Motor cortical epilepsia partialis continua in a patient with a localized sensory cortical lesion

We describe a 33-year-old man with cyclosporine encephalopathy who showed continuous jerking in the left upper limb due to epilepsia partialis continua. Jerk-locked back averaging (JLA) of magnetoencephalogram disclosed a spike preceding the jerk localized at the hand motor area, whereas JLA of electroencephalogram revealed no premyoclonus spikes. The paired-pulse motor cortical transcranial magnetic stimulation revealed motor cortical hyperexcitability, while the paired-pulse somatosensory evoked potential showed no sensory cortical hyperexcitability. The brain MRI showed a high intensity lesion localized at the hand sensory area. These results suggest that the jerks were produced by discharges at the motor cortex probably disinhibited by the sensory cortical lesion.     

79例成人连续部分性癫痫患者临床分析

目的探讨成人连续部分性癫痫(EPC)的临床特征。方法回顾性分析79例成人EPC患者的临床特征,评价治疗结果。结果所有患者均表现为不伴意识障碍的运动性部分性癫痫持续状态。EPC平均持续时间6.3d(1~28d),最常累及部位为面臂部(33例,41.8%)。最常见病因为高血糖(35例,44.3%)。预后良好54例(68.4%),预后不良25例(31.6%)。所有病因为高血糖的患者预后良好,无需长期抗癫痫治疗。预后良好的相关因素为代谢性疾病或高血糖导致的EPC、单药治疗和EPC持续时间。结论 EPC最常见病因为高血糖,预后依赖于病因、使用的抗癫痫药物种类和EPC持续时间。     

Long-term follow-up for patients with nonprogressive epilepsia partialis continua in a single center in China

Epilepsia partialis continua (EPC) is a rare variant of epilepsy. Cases from China are rare. We present a case series of seven patients to analyze its clinical features, imagining findings, etiology, drug use, and long-term outcome in a single epilepsy center. We made assessments of drug effects twice (Stage I – when they left our hospital; Stage II in March 2017 – by telephone interviews to rate their long-term outcome). The mean duration of the second follow-up was 4.8 years. Of the seven patients, four patients characterized motor and sensory EPC and three motor EPC. Local distributions of EPC were: the left face (2 patients), right face (1 patient), left leg (3 patients), right leg and arm (1 patient). CT/MR was abnormal in four, normal in two, and not available in one patient. EEG abnormalities commonly consisted of spike-waves, sharp-waves (or) slow wave activity, and periodic lateralized epileptiform discharges. They were all nonprogressive EPC (encephalitis: 2; tumor: 2; head trauma: 1; and not found in 2 cases). In our observations, topiramate might be effective in patients with facial muscles continuous jerking, while carbamazepine in cases of limbs continuous myotonia. Our cases had favorable long-term outcome. Thus, our cases’ etiology differentiated from other regions. Some drugs used by referring to EPC distributions might help to control EPC and their outcome were usually favorable.     

Clinical course and variability of non-Rasmussen, nonstroke motor and sensory epilepsia partialis continua: a European survey and analysis of 65 cases

Purpose: To gain new insights into the clinical presentation, causes, treatment and prognosis of epilepsia partialis continua (EPC), and to develop hypotheses to be tested in a prospective investigation. Methods: In this retrospective multicenter study, all cases were included that fulfilled these criteria: constantly repeated fragments of epileptic seizures, with preserved consciousness, lasting ≥1 h and representing locally restricted motor or sensory epileptic activity. Single episodes were included when they lasted for a minimum of 1 day. EPC with Rasmussen syndrome and acute stroke were excluded. Key Findings: Three time courses with two subtypes each were distinguished, that is, EPC as a solitary event (de novo or in preexistent epilepsy); chronic repetitive nonprogressive EPC (with frequent or rare episodes); and chronic persistent nonprogressive EPC (primarily or evolving out of an episodic course). These were unrelated to etiologies (morphologic lesions 34%, inflammatory 29%, systemic disorders 9%, idiopathic 5%, unknown 23%). Precipitation and inhibition of seizures is a frequent feature of EPC. Levetiracetam and topiramate have improved the possibilities for pharmacotherapy. Topiramate seems to be particularly effective with dysontogenetic etiologies. Significance: The existence of several clearly distinct courses of nonprogressive EPC is a new finding. These distinctions will be further investigated in a prospective study with precise protocols for electroencephalography (EEG), imaging, and other studies. This should better establish the relation of motor and somatosensory EPC; further clarify the relations, pathogenesis, and significance of the different types and their etiologies; and possibly identify more semiologic variants. It should also provide more precise knowledge about therapy and modification of ictogenesis by external stimuli.     

