Differentiation of myoclonic seizures in epileptic syndromes: A video-polygraphic study of 26 patients

Objective:   We conducted a video-polygraphic study of myoclonic seizures (MS) in different epileptic syndromes to clarify semiologic and electroencephalography (EEG) differences among them.
Subjects and methods:   The subjects were 26 children with MS, including benign myoclonic epilepsy in infants (BME) in 10, severe myoclonic epilepsy in infants (SME) in 6, idiopathic epilepsy with myoclonic-astatic seizures (IEMAS) in 4, and juvenile myoclonic epilepsy (JME) in 6. We reviewed the video-polygraphs of MS, including the predominant area of muscle involvement (neck, trunk, and proximal or distal upper extremities), postural changes including astatic falling, and mode of appearance. We also analyzed the frequency of a corresponding generalized spike-and-wave complex (GSW) and the duration of myoclonic electromyography (EMG) activity.
Results:   A total of 550 MS were documented in the 26 cases. MS manifested with proximal predominance/forward flexion/single occurrence in BME, proximal predominance/forward astatic flexion/single occurrence in IEMAS, proximal predominance/extension/succession in SME, and distal predominance/extension/succession in JME. The median frequency of GSW was 1.5, 1.3, 3.2, and 3.1 Hz, respectively, and the median duration of the myoclonic EMG activity was 387, 587, 81, and 65 ms, respectively.
Conclusion:   MS in the four different epileptic syndromes show significant semiologic and EEG differences, as well as similarities. Although our study has the limitations of the small number of patients and retrospective methodology, these results should be considered in the classification and differential diagnosis of myoclonic epileptic syndromes.     

病理证实的淀粉样脑血管病26例临床分析

目的 总结26例尸检证实的淀粉样脑血管病的临床资料特点,以提高对本病的认识和诊断水平.方法 回顾性分析我院1983--1999年收治的经尸检证实的26例淀粉样脑血管病患者,总结其既往史、临床表现、实验室检查等临床资料.结果 26例淀粉样脑血管病中男17例,女9例,年龄45 ～78岁,有高血压病史者8例(30.7％),糖尿病病史者6例(23.1％).2例患者(7.6％)正服用抗凝或扰血小板剂,均为多发脑叶出血.26例中脑出血20例,其中单发脑叶出血2例,多灶性脑叶出血8例,壳核出血5例,丘脑及小脑出血各2例,脑干出血1例.蛛网膜下腔出血2例,出血性脑梗死、基底节区梗死、椎基底动脉闭塞及硬膜下血肿各1例.20例淀粉样脑血管病脑出血患者临床表现为头痛、肢体瘫痪、昏迷、抽搐等.结论 淀粉样脑血管病临床上常以脑血管病表现形式起病,伴有或不伴有高血压.脑叶出血是淀粉样脑血管病最常见临床表现,但少数也可见基底节、小脑和脑干出血.淀粉样脑血管病也可表现为脑梗死、蛛网膜下腔出血.抗凝剂(如华法林)及抗血小板药物(如阿司匹林)的应用对淀粉样脑血管病脑出血可能具有一定的促发作用.     

Ictal epileptic headache in adult life: Electroclinical patterns and spectrum of related syndromes

