Summary Surgery is widely accepted as an effective therapy for selected individuals with medically refractory epilepsy. Numerous studies in the past 20 years have reported seizure freedom for at least 1 year in 53–84% of patients after anteromesial temporal lobe resections for mesial temporal lobe sclerosis, in 66–100% of patients with dual pathology, in 36–76% of patients with localised neocortical epilepsy, and in 43–79% of patients after hemispherectomies. Reported rates for non-resective surgery have been less impressive in terms of seizure freedom; however, the benefit is more apparent when reported in terms of significant seizure reductions. In this Review, we consider the outcomes of surgery in adults and children with epilepsy and review studies of neurological and cognitive sequelae, psychiatric and behavioural outcomes, and overall health-related quality of life.

https://www.thelancet.com/pdfs/journals/laneur/PIIS1474-4422(08)70109-1.pdf

Outcomes of epilepsy surgery in adults and children

Myorhythmia is defined as repetitive, rhythmic, slow (1-4 Hz) movement affecting chiefly cranial and limb muscles. When occurring in the limbs it may be oscillatory and jerky, whereas oculo-masticatory myorhythmia, typically associated with Whipple's disease, is a slow, repetitive, often asymmetrical, facial and ocular movement. Thus, myorhythmia overlaps phenomenologically with tremor and segmental myoclonus. Although often present at rest, it must be differentiated from parkinsonian or dystonic tremor. Recognition of this movement disorder is important because it is usually associated with lesions involving the brainstem, thalamus, or other diencephalic structures with potentially treatable etiologies. In addition to Whipple's disease, myorhythmia has been described in patients with cerebrovascular disease, listeria encephalitis, anti-N-methyl-d-aspartate receptor encephalitis, steroid-responsive encephalopathy associated with autoimmune thyroiditis, multiple sclerosis, and other disorders. In addition to our own experience, we have systematically reviewed the medical literature, focusing on the phenomenology, pathophysiology, and etiology of this poorly recognized movement disorder. In this review, we aim to highlight the clinical features that differentiate myorhythmia from other movement disorders. Treatment should be directed against the underlying etiology.

Myorhythmia: phenomenology, etiology, and treatment.

Purpose Corpus callosum (CC) is the largest forebrain commissure. This review focuses on the significance of CC for seizure disorders, the role of CC in seizure spread and the surgical disruption of callosal fibers (callosotomy) for treatment of patients with drug-resistant epilepsy. Methods Personal experience/extensive literature review. Results Structural CC pathologies comprise developmental abnormalities, callosal involvement in identified disorders, transient imaging findings and microstructural changes. Epilepsies are reported in up to two thirds of patients with complete or partial CC agenesis (AgCC). However, AgCC per se is not indicative for seizure disorders. Moreover, additional malformations of cortical development (MCD) are causal. Microstructural CC abnormalities are detected by advanced imaging techniques, are part of diffuse white matter disturbances and are related to cognitive deficits. The etiological significance remains unexplained. However, they are also found in non-epileptic benign and transient disorders. In drug-resistant epilepsies with violent drops to the floor ("drop seizures") callosotomy may be beneficial in seizure reduction. Since the EEG after callosotomy exhibits a single seizure focus in up to 50% of patients, consecutive resective surgical methods might be successful. Conclusion CC is part of cerebral white matter and anomalies cannot act per se as seizure onset zone. Imaging techniques demonstrate additional lesions in patients with epilepsies. CC is the major pathway for seizure generalization. Therefore, callosotomy is used to prevent generalized drop seizures.

Corpus callosum and epilepsies

BACKGROUND Medically intractable epilepsy involving drop attacks can be difficult to manage and negatively affect quality of life. Most studies investigating the effect of corpus callosotomy (CC) on seizures have been limited, focusing on the pediatric population or drop seizures alone, with little attention to other factors influencing seizure outcome. OBJECTIVE To assess seizure outcomes after CC in adults and children. METHODS Retrospective analysis was performed on all patients who underwent CC (anterior two thirds, 1- or 2-stage complete) at our institution between 1990 and 2011. Change in frequency after CC was assessed for drop seizures and other seizure types. Multiple factors were evaluated for impact on seizure outcome. RESULTS Fifty patients met inclusion criteria. The median age was 1.5 years at seizure onset and 17 years at time of surgery. Anterior two-thirds CC was performed in 28 patients, 1-stage complete in 17, and 2-stage complete in 5. All 3 groups experienced a significant decrease in drop seizures (P < .001, P < .001, and P = .020, respectively), with 40% experiencing complete resolution, and 64% dropping at least 1 frequency category. Other seizure types significantly decreased in anterior two-thirds CC and 1-stage complete (P = .0035, P = .001, respectively). Younger age at surgery correlated with better seizure outcomes (P = .043). CONCLUSION CC for medically refractory generalizing epilepsy is effective for both drop seizures and other seizure types. CC should be considered soon after a patient has been deemed medically refractory because earlier age at surgery results in lower risk and better outcome.

Seizure outcomes after corpus callosotomy for drop attacks.

Spinal-generated movement disorders (SGMDs) include spinal segmental myoclonus, propriospinal myoclonus, orthostatic tremor, secondary paroxysmal dyskinesias, stiff person syndrome and its variants, movements in brain death, and painful legs-moving toes syndrome. In this paper, we review the relevant anatomy and physiology of SGMDs, characterize and demonstrate their clinical features, and present a practical approach to the diagnosis and management of these unusual disorders.

/pdf/spinal-generated-movement-disorders-a-clinical-review-4woy9ppv0k.pdf

Spinal-generated movement disorders: a clinical review

Objective: The purpose of this study was to evaluate seizure outcome in children with intractable secondary generalized epilepsy without a resectable focus who underwent complete co

Corpus callosotomy for treatment of pediatric epilepsy in the modern era

We report an 18-year-old man with paroxysmal jerking movements of the left arm since age 7 years. These were invariably precipitated by startle or sudden movements. He was subsequently diagnosed with a cervical cord anaplastic astrocytoma on MRI. We could not identify previous reports of paroxysmal myoclonus secondary to a spinal cord neoplasm. We have coined the term Paroxysmal Kinesigenic Segmental Myoclonus to describe this entity.

Paroxysmal Kinesigenic Segmental Myoclonus due to a spinal cord glioma.

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Corpus callosotomy for treatment of pediatric epilepsy in the modern era

Paroxysmal Kinesigenic Segmental Myoclonus due to a spinal cord glioma.