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Critical care, neurology and analgesia
Published in Evelyne Jacqz-Aigrain, Imti Choonara, Paediatric Clinical Pharmacology, 2021
Evelyne Jacqz-Aigrain, Imti Choonara
Whether seizures will respond to treatment and whether the epilepsy will spontaneously remit is determined by the specific epilepsy syndrome, underlying aetiology and, almost certainly (and as yet unidentified), genetic factors [4]. There is no evidence that the use of antiepileptic drugs influences the natural history of the vast majority of the epilepsies and epilepsy syndrome; the drugs simply suppress the seizures, whilst the epilepsy is active within an individual’s brain. It is possible that, in some of the rare, early onset, infantile epileptic encephalopathies (specifically West syndrome, characterised by infantile spasms and hypsarrhythmia on the EEG [5] and the Landau-Kleffner syndrome), early and aggressive treatment may improve developmental and cognitive outcome. Further data, however, are required to confirm or refute these limited findings.
Epilepsy
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
Donald C. Barr, Andres M. Kanner
The severity of cognitive disturbances can range from very mild, which may only be detected with formal neuropsychological testing to very severe ID. These IDs may affect cognitive functions globally or involve very selective cognitive functions. Genetically mediated epileptic encephalopathies presenting as Lennox–Gastaut syndrome exemplify the former, while the latter is illustrated by the acquired aphasia of epilepsy of childhood (also known as Landau–Kleffner syndrome).20 In this syndrome, a de novo acquired receptive aphasia and auditory agnosia, which can evolve into a global aphasia, occurring between the ages of 3 and 8, is the most important clinical manifestation. Nonverbal cognitive functions may be preserved. They are associated with a typical nocturnal electrographic recording consisting of continuous bisynchronous spikes and slow waves in perisylvian regions during sleep. In contrast to the severe cognitive deficit, seizures may be subtle consisting of behavioral arrest, though secondarily GTC seizures can also occur. In 30% of cases, no clinical seizures are identified.
Convulsive disorders
Published in James Law, Alison Parkinson, Rashmin Tamhne, David Hall, Communication Difficulties in Childhood, 2017
It has been accepted for at least half a century that epilepsy in the young child can have an adverse effect on the child's speech and language development. The prevalence rate is unknown, although one specific disorder in which epilepsy and language disorder co-occur, the Landau±Kleffner syndrome (LKS), is generally considered to be rare. However, epilepsy is itself common in childhood with a prevalence of about 5 per 1000.2 It is recognised that there is an increasing prevalence of epilepsy in children with learning difficulties. Corbett3 gives figures of 6% for children with IQs in the range 50±70 and nearly 50% for those children with IQs below 20.
Treatment response in newly diagnosed epilepsy: a syndromic approach
Published in Neurological Research, 2022
Ali A. Asadi-Pooya, Mohsen Farazdaghi
The treatment strategy included monotherapy with an ASM in 408 (99.5%) patients; two patients with SGE received polytherapy at the first visit (one child with Landau-Kleffner syndrome received diazepam plus valproate and one over-weight patient with Lennox-Gastaut syndrome and frequent generalized tonic-clonic seizures and atypical absences received topiramate plus lamotrigine). The two most commonly prescribed ASMs were as follows: for focal epilepsies [Carbamazepine (148; 71%) and Lamotrigine (18; 9%)]; for IGEs [Valproate (86; 66%) and Lamotrigine (26; 20%)]; for SGE [Valproate (24; 49%) and Topiramate (17; 35%)]; and for unclassified epilepsies [Carbamazepine (13; 57%) and Lamotrigine (5; 22%)]; the difference in treatment strategy between the syndromes was significant (p = 0.0001; Pearson Chi-Square test).
A review of the pharmacotherapeutic considerations for managing epilepsy in people with autism
Published in Expert Opinion on Pharmacotherapy, 2022
Lance V Watkins, Maire O’Dwyer, Rohit Shankar
Epileptic encephalopathies in association with the autism phenotype are a more robustly researched area and treatment should be aligned to epilepsy syndrome. There is an evidence base to support treatment pathways and ASM classification choice when clinical epilepsy syndromes are identified. First-line, adjunctive treatment options, and ASMs to avoid for some of the epileptic encephalopathies do have a limited evidence base, but with limited success and high levels of treatment resistance. Lennox–Gastaut Syndrome (LGS): Sodium Valproate may be considered 1st line treatment, with Lamotrigine, clobazam, corticosteroids, and Levetiracetam as adjunctive ASMs.Continuous Spikes and Wave during Sleep (CSWS): Corticosteroids and clobazam may be considered first line with sodium valproate, ethosuximide, and sulthiame as adjunctive.Landau–Kleffner Syndrome (LKS): Corticosteroids may be considered first line, with valproate, clobazam, and sulthiame as adjunctive [30,47,48].
Central deafness: a review of past and current perspectives
Published in International Journal of Audiology, 2019
Frank E. Musiek, Gail D. Chermak, Barbara Cone
The auditory middle latency response (AMLR) is generated by the auditory thalamus (MGB), the fibre tract (auditory radiation) to the cortex from thalamus and the primary auditory cortex on Heschl’s gyrus. The AMLR is reflective of dendritic post-synaptic potentials and so is less subject to the neural synchrony demands of CAP or ABR. For this reason, it is possible to obtain an MLR in some cases when CAP or ABR is absent due to lack of neural synchrony as in multiple sclerosis or auditory neuropathy. Lesions of the primary auditory cortex will reduce or eliminate the AMLR, and thus, the absence of AMLR when CAP and ABR are present is consistent with CD. AMLR abnormality has also been observed in cases of word deafness following thalamic or putamen haemorrhage (Hayashi and Hayashi 2007), CD (Musiek et al. 2007) and in cerebrovascular disorder known as Moyamoya disease: the spontaneous, progressive occlusion of the circle of Willis (Setzen et al. 1999). The AMLR is absent, but ABR is spared (Musiek et al. 2007; Plyler and Harkrider 2013) in cases of Landau–Kleffner Syndrome, a form of temporal lobe epilepsy that is associated with a progressive aphasia including a severe form of auditory language comprehension disorder, CD and word deafness. Resolution or improvement in this condition is also accompanied by re-establishment of normal AMLRs (Wioland, Rudolf, and Metz-Lutz 2001).