1/24. Primary ciliary dyskinesia syndrome and primary generalised epilepsy. A child with primary ciliary dyskinesia syndrome (PCDS) developed primary generalised epilepsy (PGE). The PGE had characteristics of both childhood absence epilepsy (CAE) and juvenile myoclonic epilepsy (JME). The association of PCDS and PGE has not previously been recorded. ( info) |
| We describe two patients with psychogenic seizures of rare semiology. Both patients (a 23-year-old man and a 26-year-old woman) attended the emergency department because status epilepticus with myoclonic seizures had been diagnosed. seizures were documented with video-electroencephalography. Semiology of seizures were brief myoclonia of both arms resulting in a short elevation of both arms without impairment of consciousness. Ictal EEG registration was without abnormal finding. Psychiatric diagnostic assessment suggested a dissociative disorder and mild depression, respectively. During psychiatric treatment seizures occurred only rarely within a 3-5 months follow-up. One should be aware that juvenile myoclonic epilepsy may be mimicked by psychogenic seizures copyright 2001 BEA Trading Ltd. ( info) |
3/24. Myoclonic status epilepticus: video presentation. A young woman with juvenile myoclonic epilepsy had recurrent attacks of myoclonic status epilepticus related to a long history of limited compliance and irregular sleep. The diagnosis of this clinical pattern is based mainly on clinical description. A home video captured an attack. ( info) |
4/24. Suppression of cortical myoclonus by levetiracetam. A 16-year-old boy suffered severely disabling posthypoxic myoclonus. Neurophysiological investigation showed cortical but not reticular reflex myoclonus. Add-on therapy with levetiracetam significantly improved the patient's clinical condition, suppressed cortical myoclonus-associated spikes, and enabled further neurorehabilitation. ( info) |
5/24. The long-term course of seizure susceptibility in two patients with juvenile myoclonic epilepsy. We have observed epileptic seizures of juvenile myoclonic epilepsy (JME) to be surprisingly sensitive to higher mental activity. The purpose of the present study was to examine changes over time in seizure susceptibility in two patients with JME who we followed-up for over 20 years. During the period, they were repeatedly subjected to provocative cognitive tasking, that is, to 'neuropsychological EEG activation'. Tasks included reading, speaking, writing, written arithmetic, mental calculation, and spatial construction.During the first 15 years after the onset of symptoms, higher mental activities, mainly associated with use of the hands, i.e. writing, written calculation, and spatial construction, as well as physiological factors, such as sleep deprivation, awakening, and fatigue, precipitated the seizures. Generalized tonic-clonic and absence seizures but not myoclonic seizures disappeared almost completely after antiepileptic treatment. After age 30, the provocative effect of higher mental activities persisted, and the myoclonic seizures decreased under same drug regimen. These observations suggest that the pathophysiology of JME improves with time but persists for a long time, and that it is closely related to a neural network involved in higher mental activities mainly associated with use of the hands rather than in physiological factors emphasized in prior reports. ( info) |
6/24. Low dose sodium valproate in the treatment of juvenile myoclonic epilepsy. Fourteen patients with juvenile myoclonic epilepsy (JME) were treated with a single low dose of a sustained-release preparation of sodium valproate (VPA, 500 mg daily). The mean age of the onset of the low dose treatment was 19.2 years (range 14-26). Before this treatment, six patients had been treated with high dose VPA for a period of more than 2 years, three patients for 1 to 2 years, three patients less than 1 year and two patients initiated the treatment from the beginning with a low dose. The mean duration of low dose treatment is 35.6 months (range 25-59 months). (All patients are still under medication). Generalized tonic-clonic and absence seizures were controlled in all patients. Myoclonic jerks relapsed only in one patient, a young mother who was looking after her newly born baby and was deprived of sleep. No adverse reactions have been reported. We suggest that JME patients can effectively be treated with single low VPA dose (500 mg daily), while at the same time seizure precipitating factors, such as sleep deprivation and alcohol ingestion, should be avoided. ( info) |
7/24. Source analysis of polyspike and wave complexes in juvenile myoclonic epilepsy. We applied dipole modeling and brain distributed source analysis to find current sources comprising spikes and slow waves of polyspike and wave complexes (PSWC) in patients with juvenile myoclonic epilepsy (JME). The dipoles were localized in frontal, parietal and temporal lobes. The frontal dipoles were clustered in the frontal medial gyrus and fronto-orbital region. A midsagittal frontal current source was observed using brain distributed source analysis in all patients. When the slow wave was analyzed, multiple sources in different cortical regions were detected using dipole modeling and brain distributed analysis. These results show pre-frontal medial current sources corresponding to spikes and many diffuse sources in cortical regions corresponding to wave components of PSWC in patients with JME. ( info) |
8/24. Nonconvulsion status epilepticus in patients with juvenile myoclonic epilepsy: types and frequencies. Juvenile myoclonic epilepsy (JME) is an idiopathic, age-related generalized epileptic syndrome, featuring generalizedtonic-clonic and absence seizures as well as myoclonic jerks. Except for some case reports, little is known about type and frequency of nonconvulsive status epilepticus (NCSE) in adult patients with JME. In a retrospective study we therefore reviewed all patients with JME, who had been referred to our hospital between 1994 and 1999 for the occurrence of NCSE. Of the 69 patients with JME seen within these 6 years three women had typical absence status-one of them two times-and one woman had impulsive-petit-mal status. All four patients had a history of myoclonic, absence and generalized tonic-clonic seizures. Because of the extreme rarity of impulsive-petit-mal status the corresponding case history is presented in detail. According to our findings the prevalence of NCSE in JME can be estimated at 5.8%, the incidence at 1.2% per year with a clear preponderance of female gender. ( info) |
9/24. Myoclonic seizures as a main manifestation of Epstein-Barr virus infection. A 15-year-old girl with clinical and serologic evidence of active Epstein-Barr virus infection presented with a new onset of myoclonic jerks, confirmed on electroencephalogram (EEG) by generalized polyspike-and-wave epileptiform activity. family history for juvenile myoclonic epilepsy was negative, and brain magnetic resonance imaging was normal. The myoclonic jerks resolved spontaneously after 5 days, with gradual normalization of the EEG during the ensuing month and since then no resumption over the last 2 years. Previous reports have already linked Epstein-Barr virus infection to opsoclonus-myoclonus syndrome and hypsarrhythmia, but to the best of our knowledge, this is the first presentation of epileptic myoclonic jerks associated with acute Epstein-Barr virus infection. We suggest that acute myoclonic seizures with a transiently abnormal EEG and benign course can be associated with acute Epstein-Barr virus infection. ( info) |
10/24. Graphogenic epilepsy: a variant of language-induced epilepsy distinguished from reading- and praxis-induced epilepsy. We report a case of graphogenic epilepsy as a variant of language-induced epilepsy. A 25-year-old, right-handed woman had noticed for the previous 10 years that writing almost always provoked jerks or a jerking sensation in her right hand. No other triggers, including facets of language function such as reading and speaking, elicited any epileptic seizures. Ictal EEG demonstrated an initial left central abnormality. In our report, we stress that graphogenic seizures as an expression of language-induced epilepsy should not be confused with those that are manifestations of juvenile myoclonic epilepsy, and that writing and reading trigger different facets of motor manifestations. ( info) |