ObjectivesBoth headache and epilepsy are frequent paroxysmal disorders that often co-occur or are related in numerous ways. Although ictal epileptic headache has become the focus of several studies, this remains a very rare and not well-known phenomenon. Electroclinical features, pathophysiology, and syndromic context are heterogeneous. We investigated the electroclinical and neuroimaging findings in a population of adult patients with ictal epileptic headache.MethodsWe retrospectively examined 8800 EEG recordings of almost 4800 patients admitted to our video-EEG laboratory from 2010 to 2013 with a history of well-documented epilepsy. We selected patients who reported headache closely related to a seizure documented by video-EEG or 24-hour ambulatory EEG. We analyzed ictal electroclinical features of headache, and we defined the related epileptic syndromes.ResultsWe identified five patients with ictal epileptic headache. Two patients described tension headache during an epileptic seizure. In three patients, the headache was accompanied by other “minor” neurological symptoms mimicking a migrainous aura. In all cases, the headache stopped with the end of the epileptic activity. Three patients had a history of partial symptomatic epilepsy with cerebral lesions (low grade glioma, astrocytoma, porencephalic cyst) in the left posterior regions, whereas two patients were affected by idiopathic generalized epilepsy.ConclusionThis study confirms the rarity of ictal epileptic headache. To date, well-documented video-EEG cases remain as exceptional reports, especially in cases of idiopathic generalized epilepsies. Moreover, we confirm the main involvement of posterior regions in patients with ictal epileptic headache affected by partial symptomatic epilepsies.     

Late onset epileptic seizures A retrospective study of 250 patients

A retrospective study of 250 patients with late-onset epilepsy was carried out. The ages ranged from 22 to 88. The seizures were partial in 104 patients and generalized in 146. The neurological examination was abnormal in 41 patients and normal in 209. The EEG studies and CT scan revealed abnormalities in 76.5% and 50.8% respectively. The most frequent CT scan findings were diffuse atrophy (19.2%), tumors (16.4%) and cerebral infarct (8.8%). The clinical parameters which best predicted the CT scan abnormalities were an abnormal neurological examination and simple partial seizures. In seven of the 45 patients with space-occupying lesions, the clinical examination and EEG were normal. The etiology of the convulsions was established in 201 patients, the most frequent cause being chronic alcoholism (62 cases), tumors (41 cases), postischemic vascular epilepsy (33 cases) and postraumatic epilepsy (28 cases). We conclude that a CT scan is essential in the assessment of patients with epileptic convulsions of late onset, even when the EEG and clinical examination are normal.     

Lobar cerebral hemorrhages: Acute clinical syndromes in 26 cases

The acute syndromes and CT findings are described in 26 cases of spontaneous cerebral hemorrhage. Occipital hemorrhage (11 cases) caused severe pain around the ipsilateral eye and dense hemianopia. Left temporal hemorrhage (7 cases) began with mild pain in or just anterior to the ear, fluent dysphasia with poor auditory comprehension but relatively good repetition, and a visual deficit subtending less than a hemianopia. Frontal hemorrhage (4 cases) caused a distinctive syndrome beginning with severe contralateral arm weakness, minimal leg and face weakness, and frontal headache. Parietal hemorrhage (3 cases) began with anterior temporal (“temple”) headache and hemisensory deficit, sometimes involving the trunk to the midline. One patient had a right temporal hemorrhage. Spontaneous lobar hemorrhage and branch artery embolism in the same region produce similar clinical syndromes. Headache is a first and prominent symptom. A rapid but not instantaneous onset over several minutes, when combined with one of the typical syndromes, suggests lobar hemorrhage rather than other types of stroke. Ancillary investigations (including CT scanning, angiography in 11 patients, and autopsy in 4) disclosed 2 patients with bleeding diatheses due to warfarin, 2 with arteriovenous malformations, and 1 with metastatic tumor. Only 8 of the 26 patients had chronic hypertension (blood pressure greater than 130/85 mm Hg), suggesting that hypertension is not an etiological factor in most lobar hemorrhages.     

Transient epileptic amnesia: a retrospective cohort study of 127 cases,including CSF amyloid and tau features

Journal of Neurology - Transient epileptic amnesia (TEA) is a late-onset epilepsy syndrome encompassing transient iterative amnesias and interictal cognitive impairment, two features that overlap...     

Pilocytic astrocytoma: A retrospective study of 32 cases

Pilocytic astrocytoma (PA) is a neoplasia which is considered as a grade I astrocytoma by the World Health Organization (WHO). Its most common location is the cerebellum and it develops during the first two decades of life. Prognosis is mostly excellent if gross-total resection can be achieved, with 10-year survival rates of up to 95%. In rare cases, however, the patient has a bad outcome.     

