Cases reported "Epilepsy, Rolandic"

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1/13. Idiopathic rolandic epilepsy with "interictal" facial myoclonia and oromotor deficit: a longitudinal EEG and PET study.

    PURPOSE: The prognosis of benign epilepsy with centrotemporal spikes (BECTS) is always favorable as far as the epilepsy is concerned. However, some data suggest that affected children may be at risk for minor cognitive impairment. We report here the longitudinal study of a young girl demonstrating that BECTS also may be associated with severe motor disturbances. methods: BECTS (rare left oromotor seizures, right rolandic spike-waves activated during sleep) started at the age of 3 years 6 months in a girl with normal initial psychomotor development. Her clinical, neuropsychological, and EEG status was assessed every 3-6 months. Regional cerebral glucose metabolism was measured by using the [18F]fluorodeoxyglucose-positron emission tomography (FDG-PET) method. RESULTS: Between the age of 5 and 6 years, the girl had (a) increased seizure frequency; (b) brief perioral and palpebral myoclonic jerks, concomitant with the spike component of interictal spike-waves, and (c) persistent but fluctuating oromotor deficits (drooling, dysarthria, dysphagia). The EEG showed a marked increase in abundance and amplitude of wake and sleep interictal abnormalities, which became bilateral. Awake FDG-PET revealed a bilateral increase of glucose metabolism in opercular regions. A complete and definitive EEG and clinical remission occurred at age 5 years 11 months and has persisted since (present age, 7 years 9 months). CONCLUSIONS: This case confirms that during BECTS, epileptiform dysfunctions within rolandic areas may induce "interictal" positive or negative oromotor symptoms, independent of classic seizures.
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keywords = status
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2/13. Paradoxic reaction to lamotrigine in a child with benign focal epilepsy of childhood with centrotemporal spikes.

    A girl aged 5 years developed nocturnal seizures (mouth twitching, salivation, anarthria, with right arm jerking and occasional secondary generalisation), with frequent focal sharp waves over the left centrotemporal region in her EEG, suggesting benign childhood epilepsy with centrotemporal spikes (BECTS). seizures became diurnal and frequent, not modified by carbamazepine (CBZ) or valproate (VPA) but responding to VPA and lamotrigine (LTG) with recommended dosage schedules for this combination. Her school performance then deteriorated insidiously, with poor memory and concentration, clumsiness, stuttering, and emotional lability. After 4 months, new episodes, < or =10 per day, occurred. These lasted a few seconds; she stared into space, her jaw dropped, her head dropped to the right, and her eyelids flickered. She usually maintained awareness. Attacks were often provoked by blowing or sneezing. Ictal EEG showed anterior-predominant 3/s sharp-slow wave complexes lasting < or =8 s, with bilateral rolandic discharges interictally. Withdrawal of LTG resulted in rapid improvement in cognitive function and gradual remission of the new attacks. CONCLUSIONS: This appears to be a paradoxic reaction to LTG in the setting of BECTS.
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ranking = 0.0033648444569925
keywords = complex
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3/13. epilepsy and perisylvian lipoma/cortical dysplasia complex.

    Intracranial lipomas are congenital malformations composed of mature adipocytes. They are usually located in the midline, particularly in the pericallosal region, a hemispheric location accounting for only 3 to 7% of cases. review of the literature found 21 previous cases of hemispheric lipoma. Although hemispheric cerebral lipomas are rare, association with epilepsy appears to be frequent. We have recently studied two patients in whom epilepsy was the first clinical manifestation of hemispheric cerebral lipoma in the sylvian region. The patients presented with simple motor partial seizures as the first manifestation of the lesion. Neurological examination was normal. MRI disclosed in both cases a lesion involving the sylvian fissure with characteristics of the lipid signal. MRI also demonstrated abnormalities involving the cerebral cortex in the vicinity of the lesion (pachygyria-like aspect). Partial excision of the lesion was achieved in one patient but was followed by a worsening of seizures and neurological condition (hemiparesis). According to the literature, the prognosis for epilepsy in patients with hemispheric lipoma appears good. Several other arguments support non-surgical management: the lesion is benign and can be identified with a high degree of certainty by imaging; surgery is technically difficult due to adherence to adjacent vascular and cerebral structures and hypervascularity; location near functional brain tissue increases the risk of postoperative sequelae. In addition, mechanisms of epilepsy probably involve vascular and cortical dysplasic abnormalities. In consideration of the complexity of the lesion, hemispheric lipomas are more appropriately classified with localized cortical malformations rather than as simple extracerebral malformations.
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ranking = 0.027577108912993
keywords = simple, complex
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4/13. Autosomal dominant rolandic epilepsy with speech dyspraxia.

