Treatment of childhood epilepsy with dipropylacetic acid (DPA)

Lagenstein I, Sternowsky HJ, Blaschke E, Rothe M, Fehr R. Treatment of childhood epilepsy with dipropylacetic acid (DPA). Archiv für Psychiatrie und Nervenkrankheiten Vereinigt mit Zeitschrift f�r die Gesamte Neurologie und Psychiatrie. 1978;226(1):43-55.Dipropylacetate (DPA) was used in the treatment of different types of epilepsy in 112 children aged 1–20 years, with a mean age of 9.2 years, for a period of 19.8 months, ranging from 1 to 49 months. Of this group, 64 children were therapy-resistant to other antiepileptic medications prior to the introduction of DPA; 31 were treated for the first time with an antiepileptic drug, which was DPA; 44 were treated with DPA alone; and 68 had one or more additional antiepileptic medication. The following results were found while DPA was administered in a relatively high dosage with a mean of 48 mg/kg body weight/day an ranging from 7 to 125 mg/kg/day. 1. Statistically, the results are significantly better in primary generalized epilepsy than in partial or in secondary generalized epilepsy. 2. Ninety-two percent of 51 patients who had absences were treated successfully. The same applies to 87% of 30 patients with primary generalized grand mal with spike wave, to all four patients who had impulsive petit mal, and to 47% of the 15 patients who had centrencephalic myoclonic-astatic petit mal. 3. Positive effect of DPA in partial epilepsy and secondary generalized epilepsy was seen only if the EEG pattern was ‘centrencephalic’ besides focal changes. During therapy with DPA, five patients with pure focal EEG showed an increase in seizure frequency, which demonstrated complete therapeutic failure. 4. Centrencephalic seizure activity (irregular spike wave, 3/s spike wave, and more than 3.5/s spike wave) were treated successfully (P< 0.001). Focal changes or focal sharp wave with tendency to spread or generalization were treated unsuccessfully.112 Kinder mit unterschiedlichen Epilepsien wurden im Alter von durchschnittlich 9,2 Jahren (1–20 Jahre) 19,8 Monate (1–49 Monate) mit Dipropylacetat (DPA) behandelt. 64 Kinder waren vorbehandelt, hatten jedoch auf die vorausgegangene Medikation nicht angesprochen, 31 erhielten DPA als erstes Medikament. Insgesamt wurden 44 Kinder allein mit DPA, 68 in Kombination mit anderen Medikamenten behandelt. Die Dosierung betrug durchschnittlich 48 mg/kg/die (7–125 mg/kg/die). Folgende Ergebnisse wurden erzielt: 1. Generalisierte primäre Epilepsien wurden statistisch signifikant günstiger beeinflußt als generalisierte sekundäre oder fokale Epilepsien. 2. Bei den generalisierten primären Epilepsien fand sich eine Wirksamkeit von 47–100%: centrencephale myoklonisch-astatische Anfälle 47%, Spikewave-Absencen 92%, Grand-mal-Anfälle mit Spike-wave-Gruppen 87%, Impulsiv-Petit-mal-Anfälle 100%. 3. Positive Effekte bei den generalisierten sekundären (Lennox-Syndrom) und fokalen Epilepsien traten nur dann auf, wenn im EEG „centrencephale“ EEG-Muster nachweisbar waren. Bei 5 Patienten mit alleinigen herdförmigen EEG-Veränderungen wurden erhebliche Verschlechterungen beobachtet. 4. „Centrencephale“ EEG-Muster (irreguläre Spike-wave-Gruppen, 3/s und >3,5/s Spike wave) wurden signifikant beeinflußt (P<0,001), herdförmige Veränderungen und Sharp-wave-Foci mit Ausbreitung oder Generalisation zeigten keine Veränderung

Juvenile Type of Generalized Ceroid-Lipofuscinosis (Spielmeyer-Sjögren Syndrome), I. Clinical Findings

Thirteen patients with the clinical course of the juvenile type of generalized ceroid-lipofuscinosis were examined clinically, ophthalmologically, neurologically and psychiatrically. This included registration of EEG, x-ray, brain scintigraphy, motor nerve conduction velocity, ERG, fundus photography and bioptical investigations including electron microscopy. The children suffered from progressing decrease of visual acuity, decline of mental capacities, and later on speech and gait disturbances. The most characteristic findings are presented in case reports, two tables and fourteen pictures

Juvenile type of generalized ceroid-lipofuscinosis (Spielmeyer-Sjögren syndrome): II. biopsy findings

Rectal biopsies were obtained from 11 children, clinically presumed to suffer from the juvenile type of generalized ceroid-lipofuscinosis. In addition, sural nerve biopsies were performed in 4 cases. Of the various staining procedures applied for light microscopy, Sundan Black B was found to be the most useful, revealing in each of the rectal biopsies at least some nerve cells with coarse black droplets as well as numerous mucosal macrophages with a more greyish, dustlike material. In sural nerve biopsies a slight reduction of the thick myelinated nerve fibers was seen in two cases, accompanied by regeneration phenomena. The latter were also detectable in one other case, the remaining sural nerve biopsy being histologically unremarkable. By electron microscopy neuronal perikarya revealed lipopigment bodies with a fingerprint-like pattern in each of the rectal biopsies. In one case only was this pattern combined with distinct curvilinear profiles. In 4 cases, however, the fingerprint-like deposits were intermingled with different lamellated bodies, some of them reminiscent of MCB's or zebra bodies. In two of the 4 cases, these were even seen exclusively in some neurons. In each of the rectal as well as the sural nerve biopsies, most of the non-neuronal cell types presented lipopigment bodies, likewise, displaying both curvilinear and fingerpring-like profiles, with some characteristics related to the various cell types. With regard to generalized ceroid-lipofuscinosis the diagnostic value of rectal biopsies, though being disputed in the literature, appears reestablished by the findings presented

Quantitative magnetic resonance imaging in multiple sclerosis: the effect of high dose intravenous methylprednisolone.

Magnetic resonance imaging was performed on 50 patients with clinically definite or probable multiple sclerosis before and 15 days after starting treatment with intravenous methylprednisolone (0.5 g daily for 5 days). Scans were abnormal in 49 patients. New lesions had appeared on the second scan in nine individuals and in seven a single pre-existing lesion appeared to have become smaller but in no case were lesions seen to disappear. Two patients showed both reduction in the size of an abnormal area and development of a single new lesion indicating that corticosteroids do not appear rapidly to alter the process underlying plaque formation. Measurements of relaxation times were performed in 12 randomly selected patients. All showed elevated values in normal appearing white matter but not cortex before treatment compared with 18 healthy controls. After treatment a significant decrease of T1 and T2 was observed in cortex, and of T1 alone in normal appearing white matter. No significant change could be detected within lesions, a finding attributed to the wide range of relaxation values observed at these sites before treatment. Since brain water content is increased in normal appearing white matter of multiple sclerosis patients, and is significantly reduced by high-dose methylprednisolone, resolution of oedema may contribute to the rapid spontaneous or corticosteroid induced symptomatic recovery that characterises the disease in its early stages