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We report on the preliminary clinical and electrophysiological aspects of an in-patient possibly presenting epilepsia partialis continua (Koshevnikov). We discuss the different etiologies and emphasize on the possible idiopathic form in this case.

Background: The characteristic clinical feature of epilepsia partialis continua (EPC) is chronic focal myoclonus, usually involving the distal part of one extremity. A variety of pathogenetic factors have been implicated in EPC. In children, the most common cause is Rasmussen encephalitis; in adults, it is vascular disease or tumor involving the sensorimotor cortex. Epileptic seizures are a relatively common manifestation of central nervous system involvement in patients infected with human immunodeficiency virus (HIV), but, to our knowledge, isolated, chronic EPC has not been previously reported., Objective: To describe a case of typical EPC in a patient infected with HIV., Design and Setting: Case report from an epilepsy center., Patient: A 58-year-old man infected with HIV had continuous myoclonus that involved the right arm and was associated with intermittent motor seizures. The electroencephalographic findings were normal at the onset of the symptoms, but left central theta rhythm appeared later. Serial magnetic resonance imaging scans obtained over a 3-month period showed a progressively increasing left rolandic T2-weighted hypersignal. Histologic study of a stereotactic biopsy specimen demonstrated inflammation characterized by perivascular mononuclear cell infiltration. The only detectable cause was HIV infection. Immunocytochemical tests ruled out JC virus. Neuropsychological testing showed no evidence of cognitive impairment. An electroencephalographic-electromyographic "back-averaging" study showed a reproducible transient left biphasic complex preceding the bursts by about 30 milliseconds on the C3 and F3 electrodes, thus demonstrating that the myoclonus was of cortical origin. High-dose corticosteroid (prednisone, 100 mg/d) and anti-HIV- 1 therapy led to marked radiological and clinical improvement. Infection with HIV enhances the risk of seizures, but, to our knowledge, this is the first reported case of "inflammatory" EPC., Conclusions: The present case suggests that the possibility of central nervous system involvement by HIV-1 should be taken into account in the diagnostic workup of patients with EPC. This case also indicates that treatment can be effective.

Two patients with human immunodeficiency virus (HIV) type 1 infection presented new-onset epilepsia partialis continua (EPC) as an early manifestation of progressive multifocal leukoencephalopathy (PML). EPC occurred with no other seizures and was associated with negative radiographic and electrophysiological findings for several weeks. PML represents an increasingly recognized cause of new-onset seizures in both seropositive and seronegative patients, with no report of EPC as a presenting complaint.

Five patients had electroencephalograms (EEGs) that showed periodic epileptiform discharges in the midline (PEDIM); other than location, this activity has the same characteristics as periodic lateralized epileptiform discharges (PLEDs). All 5 patients had acute onset of partial motor seizures involving the lower extremity. The location of the PEDIM corresponded to the seizure type and focal neurologic deficits. All 5 patients had sustained a cerebrovascular insult, either old or new, and the PEDIM and seizures suggested an origin from the watershed area between the anterior, middle, and posterior cerebral arteries, involving predominantly the parasagittal region of the cerebral hemisphere.

Rasmussen syndrome (RS) is a severe and progressive focal epilepsy of unknown etiology that leads to deterioration of motor and cognitive function. We report a 14-year-old girl who developed epilepsia partialis continua involving the left hand, mild hemiparesis, and secondarily generalized seizures. RS was confirmed by brain biopsy. The patient has been treated with intravenous gamma globulin every 4 months for 46 months. The clinical course throughout this time has been distinctly atypical for RS, with no progression in motor or cognitive deficits and rare secondarily generalized seizures. Although the mechanism for action for gamma globulin in RS is not known, an immunomodulatory role has been postulated. Evidence of an immunologically mediated process in RS and clinical experience with a growing number of patients who benefit from immunomodulatory therapy suggest that a systematic study of the efficacy of gamma globulin in comparison with other forms of medical therapy is warranted.

