NevSom – Norwegian Centre of Expertise for Neurodevelopmental Disorders and Hypersomnias

Clinical organisation/ follow-up of narcolepsy in Norway Ragnhild Berling Grande

MD, PhD, Neurologist

NevSom, Oslo University Hospital

Nordic Narcolepsy Symposium, Oslo, 6.febr. 2020

NevSom – Norwegian Centre of Expertise for Neurodevelopmental Disorders and Hypersomnias

NevSom – Norwegian Centre of Expertise for Neurodevelopmental Disorders and Hypersomnias

NevSom – Norwegian Centre of Expertise for Neurodevelopmental Disorders and Hypersomnias

Daily challenges i Narcolepsy

NevSom – Norwegian Centre of Expertise for Neurodevelopmental Disorders and Hypersomnias

Case-history (slides not available) : what to learn?

• This is a typical narcolepsy patient- complex, with psykosocial and educational challenges

• We adviced:

• Intensify treatment, both night and day

• treat parmacological and non-pharmacological

• Lifestyle changes: «power-naps», daylight, fysical activity, food ++

• Frequent follow-up, experienced neurologist

• Multidiciplinary care: social advisor, nutricionist, physio

• Focus on aims for the treatment:

• - reduce further educational delay

• - transport

• -social life

• -economy

• quality of life

NevSom – Norwegian Centre of Expertise for Neurodevelopmental Disorders and Hypersomnias

NevSom – Norwegian Centre of Expertise for Neurodevelopmental Disorders and Hypersomnias

The norwegian way: from diagnosis to treatment -In general we have a large variation in health care for narcolepsy

1. GP x ?

2. Privat health care?

3. Referral to sleep investigations

4. Results back to GP/ referral to ped/neur

5. If diagnosis unclear: complete the diagnostics/ private health care

6. If diagnosis clear: Ped/Neurologist start medication – (Modafinil 100mg x2)

7. Ped/Neur: follow-up, big variation in follow-up habits, the doctor decides

8. When medication «stable» – back to GP or Ped/Neur/privat- yearly follow-neurology dept.

NevSom – Norwegian Centre of Expertise for Neurodevelopmental Disorders and Hypersomnias

Multidiciplinary care

NevSom – Norwegian Centre of Expertise for Neurodevelopmental Disorders and Hypersomnias

Multidiciplinary care – never/ rarely offered

NevSom – Norwegian Centre of Expertise for Neurodevelopmental Disorders and Hypersomnias

• PAKKEFORLØP: Standardized normative patient treatment-and follow-up i Norway

NevSom – Norwegian Centre of Expertise for Neurodevelopmental Disorders and Hypersomnias

• PAKKEFORLØP: Standardized normative patient treatment-and follow-up i Norway – no such system exists for Narcolepsy

NevSom – Norwegian Centre of Expertise for Neurodevelopmental Disorders and Hypersomnias

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NevSom – Norwegian Centre of Expertise for Neurodevelopmental Disorders and Hypersomnias

CNS-hypersomnias/ Narcolepsy NOT AT ALL mentioned in a new and very important brain-health-strategy. Sleep-disorders are only mentioned as « Sleep-disorders» on some lists of diagnoses. This is very dissappointing.

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NevSom – Norwegian Centre of Expertise for Neurodevelopmental Disorders and Hypersomnias

Norwegian online clinical «handbooks/ guidelines on Narcolepsy»: Generell veileder i pediatri and NevroNEL

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NevSom – Norwegian Centre of Expertise for Neurodevelopmental Disorders and Hypersomnias

NevroNEL: the list is very long….

