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1.
Semin Neurol ; 2024 Jun 24.
Article in English | MEDLINE | ID: mdl-38914127

ABSTRACT

Management of severe (drug-resistant) epilepsy and epilepsy in other serious illnesses is multidimensional and requires consideration of both physical symptoms and psychosocial distress that require individualized treatment. Palliative care offers a holistic approach to disease that focuses on all dimensions of suffering to maintain quality of life. Integration of a palliative care mind- and skillset in the management of severe epilepsy and epilepsy in other serious illnesses can provide person-centered care and support for families and caregivers.

2.
Brain Sci ; 13(12)2023 Nov 22.
Article in English | MEDLINE | ID: mdl-38137064

ABSTRACT

Idiopathic intracranial hypertension (IIH) has its highest prevalence among women of childbearing age and therefore frequently coincides with pregnancy. This retrospective cohort study aimed to explore the impact of pregnancy on the clinical course, ophthalmologic findings and on the therapeutic management of IIH patients. Individual patient records were reviewed for neuro-ophthalmologic findings, treatment strategy, adherence to therapy and pregnancy complications. Sixteen patients with 19 documented pregnancies were identified. The visual acuity, visual field defects and the grade of papilledema at baseline and after pregnancy were compared. The visual acuity and visual field mean deviation at baseline and at follow-up after pregnancy did not significantly differ. Papilledema at baseline was more pronounced in patients who had been diagnosed with IIH during pregnancy than in patients with established IIH. In this cohort, the visual acuity and the visual field were not lastingly impacted by pregnancy. The adherence to therapy was low, with 69% discontinuing treatment or medication.

3.
Neurol Sci ; 44(6): 2131-2135, 2023 Jun.
Article in English | MEDLINE | ID: mdl-36689008

ABSTRACT

BACKGROUND: Neurofilament light chain (NfL) reflects axonal damage in neurological disorders. It has recently been evaluated in idiopathic intracranial hypertension (IIH). A biomarker indicating the severity of optic nerve damage in IIH could support diagnostic accuracy and therapeutic decisions. METHODS: We retrospectively reviewed NfL concentrations in the cerebrospinal fluid (CSF) of 35 IIH patients and 12 healthy controls, who had received diagnostic workup for IIH in our clinic. The diagnosis of IIH was made according to the modified Friedman criteria for IIH and for IIH without papilledema Friedman DI et al Neurol 81:1159-1165 (2013) [1]. NfL in the CSF (CSF-NfL) was correlated with the severity of papilledema and with CSF opening pressure. RESULTS: CSF-NfL correlated with CSF opening pressure at the time of collection. In patients with IIH and moderate or severe papilledema, CSF-NfL was significantly increased compared to patients with mild or no papilledema. Healthy controls with raised intracranial pressure showed no relevant elevation of CSF-NfL. CONCLUSION: CSF-NfL appears to correlate with the severity of papilledema in IIH and with CSF opening pressure and may therefore be a predictor of optic nerve damage in IIH patients.


Subject(s)
Intracranial Hypertension , Papilledema , Pseudotumor Cerebri , Humans , Intermediate Filaments , Intracranial Hypertension/complications , Intracranial Hypertension/diagnosis , Papilledema/complications , Papilledema/diagnosis , Pseudotumor Cerebri/complications , Pseudotumor Cerebri/diagnosis , Retrospective Studies
4.
Brain Sci ; 12(12)2022 Dec 10.
Article in English | MEDLINE | ID: mdl-36552156

ABSTRACT

Palliative care adds significant burdens to healthcare workers. In neuropalliative care (NPC), additional challenges include patient symptom burdens, such as impairments in mobility, cognition, and communication. After one year of operating the first NPC ward in Germany, we assessed burdens, resources, and the number of deaths perceived as stressful. NPC physicians and nursing staff were compared with the team of other neurological wards, including a stroke unit. The assessment took place between March 2022 and May 2022. All 64 team members were contacted; the responder rate was 81%. The perceived burden was high but did not differ between groups. There were no differences between the NPC- and the neurological wards in the number of deaths perceived as stressful. However, rather than the number of deaths, the circumstances of dying influence the perceived distress. The resources mentioned were similar between groups, with the team and private life being most important. Communication difficulties were frequently cited as stressful, whereas successful communication was identified as a resource.

