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Hippocampal sclerosis (HS) has been recognized as the most commonly encountered pathological substrate of mesial temporal lobe epilepsy (MTLE). The pathological condition was initially described by early neuropathologists based on postmortem material. Studies in familial MTLE are also important for better understanding of the pathogenesis of HS. HS identified by magnetic resonance imaging (MRI) has been associated with poor medical control of seizures. Neurologic examination is usually normal except for facial asymmetry and memory deficits, which are material-specific for the side of ictal onset. Interictal electroencephalogram (EEG) findings in patients with MTLE typically include unilateral or bilaterally independent mesial temporal spikes, best seen with basal derivations. Treatment should start with a first-line antiepileptic drug (AED) in monotherapy, the dose of which is increased until seizure freedom or the occurrence of side effects such as tiredness, dizziness, diploplia, or gait disturbance.
This chapter focuses on the influence of multiple sclerosis (MS) on school and psychosocial aspects of life in children and adolescents, offering practical information about how to address potential problems. The MS has substantial impacts on the lives of those diagnosed with the disorder. Both attacks and progressive symptoms associated with MS can contribute to the net burden of disease. The Expanded Disability Status Scale (EDSS) is a tool that provides a nonlinear score based predominantly on the neurologic examination to quantify this burden. MS can affect a child's cognitive functioning, including reasoning, processing, attention span, information processing and retrieval, and other thinking abilities. Cognitive dysfunction can, in turn, impact the learning and memory and, consequently, the academic performance of children with MS. Signs of psychosocial distress in children and teens include unexplained medical complaints, poor compliance with treatment plans, school refusal, and risk-taking behaviors.
Paroxysmal dyskinesias are a heterogeneous group of disorders characterized by intermittent attacks of hyperkinetic involuntary movements without loss of consciousness. The paroxysmal movements may affect the arm or leg on one or both sides of the body, or all extremities. All kinds of paroxysmal dyskinesias may be symptomatic in origin. Twenty-two percent of one series of patients with paroxysmal movement disorders had underlying causative diseases. History and neurologic examination are the cornerstones on the way to the diagnosis of paroxysmal dyskinesias. The paroxysmal nature and predominantly short duration of the attacks, the aura symptoms and the response to even low doses of anticonvulsant drugs suggest that paroxysmal dyskinesias closely resemble epilepsy. Sandifer syndrome is a rare movement disorder in toddlers. During or immediately after feeding, affected children exhibit severe dystonic movements or postures of the head and neck. They often vomit and are malnourished.
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