Epilepsia partialis continua in Creutzfeldt-Jakob disease

OBJECTIVES: We describe a patient with Creutzfeldt-Jakob disease (CJD) with epilepsia partialis continua (EPC) and complex partial seizures. MATERIAL, METHODS AND RESULTS: The patient presented with semi-rhythmic jerking movements of the right upper extremity. Serial EEG findings showed progressive changes with initial periodic lateralizing epileptiform discharges (PLEDs) on the left hemisphere which evolved into more generalized periodic sharp wave complexes (PSWCs) at later stage. Video-EEG monitoring recorded complex partial seizures originating from the left hemisphere in addition to EPC. CONCLUSIONS: A diagnosis of CJD should be considered when a rapidly progressive dementia is accompanied by abnormal movements. EPC, although rare, may present as an initial manifestation of CJD.     

Epilepsia partialis continua in multiple sclerosis

Epilepsia partialis continua (EPC) in multiple sclerosis is extremely rare. We report a case of a patient with clinically definite multiple sclerosis who developed EPC. Magnetic resonance imaging (MRI) depicted high signal in subcortical white matter and basal ganglia. A combination of lesions may be important in the generation of EPC.     

Epilepsia partialis continua studied by PET

We report an [18F]fluordeoxyglucose (FDG)-PET study performed in an 11-year-old girl with a 5-month history of epilepsia partialis continua (epc). Visual inspection of PET images showed a hypermetabolic focus in the right central cortex and in the ipsilateral thalamus, which was confirmed by the absolute values of regional cerebral glucose metabolism (rCMRGlu). The thalamic hypermetabolism provides evidence for an involvement of thalamic nuclei in this ictally epileptic process. The scalp EEG revealed a theta-delta and sharp wave focus in the right Rolandic cortex at the same location as the hypermetabolic zone seen in PET. Simultaneously recorded EMG of the left tibialis anterior muscle showed regular jerks, time-locked to the sharp waves at the right central region, and myoclonic 'storms' during focal motor seizures. The results of the brain biopsy and the child's clinical course led us to a diagnosis of 'chronic encephalitis' of Rasmussen.     

Epilepsia partialis continua following pertussis infection

Clinical, electrophysiological and CT findings of a patient with epilepsia partialis continua following pertussis infection are presented. The EEG showed lateralized abnormal slow waves and CT revealed global atrophy around the Sylvian fissure. The findings favor the primary role of the cerebral cortex in EPC.     

Epilepsia partialis continua associated with levamisole

Epilepsia partialis continua is defined as a spontaneous regular or irregular clonic muscular twitching affecting a limited part of the body, occurring for a minimum of 1 hour and recurring at intervals of less than 10 seconds. Levamisole is used as an immunomodulating medication in patients with recurrent aphthous ulcers. Evidence suggests that it can induce multifocal inflammatory leukoencephalopathy. We describe the clinical neuroimaging and ictal electroencephalographic findings in an adolescent with epilepsia partialis continua caused by the administration of levamisole with cortical and subcortical lesions. To our knowledge, this is the first report that describes the association of epilepsia partialis continua cortical lesions detected by magnetic resonance imaging and levamisole that were not previously described.     

Epilepsia partialis continua and neuronal migration anomalies.

Neuronal migration anomalies commonly cause seizures that are partial in type and generally refractory to medical treatment. Epilepsia partialis continua (EPC), an unusual form of epilepsy commonly related to acute damage of the cerebral cortex or to a chronic lesion, has never been described in a patient with neuronal migration anomalies. In 50 children with epilepsy due to neuronal migration anomalies, we observed two cases of EPC. These two children had unilateral neuronal migration abnormalities with partial seizures other than EPC and contralateral hemiparesis. Epilepsia partialis continua appeared two to three years after the onset of partial attacks and was accompanied by a worsening of the children's previous hemiparesis. Although a rare seizure manifestation in children with neuronal migration anomalies, when it does appear, EPC can aggravate the clinical neurological condition and should always be investigated for in these cases. Because its clinical appearance is often subtle, as in these two children, EPC may easily remain undiagnosed.     

Epilepsia partialis continua following metrizamide cisternography

Evidence indicating cell membrane fusion among cells and their processes where subacute sclerosing panencephalitis (SSPE) virus is present is shown in an ultrastructural study of a brain biopsy. Virus budding from plasma membrane was not seen. These rare findings reinforce the hypothesis of cell-to-cell transmission as the most likely mechanism of SSPE virus infection. The presence of fused membranes between cell processes and cell soma probably explains the rapid spread of this virus throughout the nervous system.     

Epilepsia partialis continua: semiology and differential diagnoses.