5.
Brain Sci ; 12(11)2022 Nov 04.
Article in English | MEDLINE | ID: mdl-36358424

ABSTRACT

A unique structure of care for neurological inpatients with significant palliative care (PC) needs was established in the Department of Neurology at the Charité-Universitätsmedizin Berlin in 2021: a specialized neuropalliative care (NPC) unit. After one year, we provide an overview of the concept and the patients' characteristics. METHODS: We retrospectively analyzed the characteristics of patients treated in our NPC unit between February 2021-February 2022. Data were extracted from medical records and PC assessment including diagnosis, mode of admission and discharge, length of stay, and palliative symptoms. Data are presented as averages with a 95% confidence interval [lower limit; upper limit] or percentage (absolute number). RESULTS: We included 143 patients (52% (75) female, 67.9 years [65.6; 70.2]). Patients were admitted from general wards (48%; 68), their homes (22%; 32), intensive care units (16%; 23) or emergency departments (14%; 20). The main diagnoses were tumors of the nervous system (39%; 56), neurodegenerative diseases (30%; 43), neurologic complications (13%; 19) and cerebrovascular diseases (12%; 17). Complaints most frequently rated as severely to overwhelmingly burdensome were motor- or fatigue-associated problems, problems communicating, dysphagia and pain. The average length of stay was 13.7 days [12.2; 15.2]. Forty-five percent (64) of patients were discharged without further PC, 17% (24) were referred to a hospice and 13% (18) were discharged with outpatient PC. Five percent (7) were referred to neurorehabilitation and 21% (30) of patients died. CONCLUSIONS: Our NPC unit is a new model of care for neurological patients with substantial PC needs especially within the structures of a highly specialized and individualized medicine.

6.
J Neurol ; 269(5): 2707-2714, 2022 May.
Article in English | MEDLINE | ID: mdl-34727204

ABSTRACT

BACKGROUND: When treating patients with epileptic seizures in the emergency room (ER), it is of paramount importance to rapidly assess whether the seizure was acute symptomatic or unprovoked as the former points to a potentially life-threatening underlying condition. In this study, we seek to identify predictors and analyze characteristics of acute symptomatic seizures (ASS). METHODS: Data from patients presenting with seizures to highly frequented ERs of two sites of a university hospital were analyzed retrospectively. Seizures were classified as acute symptomatic or unprovoked according to definitions of the International League Against Epilepsy. Univariate and multivariate analysis were conducted to identify predictors; furthermore, characteristics of ASS were assessed. RESULTS: Finally, 695 patients were included, 24.5% presented with ASS. Variables independently associated with ASS comprised male sex (OR 3.173, 95% CI 1.972-5.104), no prior diagnosis of epilepsy (OR 11.235, 95% CI 7.195-17.537), and bilateral/generalized tonic-clonic seizure semiology (OR 2.982, 95% CI 1.172-7.588). Alcohol withdrawal was the most common cause of ASS (74.1%), with hemorrhagic stroke being the second most prevalent etiology. Neuroimaging was performed more often in patients with the final diagnosis of ASS than in those with unprovoked seizures (82.9% vs. 67.2%, p < 0.001). Patients with ASS were more likely to receive acute antiseizure medication in the ER (55.9% vs. 30.3%, p < 0.001). CONCLUSIONS: In one quarter of patients presenting to the ER after an epileptic fit, the seizure had an acute symptomatic genesis. The independently associated variables may help to early identify ASS and initiate management of the underlying condition.


Subject(s)
Alcoholism , Epilepsy , Substance Withdrawal Syndrome , Alcoholism/complications , Emergency Service, Hospital , Epilepsy/diagnosis , Humans , Male , Retrospective Studies , Seizures/diagnosis , Seizures/drug therapy , Seizures/epidemiology
8.
Front Neurol ; 12: 674114, 2021.
Article in English | MEDLINE | ID: mdl-34408720

ABSTRACT

Background: Acute and unexpected hospitalization can cause serious distress, particularly in patients with palliative care needs. Nevertheless, the majority of neurological inpatients receiving palliative care are admitted via an emergency department. Objective: Identification of potentially avoidable causes leading to acute hospitalization of patients with neurological disorders or neurological symptoms requiring palliative care. Methods: Retrospective analysis of medical records of all patients who were admitted via the emergency department and received palliative care in a neurological ward later on (n = 130). Results: The main reasons for acute admission were epileptic seizures (22%), gait disorders (22%), disturbance of consciousness (20%), pain (17%), nutritional problems (17%), or paresis (14%). Possible therapy limitations, (non)existence of a patient decree, or healthcare proxy was documented in only 31%. Primary diagnoses were neoplastic (49%), neurodegenerative (30%), or cerebrovascular (18%) diseases. Fifty-nine percent were directly admitted to a neurological ward; 25% needed intensive care. On average, it took 24 h until the palliative care team was involved. In contrast to initially documented problems, key challenges identified by palliative care assessment were psychosocial problems. For 40% of all cases, a specialized palliative care could be organized. Conclusion: Admissions were mainly triggered by acute events. Documentation of the palliative situation and treatment limitations may help to prevent unnecessary hospitalization. Although patients present with a complex symptom burden, emergency department assessment is not able to fully address multidimensionality, especially concerning psychosocial problems. Prospective investigations should develop short screening tools to identify palliative care needs of neurological patients already in the emergency department.