Abdominal epilepsia partialis continua in neurocysticercosis

Epilepsia partialis continua (EPC) of abdominal muscles is a rare entity with variable clinical localization and aetiology. A 25‐year‐old man presented with sudden onset of intermittent focal myoclonic movements involving the abdominal muscles on the right side exclusively, lasting from 20 minutes to an hour. Brain MRI revealed a ring‐enhancing lesion, suggestive of cysticercal granuloma over the left precentral gyrus. The patient fulfilled the revised diagnostic criteria for definitive diagnosis of neurocysticercosis. EEG did not show focal abnormalities during the events. Episodes of EPC were controlled with difficulty using 600 mg oxcarbazepine, 200 mg lacosamide, and 2,000 mg levetiracetam. The patient received antiparasitic therapy with albendazole (15 mg/kg for two weeks) and oral dexamethasone (0.1 mg/kg) for two weeks which was then tapered. The involvement of the primary motor cortex during ictal propagation may account for this curious phenomenon. This is the first report of abdominal EPC in a patient with inflammatory granuloma as a result of neurocysticercosis.     

Anomalies of the cerebral cortex in a case of epilepsia partialis continua

Morphological and biochemical findings are described from the brain biopsy of an 11-year-old girl with intractable type II epilepsia partialis continua. Computerized tomography scan showed severe progressive central and cortical atrophy, mainly of the right hemisphere. Brain biopsy revealed microangiopathy of the cortex-penetrating arteries, patchy necrosis of the cortex, and small loose infiltrates of lymphocytic cells. Biochemical analysis showed normal pyruvate metabolism and citric acid cycle in gray and white matter.     

Epilepsia partialis continua possibly caused by cerebellar lesion

The cerebellum is known to have inhibitory effects on seizures. Nevertheless, cerebellar dysplastic lesions can be epileptogenic. We report a patient presenting with epilepsia partialis continua (EPC) following a hemorrhagic cerebellar lesion. Possible pathophysiological mechanisms are discussed.     

Alcohol‐responsive epilepsia partialis continua

Epilepsia partialis continua is typically associated with lesions of the cerebral cortex. However, subcortical lesions can also cause this condition. We present a patient with epilepsia partialis continua who failed to respond to conventional anticonvulsant medications but experienced a dramatic transient response to alcohol and a subsequent response to primidone. This pattern of sensitivity, which is similar to that seen in essential tremor, has led to the hypothesis that the two disorders are associated with pathology within the same anatomical network. A new pathophysiological model is thus proposed for the occurrence of epilepsia partialis continua in both cortical and subcortical disease processes. [Published with video sequences]     

Epilepsia partialis continua with dystonic hand movement in a patient with a malformation of cortical development.

Malformations of cortical development (MCD) with polymicrogyria and schizencephaly are due to abnormal cortical organization and usually manifest by intractable epilepsy and mental retardation. Epileptical activity is often hard to register and focal dystonia associated with such MCD has previously been described but without any metabolic imaging. We report here a 46-year-old man presenting with late-onset atypical abnormal movements of his left hand associated with right central region MCD. To demonstrate the involvement of an epileptical focus, we performed [(18)F]FDG-PET and fMRI both before and after a single dose of clobazam and diazepam, respectively. Characteristics of the abnormal hand movements, clinical response to the medication, and the result of the [(18)F]FDG-PET and fMRI investigations all favor the diagnosis of epilepsia partialis continua. We conclude that the dystonic movement is part of the partial seizure.     

Paradoxical lateralization of parasagittal spikes revealed by back averaging of EEG and MEG in a case with epilepsia partialis continua.

Our aim was to localize the generator site of parasagittal epileptiform discharges in a patient with epilepsia partialis continua (EPC) in the right leg. We examined a 32-year-old woman with EPC whose conventional EEG did not show any epileptic discharge. We performed the jerk-locked back averaging (JLA) of EEG and magnetoencephalography (MEG) to localize the dipole source of sharp transients. The myoclonic discharges in the right soleus muscle were used as a trigger pulse. JLA revealed consistent EEG and MEG sharp transients that coincided consistently and constantly preceded the myoclonic jerks. JLA of EEG demonstrated sharp waves paradoxically distributed over the vertex and right hemisphere. However, the estimated dipoles of MEG were localized in a restricted area in the primary leg motor area in the left hemisphere, which was closely located in the abnormal lesion on the brain MRI. JLA of MEG is considered to be a useful non-invasive method for localizing the epileptogenic area in EPC even when paradoxical lateralization of electroencephalographic discharges was noted.     

Epilepsia partialis continua responsive to neocortical electrical stimulation

Epilepsia partialis continua (EPC), defined as a syndrome of continuous focal jerking, is a rare form of focal status epilepticus that usually affects a distal limb, and when prolonged, can produce long‐lasting deficits in limb function. Substantial electrophysiologic evidence links the origin of EPC to the motor cortex; thus surgical resection carries the risk of significant handicap. We present two patients with focal, drug‐resistant EPC, who were admitted for intracranial video‐electroencephalography monitoring to elucidate the location of the epileptogenic focus and identification of eloquent motor cortex with functional mapping. In both cases, the focus resided at or near eloquent motor cortex and therefore precluded resective surgery. Chronic cortical stimulation delivered through subdural strips at the seizure focus (continuous stimulation at 60–130 Hz, 2–3 mA) resulted in >90% reduction in seizures and abolition of the EPC after a follow‐up of 22 months in both patients. Following permanent implantation of cortical stimulators, no adverse effects were noted. EPC restarted when intensity was reduced or batteries depleted. Battery replacement restored previous improvement. This two‐case report opens up avenues for the treatment of this debilitating condition.     