Relation of photosensitivity to epileptic syndromes.

Photosensitivity is the most common mode of seizure precipitation. It is age-related, more frequent in females, and most often found in generalised epilepsies. Little is known about its relation to individual epileptic syndromes. This study on 1062 epileptic patients who had 4007 split screen video EEG investigations revealed that the relation to generalised epilepsy is even more close than generally believed. Versive seizures with visual hallucinations was the only focal seizure type related to photosensitivity. Of the syndromes of generalised epilepsy, only childhood absence epilepsy, juvenile myoclonic epilepsy, and epilepsy with grand mal on awakening were related to photosensitivity. The closest correlation was with juvenile myoclonic epilepsy. This is confirmed by a relation to the poly-spike wave pattern, and by an increase of myoclonic seizures by intermittent light stimuli. No relation was found with early childhood syndromes of generalised epilepsy, or generalised tonic-clonic seizures in the evening, or, most remarkably, with juvenile absence epilepsy. In generalised epilepsies with onset around puberty, photosensitivity could thus act as a pathoplastic factor. The female preponderance in both childhood absences and photosensitivity could be due to the same unknown factor.     

Hemispherectomy in pediatric patients with epilepsy: a study of 45 cases with special emphasis on epileptic syndromes

Objective

In this study we report the clinical outcomes of hemispherectomy for epilepsy in pediatric patients with special emphasis on the epileptic syndromes and their etiologies.

Material and methods

We retrospectively studied 45 patients with medically refractory epilepsy with hemispheric lesions who underwent hemispherectomy at the ??Hospital de Pediatría Prof. Dr. Juan P. Garrahan??, Buenos Aires, Argentina between February 1990 and February 2010. Patients had been assessed using a standard protocol involving clinical, neuroradiological, neurophysiological, and neuropsychological teams.

Results

Twenty-seven males and 18 females with a mean age of 8.5?years (range, 2?months to 18?years) who underwent epilepsy surgery for refractory epilepsy were assessed. The mean time of follow-up was 9.5?years (range, 1 to 16?years). The following epileptic syndromes were recognized: West syndrome in 15 patients (33.5%), Rasmussen syndrome in 13 (29%), focal symptomatic epilepsy in 8 (17.5%), startle epilepsy in 6 (13.5%), Lennox?CGastaut syndrome in 2 (5%), and continuous spikes and waves during slow sleep in 1 (2%). The surgical specimens revealed malformations of cortical development in 18 patients (40%), Rasmussen encephalitis in 13 (29%), porencephalic lesions in 10 (22%), gliosis in 2 (4.4%), tumor in 1 (2.2%), and Sturge?CWeber syndrome in 1 (2.2%).

Conclusion

The outcome of hemispherectomy in pediatric patients is good for those with refractory epilepsies, such as West syndrome, Lennox?CGastaut syndrome, epileptic encephalopathy with continuous spikes and waves during slow sleep, and startle epilepsy arising from a hemispheric lesion associated with hemiplegia.     

Infantile spasms. A retrospective study of 105 cases]

This paper is a summary of our observations on 105 cases of infantile spasms. The age of onset was around six months after birth, but the patients came for treatment mainly about one year after onset. Fever of unknown cause, asphyxia, birth injury, infection of the central nervous system, tuberous sclerosis, phenylketonuria and recent immunization etc. were possible etiology. Clinically, it is characterized by head nodding, mental retardation, myoclonic jerks and various neurologic deficits. EEG findings showed classical or modified arrhythmia or other epileptiform patterns. About one third of 22 cases examined had abnormal brain stem auditory evoked potentials. Among 42 patients who underwent CT scanning before ACTH treatment, 18 were normal and 7 abnormal; during ACTH treatment 3 normal and 4 abnormal; after completion of treatment, 4 normal and 6 abnormal, suggesting no further atrophy of the brain. Examination of trace elements of the hair by proton-induced X-ray emission (PIXE) method in 23 patients revealed a significant difference in lead, calcium and zinc contents between patients and 101 controls, but no statistical difference in iron and copper contents between the two groups. Sodium valproate, prednisone and ACTH appear to be effective in the treatment of infantile spasms. Eight patients fully recovered, and they can go to school without difficulty. Many patients derived various degrees of improvement of the satisfaction of their parents. Two patients were still amended and often attacked by myoclonus. The effects, side effects of these drugs, and the possible pathogenesis were discussed.     

