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Hyperkinetic Movement Disorders
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
Morales-Briceno Hugo, Victor S.C. Fung, Annu Aggarwal, Philip Thompson
Fragments of epilepsy: Epilepsia partialis continua.Photosensitive myoclonus.Jeavons' syndrome (eyelid myoclonia, photosensitivity, and absence seizures).Myoclonic absence in petit mal.Angelman's syndrome (happy puppet syndrome).Familial cortical tremor and epilepsy.
Measles and its neurological complications
Published in Avindra Nath, Joseph R. Berger, Clinical Neurovirology, 2020
Benedikt Weissbrich, Jürgen Schneider-Schaulies
Typically the neurological disease begins with seizures that are often severe and refractory to treatment. They may take the form of epilepsia partialis continua. Only one patient has been reported to have had fever. As the disease evolves the patients develop neurological deficits such as hemiparesis, ataxia, aphasia, and visual symptoms. Eventually all patients exhibit altered levels of consciousness, which can progress to stupor and coma. The course of the disease is usually rapid and death follows in 75% of the patients within days to a few months. Some patients die of the underlying disease. The surviving patients are left with permanent neurological deficits [159].
Mitochondrial Dysfunction and Epilepsies
Published in Shamim I. Ahmad, Handbook of Mitochondrial Dysfunction, 2019
Bindu Parayil Sankaran, Arun B. Taly
Most common seizure type in juvenile and adult-onset patients is focal motor seizures-particularly affecting the upper limbs, neck or proximal muscles or trunk which evolves into bilateral convulsive seizures. There is an occipital predilection in the early stages, characterized by visual disturbances including coloured light scotoma, visual blurring, ictal visual loss and oculo clonus. (Engelsen et al. 2008) Status epilepticus and epilepsia partialis continua are common. The onset is frequently explosive and fulminant. Additional neurological features include migraine neuropathy ataxia and chronic progressive external ophthalmoplegia. The specific EEG findings include rhythmic high amplitude delta with superimposed spikes and polyspikes (RHODES) (Wolf et al. 2009).
Pharmacotherapeutic management of epilepsy in MERRF syndrome
Published in Expert Opinion on Pharmacotherapy, 2019
MERRF is one of the most frequent causes of myoclonic epilepsy [66]. Thus, the most frequent seizure types in MERRF are focal or generalized myoclonic seizures (Table 1) [1]. Myoclonus in MERRF may be constant or intermittent, photosensitive, or enhanced by action (writing, eating) [67]. Myoclonic jerks in MERRF may or may not correlate with spike or polyspike activity on electroencephalography (EEG), and there may be suppression of epileptic activity upon eye-opening [67]. Myocloni in MERRF may not only result from epileptic activity but also from cerebellar dysfunction [9] or spinal cord affection. Other seizure types found in MERRF patients include focal atonic or focal clonic seizures, generalized tonic-clonic, generalized atonic, or generalized myoclonic-atonic seizures, or typical absences [68] or myoclonic absences (Table 2) [69–73]. In a single patient from Japan carrying the m.8344A>G variant, astatic seizures have been reported [68]. Not only single seizures but also epileptic states have been described in MERRF patients. In a study of 24 Italian MERRF patients, 2 had a status epilepticus [9]. Unfortunately, no detailed description of the status was provided. Status epilepticus has been also reported in a patient from Thailand [70]. In one patient from India, epilepsia partialis continua has been described [74].
An update on Alpers-Huttenlocher syndrome: pathophysiology of disease and rational treatment designs
Published in Expert Opinion on Orphan Drugs, 2018
Alpers-Huttenlocher syndrome (AHS) is an autosomal recessive disorder of mitochondrial DNA (mtDNA) maintenance. This syndrome is characterized by a triad of medically refractory seizures, psychomotor regression, and hepatopathy [1,2]. The typical clinical presentation occurs in a normally developing child or young adult with onset of seizures, which can be explosive and may present with status epilepticus [3]. Seizure semiology of early seizures is mostly focal motor seizures and/or myoclonus [4,5]. Seizure onset often heralds the progression of disease. As the disease progresses, most patients have repeated episodes of status epilepticus and epilepsy partialis continua (EPC). Over time, seizures become more refractory to medical management with myoclonus and myoclonic seizures becoming more prominent. Devastating encephalopathy becomes apparent during the progression to medically intractable seizures [1,6]. Liver involvement is variable during the course of the syndrome, some patients’ hepatopathy occurs early in the disease and in others, it is not present until late or even not at all at the time of death [1,5]. The reason for the variability in the time course of liver involvement is not fully understood. Early death due to status epilepticus or other events such as respiratory failure may truncate the full natural history of the disease and liver involvement (see discussions below).
Takotsubo cardiomyopathy following a simple partial seizure
Published in Baylor University Medical Center Proceedings, 2022
Marc Saouma, Chadi Allam, Michel Chedid El Helou, Zeina Kadri, Georges Badaoui
Stöllberger et al screened the literature for all SIC cases occurring in the setting of seizure. In 75% of cases, there was an underlying neurologic disease or a direct cause of epilepsy, and generalized tonic-clonic and complex partial were the most commonly involved seizure types, with no report of SIC following a simple partial seizure.3 This unique case reports the first SIC following a simple partial seizure in a patient with a prior history of partially resected meningiomatosis. The long seizure duration in our patient, which defines epilepsia partialis continua, may have caused SIC.