Juvenile myoclonic epilepsy

CONCLUSION: We conclude that despite inevitable variability the clinical picture of JME is characteristic. It is easy to diagnose JME if one thinks of it while the history should be thoroughly analyzed. An EEG recording during sleep confirms the diagnosis. An early diagnosis of JME permits adequate prognosis of the subsequent course of epilepsy, and adequate therapy brings remission in most of the patients. If treatment starts following the large number of severe GTC seizures, the response to therapy is incomplete. The persistency of the illness throughout the life, the need for continuous medication and therapeutic unresponsiveness in cases with late diagnosis, do not justify the increasing misconception that JME is of benign nature. Diagnosis of JME is rare because of insufficient familiarily of physicians with the illness. BACKGROUND: Juvenile myoclonic epilepsy (JME) is an idiopathic generalized epileptic syndrome characterized with the combination of myoclonic, generalized tonic-clonic (GTC) and absence seizures that are readily provoked by sleep deprivation. PATIENTS: Forty-three patients, aged from 14 to 51 years, participated in a 5-year follow-up study. Diagnosis was made according to the criteria (Table 1) for diagnosis of JME set by Panayiotopoulos et al. (1994). Nineteen patients made their first contact with a neurologist at the Institute of Neurology and were diagnosed as JME, while the remaining 24 were referred to from other medical institutions with a diagnosis of therapy resistant to focal epilepsy. All patients underwent a somatic and neurological examination, "mini mental test," EEG in waking and CT scan of the brain. Some patients had EEG performed during sleep and some had MRI of the head. RESULTS: JME began between 9 and 26 (average 17) years. All patients had myoclonic seizures, 98% had GTC and 23% absence seizures. The first myoclonic seizure occurred between 9 and 24 years while the frst GTC seizure occurred between 10 and 32 years. Myoclonic seizures (83% of patients) and GTC seizures (70% of patients) occurred most often immediately after awaking. The most frequent provocative factors were insufficient sleep, alcohol abuse and tiredness. Epilepsy in the family was present in 39%, focal neurological deficiency in 9% and pathological findings on of CT and MRI in 7% of patients. Waking EEG was pathological in 77% of patients; it included generalized spike-wave discharges in 73%, multiple spike-wave complexes in 33% and focal discharges in 12% of patients, respectively. In all 26 patients tested, sleep EEG was pathological most often with multiple spike-wave complexes in 85% and 3-4 Hz spike-wave complexes in 57% of patients. The correct diagnosis of JME following a comprehensive examination was made in 24 (56%) patients after a delay of 1 to 35 years. In 24 patients with delayed diagnosis of JME the replacement of earlier medication with valproic acid (VPA) induced remission in 18 patients (75%) while 1 patient (4%) experienced a reduction in the number of seizures. Five patients (21%) did not respond to VPA medication: 2 due to a weak compliance, another 2 due to inefficient medication and 1 because of the preexistent malabsorption syndrome. In 19 patients (44%) with initial diagnosis of JME, VPA was introduced immediately upon diagnosis. Of them, 15 (79%) had excellent response to VPA, 1 refused therapy and for 3 patients there is no information. In 2 patients VPA was substituted due to side effects (hepatotoxicity and alopetia) with lamotrigine (low doses), which brought about decrease in frequency and mitigation in myoclonic seizures.     

Application of the scaling factor method to estimation of beta dose distributions for dissimilar media separated by a planar interface

Reliable methods of estimating doses are essential for the use of beta emitting radionuclides for radiotherapy. The passage of electrons through matter is a very complex phenomenon due to the large number of elastic and inelastic interactions resulting in scattering and energy losses. The analytical solution for the electron transport being intractable, the problem has been addressed by the Monte Carlo technique. Empirical or semiempirical less time consuming methods, such as the scaling factor method, may appear more preferable in practice when dealing with complicated source distributions. The method, proposed by Cross and co-workers [AECL Report Nos. AECL-1617 (1982), AECL 10521 (1992)] consists in the derivation of beta-ray dose distribution in other media from those in water by using a "scaling factor" or "relative attenuation factor" on distance and a closely related renormalization factor imposed by the energy conservation. This work investigates the accuracy of the scaling factor method using a statistical approach, a generalized chi 2 test, focusing on the particular case of potential interest, the scaling factor for water to bone. The direct comparison of the shapes of the depth dose deposition curves in the two media indicates discrepancies of less than 5% up to at least 60% of the range in bone, a depth within which 95% of the initial energy is deposited. The scaling factor derived by this method, 0.9720 +/- 0.0012, confirms the existing experimentally determined value of 0.973 +/- 1% [AECL Report No. AECL-10521 (1992)]. The accuracy of the determination is increased by almost a factor of 10. A way of improving the scaling method, especially for depth over the 60% continuous slowing down approximation range, by using a modulation function is also proposed.     