Eye closure sensitivity in juvenile myoclonic epilepsy and its effect on prognosis

PurposeTo investigate eye closure sensitivity (ECS) in the EEGs of patients diagnosed with juvenile myoclonic epilepsy (JME) and its relationship to prognosis.MethodsWe included 76 JME patients with a minimum follow-up of one year and evaluated a total of 254 EEGs to obtain evidence of ECS. The patients were grouped according to their response to treatment, and these subgroups were compared in relation to ECS and other clinical and EEG features.ResultsThere were 12 patients (15.8%) with poor prognosis who showed resistance to appropriate anti-epileptic drug treatment, 15 (19.7%) patients with pseudo-resistance, and 49 (64.5%) patients with good prognosis. The EEGs of only four of the patients displayed pure ECS (5.3%), and only one of these exhibited poor prognosis. Furthermore, 11 patients (14.5%) had both ECS and photosensitivity, and two of these patients exhibited poor prognosis. Thus, neither pure ECS nor ECS with photosensitivity correlated with poor prognosis. A family history of epilepsy and focal findings on the EEG was correlated with poorer prognosis.ConclusionsECS is a rare EEG finding in JME and does not appear to be a marker for poor prognosis.     

Time-dependent risk factors for epileptic seizures in glioblastoma patients: A retrospective analysis of 520 cases

Objective

Epilepsy is a common comorbidity of glioblastoma. Seizures may occur in various phases of the disease. We aimed to assess potential risk factors for seizures in accordance with the point in time at which they occurred.

Methods

We retrospectively analyzed medical files of adult patients with de novo glioblastoma treated at our institution between January 2006 and January 2020. We categorized seizures as preoperative seizures (POS), early postoperative seizures (EPS; before initiation of radio[chemo]therapy [RCT]), seizures during radiotherapy (SDR; during or <30 days after RCT), and posttherapeutic seizures (PTS; ≥30 days after completion of RCT). We addressed associations between patients' characteristics and their seizures.

Results

In the final cohort (N = 520), 292 patients experienced seizures. POS, EPS, SDR, and/or PTS occurred in 29.6% (154/520), 6.0% (31/520), 13.8% (70/509), and 36.1% (152/421) of patients, respectively. POS occurred more frequently in patients with higher Karnofsky Performance Scale scores (odds ratio [OR] = 3.27, p = .001) and tumor location in the temporal lobe (OR = 1.51, p = .034). None of the parameters we analyzed was related to the occurrence of EPS. SDR were independently associated with tumor location (parietal lobe, OR = 1.86, p = .027) and POS, but not EPS, and were independent of RCT. PTS were independently associated with tumor progression (OR = 2.32, p < .001) and with occurrence of SDR (OR = 3.36, p < .001), and negatively correlated with temporal lobe location (OR = .58, p < .014). In patients with tumors exclusively located in the temporal lobe, complete tumor resection was associated with a decreased risk of postoperative seizures.

Significance

Seizures in glioblastoma patients have various, time-dependent risk factors. Temporal lobe localization was a risk factor for preoperative seizures; surgery may have had a protective effect in these patients. RCT did not have dose-dependent pro- or anticonvulsive effects. PTS were associated with tumor progression.     

Somatosensory evoked spikes and epileptic seizures: a study of 385 cases.