• Lots of disorders:

• ADEM (akutt disseminert encefalomyelitt)

• Akutt hemorrhagisk leukoencefalitt (AHLE) Camptocormia og dropped head

• Centromedullært syndrom

• Cerebral amyloid angiopati rev • Cerebral fett emboli

• Cerebral småkarsykdom

• Cerebral vaskulitt rev

• Cerebral venetrombose

• Cerebrale vaskulære malformasjoner

• Cerebrovaskulære syndromer • Cervikal radikulopati

• Cervikal spondylotisk myelopati

• Charles Bonnet syndrom • Chiari-malformasjon og syringomyeli

• Chorea

• CIDP - kronisk inflammatorisk demyeliniserende polynevropati rev • CIS (clinically isolated syndrome) rev

• Clippers rev

• Cogan syndrom ny

• Creutzfeldt-Jacob og andre prionsykdommer

• Critical illness nevropati og myopati

• Delir • Demens

• Dermoid cyste

• Diabetisk nevropati • Diafragmaparese

• Diagnostikk av metabolske sykdommer hos voksne

• Diplopi • Dolikoektasi av intrakraniale arterier

• Doparesponsiv dystoni

• Drop attakk hos eldre rev • Drop fot

• Drop hand

• Dystoni rev • Dystrofia myotonica

• Eagles syndrom

• Empty sella • Epidermoid cyste

• Epilepsi

• Epilepsi hos eldre

• Epilepsi og kvinner rev

• Epilepsi og utviklingshemming

• Epileptiske syndromer/encefalopatier • Exploding head syndrome rev

• Fabrys sykdom

• Facial-onset sensory-motor nevronopati - FOSMN syndrom

• Farmakoresistent epilepsi

• Fascikulasjoner

• Femoralisnevropati

• Feokromocytom

• Fokal myositt

• Forsinket søvnfaselidelse

• Fragilt X tremor og ataksi syndrom

• Frontotemporal demens

• Funksjonelle nevrologiske symptomer

• Første krampeanfall rev

• Generelt om hodepine

• Gliomer

• Guillain-Barrè Syndrom rev

• Hashimoto's encefalopati/steroid responsiv encefalopati assosiert med thyroiditt (SREAT)

• Hemifacialisspasme

• Hepatisk encefalopati

• Hereditær spastisk paraparese HSP

• Herpes Simplex Encefalitt

• Hirayama sykdom

• Hjerneabscess

• Hjerneblødning rev

• Hjerneinfarkt - Akutt utredning og behandling

• Hjerneinfarkt trombolyse

• Hodepine av spenningstype (HST)

• Hodepine postpartum

• Horner syndrom

• Akutt høretap

• Akutt intermitterende porfyri (AIP)

• Akutt perifer facialisparese

• Alkoholrelaterte kramper

• Alzheimer demens

• Amyotrofisk Lateral Sklerose (ALS)

• Andre flåttbårne sykdommer rev

• Anisokori

• Ansiktssmerter ny

• Anterior interosseus syndrom

• Antifosfolipid syndrom

• Anti-NMDA receptor encefalitt rev

• Antistoffassosierte bevegelsesforstyrrelser

• Araknoidal cyste hos voksne

• Arteriell disseksjon

• Arvelige nevrodegenerative ataksier

• Arvelige nevropatier (CMT og andre)

• Ataksi

• Atypisk parkinsonisme

• Autisme (Aspergers syndrom)

• Autoimmun encefalitt - limbisk encefalitt rev

• Autonom svikt

• Autonome nevropatier (kroniske)

• Autonome nevropatier (subakutte)

• Balo´s concentrisk sklerose ny

• Basilaris trombose

• Behcet´s sykdom - Nevrobehcet

• Benign paroksysmal posisjonsvertigo BPPV

• Bilateral facialisparese

• Bildeundersøkelser i svangerskap

• Biotin-thiamin responsiv basalganglie sykdom

• Botulisme

• Human T celle Leukemi Virus type 1 HTLV1

• Huntingtons sykdom

• Hvit substans patologi på MR ny

• Hydrocefalus

• Hyperakutt hodepine rev

• Hyperperfusjons syndrom cerebralt

• Hypofyse adenom

• Hypofyse apoplexi

• Hypoglossusparese

• Hypoksisk iskemisk hjerneskade rev

• Hyponatremi

• Høy CK - oligo/asymptomatisk

• Høy CK - rhabdomyolyse

• Idiopatisk intrakraniell hypertensjon

• Indremedisinske tilstander og nevrologi

• Infeksiøs encefalitt rev

• Infeksiøse polynevropatier

• Inndeling av søvnsykdommer

• Insomni

• Intensivpasient med nevrologiske utfall

• Intrakraniale aneurismer

• Isjias og cauda equina syndromet

• Isjiasnevropati

• Juvenil myoklon epilepsi (JME)