9.
Epilepsia ; 62(9): 2133-2141, 2021 09.
Article in English | MEDLINE | ID: mdl-34328218

ABSTRACT

OBJECTIVE: In focal epilepsy, data on the etiology-specific response to antiseizure medication (ASM) are surprisingly sparse. In this study, we sought to reappraise whether seizure outcome of pharmacological treatment is linked to the underlying etiology. Furthermore, we assessed ASM load with respect to the cause of epilepsy. METHODS: Data were retrospectively obtained from the electronic database of the three sites of an academic adult epilepsy outpatient clinic. For each patient, presumed cause of epilepsy was categorized into one of nine etiological groups. Individual drug loads were calculated according to the 2020 World Health Organization Center for Drug Statistics Methodology ATC/DDD Index. Univariate and multivariate analyses were conducted to explore the association between different etiologies and outcome regarding 12-month seizure freedom as well as ASM load. RESULTS: A total of 591 patients with focal epilepsy were included in the final analysis. Ischemic stroke was the etiology with the highest rate of 12-month terminal seizure freedom (71.2%, 95% confidence interval [CI] = 57.9-82.2) and, considering all etiological groups, was an independent predictor of seizure freedom (odds ratio = 2.093, 95% CI = 1.039-4.216). The lowest rates of seizure freedom were observed in patients with hippocampal sclerosis (28.2%, 95% CI = 15.0-44.9) and malformation of cortical development (16.7%, 95% CI = 2.1-48.4). In patients with ischemic stroke, median ASM load (1.0, interquartile range [IQR] = .5-1.8) was significantly lower compared to that in patients with hippocampal sclerosis (median = 1.8, IQR = 1.2-3.0, p = .008) and brain tumors (median = 1.7, IQR = .7-3.2, p = .049). SIGNIFICANCE: Response to treatment with ASM is highly etiology-specific and best in patients with epilepsy due to ischemic stroke. Interestingly, this most favorable treatment outcome can be achieved by the lowest ASM load considering all etiological groups. In focal epilepsy, etiology should be taken into account when counseling patients about their expected seizure outcome with pharmacological treatment and when tailoring initial ASM doses.


Subject(s)
Epilepsies, Partial , Anticonvulsants/therapeutic use , Epilepsies, Partial/drug therapy , Epilepsies, Partial/etiology , Epilepsy/drug therapy , Humans , Ischemic Stroke , Pharmaceutical Preparations , Retrospective Studies , Sclerosis , Seizures/drug therapy , Seizures/etiology , Stroke/complications , Stroke/drug therapy , Treatment Outcome
10.
Acta Neurol Scand ; 144(3): 288-295, 2021 Sep.
Article in English | MEDLINE | ID: mdl-33977526

ABSTRACT

OBJECTIVES: In genetic generalized epilepsies (GGE), valproic acid (VPA) is the most efficacious compound. However, due to teratogenicity and increased risk for impaired cognitive development after intrauterine exposure, its use in women of fertile age is strictly regulated but sometimes unavoidable. METHODS: All patients with GGE treated at the outpatient clinic of a tertiary epilepsy center with at least one visit between January 2015 and April 2020 were included in this retrospective study. The rate of women aged 18 to 49 years taking VPA was compared to that of men of the same age group and to women > 49 years. Furthermore, in each group, clinical variables associated with VPA use were sought. RESULTS: Twenty-eight out of 125 women of fertile age (22%) were treated with VPA, compared to 28 out of 56 men ≤ 49 years (50%; p = .002) and to 22 out of 40 female patients > 49 years (55%; p < .001). VPA dose was lower in fertile women compared to men, with no difference in seizure freedom rates. In women ≤ 49 years, multivariate analysis demonstrated age as the only variable independently associated with VPA use (OR 1.095; 95% CI 1.036-1.159). In the other two groups, no associated variables were identified. CONCLUSIONS: Despite warnings with respect to teratogenicity and impaired cognitive development with VPA, from 2015 to 2020, almost every fourth women of fertile age with GGE received this compound. Inevitably lower VPA doses in these women seem sufficient for favorable seizure freedom rates.


