healthy subjects. SIGNIFICANCE: These data could be useful in further studies on cortical excitability changes, such as those induced by therapeutic interventions (e.g. rehabilitative treatment).

[Dissection of epiaortic vessels: clinical appearance and potentiality of imaging techniques].

PURPOSE: To analyse the major radiological and clinical features of dissections of the epiaortic vessels and evaluate the potential of imaging techniques on the basis of the findings reported in the literature over the last few years. MATERIALS AND METHODS: We evaluated 9 dissections (7 carotid lesions and 2 vertebral lesions) in 9 patients (5 women and 4 men; age range: 30-56 years) who came to our attention between September 1999 and June 2002. These were all stenoses and obstructions located in the cervical region and, in two patients, extended intracranially. The morphologic features were assessed in each case by colour-Doppler US, CT, MR or conventional angiography and correlated with clinical, therapeutic and distant progression patterns. Colour-Doppler US of the neck vessels was performed in 4 patients; CT angiography of neck and intracranial vessels was performed in 4 patients using a single-slice spiral scanner and a single contrast medium bolus injection, following unenhanced examination of the brain; MR angiography was performed in 6 patients after baseline examination using flow-dependent and/or angiographic sequences; conventional angiography was performed in 7 patients. Clinical manifestations at onset were peripheral neurological lesions in 5 cases and central ischaemic lesions in the remaining 4 cases. All patients underwent medical therapy (anticoagulants or antiaggregants). The follow-up was done by colour-Doppler US, MR and/or MR angiography; follow-up conventional angiography at 6-9 months was also performed in 6 patients. Distant progression was assessed on the basis of the degree of vascular re-canalization and the residual signs and symptoms. RESULTS: MR- and CT-angiography were diagnostic in all cases. In particular, diagnosis was obtained by MR-angiography in 4 patients and by CT-angiography in 3 patients. Conventional angiography yielded a diagnosis in 2 cases which had previously undergone non-contrast brain CT or MR without examination of neck vessels alone. Colour-Doppler US revealed non-specific wall and flow alterations in 2 cases, thus requiring further diagnostic studies. In patients with central neurological symptoms at onset the diagnosis was obtained within 48-72 hours, whereas in patients with initial peripheral neurological symptoms the time to diagnosis was significantly longer (up to 10 days) due to the poor specificity of the clinical picture and/or failed detection of the warning symptoms. Medical treatment gave rise to no major complications. Overall, distant progression was good: 6 patients recovered completely and 3 had mild to moderate residual neurological deficits. DISCUSSION AND CONCLUSIONS: Dissection of epiaortic vessels should always be considered as a probable cause of cerebral ischaemia in adults aged 20-50 years, particularly in the absence of vascular risk factors. Clinical assessment is fundamental for the diagnosis and treatment. Currently, CT and MR imaging techniques providing similar information are reliable diagnostic tools that can rapidly and non-invasively clarify suspicious clinical cases. Nevertheless, angiography continues to have an important role in the overall evaluation of disease severity in view of the possible endovascular or surgical treatment of complications.