• Kalk i hjernen

• Karcinoid syndrom

• Karotisstenose

• Karpaltunnelsyndrom

• Klasehodepine og andre trigeminal-autonome hodepiner

• Kleine-Levin Syndrom (KLS)

• Kolloid cyste

• Komplekst regionalt smertesyndrom (CRPS)

• Kongenitt myasthent syndrom

• Kronisk idiopatisk aksonal polynevropati

• Kryoglobulinemi og polynevropati

• Lambert-Eaton Myastenisk Syndrom LEMS

• Late-onset nemalin myopati

• Lette og moderate hodeskader

• Leukoencefalopatier (med debut i voksen alder) rev

• Lewy body demens

• Lumbal spinal stenose

• Lymfom i CNS

• Lymfom i PNS rev

• Lysosomale sykdommer

• Marburg´s variant av MS

• Marchiafava-Bignami

• Medikamentoverforbrukshodepine (MOH)

• Meningeom

• Meningitt - CSF pleocytose

• Meralgia Paresthetica

• Metabolske muskelsykdommer

• Metastaser i CNS rev

• Metformin og kontrast

• Migrene

• Mild kognitiv svikt (MCI)

• Mitokondriesykdommer rev

• MOG-antistoff sykdom

• Multifokal motor nevropati

• Multippel sklerose (MS)

• Musculocutaneusnevropati

• Muskeldystrofier

• Muskelkramper

• Muskelsykdom (myopati)

• Myasten krise

• Myasthenia Gravis (MG)

• Myelitt (transvers myelitt)

• Myelopati

• Myoklonus

• Myositt

• Myotoni ny

• Myotonier (nondystrofe)

• Narkolepsi

• Nedre motornevron syndromer

• Neuromyelitis optica (NMO)/Nevromyelitis optica spectrum disorders (NMOSD)

• Nevroakantocytose

• Nevroborreliose rev

• Nevrocysticercose

• Nevrodegenerasjon med jernavleiring (NBIA)

• Nevrofibromatose

• Nevrologi i svangerskap

• Nevromyotoni

• Nevropatisk kløe

• Nevropatisk smerte

• Nevrosarkoidose

• Normaltrykkshydrocefalus (NPH) rev

• Obstruktiv søvnapné

• Optikus nevritt

• Painful legs and moving toes

• Paraneoplastisk polynevropati

• Paraneoplastiske nevrologiske syndromer - en oversikt

• Paraproteinemisk polynevropati

• Parasomnier

• Parkinsons sykdom

• Paroxysmale dyskinesier

• Parry-Romberg syndrom

• Perifer nerveskade

• Periodiske paralyser

• Pineal cyste

• Plexus brachialis nevritt

• Plexus lumbosacralis nevritt

• PML (progressiv multifokal leukoencefalopati)

• Polynevropati - utredning rev

• Polynevropati med affeksjon av hud, hår, negl

• Polynevropati med facialisparese

• Polynevropatier med mye smerte rev

• Posterior reversibelt encefalopati syndrom (PRES)

• Postpolio syndrom

• Postpunksjons hodepine

• PPPD (persistent postural-perceptual dizziness) rev

• Progressiv myoklonus epilepsi (PME)

• Psykisk utviklingshemming

• Psykogene, ikke-epileptiske anfall (PNES)

• Ptose

• REM-søvnatferdsforstyrrelse

• Restless Legs Syndrome - RLS

• Reversibel cerebral vasokonstriksjons syndrom (RCVS)

• Riboflavin transporter defekt (Brown-Vialetto-Van Laere syndrom)

• Sekundærprofylakse ved hjerneslag/TIA rev

• Sentral pontin og/eller ekstrapontin myelinolyse (Osmotisk demyeliniseringssyndrom)

• Septisk encefalopati

• Sjøgren og nevrologiske komplikasjoner

• Skapula alata

• Skogflåttencefalitt TBE rev

• Spastisitet

• Spinal muskel atrofi rev

• Spinale vaskulære tilstander

• Spontan intrakranial hypotensjon

• Status epilepticus

• Stiff person syndrom

• Subaraknoidal blødning (SAB)

