表现有立毛运动的复杂部分性癫痫(附3例报告及文献复习)

目的了解立毛运动性发作的病理生理、病因及临床特点。方法结合3例立毛运动性发作病例及文献复习(已报过21例)，探讨立毛运动性发作的特点。结果立毛运动性发作的最重要临床表现是反复的立毛运动，可能与右颞叶额叶(眶面皮层，运动前区)下丘脑和边缘系统学结构有关，脑电图(EEG)表现为非特异性异常，但是大多数病例有发作期发放或癫痫样波，视频监测脑电图(Video—EEG)对于评价其分型及发作期、发作间期变化有帮助。结论立毛运动性发作极罕见。但是注意结合病史，体检和EEG检查还是可以发现的。     

Hyperthermic seizures and hyperthermia in immature rats modify the subsequent pentylenetetrazole-induced seizures

It has been described that febrile seizures during infancy increase risk of subsequent non-febrile seizures during the adulthood. However, latency period between febrile seizure and the onset of the first spontaneous seizure has not been evaluated. The present study was designed to investigate the susceptibility to subsequent seizures in immature rats that had experienced early-life hyperthermic seizures and before they achieved the adult age. The results were compared with those induced by hyperthermia alone. Pentylenetetrazol (PTZ) was applied 24 h or 20 days after hyperthermic seizures or hyperthermia were induced in 10-day-old rats by a regulated stream of moderately heated air. One day after hyperthermic seizures or hyperthermia, animals demonstrated enhanced latency to the PTZ-induced myoclonic (88% and 53%, respectively), clonic (60% and 60%, respectively) and tonic seizures (233% and 659%, respectively). The incidence of myoclonic and clonic seizures was similar to that in control group (100%). However, hyperthermic seizures reduced (50%) the incidence of tonic seizures. Twenty days after hyperthermic seizures there was an augmented latency to tonic seizures (123%) and reduced incidence for all the PTZ-induced seizures (71% myoclonic; 71% clonic seizures; 57% tonic seizures) when compared with control group (100%). In contrast, hyperthermia enhanced only the latency to myoclonic (133%) and clonic seizures (659%). Our data indicate that hyperthermic seizures or hyperthermia induces a protective effect against PTZ-induced seizures during a latency period. A possible involvement of γ-aminobutyric acid (GABA) system is discussed.     

Febrile seizures in patients with complex partial seizures

Febrile seizures occurred in 14 of 155 (9%) out-patients with complex partial seizures. Twelve patients had prolonged or recurrent febrile seizures, convulsive status epilepticus or a transient postictal neurological deficit. Febrile seizures were associated with perinatal abnormalities, an earlier onset of epilepsy and with a poor seizure control. Recurrent febrile seizures or those with complicating features are associated with an unfavourable therapeutic outcome in adult patients with complex partial seizures.     

Partial seizures, cerebral calcifications and celiac disease

We describe the case of a 25 year old woman who has been clinically and instrumentally examined over a period of about 20 years. A diagnosis of celiac disease was made when she was four years old and, ten years later, CAT revealed the presence of bilateral cerebral calcifications. The partial occipital seizures were controlled by adopting a gluten-free diet, which is still being followed four years after the discontinuation of anti-epileptic treatment.

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Sommario Viene descritto il caso di una donna dell'età di 25 anni seguita attraverso controlli clinici e strumentali per circa vent'anni. All'età di 4 anni venne fatta diagnosi di celiachia, dieci anni più tardi vennero riscontrate alla TAC calcificazioni cerebrali bilaterali.Il controllo delle crisi parziali occipitali è stato ottenuto con una dieta priva di glutine instaurata precocemente e che tuttora viene seguita dopo che da quattro anni è stata sospesa la terapia antiepilettica.

     

Serum neuron-specific enolase, prolactin, and creatine kinase after epileptic and psychogenic non-epileptic seizures

PURPOSE: To evaluate the discriminative power of serial, simultaneous determinations of serum neuron-specific enolase (NSE), prolactin (PRL) and creatine kinase (CK) in differentiating psychogenic non-epileptic seizures (PNES) from epileptic seizures (ES). METHODS: Prospective measurement of the three markers after 44 single seizures (32 ES and 12 PNES) during continuous video-EEG monitoring at seven different sampling points. RESULTS: Patients with ES had a significantly greater increase in PRL at 10, 20, 30 min, 1 and 6 h. The sensitivity for elevated NSE and CK was low. PRL showed a higher sensitivity. However, the corresponding positive predictive value was lower than in CK and NSE. Additionally, PRL had the lowest specificity of all parameters. CONCLUSIONS: The limited discriminative power of PRL, CK, and NSE calls into question if these markers are helpful in differentiating PNES and ES.     

