Introduction

Although epilepsy syndromes have been primarily defined by their seizures, the pervasive additional impairments, particularly cognitive and behavioural, are increasingly recognized as being integral to the condition, e.g. cognitive and autistic regression in West syndrome. Landau–Kleffner syndrome (LKS) could hardly be described without the cognitive impairments. Motor impairments have been largely ignored from descriptions of epilepsy syndromes. Such motor phenomena are, however, common. This chapter summarizes the data on five patients with epilepsy-related movement problems that were present over long periods and were quite unrelated to individual seizures. Such impairments, which are present over time, are designated epileptic encephalopathies. They were all responsive to cortico-steroids which were used by analogy with their use in other epileptic encephalopathies, particularly West syndrome and Landau–Kleffner syndrome. The intention in treating these disorders with cortico-steroids is that such drugs are highly effective antiepileptic agents, rather than that they have any other modes of action, but this statement is open to challenge. The potential reversibility of such neurological impairments, which we would usually regard as evidence of fixed brain lesions in association with epilepsy, is of great theoretical interest, since it points to different pathophysiological mechanisms for the production of classical neurological signs (Neville, 1999).

CASE REPORT 1 LANDAU-KLEFFNER SYNDROME WITH GAIT APRAXIA (NEVILLE & BOYD, 1995)

The first child developed complex partial seizures at the age of 5.6 years with rolandic characteristics and, over a period of 8 weeks, she lost all speech and comprehension.