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  1. Book ; Conference proceedings: 7th European Congress on Epileptology

    Kälviäinen, Reetta

    Helsinki, Finland, 2 - 6 July 2006

    (Epilepsia ; 47,S3)

    2006  

    Event/congress European Congress on Epileptology (7, 2006, Helsinki)
    Author's details guest ed. Reetta Kälviäinen
    Series title Epilepsia ; 47,S3
    Collection
    Language English
    Size 272 S.
    Publisher Blackwell Publ
    Publishing place S.l.
    Publishing country United States
    Document type Book ; Conference proceedings
    Note Papierausdruck der Internetausg.
    HBZ-ID HT015752077
    Database Catalogue ZB MED Medicine, Health

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  2. Article ; Online: Comorbidities in patients with Unverricht-Lundborg disease (EPM1).

    Sipilä, Jussi O T / Kälviäinen, Reetta

    Acta neurologica Scandinavica

    2022  Volume 146, Issue 5, Page(s) 690–693

    Abstract: Background: Unverricht-Lundborg disease (EPM1) typically leads to accumulating disability. Disability may also be caused by comorbidities but there are no data available on these.: Aims of the study: To investigate the frequency of comorbidities in ... ...

    Abstract Background: Unverricht-Lundborg disease (EPM1) typically leads to accumulating disability. Disability may also be caused by comorbidities but there are no data available on these.
    Aims of the study: To investigate the frequency of comorbidities in EPM1.
    Methods: Comorbidity data of a previously described cohort of 135 Finnish patients with EPM1 were retrieved from neurological, surgical (including subspecialities), internal medicine (including subspecialities) and intensive care patient charts of the treating hospitals.
    Results: Mean follow-up time was 31.4 years (SD 12.4 years, range 6.8-57.8 years), during which at least one comorbidity was observed in 107 patients (79%) and three or more in 53 (39%). The most common diagnostic categories were external injuries, mental and behavioural disorders and endocrine, nutritional and metabolic diseases. The most common single comorbid diagnosis was a fracture of the ankle (in 19% of all patients). The second most common single comorbid diagnosis in the cohort was diabetes (in 13% of all patients), and the third was depression, recorded for 13% of the cohort. Malignancies and cardiovascular end-organ damage were rare, whereas phimosis/paraphimosis appeared more common than in general population.
    Conclusions: Patients with EPM1 often have comorbidities. Trauma and mental health risks should be especially followed and acted upon. Further studies are needed to more accurately comorbidity risks, characteristics and patient needs.
    MeSH term(s) Cohort Studies ; Comorbidity ; Finland/epidemiology ; Humans ; Male ; Unverricht-Lundborg Syndrome/pathology
    Language English
    Publishing date 2022-09-13
    Publishing country Denmark
    Document type Journal Article
    ZDB-ID 90-5
    ISSN 1600-0404 ; 0001-6314
    ISSN (online) 1600-0404
    ISSN 0001-6314
    DOI 10.1111/ane.13706
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  3. Article ; Online: When is it time for palliative and end-of-life care in status epilepticus?

    Kälviäinen, Reetta / Allal, Zakarya / Kantanen, Anne-Mari

    Epilepsy & behavior : E&B

    2023  Volume 141, Page(s) 109058

    Abstract: Status epilepticus (SE) is a neurological emergency characterized by high rates of short-term and long-term morbidity and mortality. Status epilepticus seems to be a marker of the severity of other underlying conditions rather than a determinant of death ...

    Abstract Status epilepticus (SE) is a neurological emergency characterized by high rates of short-term and long-term morbidity and mortality. Status epilepticus seems to be a marker of the severity of other underlying conditions rather than a determinant of death on its own. Careful diagnosis and acute treatment of complications and causes of death to SE or its underlying etiology will enable the differentiation of SE patients that would benefit from different levels of treatment intensity. All SE patients should be treated actively with first- and second-line drugs as early as possible. For cases in which seizures continue after second-line treatment, the current guidelines fail to offer possibilities other than the active path with general anesthesia and intensive care unit (ICU) care. However, the intensity of care should be evaluated before starting ICU care or in unclear cases with the time-limited trial at ICU. There are now multiple possibilities for specialty palliative SE care that include sequential and add-on use of second-line drugs and palliative sedation at the ward. If ICU care is prolonged, the patient's status needs to be constantly re-evaluated and communicated to the family. When patients exhibit multiple predictors of mortality and poor functional outcomes, they should be allowed to have a natural death in a peaceful environment without unnecessarily prolonged suffering. This paper was presented at the 8th London-Innsbruck Colloquium on Status Epilepticus and Acute Seizures held in September 2022.
    MeSH term(s) Humans ; Status Epilepticus/therapy ; Status Epilepticus/drug therapy ; Seizures/diagnosis ; Terminal Care ; Palliative Care ; Intensive Care Units ; Anticonvulsants/therapeutic use
    Chemical Substances Anticonvulsants
    Language English
    Publishing date 2023-01-04
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 2010587-3
    ISSN 1525-5069 ; 1525-5050
    ISSN (online) 1525-5069
    ISSN 1525-5050
    DOI 10.1016/j.yebeh.2022.109058
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  4. Article ; Online: Adult onset epilepsy incidence in Finland over 34 years: A nationwide registry study.