Epilepsia partialis continua (EPC) is a rare form of focal status epilepticus. It may have vascular, immune-mediated, neoplastic or metabolic-toxic causes. The origin of EPC has been linked with the motor cortex. This has been solidly supported by sophisticated electrophysiological studies. Here, a series of video sequences from patients with EPC (due to Rasmussen encephalitis, early-stage multiple sclerosis, and steroid responsive encephalopathy with autoimmune thyroiditis), and other cases with repetitive myoclonic jerks or movement disorders (myoclonic epilepsy associated with ragged-red fibers, Jacksonian march, myoclonic seizures in other types of frontal lobe or idiopathic generalized epilepsies, and different types of tremor) is presented. [Published with video sequences].     

Epilepsia partialis continua. A review of 32 cases.

Clinical, EEG, and autopsy findings were studied in 32 patients with epilepsia partialis continua. The seizures occurred at different ages and with diverse diseases of the brain. Facial and distal limb muscles were preferentially involved. Often, seizures suggested multifocal origin within a limited cerebral region, with low-amplitude, irregular, asynchronous contractions that could resemble other movement disorders. Twitching varied in rate, rhythm, intensity, and territorial extent continued sometimes for decades and was poorly responsive to therapy. Focal EEG abnormalities commonly consisted of discrete spikes, sharp waves, or slow-wave activity. Autopsy findings in eight patients showed consistent involvement of the motor cortex or closely adjacent areas. Clinical, EEG, and pathologic data favor a cortical origin of epilepsia partialis continua.     

Eneephalographische Befunde bei Epilepsia partialis continua Kojewnikoff

Zusammenfassung Bei den in der hiesigen Klinik beobachteten Kranken mit sog.Epilepsia partialis continua Kojewnikoff waren fast ausnahmslos erhebliche encephalographische Veränderungen nachzuweisen, und zwar vorwiegend einseitige Ventrikelverziehungen und vermehrte Oberflächenzeichnung auf der gleichen Seite. Die Encephalogramme lassen Hirnschrumpfungen annehmen, die sowohl die Rinde als auch subcorticale Gebiete betreffen. Das Zentrum dieser Hirnschrumpfungen befindet sich etwa in der Gegend des Thalamus opticus. Diese Peststellung macht es im Verein mit den klinischen Beobachtungen wahrscheinlich, daß dasKojewnikoffsche Syndrom, wie zuletztKautzky undStengel vermuteten, auf einer Schädigung vonBinden- und subcorticalen Zentren beruht. Als Ursache der encephalographischen Veränderungen kommen früher durchgemachte entzündliche Hirnschädigungen in Betracht, ohne daß das akute Stadium eines entzündlichen Hirnleidens immer nachweisbar war.Auf Grund eines Falles mit encephalographisch nachgewiesener Hirnschwellung einer Großhirnhälfte, der bei der Beobachtungvor Ausbruch der K.E. nur eine einseitige Hirnschrumpfung aufwies, ist anzunehmen, daß der Hirnschwellung, anscheinend vorwiegend bei älteren Kranken, eine besondere Bedeutung für die Auslösung der K.E. beizumessen ist.     

Epilepsia partialis continua responsive to intravenous levetiracetam

We report on a case of epilepsia partialis continua with rapid response to intravenous bolus administration of levetiracetam. A 60-year-old woman presented with continuous jerking of the right foot and hallux persisting for more than two days. She had a 9-year history of epilepsy due to a left temporoparietal oligodendroglioma with occasional focal seizures clinically presenting as speech arrest, which was treated with levetiracetam and oxcarbazepine administered orally. After hospital admission, the twitching of the foot and toe was refractory to add-on treatment with lorazepam and diazepam but stopped within 15 min after intravenous bolus administration of 2000 mg levetiracetam. This observation suggests that intravenous bolus administration of levetiracetam may be an effective therapeutic option in epilepsia partialis continua.     

Epilepsia partialis continua successfully treated with levetiracetam

Journal of Neurology -     

Epilepsia partialis continua of the abdominal muscles due to cerebrovascular disease

Two elderly men, with previous history of cerebrovascular disease, were admitted to the emergency department due to focal motor status epilepticus with persistent myoclonic jerks of one side of the body. In both cases, the clinical picture evolved into a unilateral and isolated arrhythmic myoclonus of the abdominal muscles with preserved consciousness. These involuntary movements resolved with antiepileptic drugs. Although cerebrovascular disease is one of the most common causes of epilepsia partialis continua, reported cases in the literature with predominant abdominal involvement have a different aetiology. The neuroimaging and electroencephalographic findings showed a wide spectrum of different localizations and aetiologies associated with this particular type of epileptic seizure. Indeed, the pathophysiology of focal motor seizures involving the abdominal muscles is still a matter of discussion. In our second case, we present a patient with epilepsia partialis continua of the abdominal wall with an occipital focus, which, to the best of our knowledge, has not been previously reported. [Published with video sequences]     

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.