Status epilepticus (SE) is one of the most common neurologic emergencies in children, adolescents, and young adults. SE may be due to acute neurologic conditions such as meningitis, encephalitis, or stroke, complicated febrile seizures, intractable epilepsy, degenerative diseases, intoxication, or may be the first manifestation of epilepsy. Initial treatment of convulsive SE is usually with an intravenous benzodiazepine (BZD) [lorazepam (LZP) or diazepam (DZP)], phenobarbital (PB), or phenytoin (PHT). LZP is less likely to cause respiratory depression than DZP and is therefore preferred. Sequelae and risk for recurrence of SE are primarily related to the underlying cause. Refractory SE (RSE) is most often symptomatic of an acute neurologic condition or neurodegenerative disease. Treatment for RSE is difficult, usually requiring intensive support of vital functions. Reported treatments for RSE include very high dose PB, continuous infusions of pentobarbital or BZDs (DZP, midazolam), lidocaine, inhalation anesthesia, and propofol. Outcome is related to underlying cause. Nonconvulsive SE may present as confusion or may mimic psychiatric illness. Response to BZDs is usually rapid but may not be sustained. Rapid initiation of oral or rectal valproate may be useful. Epilepsia partialis continua (EPC) is almost always due to an acute or chronic destructive lesion. Surgical treatment may be the only effective modality in some children with EPC. Acute treatment of breakthrough seizures and clusters of seizures at home with rectal BZDs (usually DZP, 0.2-0.5 mg/kg) may prevent progression to SE in some children and adolescents and reduce the need for visits to emergency facilities.

We reported a 31-year-old woman with epilepsia partialis continua (EPC) whose epileptic focus was delineated by PET. Neurologic examination revealed EPC in the face around the corner of her mouth, her thumb and forefinger on the left, slight exaggeration in the left biceps brachii reflex, and the digiti quiniti sign on the left. Laboratory tests showed pancytopenia, positive anti-nuclear and anti-DNA antibodies and decreased levels of serum complements, being consistent with systemic lupus erythematosus (SLE). Electroencephalogram showed paroxysmal discharges over the right frontoparietal region. PET with 2-18F-fluoro-2-deoxy-D glucose (FDG) clearly demonstrated a hypermetabolic area in the right frontoparietal cortex. T2-weighted images in MRI revealed reversible high signal intensity area in the right insula, postcentral gyrus and angular gyrus, while there were no abnormal findings in T1-weighted images with or without Gd-DTPA. There are only a few reports of EPC with the epileptic focus delineated by PET. Since a focal increase in glucose metabolism observed in them as well as in the present patient is a consistent indicator of the epileptic focus, the PET study is likely to provide significant information available not only for the diagnosis but also for the treatment of the condition. Finally, to our knowledge, this is the first patient with SLE presenting EPC. It is important to realize that vasculopathy associated with SLE may cause EPC and brain lesions with reversible high signal intensities shown by MRI.

The case of a young woman with EPCK is described in which neoplastic and vascular disorders were excluded. Supported by EEG and PET, EPCK was imputed to multifocal encephalitis notwithstanding serological and CSF negativity. Cerebral biopsy confirmed the inflammatory nature of the affection, although the etiologic agent was not identified. High dose intravenous immunoglobulin therapy was followed by the prompt disappearance of EPCK and the remission of the other neurological deficits.

We report the case of a young female patient with photosensitive primary epilepsy who presented partial motor status epilepticus provoked by the act of shutting the eyes. Clinical, EEG and neuroimage data are presented and discussed.

The authors describe a case of a 13-year-old adolescent. Being seven years old, he developed the epileptic syndrome associated with persistent focal and secondary generalized fits refractory to the treatment. Later the clinical picture became very suggestive of the syndrome Epilepsia partialis continua. In the course of the illness, the patient developed the diencephalic syndrome associated with arterial hypertension and early puberty, right-handed central hemiparesis, contractures, partial motor aphasia, and a decrease of the intellect. Computer-aided axial tomography revealed progressive development of atrophy of the left hemisphere and compensatory dilatation of the ventricles. Panangiography demonstrated hypoplasia of the left internal carotid artery.