Subject(s)
Epilepsy, Generalized , Epilepsy , Valproic Acid , Anticonvulsants/adverse effects , Epilepsy/drug therapy , Epilepsy/genetics , Epilepsy, Generalized/drug therapy , Epilepsy, Generalized/genetics , Female , Humans , Infant, Newborn , Male , Retrospective Studies , Valproic Acid/adverse effects
11.
Ann Clin Transl Neurol ; 8(5): 1049-1063, 2021 05.
Article in English | MEDLINE | ID: mdl-33792208

ABSTRACT

OBJECTIVE: To determine whether serum creatine kinase activity (CK) and serum creatinine concentration (Crn) are prognostic and predictive biomarkers for disease severity, disease progression, and nusinersen treatment effects in adult patients with 5q-associated spinal muscular atrophy (SMA). METHODS: Within this retrospective, multicenter observational study in 206 adult patients with SMA, we determined clinical subtypes (SMA types, ambulatory ability) and repeatedly measured CK and Crn and examined disease severity scores (Hammersmith Functional Motor Scale Expanded, Revised Upper Limb Module, and revised Amyotrophic Lateral Sclerosis Functional Rating Scale). Patients were followed under nusinersen treatment for 18 months. RESULTS: CK and Crn differed between clinical subtypes and correlated strongly with disease severity scores (e.g., for Hammersmith Functional Motor Scale Expanded: (CK) ρ = 0.786/ (Crn) ρ = 0.558). During the 18 months of nusinersen treatment, CK decreased (∆CK = -17.56%, p < 0.0001), whereas Crn slightly increased (∆Crn = +4.75%, p < 0.05). INTERPRETATION: Serum creatine kinase activity and serum creatinine concentration reflect disease severity of spinal muscular atrophy and are promising biomarkers to assess patients with spinal muscular atrophy during disease course and to predict treatment response. The decrease of creatine kinase activity, combined with the tendency of creatinine concentration to increase during nusinersen treatment, suggests reduced muscle mass wasting with improved muscle energy metabolism.


Subject(s)
Creatine Kinase/blood , Creatinine/blood , Muscular Atrophy, Spinal/blood , Muscular Atrophy, Spinal/drug therapy , Oligonucleotides/pharmacology , Adolescent , Adult , Aged , Biomarkers/blood , Disease Progression , Female , Humans , Male , Middle Aged , Muscular Atrophy, Spinal/diagnosis , Muscular Atrophy, Spinal/physiopathology , Patient Acuity , Prognosis , Retrospective Studies , Young Adult
12.
J Neurol ; 268(11): 4248-4257, 2021 Nov.
Article in English | MEDLINE | ID: mdl-33871711

ABSTRACT

BACKGROUND: Electroencephalography (EEG) significantly contributes to the neuroprognostication after resuscitation from cardiac arrest. Recent studies suggest that the prognostic value of EEG is highest for continuous recording within the first days after cardiac arrest. Early continuous EEG, however, is not available in all hospitals. In this observational study, we sought to evaluate the predictive value of a 'late' EEG recording 5-14 days after cardiac arrest without sedatives. METHODS: We retrospectively analyzed EEG data in consecutive adult patients treated at the medical intensive care units (ICU) of the Charité-Universitätsmedizin Berlin. Outcome was assessed as cerebral performance category (CPC) at discharge from ICU, with an unfavorable outcome being defined as CPC 4 and 5. RESULTS: In 187 patients, a 'late' EEG recording was performed. Of these patients, 127 were without continuous administration of sedative agents for at least 24 h before the EEG recording. In this patient group, a continuously suppressed background activity < 10 µV predicted an unfavorable outcome with a sensitivity of 31% (95% confidence interval (CI) 20-45) and a specificity of 99% (95% CI 91-100). In patients with suppressed background activity and generalized periodic discharges, sensitivity was 15% (95% CI 7-27) and specificity was 100% (95% CI 94-100). GPDs on unsuppressed background activity were associated with a sensitivity of 42% (95% CI 29-46) and a specificity of 92% (95% CI 82-97). CONCLUSIONS: A 'late' EEG performed 5 to 14 days after resuscitation from cardiac arrest can aide in prognosticating functional outcome. A suppressed EEG background activity in this time period indicates poor outcome.


