Later childhood and adolescence

Progressive myoclonus epilepsies have the nosological picture of an evolving syndrome of symptoms including massive and segmental myoclonus, myoclonic or tonic-clonic seizures, partial seizures, cerebellar impairment, and higher neurological dysfunctions. (19 Baltic or non-Lafora progressive myoclonus epilepsy is most common in the Finnish population. The age of onset is around 7 years and the disease starts with myoclonus or nocturnal tonic-clonic seizures. The life course of this disease is not as poor as in Lafora disease. The longest lifespans are more than 60 years. The intelligence level is slightly lowered or even normal. Patients with severe retardation have often had drug intoxication. (14>

Northern epilepsy syndrome resembles progressive myoclonus epilepsy but it always includes mental retardation and its gene defect is different. (19 Sialidosis and mitochondrial encephalopathy with ragged red fibres may also show myoclonic seizures. Epilepsy is quite common in girls with Rett syndrome, affecting about 90 per cent of the patients. They may have several seizure types including partial, generalized tonic-clonic, and myoclonic seizures, atypical absences, short flexion or extensor spasms, and drop attacks or various combinations of such seizures.(!6)

Of the progressive partial epilepsies, epilepsia partialis Kojewnikow or Rasmussen syndrome type 2 is especially important because the disease is fatal if untreated. The classical model of the association between frequent epileptiform discharges and permanent loss of function is provided by the Landau-Kleffner syndrome or acquired epileptic aphasia. There is increasing evidence that frequent epileptiform discharges, perhaps particularly overnight in the form of continuous spikes and waves during slow sleep, also called electrical status epilepticus, is associated with permanent intellectual impairment if allowed to continue for long periods/1?

Break Free From Passive Aggression

Break Free From Passive Aggression

This guide is meant to be of use for anyone who is keen on developing a better understanding of PAB, to help/support concerned people to discover various methods for helping others, also, to serve passive aggressive people as a tool for self-help.

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