Epilepsy duration, febrile seizures, and cerebral glucose metabolism

PURPOSE: Studies using magnetic resonance imaging have shown that reduced hippocampal volume is associated with a history of febrile seizures, the duration of epilepsy, and the number of generalized tonic-clonic seizures. It is uncertain whether these factors have the same influence on functional as on structural measures of the integrity of the epileptogenic zone. METHODS: We used positron emission tomography (PET) with fluorine 18 2-deoxyglucose to study 91 patients with temporal lobe seizure foci localized by ictal video-EEG. PET was performed in the awake interictal resting state with ears plugged and eyes patched. We recorded surface EEG during injection (5 mCi) and the 30-min uptake period. We used a standard template to analyze PET scans. RESULTS: A significant negative relation was found between the duration of epilepsy and hippocampal glucose metabolism ipsilateral to the epileptic focus. Patients with a history of either any febrile seizures, or complex, or prolonged febrile seizures, did not have greater hypometabolism ipsilateral to the epileptic focus than did patients without a febrile seizure history. We found no effect of generalized tonic-clonic seizure history. CONCLUSIONS: Longer epilepsy duration is associated with greater hypometabolism, suggesting that epilepsy is a progressive disease.     

Tonic and atonic seizures: Medical therapy and ketogenic diet

Tonic and atonic seizures are typically seen in what are often referred to as the catastrophic epilepsies. In simply considering what each of the terms signifies (either the complete loss of tone or a marked increase in tone), they would appear to be at the most diverse ends of the spectrum. They would appear to be as opposite as hot and cold or hard and soft. And yet they are often found to occur in the same patient. This article examines the nature of these seizures and discusses some of the syndromes in which they are seen. Although these seizures are often very difficult to control, some of our medications/therapies have been shown to be effective. Recommendations concerning the efficacy of these therapies and a review of the newer therapies are provided. In addition, the ketogenic diet has been particularly successful in treating these seizures; this is discussed in some detail. Finally, although outcomes for these children are generally less than ideal, many can be helped with a persistent approach that balances seizure control against the side effects of medication.     

Evoked and psychogenic epileptic seizures. I. Precipitation

The precipitation of seizures by external stimulation (evoked seizures) is well known. Less well known is the precipitation of seizures by a change in the patient's thinking or feelings. This artick uses Lockhart's monkey model of focal epilepsy to propose that there is a close relationship between seizures and ongoing brain activity. Thus, seizures precipitated by both voluntary and spontaneous changes in behaviour and thinking must commonly occur. Clinical examples of such seizure precipitation is described.     

Treatment of febrile seizures: Historical perspective,current opinions,and potential future directions

Although most febrile seizures do no harm and two-thirds of initial cases have no witnessed recurrence, the seizures cause much family anxiety, and are sometimes prolonged. In rare cases they are the first evidence of important epilepsy syndromes or are implicated in the development of epilepsy with mesial temporal sclerosis in later life. There have been trials of prophylactic treatment with antiepileptic drugs including carbamazepine, diazepam, phenobarbital, phenytoin, and sodium valproate. Several strategies have been employed with these drugs, including continuous secondary prophylaxis, intermittent secondary prophylaxis in response to later episodes of fever, and rescue medication early in the course of further seizures. Another treatment strategy has been using one or more antipyretic agents in early response to fever using agents such as acetaminophen and ibuprofen. Over the years, researchers have identified a variety of clinical, genetic, and environmental risk factors for more severe or prolonged febrile seizures and higher risk of recurrence. This review evaluates the rationale for secondary prophylaxis of febrile seizures, the potential effectiveness of such treatment, and whether it can be recommended as a general approach to treating febrile seizures or as an approach to be used in groups identified to be at increased risk.     