    Sipilä, Jussi O T / Kälviäinen, Reetta

    European journal of neurology

    2021  Volume 29, Issue 2, Page(s) 605–608

    Abstract: Background and purpose: The incidence of epilepsy is decreasing among the working-aged in high-income countries, but previous studies have reported conflicting results in Finland.: Methods: A nationwide population-based cross-sectional analysis was ... ...

    Abstract Background and purpose: The incidence of epilepsy is decreasing among the working-aged in high-income countries, but previous studies have reported conflicting results in Finland.
    Methods: A nationwide population-based cross-sectional analysis was made of annual epilepsy drug reimbursement rights frequency data from the Social Insurance Institution of Finland, the national authority, between 1986 and 2019. All persons at least 20 years of age living in Finland during the study period were included.
    Results: Based on the analysis of 77,939 new reimbursement rights, crude incidence was 57.4/100,000 (95% confidence interval [CI] = 57.0-57.8) person-years, and age-standardized (to the European Standard Population 2013) incidence was 51.6/100,000 person-years. Both crude (r = 0.62, p = 0.00009) and standardized (r = 0.65, p = 0.00003) incidence increased over time. Incidence increased in both men (from 66.4 to 71.6/100,000, r = 0.51, p = 0.002) and women (from 51.5 to 55.3/100,000, r = 0.68, p < 0.00001). The mean male to female incidence rate ratio was 1.28 (95% CI = 1.26-1.30, range = 1.15-1.41), but decreased during the study period (r = -0.47, p = 0.006). Incidence decreased in those 20-59 years old but increased in all older age groups. This development was similar between sexes.
    Conclusions: The incidence of adult onset epilepsy in Finland increased in people older than 60 years and decreased in the 20-59-year age group during the study period. These trends were similar between sexes. Therefore, etiological epilepsy trends in the elderly need to be studied further to plan public health measures to prevent epilepsy in this age group.
    MeSH term(s) Adult ; Aged ; Cross-Sectional Studies ; Epilepsy/epidemiology ; Female ; Finland/epidemiology ; Humans ; Incidence ; Male ; Middle Aged ; Registries ; Young Adult
    Language English
    Publishing date 2021-10-29
    Publishing country England
    Document type Journal Article
    ZDB-ID 1280785-0
    ISSN 1468-1331 ; 1351-5101 ; 1471-0552
    ISSN (online) 1468-1331
    ISSN 1351-5101 ; 1471-0552
    DOI 10.1111/ene.15141
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  5. Article: In depth behavioral phenotyping unravels complex motor disturbances in

    Pollari, Eveliina / Tegelberg, Saara / Björklund, Harry / Kälviäinen, Reetta / Lehesjoki, Anna-Elina / Haapalinna, Antti

    Frontiers in behavioral neuroscience

    2023  Volume 17, Page(s) 1325051

    Abstract: Progressive myoclonus epilepsy type 1 (EPM1) is an autosomal recessively inherited childhood-adolescence onset neurodegenerative disease caused by mutations in the cystatin B ( ...

    Abstract Progressive myoclonus epilepsy type 1 (EPM1) is an autosomal recessively inherited childhood-adolescence onset neurodegenerative disease caused by mutations in the cystatin B (
    Language English
    Publishing date 2023-12-21
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2452960-6
    ISSN 1662-5153
    ISSN 1662-5153
    DOI 10.3389/fnbeh.2023.1325051
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  6. Article ; Online: Progressive Myoclonus Epilepsies.

    Kälviäinen, Reetta

    Seminars in neurology

    2015  Volume 35, Issue 3, Page(s) 293–299

    Abstract: The progressive myoclonus epilepsies (PMEs) comprise a group of rare and heterogeneous disorders defined by the combination of action myoclonus, epileptic seizures, and progressive neurologic deterioration. Neurologic deterioration may include ... ...