    Autosomal Dominant Rolandic epilepsy with speech Dyspraxia (ADRESD) is a rare disorder which highlights the relationship between Benign Rolandic epilepsy (BRE) and speech and language disorders. Subtle speech and language disorders have recently been well characterised in BRE. ADRESD is associated with long term, more severe speech and language difficulties. The time course of rolandic epilepsy in ADRESD is typical of that of BRE. ADRESD is inherited in an autosomal dominant manner with anticipation. It is postulated that the anticipation may be due to an, as yet unidentified, triplet repeat expansion in a gene for rolandic epilepsy. BRE follows complex inheritance but it is possible that ADRESD may hold some valuable clues to the pathogenesis of BRE.
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ranking = 0.0033648444569925
keywords = complex
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5/13. Seizure's outcome after cortical resections including the face and tongue rolandic areas in patients with refractory epilepsy and normal MRI submitted to subdural grids' implantation.

    PURPOSE: To study the seizure's outcome in patients with refractory epilepsy and normal MRI submitted to resections including the rolandic cortex. methods: Four adult patients were studied. All patients had motor or somatosensory simple partial seizures and normal MRI and were submitted to subdural grids' implantation with extensive coverage of the cortical convexity (1 in the non-dominant and 3 in the dominant hemisphere). RESULTS: ECoG was able to define focal areas of seizures' onset in every patient. All patients were submitted to resection of the face and tongue motor and sensitive cortex; two patients had resections including the perirolandic cortex and 2 had additional cortical removals. Three patients are seizures' free and one had a greater then 90% reduction in seizure frequency. CONCLUSION: Resections including the face and tongue rolandic cortex can be safely performed even within the dominant hemisphere.
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ranking = 0.0053764433140154
keywords = simple
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6/13. Atypical evolution of Panayiotopoulos syndrome: a case report.

    Panayiotopoulos syndrome is a relatively common condition with susceptibility to early onset benign childhood seizures, which manifests primarily with autonomic and mainly emetic symptoms. It predominantly affects children of 3-6 years of age (13% of those with one or more non-febrile seizures). EEG shows great variability, with occipital, extra-occipital spikes or brief generalised discharges alone or in combination; it may also be consistently normal. Occipital spikes do not occur in one third of children. Despite the high prevalence of autonomic status epilepticus, the prognosis of Panayiotopoulos syndrome is usually excellent. Remission usually occurs within 1-2 years from onset, one third have a single seizure but 5-10% may have more than 10 seizures or a more prolonged course. Atypical evolutions with absences, atonic seizures and intellectual deterioration are exceptional; only two cases have been previously reported. We present a girl who initially had a prolonged autonomic status epilepticus typical of Panayiotopoulos syndrome, followed by seizures, with concurrent symptoms of Rolandic epilepsy. She then had an atypical evolution with atypical absences, absence status epilepticus, atonic seizures and mild impairment of scholastic performance. The case emphasises the close links between Panayiotopoulos syndrome and Rolandic epilepsy, both of which probably represent different clinical phenotypes of a maturational-related benign childhood seizure susceptibility syndrome [published with videosequences].
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ranking = 475.38186535213
keywords = status epilepticus, epilepticus, status
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7/13. Partial status epilepticus in benign childhood epilepsy with centrotemporal spikes: are independent right and left seizures a risk factor?

    PURPOSE: To describe an association between continuous simple partial seizures and independent right and left partial seizures in children with benign childhood epilepsy with centrotemporal spikes (BCECTS). methods: Three children with BCECTS and episodes of continuous simple partial seizures are described. RESULTS: All three children had a history of typical rolandic seizures occurring on the right and left sides of the body on different occasions. CONCLUSIONS: The occurrence of independent right and left rolandic seizures in children with BCECTS may be a predisposing factor for the development of partial status epilepticus.
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ranking = 792.31386180684
keywords = status epilepticus, epilepticus, status, simple
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8/13. epilepsy with reversible bulbar dysfunction.