甲状腺机能亢进与癫痫样发作(附2例报道)

目的探讨甲状腺机能亢进伴发癫痫样发作的临床特点和发病机制。方法通过中文医学期刊全文数据库及Medline检索到从1974年至今国内外相关的文献报道共17例,附我院癫痫中心2例报道。结果甲状腺机能亢进伴发癫痫样发作的临床表现有3种形式:(1)以痫样发作为首发症状出现;(2)痫样发作为继发临床症状;(3)癫痫患者合并甲亢时痫样发作加重。发作类型以全面性强直阵挛发作为主。结论(1)甲状腺机能亢进可引起脑损害而导致癫痫样发作,发病机制有待于进一步研究证实;(2)癫痫与甲亢可能存在某些共同的免疫发病机制。     

Idiopathic generalized epilepsies with versive or circling seizures

OBJECTIVES: To describe the electroclinical features of the idiopathic generalized epilepsies (IGEs) with versive or circling seizures. METHODS: Sixteen patients with versive or circling seizures and interictal electroclinical features of IGE were studied. Patients with insufficient clinical or imaging data, with a follow-up period less than 1 year or with partial seizures in addition to the versive or circling ones were excluded from the study. All patients underwent full interictal clinical and neurophysiological studies. The EEG patterns of 13 versive or circling seizures from 4 patients were also analyzed. RESULTS: A specific IGE syndrome was recognized in 9 out of the 16 patients (56%). More specific, 1 patient had childhood absence epilepsy (CAE), 4 had juvenile absence epilepsy (JAE), and 4 had juvenile myoclonic epilepsy (JME). No specific IGE syndrome was recognizable in the remaining 7 patients (44%). These 7 patients had a juvenile epileptic syndrome (mean age at onset of seizures was 15.7 years) characterized by versive or circling seizures followed or not by generalized tonic-clonic fits. Three main EEG patterns were identified during versive or circling seizures: 1) generalized spike-and-wave discharges at 3-4 cps; 2) generalized polyspike-and-wave discharges at 1 to 2.5 cps beginning with generalized fast activity at 12-14 cps, and 3) generalized spike-and-wave discharges at 3-4 cps intermingled with fast activity at 12-14 cps. Most patients had good response to treatment on a single drug regimen (mainly valproic acid). CONCLUSIONS: Versive or circling seizures may occur in the context of an IGE. Although many individuals share the features of different IGE syndromes including CAE, JAE and JME, a consistent number of patients, who show circling or versive seizures solely, remain without a specific syndromic diagnosis. When occurring in the context of IGE, circling or versive seizures do not worsen the prognosis.     

Trauma, stress, and preconscious threat processing in patients with psychogenic nonepileptic seizures

Purpose:   Psychogenic nonepileptic seizures (PNES) have long been considered as paroxysmal dissociative symptoms characterized by an alteration of attentional functions caused by severe stress or trauma. Although interpersonal trauma is common in PNES, the proposed relation between trauma and attentional functions remains under explored. We examined the attentional processing of social threat in PNES in relation to interpersonal trauma and acute psychological stress.
Methods:   A masked emotional Stroop test, comparing color-naming latencies for backwardly masked angry, neutral, and happy faces, was administered to 19 unmedicated patients with PNES and 20 matched healthy controls, at baseline and in a stress condition. Stress was induced by means of the Trier Social Stress Test and physiologic stress parameters, such as heart rate variability (HRV) and cortisol, were measured throughout the experiment.
Results:   No group differences related to the acute stress induction were found. Compared to controls, however, patients displayed a positive attentional bias for masked angry faces at baseline, which was correlated to self-reported sexual trauma. Moreover, patients showed lower HRV at baseline and during recovery.
Discussion:   These findings are suggestive of a state of hypervigilance in patients with PNES. The relation with self-reported trauma, moreover, offers the first evidence linking psychological risk factors to altered information processing in PNES.     

Ictal cerebral haemodynamic characteristics during typical absence seizures,compared to focal seizures with brief alteration of awareness