    Abstract The progressive myoclonus epilepsies (PMEs) comprise a group of rare and heterogeneous disorders defined by the combination of action myoclonus, epileptic seizures, and progressive neurologic deterioration. Neurologic deterioration may include progressive cognitive decline, ataxia, neuropathy, and myopathy. The gene defects for the most common forms of PME (Unverricht-Lundborg disease, Lafora disease, several forms of neuronal ceroid lipofuscinoses, myoclonus epilepsy with ragged-red fibers [MERRF], and type 1 and 2 sialidoses) have been identified. The prognosis of a PME depends on the specific disease. Lafora disease, the neuronal ceroid lipofuscinoses, and the neuronopathic form of Gaucher disease have an invariably fatal course. In contrast, Unverricht-Lundborg disease has a much slower progression, and with adequate care many patients have a normal life span. The specific diseases that cause PME are diagnosed by recognition of their age of onset, the associated clinical symptoms, the clinical course, the pattern of inheritance, and by special investigations such as enzyme measurement, skin/muscle biopsy, or gene testing.
    MeSH term(s) Humans ; Myoclonic Epilepsies, Progressive/classification ; Myoclonic Epilepsies, Progressive/complications ; Myoclonic Epilepsies, Progressive/diagnosis ; Myoclonic Epilepsies, Progressive/genetics ; Nervous System Diseases/etiology
    Language English
    Publishing date 2015-06
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 603165-1
    ISSN 1098-9021 ; 0271-8235
    ISSN (online) 1098-9021
    ISSN 0271-8235
    DOI 10.1055/s-0035-1552620
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  7. Article ; Online: Intranasal therapies for acute seizures.

    Kälviäinen, Reetta

    Epilepsy & behavior : E&B

    2015  Volume 49, Page(s) 303–306

    Abstract: Most seizure emergencies occur outside of the hospital, and there is a need for treatment interventions that can be administered quickly and safely by nonclinical caregivers. Intranasal benzodiazepine administration does not require intravenous access ... ...

    Abstract Most seizure emergencies occur outside of the hospital, and there is a need for treatment interventions that can be administered quickly and safely by nonclinical caregivers. Intranasal benzodiazepine administration does not require intravenous access and offers rapid seizure cessation. Intranasal midazolam is faster at aborting seizure activity than rectal diazepam and quicker to administer than intravenous diazepam. Although time to seizure cessation varies from study to study, intranasal midazolam is efficacious when administered not only by emergency department personnel but also by paramedics and caregivers in out-of-hospital and home settings. Absorption of midazolam intranasal formulations appears to be relatively rapid compared to diazepam formulations. Its shorter elimination half-life may also be beneficial in that patients may more quickly return to normal function because of rapid offset of effect. On the other hand, the faster rate of elimination of midazolam may expose patients to a higher rate of seizure recurrence compared with diazepam. Two diazepam formulations and one midazolam formulation are being currently developed for intranasal use. This article is part of a Special Issue entitled "Status Epilepticus".
    MeSH term(s) Acute Disease ; Administration, Intranasal ; Anticonvulsants/administration & dosage ; Diazepam/administration & dosage ; Humans ; Lorazepam/administration & dosage ; Midazolam/administration & dosage ; Seizures/drug therapy
    Chemical Substances Anticonvulsants ; Lorazepam (O26FZP769L) ; Diazepam (Q3JTX2Q7TU) ; Midazolam (R60L0SM5BC)
    Language English
    Publishing date 2015-08
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 2010587-3
    ISSN 1525-5069 ; 1525-5050
    ISSN (online) 1525-5069
    ISSN 1525-5050
    DOI 10.1016/j.yebeh.2015.04.027
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  8. Article ; Online: Cenobamate in refractory epilepsy: Overview of treatment options and practical considerations.

    Schmitz, Bettina / Lattanzi, Simona / Vonck, Kristl / Kälviäinen, Reetta / Nashef, Lina / Ben-Menachem, Elinor

    Epilepsia open

    2023  Volume 8, Issue 4, Page(s) 1241–1255

    Abstract: Management of drug resistant epilepsy (DRE) represents a challenge to the treating clinician. This manuscript addresses DRE and provides an overview of treatment options, medical, surgical, and dietary. It addresses treatment strategies in polytherapy, ... ...

    Abstract Management of drug resistant epilepsy (DRE) represents a challenge to the treating clinician. This manuscript addresses DRE and provides an overview of treatment options, medical, surgical, and dietary. It addresses treatment strategies in polytherapy, then focuses on the role cenobamate (CNB) may play in reducing the burden of DRE while providing practical advice for its introduction. CNB is a recently approved, third generation, anti-seizure medication (ASM), a tetrazole-derived carbamate, thought to have a dual mechanism of action, through its effect on sodium channels as well as on GABA
    MeSH term(s) Humans ; Anticonvulsants ; Drug Resistant Epilepsy/drug therapy ; Epilepsies, Partial/drug therapy ; Epilepsies, Partial/chemically induced ; Carbamates/pharmacology ; Carbamates/therapeutic use ; Tetrazoles/adverse effects
    Chemical Substances Anticonvulsants ; Cenobamate (P85X70RZWS) ; Carbamates ; Tetrazoles
    Language English
    Publishing date 2023-10-03
    Publishing country United States
    Document type Journal Article ; Review
    ISSN 2470-9239
    ISSN (online) 2470-9239
    DOI 10.1002/epi4.12830
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  9. Article ; Online: The burden of chronic drug-refractory focal onset epilepsy: Can it be prevented?