We examined 385 children whose EEG showed high voltage potentials evoked by taps applied to one or both feet or hands (SES). The relationship between characteristics of SES and the occurrence of epileptic seizures and the characterization of epileptic syndromes were studied. Ninety-one children (23.6%) had epilepsy, 42 (10.9%) had only febrile convulsions and 252 children had other complaints. Epilepsy occurred in a higher proportion of cases when: SES by foot tapping were multiphasic, with high amplitude or SES were obtained by hand stimulation and there was spontaneous epileptiform activity in the EEG. The following epileptic syndromes were diagnosed: benign childhood epilepsy with centrotemporal spikes in 21 cases, benign epilepsy of childhood with occipital paroxysms in 2, benign psychomotor epilepsy in 1, "partial idiopathic others" in 43, generalized idiopathic in 8, symptomatic epilepsies in 13 and undetermined in 3 cases. In most cases SES were observed in children without evidence of cerebral organic lesion, suggesting the existence of an age-related, functional mechanism. Some characteristics of SES and the occurrence of spontaneous epileptiform activity showed a positive association with epileptic seizures. SES occurred in different types of partial and generalized epilepsies of childhood but in nearly 50% of the cases with epilepsy, there was a benign condition involving mainly the parietal lobe with versive, unilateral and sleep-generalized seizures.     

Photosensitivity in epileptic syndromes of childhood and adolescence.

PURPOSE: Photosensitivity, a reaction of the brain to external photic stimulation, can be graded from 1 to 4, and is most frequently seen in the first decades of life. This study investigated photosensitivity in children with epilepsy. METHODS: A retrospective study performed in the neuropaediatric department of the largest paediatric hospital in Kiel, treating patients at all medical care levels. The clinical data and EEG records of 566 patients with the most common epileptic syndromes were analyzed, in particular regarding photosensitivity. Their EEGs included application of intermittent light stimulation using standard techniques at twice the minimum. RESULTS: The proportion of photosensitive patients was significantly higher in the paediatric cohort than in adult patients, as published in the literature: 46% of patients with generalized epilepsies showed photosensitivity as compared to 20% with focal epilepsies. Photosensitivity was more common in idiopathic generalized epilepsy (IGE), (epilepsy with grand mal on awakening, 74%; juvenile absence epilepsy, 56%; juvenile myoclonic epilepsy, 50%; childhood absence epilepsy, 44%) than in focal types (idiopathic partial - Rolandic epilepsy, 23%; symptomatic/cryptogenic type of epilepsy, 16%), while in patients who experienced occasional seizures (neonatal/febrile seizures), this ranged between 40% and 23%, respectively. The generalized photoparoxysmal response, (PPR), grades 3 and 4 were found significantly more often in patients with IGE (92%) than in patients with focal epilepsies. Finally, the female preponderance was confirmed (37% to 27% of all epilepsies). CONCLUSIONS: Photosensitivity can be detected both in patients with IGE, with idiopathic and symptomatic/cryptogenic types of focal epilepsies, and with epileptic (occasional) seizures. PPR grades 3 and 4 are the most common in IGE.     

Cognitive functions, epileptic syndromes and antiepileptic drugs.

Cognitive function of patients on monotherapy specific for their epileptic syndrome has been studied infrequently. We evaluated 7 patients with symptomatic localised epilepsies (SEL) on phenytoin aged 30 +/- 12 (mean +/- standard deviation) years, 8 with idiopathic generalised epilepsies on sodium valproate aged 18 +/- 4 years, 16 with SEL on carbamazepine aged 28 +/- 11 years, and 35 healthy controls aged 27 +/- 11 years. All subjects were of normal intelligence, educated appropriately to age, and led productive lives in the community. Two of the patients on carbamazepine and one on valproate had less than five partial, absence or myoclonic seizures monthly, the remaining were controlled. Carbamazepine serum concentrations were 12 +/- 5 micrograms/ml, phenytoin were 23 +/- 7, and valproate were 62 +/- 23 (mean +/- sd). Tests included immediate recall and recognition for pictures, Stroop test, delayed recall and recognition of pictures. Patients on phenytoin and valproate performed significantly worse than controls on immediate recall, and patients on carbamazepine performed significantly worse than controls in Stroop test (p < 0.01). The results indicate relatively minor effects of the epileptic syndromes and of phenytoin, carbamazepine and valproate on cognition of patients with controlled epilepsy leading productive lives in the community. We conclude that the cognitive deficit found in chronic epileptic patients on poly-therapeutic drug regimen must be multifactorial, and that future studies need to control for all possible variables in order to achieve meaningful results.     