Non‐ketotic hyperglycaemia presenting as epilepsia partialis continua

Epilepsia partialis continua is a rare epileptic syndrome observed in patients with brain structural lesions and metabolic disorders. We report a patient with non‐ketotic hyperglycaemia presenting as epilepsia partialis continua with reversible focal brain lesions. An 83‐year‐old woman visited our hospital due to sudden and repetitive left facial twitching lasting for two days. Initial laboratory data revealed serum glucose, osmolality, and sodium levels of 631 mg/dl, 310 mOsm/l, and 130 mEq/l, respectively. EEG was normal. Brain MRI showed low signal changes in the right frontal subcortical area and high signal changes in the surrounding right frontal cortical areas on T2‐weighted, fluid‐attenuated inversion recovery, and diffusion‐weighted images. No seizures recurred after correcting blood glucose levels, hydrating the patient, and infusing valproate (900 mg/day). Follow‐up MRI, six months later, showed complete resolution of the signal changes in the right frontal cortical and subcortical areas and no clinical seizures. When considering non‐ketotic hyperglycaemia with epilepsia partialis continua in an elderly patient, early diagnosis and administration of the appropriate therapy is very important in order to decrease morbidity.     

Surgically intractable epilepsy associated with focal cortical dysplasia and congenital cutaneous hemangiomas

We describe a 6-month-old girl with medically intractable seizures, multiple congenital hemangiomas, and developmental delay. The patient underwent two surgical resections. Pathological findings at both the first and second resections were consistent with focal cortical dysplasia. The literature was reviewed on focal cortical dysplasia associated with cutaneous hemangiomas.     

Epilepsia partialis continua of the abdominal muscles: a detailed electrophysiological study of a case.

We describe the clinical and electrophysiological features of an elderly woman presenting with persistent clonic twitching of the abdominal muscles that were considered to represent a rare manifestation of epilepsia partialis continua due to a metastatic cortical lesion.     

Klinisch-neurophysiologische Analyse eines Falles von Epilepsia partialis continua (Kojewnikoff)

Zusammenfassung Es wurde ein Fall von Epilepsia partialis continua (Kojewni-koff) klinisch-neurophysiologisch analysiert. Der M. tibialis anterior rechts, der die rhythmischen Spontanentladungen zeigte, konnte durch Reizung des N. peronaeus rechts und links, des N. ulnaris rechts und links, sowie des N. trigeminus-facialis rechts und links über den Cortex aktiviert werden. Die gemittelten Muskelaktionspotentiale ließen Rückschlüsse auf die corticale Erregbarkeit zu. Je weiter der Reizort von dem Effector — in bezug auf das Körperschema — entfernt war, desto länger war auch die corticale Verarbeitungszeit. Im Doppelreizversuch zeigten sich unterschiedliche Hemmungsmechanismen in Abhängigkeit von der Plazierung des ersten und zweiten Reizes. Bei kontralateraler und ipsilateraler Reizung des N. peronaeus wurden über dem aktiven corticalen Areal weitgehend identische evoked-potentials abgeleitet, die sich in Amplitude und Sequenz von einem normalen evoked-potential deutlich unterschieden.Herrn Prof. Dr. P. Röttgen zum 65. Geburtstag.     

La relevancia del tiempo en la epilepsia parcial continua

IntroductionTiming is one of the most important modifiable prognostic factors in the management of status epilepticus. Epilepsia partialis continua (EPC) is a status epilepticus subtype of highly variable, occasionally prolonged, duration. The aim of this study was to analyze the relationship between EPC duration and outcomes.MethodsWe performed an observational prospective study of all patients with EPC admitted to our tertiary hospital between 1 September 2017 and 1 September 2018.ResultsThe sample included 10 patients, of whom 9 were women; median age was 74 years. The most frequent aetiology was cerebrovascular disease (n = 6). EPC onset occurred outside the hospital in 5 patients, with a median time to hospital admission of 4 hours. The median time to treatment onset for all patients was 12.3 hours. The median time from treatment onset to EPC control was 30 hours; time from treatment onset to EPC control showed a strong positive correlation with TT (Spearman's rho = 0.88). Six patients presented hyperglycaemia at onset; this was positively correlated with time from treatment onset to EPC control (rho = 0.71). All 6 patients with hyperglycaemia presented a brain injury explaining the EPC episode.ConclusionsDelays were observed in different phases of EPC management, which was related to longer duration of the episode. Glycaemia was also related to episode duration, probably acting as a triggering factor rather than as the aetiology.     

Epilepsia partialis continua and cortical motor control: insights into physiology

Motor epilepsia partialis continua is a widely described variant of simple focal motor status epilepticus. However, few studies have addressed associated pathophysiological anomalies that may help us understand the cortical organization, basic functioning and control of voluntary movement. We describe the clinical, video‐EEG and neuroimaging findings from two cases of motor epilepsia partialis continua that support the hypothesis of the coexistence of both classic body and complex motor map models in the cortical organization of voluntary movement in humans. [Published with video sequence]