Subject(s)
Coma , Heart Arrest , Adult , Electroencephalography , Heart Arrest/complications , Heart Arrest/therapy , Humans , Predictive Value of Tests , Prognosis , Retrospective Studies
13.
Acta Neurol Scand ; 143(4): 421-429, 2021 Apr.
Article in English | MEDLINE | ID: mdl-33210727

ABSTRACT

OBJECTIVES: To identify demographic and clinical variables independently associated with patients' decisions against their physicians' recommendations for resective epilepsy surgery or further scalp video-EEG monitoring (sca-VEM), semi-invasive (sem-)VEM with foramen ovale and/or peg electrodes, and invasive (in-)VEM. METHODS: Consecutive patients, who underwent presurgical assessment with at least one sca-VEM between 2010 and 2014, were included into this retrospective analysis. Multivariate analysis was used to identify independent variables associated with patients' decisions. RESULTS: Within the study period, 352 patients underwent 544 VEM sessions comprising 451 sca-, 36 sem-, and 57 in-VEMs. Eventually, 96 patients were recommended resective surgery, and 106 were ineligible candidates; 149 patients denied further necessary VEMs; thus, no decision could be made. After sca- or additional sem-VEM, nine out of 51 eligible patients (17.6%) rejected resection. One hundred and ten patients were recommended in-VEM, 52 of those (47.2%) declined. Variables independently associated with rejection of in-VEM comprised intellectual disability (OR 4.721, 95% CI 1.047-21.284), extratemporal focal aware non-motor seizures ("aura") vs. no "aura" (OR 0.338, 95% CI 0.124-0.923), and unilateral or bilateral vs. no MRI lesion (OR 0.248, 95% CI 0.100-0.614 and 0.149, 95% CI 0.027-0.829, respectively). CONCLUSIONS: During and after presurgical evaluation, patients with intractable focal epilepsy declined resections and intracranial EEGs, as recommended by their epileptologists, in almost 20% and 50% of cases. This calls for early and thorough counseling of patients on risks and benefits of epilepsy surgery. Future prospective studies should ask patients in depth for specific reasons why they decline their physicians' recommendations.


Subject(s)
Decision Making , Drug Resistant Epilepsy/psychology , Drug Resistant Epilepsy/surgery , Patient Participation/psychology , Physician's Role/psychology , Adolescent , Adult , Aged , Child , Child, Preschool , Drug Resistant Epilepsy/diagnostic imaging , Electroencephalography/methods , Female , Humans , Magnetic Resonance Imaging/methods , Male , Middle Aged , Physician-Patient Relations , Prospective Studies , Retrospective Studies , Young Adult
14.
Z Orthop Unfall ; 158(5): 535-555, 2020 Oct.
Article in German | MEDLINE | ID: mdl-32645744

ABSTRACT

Iatrogenic nerve injuries are rare complications of total hip and knee arthroplasty, which may cause chronic pain and loss of function, severely affecting the patient's daily activities and quality of life. Nerves "at risk" include the sciatic nerve, the femoral nerve, the lateral femoral cutaneous nerve and the superior gluteal nerve during total hip arthroplasty, and the infrapatellar branch of the saphenous nerve as well as the peroneal nerve during total knee arthroplasty. Multiple procedure-related and patient-related factors have been identified to modify the risk of nerve injury in the course of lower limb joint replacement surgery. These include the surgeon's skills, the surgical approach, the type of implant fixation, the intraoperative positioning of the patient, as well as pre-existing scars, the patient's sex, age and comorbidities. Diagnostic and therapeutic approaches should be based on the aetiology of the lesion: iatrogenic nerve lesions can result from direct (compression or transection) and/or indirect (traction, ischemia) trauma. The majority of nerve injuries encountered in hip or knee arthroplasty has been referred to as "minor" nerve lesions, which generally respond very well to non-operative treatment. "Major" nerve lesions, such as complete motor nerve transection, may result in lifelong impairment. Any perioperatively encountered neurological deficit requires a meticulous diagnostic work-up and an individually tailored treatment strategy, respecting aetiology and anatomic site of the nerve lesion as well as the individual patient's needs and comorbidities.


Subject(s)
Arthroplasty, Replacement, Knee , Trauma, Nervous System , Arthroplasty, Replacement, Hip/adverse effects , Arthroplasty, Replacement, Knee/adverse effects , Humans , Quality of Life , Risk Factors
15.
Ann Neurol ; 87(3): 405-418, 2020 03.
Article in English | MEDLINE | ID: mdl-31900946