    In patients with focal epilepsy, focal neurological dysfunction can occur due to status epilepticus and also as a post-ictal phenomenon. Bulbar dysfunction as evident by drooling, dysarthria, swallowing difficulties, and palatal-glossalpharyngeal weakness has been reported in conjunction with epilepsy. This is non-progressive and is correlated in its severity with the frequency of seizures. Accompanying EEG discharges are often localized to rolandic areas that cortically represent oral movements and salivation. We report a 6-year-old male and a 6 1/2-year-old female with progressive bulbar dysfunction resulting from epilepsy. Ictal EEGs in patient 1 did not confirm a diagnosis of epilepsy. With no evidence of a cortical or brainstem focus from EEG or MRI, it is very difficult to explain the mechanism of bulbar dysfunction. The complete restoration of bulbar function after treatment with antiepileptic drugs demonstrates the need to consider epilepsy in similar clinical situations.
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ranking = 158.46062178404
keywords = status epilepticus, epilepticus, status
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9/13. Electrophysiological characterization of spontaneous and carbamazepine-induced epileptic negative myoclonus in benign childhood epilepsy with centro-temporal spikes.

    OBJECTIVE: Epileptic negative myoclonus (ENM), a transient muscular atonic phenomenon time-locked to epileptiform EEG abnormalities, is often observed in children with benign childhood epilepsy with centro-temporal spikes (BECTS). In some, for unknown reasons, ENM can be worsened by carbamazepine (CBZ). We describe two children aged 11 and 15 years, in whom CBZ precipitated seizure worsening and ENM. We investigated the morphological and topographic features of the EEG abnormalities while on CBZ and after CBZ withdrawal and compared them with those from 9 children with classical BECTS. The aim of the study was to identify possible electrophysiological specificities in patients who eventually develop ENM during CBZ treatment. methods: The characterization of EEG abnormalities, related (R) and unrelated to ENM (U), in patients with ENM and rolandic discharges (RD) and in matched controls with BECTS was performed based on polygraphic digital EEG recordings. Off-line time-domain analysis included correlation coefficient between EEG and EMG channels, quantitative analysis on ENM, and topographic analysis on spike-and-wave complexes. Z-score test and paired t test were used when appropriate for statistical analysis on R, U and RD. RESULTS: Recordings in both children with BECTS and ENM while on CBZ showed frequent R discharges (mean interval between R=19.89 /-9.4 s in patient 1; 2.16 /-1.2 s in patient 2). Withdrawal of CBZ produced abatement of R (no R recorded in patient 1; 5.69 /-7.1 s in patient 2) and reduction of the slow wave component of R (P<0.01). Morphology and topography of R and RD differed in field distribution, amplitude (P<0.01) and duration (P<0.01) of the slow wave component. RD and U did not show a significantly different morphology and field distribution. CONCLUSIONS: Our findings suggest that an increased cortical inhibition could be the electrophysiological correlate of CBZ-induced ENM. If confirmed on a larger series, the presence of spike-wave (rather than sharp waves) discharges in children with BECTS might be used as an electrophysiological predictor of an abnormal response to CBZ.
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ranking = 0.0033648444569925
keywords = complex
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10/13. speech and language deterioration in benign rolandic epilepsy.

    A 5-year-old boy presented with typical clinical and electrophysiologic features of benign rolandic epilepsy. His neurodevelopment, language, and behavior prior to the onset of epilepsy were appropriately normal. He demonstrated marked deterioration of language and cognitive function during the course to a mild and then a moderate disability range. Serial sleep electroencephalographic recordings initially showed continuous and bilateral rolandic discharges with evolution to localized left rolandic spikes. Language and cognitive improvements were subsequently seen. Educational support and evolution of the electroencephalogram to a localized focus could have been contributory. It is anticipated, however, that he will have significant long-term problems in complex language.
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ranking = 0.0033648444569925
keywords = complex
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