• Subduralt hematom, kronisk

• Superfisiell siderose i CNS

• Super-refraktær status epilepticus

• Suprascapularis nevropati

• Susac syndrom

• Svimmelhet rev

• Svulster i CNS; WHO klassifisering og gradering

• Synstap

• Tardive syndromer

• Temporallappsepilepsi

• Thoracic outlet syndrome

• Thorakalt skiveprolaps

• TIA (transitorisk iskemisk attakk) rev

• Tourette syndrom

• Transitorisk Global Amnesi

• Trapezius lammelse

• Tremor

• Trigeminusnevralgi og andre kraniale nevralgier

• Tumefactiv demyelinisering ny

• Tverrsnittslesjon

• Tynnfiber nevropati

• Ulnarisnevropati

• Ureasyklus forstyrrelser

• Varizella-zoster infeksjon; nevrologiske manifestasjoner

• Vaskulitt nevropati

• Vaskulær demens

• Vegetativ status

• Vestibularisnevritt rev

• Vestibulær migrene

• Vitaminmangel polynevropati

• Wernicke (tiaminmangel) rev

• West Nile virus infeksjon

• Wilson's sykdom

• Årsaker til kognitiv svikt/forvirring

• Årsaker til polynevropati

• Dystrofia myotonica

• Eagles syndrom • Empty sella

• Epidermoid cyste

• Epilepsi

• Epilepsi hos eldre

• Epilepsi og kvinner rev

• Epilepsi og utviklingshemming • Epileptiske syndromer/encefalopatier

• Exploding head syndrome rev

• Fabrys sykdom • Facial-onset sensory-motor nevronopati - FOSMN syndrom

• Farmakoresistent epilepsi

• Fascikulasjoner • Femoralisnevropati

• Feokromocytom

• Fokal myositt • Forsinket søvnfaselidelse

• Fragilt X tremor og ataksi syndrom

• Frontotemporal demens • Funksjonelle nevrologiske symptomer

• Første krampeanfall rev

• Generelt om hodepine • Gliomer

• Guillain-Barrè Syndrom rev

• Hashimoto's encefalopati/steroid responsiv encefalopati assosiert med thyroiditt (SREAT)

• Hemifacialisspasme

• Hepatisk encefalopati

• Hereditær spastisk paraparese HSP • Herpes Simplex Encefalitt

• Hirayama sykdom

• Hjerneabscess • Hjerneblødning rev

• Hjerneinfarkt - Akutt utredning og behandling

• Hjerneinfarkt trombolyse

• Hodepine av spenningstype (HST)

• Hodepine postpartum

• Horner syndrom

• Akutt høretap

• Akutt intermitterende porfyri (AIP)

• Akutt perifer facialisparese

• Alkoholrelaterte kramper

• Alzheimer demens

• Amyotrofisk Lateral Sklerose (ALS)

NevSom – Norwegian Centre of Expertise for Neurodevelopmental Disorders and Hypersomnias

nevroNEL

NevSom – Norwegian Centre of Expertise for Neurodevelopmental Disorders and Hypersomnias

nevroNEL

NevSom – Norwegian Centre of Expertise for Neurodevelopmental Disorders and Hypersomnias

nevroNEL

NevSom – Norwegian Centre of Expertise for Neurodevelopmental Disorders and Hypersomnias

nevroNEL

NevSom – Norwegian Centre of Expertise for Neurodevelopmental Disorders and Hypersomnias

nevroNEL

NevSom – Norwegian Centre of Expertise for Neurodevelopmental Disorders and Hypersomnias

nevroNEL

NevSom – Norwegian Centre of Expertise for Neurodevelopmental Disorders and Hypersomnias

nevroNEL

NevSom – Norwegian Centre of Expertise for Neurodevelopmental Disorders and Hypersomnias

Checklist 1 - Narcolepsy. NEWLY DIAGNOSED

General information

o Explaination of what narcolepsy is- provide brochure.