ABSTRACT

OBJECTIVE: Leucine-rich glioma-inactivated 1 (LGI1) encephalitis is the second most common antibody-mediated encephalopathy, but insight into the intrathecal B-cell autoimmune response, including clonal relationships, isotype distribution, frequency, and pathogenic effects of single LGI1 antibodies, has remained limited. METHODS: We cloned, expressed, and tested antibodies from 90 antibody-secreting cells (ASCs) and B cells from the cerebrospinal fluid (CSF) of several patients with LGI1 encephalitis. RESULTS: Eighty-four percent of the ASCs and 21% of the memory B cells encoded LGI1-reactive antibodies, whereas reactivities to other brain epitopes were rare. All LGI1 antibodies were of IgG1, IgG2, or IgG4 isotype and had undergone affinity maturation. Seven of the overall 26 LGI1 antibodies efficiently blocked the interaction of LGI1 with its receptor ADAM22 in vitro, and their mean LGI1 signal on mouse brain sections was weak compared to the remaining, non-ADAM22-competing antibodies. Nevertheless, both types of LGI1 antibodies increased the intrinsic cellular excitability and glutamatergic synaptic transmission of hippocampal CA3 neurons in slice cultures. INTERPRETATION: Our data show that the patients' intrathecal B-cell autoimmune response is dominated by LGI1 antibodies and that LGI1 antibodies alone are sufficient to promote neuronal excitability, a basis of seizure generation. Fundamental differences in target specificity and antibody hypermutations compared to the CSF autoantibody repertoire in N-methyl-D-aspartate receptor encephalitis underline the clinical concept that autoimmune encephalitides are very distinct entities. Ann Neurol 2020;87:405-418.


Subject(s)
Antibodies, Monoclonal/pharmacology , Autoantibodies/pharmacology , Intracellular Signaling Peptides and Proteins/immunology , Neurons/physiology , ADAM Proteins/drug effects , Aged , Animals , Antibodies, Monoclonal/cerebrospinal fluid , Autoantibodies/cerebrospinal fluid , CA3 Region, Hippocampal/physiology , Cells, Cultured , Encephalitis/cerebrospinal fluid , Encephalitis/immunology , Female , Hashimoto Disease/cerebrospinal fluid , Hashimoto Disease/immunology , Humans , Immunoglobulin Isotypes , Intracellular Signaling Peptides and Proteins/antagonists & inhibitors , Intracellular Signaling Peptides and Proteins/genetics , Male , Mice , Mice, Knockout , Middle Aged , Nerve Tissue Proteins/drug effects , Rats , Synaptic Transmission/drug effects
16.
Epilepsia ; 60(2): 211-219, 2019 02.
Article in English | MEDLINE | ID: mdl-30653659

ABSTRACT

OBJECTIVE: Despite the obvious advantages of resective surgery in patients with drug-resistant focal epilepsy, namely high probability of seizure freedom, decreased mortality, and increased quality of life, referral rates from physicians and approval rates by patients for presurgical assessment remain constantly low. METHODS: In the outpatient clinics of a tertiary epilepsy center, checklists were implemented asking treating epileptologists whether they recommended presurgical evaluation with noninvasive video-electroencephalographic monitoring to adult patients with drug-resistant focal epilepsy and asking respective patients whether they followed this recommendation. RESULTS: Of 185 eligible patients, 80 (43%) were recommended presurgical evaluation by their epileptologists, and 24 (30%) of these patients consented. Nineteen of all patients (10%) actually underwent noninvasive presurgical assessment, and nine of these eventually proceeded to resection. The most frequent reason for nonreferral by epileptologists was their subjective appraisal of seizure frequency as low (31%), whereas patients declined most often due to overall fear of brain surgery (50%). Variables independently associated with nonreferral by epileptologists comprised older age of patients at questioning (odds ratio [OR] = 1.03), no previous evaluation for epilepsy surgery (OR = 4.04), the presence of legal guardianship (OR = 4.29), and ≥11 years of professional experience by the treating epileptologist (OR = 4.62). Independent predictors for patients' rejection of presurgical evaluation were older age at questioning (OR = 1.08), lifetime number of antiepileptic drugs ≥ 5 (OR = 4.47), presence of focal aware seizures (OR = 4.37), and absence of focal seizures with impaired awareness (OR = 11.24). SIGNIFICANCE: In both epileptologists and patients with difficult-to-treat epilepsy, we found high decision rates against presurgical assessment. Some reasons given by physicians for not recommending presurgical evaluation to patients may be understandable; others need further exploration. On the patients' side, early and thorough counseling on risks and benefits of epilepsy surgery is necessary to increase understanding and acceptance.