Classification of epileptic seizures and syndromes: new proposals

In the decade of 1980 the International League Against Epilepsy (ILAE) developed a classification system of epileptic seizures and syndromes. These classifications were established progressively and are presently used in all the world. In the following years, video-EEG monitoring became widely available and provided direct information about seizure semiology and ictal EEG that had not been so well understood during the development of the ILAE classifications. This new information demonstrated certain limitations of the old classification of epileptic seizures. In addition, improvements in brain imaging techniques and research in genetics added further information on the pathophysiology of epilepsies and epileptic syndromes. In order to integrate these advances into the practical management of epilepsies, new classification proposals have been developed. This article reviews the clinical implications of these new classifications.     

Unilateral pallidotomy in advanced Parkinson's disease: a retrospective study of 26 patients.

OBJECTIVE: To evaluate the effects of unilateral pallidotomy in patients with Parkinson's disease (PD). PATIENTS AND METHODS: Twenty-six patients with PD and disabling dyskinesias, painful and/or disabling dystonia, and/or pain as part of PD despite optimal pharmacotherapy underwent unilateral pallidotomy. For assessment, the Unified Parkinson's Disease Rating Scale (UPDRS; part II and III), Hoehn and Yahr staging, the Schwab and England scale, a Dyskinesia Rating Scale, and timed tests were used. Assessment was performed in defined "off' and "on," and on average 2 months before and 7.5 months after the unilateral pallidotomy. Adverse effects were classified as transient or permanent and as major or minor. RESULTS: In the "off' phase, the median UPDRS II score improved from 26.5 to 20.5 (23%) and the median UPDRS III score improved from 47.5 to 33.0 (31%). In the "on" phase, dyskinesias contralateral to the side of the procedure improved with 88% ipsilateral dyskinesias improved only temporarily, and the total UPDRS II and III scores remained unchanged. Thirteen patients had transient adverse effects, three patients had permanent, and two patients had a combination of transient and permanent adverse effects. The transient adverse effects in two patients were classified as major. CONCLUSION: Stereotactic unilateral pallidotomy can improve symptoms and disability in the "off' phase. In the "on" phase, dyskinesias disappeared at the side contralateral to the procedure. Permanent minor complications of pallidotomy occurred in 19% of the patients.     

Risk of aspiration pneumonia after an epileptic seizure: a retrospective analysis of 1634 adult patients

We reviewed the incidence of aspiration pneumonia secondary to seizures in three populations of patients with chronic epilepsy: 733 outpatients seen in an Epilepsy Foundation clinic; 806 adult patients admitted to two university video telemetry units; and 95 institutionalized, profoundly retarded adult patients with chronic epilepsy. Two of the 733 adults who had seizures in the outpatient setting and 2 of the 806 patients who had one or more epileptic seizures in the telemetry units developed aspiration pneumonia. In the 95 institutionalized patients, there were 17 instances of aspiration pneumonia after a generalized seizure and 32 instances of aspiration unrelated to seizures over a 12-month period. Our findings suggest that aspiration pneumonia is not a common complication of seizures in otherwise healthy adults. The increased incidence of aspiration in developmentally delayed individuals seems to derive from a combination of factors. Increased oral secretions, impaired swallowing mechanisms, and difficulty in attaining adequate patient positioning significantly increased the risk of aspiration.