Aims. To investigate ictal cerebral haemodynamic characteristics during spontaneous typical absence seizures (TAS) and hyperventilation‐evoked absence seizures in paediatric patients, relative to brief complex partial seizures (BCPS). Methods. All children diagnosed with seizures using real‐time transcranial doppler ultrasonography (TCD) and sleep‐deprived video‐EEG (vEEG) from 2015 to 2017 in our hospital were included. The seizures were diagnosed based on the video and EEG findings. Mean cerebral blood flow velocity (CBFV_m) of the unilateral middle cerebral artery was measured using TCD. TCD and vEEG data were integrated for a synchronous assessment of CBFV_m changes and epileptic status. Baseline and peak CBFV_m for TAS and BCPS were compared by T‐test. Results. Six children (two boys and four girls) with TAS and two girls with BCPS were enrolled. A total of 15 spontaneous TAS, 14 hyperventilation‐evoked absence seizures, and six BCPS were recorded using real‐time TCD‐vEEG monitoring. During spontaneous TAS, whether awake or asleep, the CBFV_m decreased by 20–40% compared to baseline. During hyperventilation‐evoked absence seizures and BCPS, the CBFV_m increased by 50–150% and 20–30% over baseline levels, respectively. Conclusions. The haemodynamic characteristics during TAS and BCPS are distinct, and thus our results may provide a new method to diagnose typical absence seizures using dynamic CBFV_m curves. Ictal cerebral haemodynamic characteristics during spontaneous typical absence seizures and hyperventilation‐evoked absence seizures may reflect different pathophysiological mechanisms and networks compared with BCPS.     

Brain activation patterns of versive, hypermotor, and bilateral asymmetric tonic seizures

Purpose: Patients who have seizure onset from different brain regions can produce seizures that appear clinically indistinguishable from one another. These clinically stereotypic manifestations reflect epileptic activation of specific networks. Several studies have shown that ictal perfusion single photon emission computed tomography (SPECT) can reveal propagated ictal activity. We hypothesize that the pattern of hyperperfusion may reflect neuronal networks that generated specific ictal symptomatology. Methods: All patients were identified who were injected with ^99mTc‐hexamethyl‐propylene‐amine‐oxime (HMPAO) during versive seizures (n = 5), bilateral asymmetric tonic seizures (BATS; n = 5), and hypermotor seizures (n = 7) in the presurgical epilepsy evaluation between 2001 and 2005. The SPECT ictal–interictal difference image pairs of each subgroup were compared with image pairs of 14 controls using statistical parametric mapping (SPM 2) to identify regions of significant hyperperfusion. Hyperperfused regions with corrected cluster‐level significance p < 0.05 were considered significant. Results: We have identified a distinct ictal perfusion pattern in each subgroup. In versive seizure subgroup, prominent hyperperfusion was present in the frontal eye field opposite to the direction of head version. In addition, there was associated caudate and crossed cerebellar hyperperfusion. The BATS subgroup showed pronounced hyperperfusion supplementary sensorimotor area ipsilateral to the epileptogenic region, bilateral basal ganglia, and contralateral cerebellar hemisphere. The hypermotor seizure subgroup demonstrated two clusters of significant hyperperfusion: one involving bilateral frontomesial regions, cingulate gyri, and caudate nuclei, and another involving ipsilateral anteromesial temporal structures, frontoorbital region, insula, and basal ganglia. Discussion: We have identified distinct hyperperfusion patterns for specific ictal symptomatology. Our findings provide further insight into understanding the anatomic basis of seizure semiology.     

New onset seizures in HIV—Seizure semiology, CD4 counts, and viral loads

Thirty-seven HIV-positive patients with new-onset seizures (NOS) were prospectively identified during a 1-year study period. The patients were categorized according to the different mechanisms causing NOS in HIV, namely focal brain lesion (FBL) in 21 patients (57%), meningitis in 6 patients (16%), metabolic derangement (no patient), and no identified cause (NIC) other than HIV itself (10 patients, 27%). Seizure semiology, CD4 counts, and blood and cerebral spinal fluid (CSF) viral loads were studied to identify any special characteristics of the different categories. With respect to seizure semiology, all NIC patients had generalized seizures. Two-thirds of the meningitis patients had generalized seizures with one-third having focal seizures. Half of the patients with FBL had generalized seizures and one-third had focal seizures. Status epilepticus was strongly associated with FBL. No significant difference could be detected between the subgroups with respect to CD4 counts and serum and CSF viral loads. The median CD4 count in all patients was 108 cells/ml, indicating advanced immunosuppression. In the FBL group this was 104 cells/ml. In the meningitis group the median CD4 count was 298 cells/ml, and in the NIC group this was 213 cells/ml. Similarly, no differences were noted in the NOS categories with respect to serum and CSF viral loads. Seizures in HIV are a nonspecific manifestation of the seizure mechanism.