Subject(s)
Drug Resistant Epilepsy/surgery , Epilepsies, Partial/surgery , Epilepsy/surgery , Seizures/surgery , Adult , Aged , Anticonvulsants/therapeutic use , Electroencephalography/methods , Female , Humans , Male , Neurosurgical Procedures/methods , Quality of Life , Treatment Outcome
17.
Epilepsia ; 59(8): 1549-1556, 2018 08.
Article in English | MEDLINE | ID: mdl-29943451

ABSTRACT

OBJECTIVE: The objective of this study was to evaluate effectiveness, retention, and tolerability of brivaracetam (BRV) in genetic generalized epilepsies (GGE) in clinical practice. METHODS: A multicenter, retrospective cohort study recruiting all patients that started BRV in 2016 and 2017. RESULTS: A total of 61 patients (mean age = 29.8, range = 9-90 years, 41 female [67%]) were treated with BRV. They were difficult to control, with 2.4 failed antiepileptic drugs (AEDs) in the past, taking 1.9 AEDs on average at baseline. The length of exposure to BRV ranged from 7 days to 24 months, with a mean retention time of 7.9 months, resulting in a total exposure time to BRV of 483 months. The retention rate was 82% at 3 months and 69% at 6 months. Efficacy at 3 months was 36% (50% responder rate), with 25% seizure-free for 3 months. Patients with juvenile myoclonic epilepsy showed a responder rate of 60%, with 40% being free of any seizures. Long-term 50% responder rate was present in 17 patients (28%; 11 seizure-free [18%]) for >6 months and in 14 patients (23%; 10 seizure-free [16%]) for >12 months. Treatment-emergent adverse events were observed in 26% of the patients, with the most common being somnolence, ataxia, and psychobehavioral adverse events. Use of intravenous BRV with bolus injection of 200-300 mg in two females with absence status epilepticus was well tolerated, but did not result in cessation of status epilepticus. SIGNIFICANCE: Use of BRV in GGE is well tolerated, and 50% responder rates are similar to those observed in the regulatory trials for focal epilepsies. An immediate switch from levetiracetam (LEV) to BRV at a ratio of 15:1 is feasible. The occurrence of psychobehavioral adverse events seems less prominent than under LEV, and a switch to BRV can be considered in patients with LEV-induced adverse events.


Subject(s)
Anticonvulsants/administration & dosage , Epilepsy, Generalized/drug therapy , Pyrrolidinones/administration & dosage , Treatment Outcome , Adolescent , Adult , Aged , Aged, 80 and over , Child , Cohort Studies , Female , Humans , Injections, Intravenous , Male , Middle Aged , Product Surveillance, Postmarketing , Young Adult
18.
Front Neurol ; 9: 401, 2018.
Article in English | MEDLINE | ID: mdl-29922217

ABSTRACT

Purpose: This study aimed to assess alcohol consumption and the occurrence of alcohol-related seizures in patients with epilepsy within the last 12 months. Methods: In an epilepsy outpatient clinic, a standardized questionnaire was used to collect data retrospectively from consecutive adult epilepsy patients who had been suffering from the disease for at least 1 year. Logistic regression analyses were performed to identify independent predictors. Results: A total of 310 patients with epilepsy were included. Of these, 204 subjects (65.8%) consumed alcohol within the last 12 months. Independent predictors for alcohol use were antiepileptic drug monotherapy (OR 1.901) and physicians' advice that a light alcohol intake is harmless (OR 4.102). Seizure worsening related to alcohol consumption was reported by 37 of the 204 patients (18.1%) who had used alcohol. All 37 subjects had consumed large quantities of alcohol prior to the occurrence of alcohol-related seizures regardless of their usual alcohol-drinking behavior. The amount of alcohol intake prior to alcohol-related seizures was at least 7 standard drinks, which is equivalent to 1.4 L of beer or 0.7 L of wine. In 95% of cases, alcohol-related seizures occurred within 12 h after cessation of alcohol intake. Independent predictors for alcohol-related seizures were generalized genetic epilepsy (OR 5.792) and chronic heavier alcohol use (OR 8.955). Conclusions: Two-thirds of interviewed subjects had consumed alcohol within the last 12 months. This finding may be an underestimate due to patients' self-reporting and recall error. In all cases, the occurrence of alcohol related-seizures was associated with timely consumption of considerably large amounts of alcohol. Thus, a responsible alcohol intake seems to be safe for most patients with epilepsy. However, subjects with epilepsy and especially those with generalized genetic epilepsy should be made aware of an increased risk for seizures related to heavy alcohol consumption. Factors accompanying acute heavy alcohol intake such as altered sleep architecture, impaired adherence to antiepileptic medication, and metabolic disturbances may further facilitate the occurrence of seizures.