Study of specific absorption rate (SAR) induced in two child head models and in adult heads using mobile phones

This paper gives a first comparison of specific absorption rate (SAR) induced in a child-sized (CS) head and an adult head using a dual-band mobile phone. In the second study, the visible human head is considered and comparison of SAR induced in a CS or child-like (CL) head and an adult head using a dual-band mobile phone is given. All the peaks of average SAR over a mass of 10 and 1 g in the head and the power budget are determined in the two comparisons using the finite-difference time-domain method. The differences between the results for adult and CS or CL heads are given at 900 and 1800 MHz. No important differences are noted for the peak SAR averaged over 10 g (SAR10 g), between the two adult head models, as well as between the two child head models. The peak SAR10 g in the brain of the CS or CL head is slightly more significant than that for the adult one.     

Room-Temperature Mid-, and Far-Infrared Absorption and Electrical Properties of Intercalated GaSe and TlInS<Subscript>2</Subscript> Crystals

We report room-temperature measurements of the mid- and far-IR absorption throughout the 400 – 4000 cm^?1 and 10 – 700 cm^?1 spectral ranges and the resistivity of layered p-GaSe and p-TlInS2 intercalated with Li⁺. Intercalation was performed by immersing Bridgman grown crystals in 0.5 M solutions of LiCl in distilled water at ambient conditions. The crystal structure and the stoichiometry of the grown crystals were determined by X-ray diffraction and XRF methods. It is shown that intercalation does not change the frequency of the IR-, and Raman active low-frequency“rigid layer” mode (GaSe), the space symmetry group or the lattice parameters of the crystals. It was found that for both crystals, the resistivity versus time dependencies are nearly the same. Three ranges in the resistivity-intercalation time dependencies were explained qualitatively. The resistivity increase due to intercalation was explained by assuming that the intercalated lithium ions act as ionized donors and compensate the host p-type crystal. The highest degree of compensation for GaSe and TlInS₂ crystals was achieved after intercalation during 12 and 10 days, respectively.     

Numerical modeling of energy balance equations in quantum well AlxGa1-xAs/GaAs p-i-n photodiodes

The energy balance equations coupled with drift diffusion transport equations in heterojunction semiconductor devices are solved modeling hot electron effects in single quantum well p-i-n photodiodes. The transports across the heterojunction boundary and through quantum wells are modeled by thermionic emission theory. The simulation and experimental current-voltage characteristics of a single p-i-n GaAs/Al _xGa_1-xAs quantum well agree over a wide range of current and voltage, The GaAs/Al_xGa_1-xAs p-i-n structures with multi quantum wells are simulated and the dark current voltage characteristics, short circuit current, and open circuit voltage results are compared with the available experimental data, In agreement with the experimental data, simulated results show that by adding GaAs quantum wells to the conventional cell made of wider bandgap Al_x Ga_1-xAs, short circuit current is improved, but there is a loss of the voltage of the host cell, In the limit of radiative recombination, the maximum power point of an Al_0.35Ga_0.65As/GaAs p-i-n photodiode with 30-quantum-well periods is higher than the maximum power point of similar conventional bulk p-i-n cells made out of either host Al_0.35Ga_0.65As or bulk GaAs material     

Photonic bandgap fibre oscillators and amplifiers

In the search for ever higher output power or energy from fibre oscillators or amplifiers a nowadays mature technology relies on enlarging the fibre mode area. Broadening of the core diameter, all other things being equal, inevitably yields a multimode fibre, thereby dramatically limiting the device usefulness. Various strategies have been deployed to design and manufacture single transverse mode fibre oscillators and amplifiers, among which making use of the so-called photonic bandgap effect to restrict the modal population seems promising. Helped by efficient and reliable numerical tools the design of large mode area singlemode photonic bandgap fibres is presented. Two fibres with 20-μm and 40-μm core diameter, both of them heavily doped with Yb³⁺ ions, have been fabricated by the widespread modified chemical vapour deposition process and are shown to behave properly when used as the core element of either continuous wave oscillators or femtosecond amplifiers. Good output beam quality (M² parameter spanning from 1.12 to 1.5 for the set of fibres studied) and high slope efficiency of 80% in cw oscillation regime are demonstrated. Furthermore the 40-μm core diameter fibre is shown to be resilient to tight bending down to 7.5-cm radius. The stack-and-draw process makes it easy to tailor the outer cladding so that a large numerical aperture can be reached. Subsequently, from this air-clad fibre, 500 fs 47 W pulses at 35 MHz are obtained from a two-stage chirped pulse amplification system.     

Remote‐Controlled Hydrogel Depots for Time‐Scheduled Vaccination

Remote‐controlled drug depots represent a highly valuable tool for the timely controlled administration of pharmaceuticals in a patient compliant manner. Here, the first pharmacologically controlled material that allows for the scheduled induction of a medical response in mice is described. To this aim, a novel, humanized biohybrid material that releases its cargo in response to a small‐molecule stimulus licensed for human use is developed. The functionality of the material in mice is demonstrated by the remote‐controlled delivery of a vaccine against the oncogenic human papillomavirus type 16. It is shown that the biohybrid depot‐mediated immunoprotection is equivalent to the classical multi‐injection‐based vaccination. These results indicate that this material can be used as a universal remote‐controlled vehicle for the patient‐compliant delivery of vaccines and pharmaceuticals.