    Ben-Menachem, Elinor / Schmitz, Bettina / Kälviäinen, Reetta / Thomas, Rhys H / Klein, Pavel

    Epilepsy & behavior : E&B

    2023  Volume 148, Page(s) 109435

    Abstract: Despite the many therapeutic options for epilepsy available today, a third of patients still have poorly controlled epilepsy. Over the years, their transition through lines of treatment exposes them to increased risk of disease progression, mortality, ... ...

    Abstract Despite the many therapeutic options for epilepsy available today, a third of patients still have poorly controlled epilepsy. Over the years, their transition through lines of treatment exposes them to increased risk of disease progression, mortality, morbidity, mental distress, and not least significantly impaired quality of life (QoL). The present review explores the multiple factors contributing to the impairment of health-related QoL in PWE-including both seizure-related and non seizure-related. The analysis aims to identify potential areas of intervention and strategies for a more holistic approach to epilepsy care and inform policy-makers and healthcare providers in their approach to this condition.
    MeSH term(s) Humans ; Anticonvulsants/therapeutic use ; Quality of Life ; Epilepsy/drug therapy ; Epilepsy/chemically induced ; Drug Resistant Epilepsy/drug therapy ; Seizures/drug therapy
    Chemical Substances Anticonvulsants
    Language English
    Publishing date 2023-09-23
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 2010587-3
    ISSN 1525-5069 ; 1525-5050
    ISSN (online) 1525-5069
    ISSN 1525-5050
    DOI 10.1016/j.yebeh.2023.109435
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  10. Article ; Online: Cognitive functioning in progressive myoclonus epilepsy type 1 (Unverricht-Lundborg Disease, EPM1).

    Äikiä, Marja / Hyppönen, Jelena / Mervaala, Esa / Kälviäinen, Reetta

    Epilepsy & behavior : E&B

    2021  Volume 122, Page(s) 108157

    Abstract: Objective: The aim of this neuropsychological study of a large cohort of patients with progressive myoclonus epilepsy type 1 (Unverricht-Lundborg disease, EPM1) was to characterize the cognitive function of EPM1 patients and to explore the association ... ...

    Abstract Objective: The aim of this neuropsychological study of a large cohort of patients with progressive myoclonus epilepsy type 1 (Unverricht-Lundborg disease, EPM1) was to characterize the cognitive function of EPM1 patients and to explore the association between the disability caused by the disease and cognitive performance.
    Method: Sixty-eight genetically verified EPM1 patients homozygous for the expansion mutation in the CSTB gene (37 males and 31 females aged 35 ± 11) participated in a neuropsychological assessment of intellectual ability, verbal memory, and executive and psychomotor function. The clinical evaluation comprised administering (and video-recording) the unified myoclonus rating scale (UMRS) to assess the severity of each patient's myoclonus. Forty-six healthy volunteers (19 males and 27 females aged 32 ± 11) served as the control group for the neuropsychological tests.
    Results: The cognitive performance of the EPM1 patient group was impaired. Verbal Intelligence Quotient (VIQ) was below the average range (VIQ < 85) in 49% of the patients; further, Performance Intelligence Quotient (PIQ) was below average in 75% of the patients. The patients performed worse than the controls in both immediate and delayed story recall (p = 0.001); however, in the word list learning task, the patients performed only slightly worse than the controls. The one-hour delayed recall of the learned words was similar in both groups, and the percentage of retained words and story contents did not differ between the patients and controls. The patients were impaired in all of the executive function tests as well as in the psychomotor speed tests (p < 0.001 for all). Also, the patients' simple psychomotor speed in the tapping task was significantly slowed in comparison to controls (p < 0.001).
    Conclusion: The patients had impaired performance in the majority of the cognitive measures; they showed the highest level of impairment in all the executive function tests and in the psychomotor speed tests. The measures of these cognitive domains are timed-therefore, it is clear that severe myoclonus limits patients' performance. In contrast, verbal memory, especially delayed recall, was the least affected cognitive domain.
    MeSH term(s) Cognition ; Female ; Humans ; Intelligence Tests ; Male ; Myoclonus ; Neuropsychological Tests ; Unverricht-Lundborg Syndrome/complications
    Language English
    Publishing date 2021-06-23
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 2010587-3
    ISSN 1525-5069 ; 1525-5050
    ISSN (online) 1525-5069
    ISSN 1525-5050
    DOI 10.1016/j.yebeh.2021.108157
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