19.
J Neurol Neurosurg Psychiatry ; 89(6): 603-610, 2018 06.
Article in English | MEDLINE | ID: mdl-29348303

ABSTRACT

OBJECTIVES: Depending on patient age at onset, absence epilepsy is subdivided into childhood and juvenile forms. Absence seizures can occur several times per day (pyknoleptic course) or less frequently than daily (non-pyknoleptic course). Seizures typically terminate before adulthood, but a quarter of patients need ongoing treatment beyond adolescence. Little is known about their long-term seizure and psychosocial outcome. METHODS: Files of 135 outpatients with absence epilepsy (76 females; 123 had additional generalised tonic-clonic seizures) were retrospectively analysed after a median follow-up of 45.4 years (IQR: 31.9-56.2). Eighty-two subjects completed an additional interview. Patients were dichotomised according to age at epilepsy onset (childhood: n=82; juvenile: n=53) and course of absence seizures (pyknoleptic: n=80; non-pyknoleptic: n=55). RESULTS: Among all patients, 53% achieved 5-year terminal seizure remission, 16% without antiepileptic medication. Median age at last seizure was lower in patients with childhood onset of absence epilepsy (37.7 years) versus juvenile onset (44.4 years; P≤0.01). However, rates and duration of terminal seizure remission were similar. Pyknoleptic versus non-pyknoleptic course of absence seizures made no difference for long-term seizure outcome. Multivariate analysis identified only higher age at investigation to be associated with terminal 5-year seizure remission. Regarding aspects of psychosocial outcome, there were no significant differences between the respective subgroups. CONCLUSIONS: These data indicate that if absence epilepsy persists beyond adolescence, long-term seizure and psychosocial outcome do not differ between childhood and juvenile onset or between pyknoleptic and non-pyknoleptic course of absence epilepsy. However, higher patient age increases the chance of terminal seizure remission.


Subject(s)
Epilepsy, Absence/epidemiology , Adolescent , Adult , Age of Onset , Aged , Aged, 80 and over , Epilepsy, Absence/diagnosis , Epilepsy, Absence/therapy , Female , Follow-Up Studies , Humans , Male , Middle Aged , Remission, Spontaneous , Retrospective Studies , Time Factors , Young Adult
20.
Epilepsia ; 58(7): 1244-1250, 2017 07.
Article in English | MEDLINE | ID: mdl-28464258

ABSTRACT

OBJECTIVE: Until now, it has been unclear if the three subsyndromes of adolescent-onset generalized genetic epilepsy (GGE) differ in long-term prognosis. Therefore, this study aimed to compare long-term seizure outcome in juvenile absence epilepsy (JAE), juvenile myoclonic epilepsy (JME), and epilepsy with generalized tonic-clonic seizures alone (EGTCS). METHODS: This retrospective study is based on the archive of an institutional tertiary care outpatient clinic for adult patients with epilepsy. Charts of 870 epilepsy outpatients were reviewed among whom 176 had adolescent-onset GGE (53 JAE, 66 JME, 57 EGTCS). Median patient age at investigation was 60 years; median follow-up time was 42.5 years. If possible, GGE patients were additionally interviewed on psychosocial and clinical variables. RESULTS: Age at first seizure was significantly higher in EGTCS patients (median 18 years) than in patients with JAE or JME (14 years each; p ≤ 0.001). Long-term seizure outcome hardly differed between the three subsyndromes. At the end of follow-up, 60% of all patients were in 5-year terminal seizure remission, and in 14%, epilepsy even had resolved (>10 years without seizures, >5 years without pharmacotherapy). Twenty percent of patients had persistent seizures during the last year of follow-up. Across all patients, 23% reported a psychiatric comorbidity, 87% had married, and 57% had achieved university entrance qualification. SIGNIFICANCE: Long-term outcome was shown to be highly similar across all subsyndromes of adolescent-onset GGE. Even in a selection of difficult-to-treat epilepsy patients still attending an adult epilepsy clinic, most become seizure-free. To confirm these findings, prospective studies are needed.


Subject(s)
Epilepsy, Absence/diagnosis , Epilepsy, Absence/genetics , Epilepsy, Generalized/diagnosis , Epilepsy, Generalized/genetics , Epilepsy, Tonic-Clonic/diagnosis , Epilepsy, Tonic-Clonic/genetics , Myoclonic Epilepsy, Juvenile/diagnosis , Myoclonic Epilepsy, Juvenile/genetics , Adolescent , Adult , Anticonvulsants/therapeutic use , Epilepsy, Absence/drug therapy , Epilepsy, Generalized/drug therapy , Epilepsy, Tonic-Clonic/drug therapy , Female , Humans , Male , Middle Aged , Myoclonic Epilepsy, Juvenile/drug therapy , Prognosis , Retrospective Studies
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