How amytal changed psychopharmacy: off-label uses of sodium amytal (1920-40).

In the early 1930s, American neurologist and psychiatrist William Bleckwenn used sodium amytal to render catatonic patients responsive, so that he could engage in talk therapy. Bleckwenn found a new, 'off-label' use for this anaesthetic and anxiolytic medication in psychiatry and, in doing so, allowed for important discoveries in the diagnosis and treatment of catatonia. Pharmacological textbooks reveal a 'label', while the Index-Catalogue of the Library of the Surgeon-General's Office reveals explorations 'off label' of barbiturates. The 'off-label' use of barbiturates facilitated talk therapy, heralding an important shift in psychopharmacy. Drugs previously only used as chemical restraints became a form of treatment for specific psychiatric diseases. The current strictures against off-label prescribing are overprescriptive and close off innovative new uses.

Doose syndrome (myoclonic-astatic epilepsy): 40 years of progress.

Doose syndrome, otherwise traditionally known as myoclonic-astatic epilepsy, was first described as a unique epilepsy syndrome by Dr Hermann Doose in 1970. In 1989, the International League Against Epilepsy classified it formally as a symptomatic generalized epilepsy, and 20 years later it was renamed 'epilepsy with myoclonic-atonic seizures'. In this review, we discuss the components of this unique disorder including its incidence, clinical features, and electroencephalographic findings. Recent evidence has suggested possible genetic links to the GEFS+ (generalized epilepsy with febrile seizures plus) family, and, additionally, some children with structural brain lesions can mimic the Doose syndrome phenotype. Treatment strategies such as corticosteroids, ethosuximide, and valproate have been described as only partially effective, but newer anticonvulsants, such as levetiracetam and zonisamide, may provide additional seizure control. The most effective treatment reported to date appears to be the ketogenic diet. Prognosis is quite varied in this disorder; however, many children can have a remarkably normal neurodevelopmental outcome.

Enfermedad de lafora: una forma poco frecuente de epilepsia mioclónica: aspectos clínicos, histológicos, inmunohistoquímicos y ultraestructurales de un caso: revisión de la literatura / Lafora's disease: an form little frequent of myclonus epilepsies: aspect clinic histological inmunohistochemical and ultrastructural: revision of a case and of the literature

Los cuerpos de poliglucosán (CPG), se encuentran en diversas entidades patológicas e incluso en condiciones normales. Su hallazgo en biopsia, cerebrales de pacientes con epilepsia mioclónica progresiva es diagnóstico de enfermedad de Lafora, siendo también encontrados en hígado, músculo esquelético, músculo cardíaco y piel. Presentamos los hallazgos clínicos, gistoquímicos, inmunohistoquímicos y ultraestructurales de la biopsia cerebral de un paciente masculino de 18 años de edad, en el que clínicamente se sospechó la enfermedad

[Lafora and neuropathology]. / Lafora y la neuropatología.

This article wants to be a memory to the figure and neuro-pathologic work of D. Gonzalo Rodríguez-Lafora. The tediousness of its neurological work allows to divide it in its slopes neurophatologic, neurophysiologic, clinic and therapy. Also, it embraces other topics outside of the field of the neurology, centered fundamentally in the psychiatry. It is without a doubt the facet neuro-histopathologic the one that provides him bigger national and international prestige and it contributes to deepen in the histopathology of the senility, picking up in a definitive way in their work critical valuation of the discoveries histopathological in the senility (1952) their thought in this respect. Mention separated deserves its more important discovery: The inclusions amylaceous in cells ganglionars, in a certain type of epilepsy myoclonic that today takes its name. Other entities like the illness of Wernicke, the hemorrhages hipofisarias, the Parkinson (for scarce months he is not early to Levy in an important discovery), or the alterations of the malaria in the cerebral fabric plows object of their attention, of the work of Lafora highlights its anatomo-pathologics works next to figures as Kraepelin, Alzheimer, Vogt, Openheim or Brodmann. Professor Lafora's figure is known internationally as neuropathologist, recognizing its contribution, collection in the world literature, to the study of the myoclonic epilepsy: 'Lafora disease. A form of progressive myoclonus epilepsy.

[Juvenile myoclonic epilepsy]. / Epilepsia mioclónica juvenil.

Since juvenile myoclonic epilepsy (EMJ) and/or Janz syndrome is a primary syndrome which persists throughout life, there should be permanent behavioural hygiene and treatment to keep it under control. Suspension of treatment is nearly always followed by recurrence. Although some researchers report relapses of 75% on suspension of treatment, Janz (1995), cited by Dreifuss points out that this rises to 91% in patients who suspend treatment after two years with no crises. This is, to date, the highest documented level of relapses in epilepsy (5,6,12).