Regard the patients Health Literacy

o Epidemiology, prognosis

o Usual challenges due to the disease

TREATMENT

-Lifestyle changes o Powernaps 15-

20min.

o Healthy lifestyle

o Good sleep rutines

o Get enough sleep

o Physical activity

Common challenges

o Concentration

o Memory

o Mood changes/

irritability

o Weight gain

o Depression/anxiety

Lifestyle o Education

o Work/ employment

o Social public support?

o Leisure

o Psychosocial/ relations

o Financial

o Driving/ licence

TREATMENT

-Medication

o Choice of drug

o Efficacy

o Side effects

o Adherence

o Drug interactions

o ECG, Bloodpress,

puls, weight

Self care: This may improve

narcolepsy?

o Powernaps!!!

o Sport, physical

activity, fresh air,

daylight!!

o Knowledge

o Daily life

arrangements/support

o Avoid big meals

o Information to

family/friends

TREATMENT

-Multidiciplinary o Social advisor (all should be referred, early)

o Sleep-nurse in outpatient clinic (give phonenumber

to all) (all)

o Psychiatrist, psychologist, nutricionist,

physiotherapist if needed

o Hospitalization/ rehab for multidisciplinary

treatment/psychosocial rehab/ work-training/change

of medication if needed

Discuss aims for the treatment and patients needs and wishes

-Comorbid disorders MUST be diagnosed and treated. Be

aware of depression.

Issues for Women

o Contraception (check

interactions)

o Pre-conception

o Pregnancy

o Breastfeeding

Information/ Support

Organisations

o Written info./

webpages/YouTube-

videos/app/e-learning

o Centre of Expertice

nevsom.no

o Patient organisation

sovnforeningen.no

FOLLOW-UP

o Specialist doctor

o Frequency due to

needs

o Multidiciplinary

o Bring a friend

o Stay in contact with

GP

NevSom – Norwegian Centre of Expertise for Neurodevelopmental Disorders and Hypersomnias

Checklist 2 – Narcolepsy. FOLLOW-UP

Current symptoms o Excessive daytime sleepiness/ sleep-attacks o Cataplexy (type, frequency, falls?) o Other symptoms

Patient education/ level of knowledge on narcolepsy o Check level of knowledge. Often need to repeat general information? Regard patients

Health Literacy

o Source of information

o Knowledge of centre of expertice? Know the website? www.nevsom.no

o Knowledge of Support Organisations? Membership? Know the website?

www.sovnforeningen.no

How is daily life? o Education (medical certificat)

o Work/employment

o Leisure

o Psychosocial

o Relationships/family life

o Living arrangements

o Financial

o Driving/ public transport

o Travelling abroad (medical certificat)

Medication (complete list, contraceptives,

herbs)

o Adherence

o Effect

o Side effects

o Interactions

Multidisiplinary o Met social advisor?

o Met/ phonecall nurse?

o Other?

Practice of self care? o Powernaps 15-20 min.

o Regular sleep rutines, no sleep deprivation

o Physical activity, sport

o Healthy lifestyle, outdoors, daylight

o Avoid big heavy meals

o Information to family/friends

Common challenges ? o Concentration

o Memory

o Mood changes

o Depression/anxiety

Women o Family planning status

o Contraception (safe?

check interactions)

Summary o Explain any changes

to treatment/

management o Agree on treatment

goal/ plan o Patients questions?

Follow-up o Specialist with

experience

o Frequency due to

needs

o Mulidiciplinary

o Stay in contact with

GP

NevSom – Norwegian Centre of Expertise for Neurodevelopmental Disorders and Hypersomnias

NevSom – Norwegian Centre of Expertise for Neurodevelopmental Disorders and Hypersomnias

How can we fill the GAP in Norway? • Mandatory education on sleep/ sleep disorders in medical education/

neuro/ped-spezialisation?

• Educate somnologists? How? Where?

• Sleep disorders into ALL already existing national health strategies?

• Copy successful models from other countries?

• Copy succesful models for «similar» complex chronic neurological diagnoses in Norway?- «Pakkeforløp», «ParkinsonNet» or 3.rd line specialist treatment centres like i.e. SSE- Epilepsy Centre in Oslo University Hospital)?

• More Patient involvement ?

• More attention?

NevSom – Norwegian Centre of Expertise for Neurodevelopmental Disorders and Hypersomnias

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NevSom – Norwegian Centre of Expertise for Neurodevelopmental Disorders and Hypersomnias