Epilepsies Vestibulaires: Des entités à part ?

Fabrice Bartolomei

Hôpital de la Timone & Hôpital Henri Gastaut/Centre Saint Paul

Université Aix Marseille IIMarseille, France

http://www.cinapse.org

Les vertiges paroxystiques épileptiques: une existence contestée

!!

Etiologie des Manifestations vertigineuses (from Dietriech et al, J Neurol (2007) 254:559–568))

Vestibular system: projection to the cortex

• The vestibular system encodes translations and rotations of the head in space

• Vestibular signals are of crucial importance for oculomotor and postural reflexes, and they are also at the basis of conscious perception and spatial cognition (Berthoz, 1996)

• Functions: self-motion perception, spatial navigation and memory (Berthoz et al., 1995; Brandt et al., 2005), perception of the vertical (Lopez et al., 2007), visual processing related to gravitational cues (Indovina et al., 2005; Lopez et al., 2009), and bodily self-consciousness (Blanke et al, 2002).

Vestibular Cortex

• Network encompassing any area of the cortex that receives inputs from the vestibular system.

• Parieto-insular vestibular cortex (PIVC), located in the posterior insula/retroinsular cortex (Grusser et al., 1990, 1994; Guldin and Grusser, 1998)

Guldin & Grusser, 1998, modified

Métanalyse Etudes d’activation en Imagerie Fonctionnelle

• Nombreuses régions corticales activées

• Mais « core » cortex insulaire postérieur et rétroinsulaire

Lopez et al, 2012

Kahane et al. 2003

1 insular site

Intracerebral Electrodes Stimulations

Epileptic manifestations with vestibular disturbances

• Aura of various type of partial seizures (Smith , 1960)

• Vestibulogenic Epilepsy (reflex)• Isolated dizziness/vertigo seizures (“dizziness

epilepsy” Kogeorgos et al, 1981; “Vestibular epilepsy”, Alpers, 1959, Hewett et al, 011 )

• Epilepsy with Epileptic Nystagmus• Rotatory/Volvular Epilepsy

Review in Brandt, 1999, Hewett & Bartolomei, 2013

Une histoire complexe

• Antiquité

- Arataeus (1°c après JC): vertige comme manifestations épileptiques

- CAurelanius (5 après JC) “petite épilepsie”

Bladin PF, 1998, Temkin O, 1994

Début 19°Siècle

• Esquirol: “Des maladies Mentales”: vertige epileptique, petit mal et grand mal (1838)

• Calmeil (1824): vertige épileptique, absence

• 1854 Todd: “If morbid material affected the hemisphere then epileptic vertigo results”

• Plutot synonyme d’accés épileptiques mineurs

Esquirol

19 ° siècle: le vertige épileptique comme un synonyme d’accés violents, médico-légaux

des crises d'épilepsie

• Falret (1860), Morel (1860) Althaus (1877)

• Lien entre Vertige épileptique et actes meurtriers pendant des ccrises épileptiques (Bucknill1879)

• Lombroso criminologue qui a influencé Emile Zola, dans “la Bête Humaine”

Approches Neurologiques des manifestations vestibulaires au

XIX°s

• JH Jackson: considéra les manifestations vertigineuses comme d’origine épileptique potentielle

• Mais insistera surtout sur les formes périphériques

• Description de la maladie de Ménière en 1872 et souligne le rôle de l’oreille interne

• Charcot suit la même idée (1874)

From selected writings of JH Jackson

Gowers (1877,1903)

• Rejete le terme epileptic vertigo pour désigner les manifestations psychiatriques épileptiques

• Propose que les manifestations vertigineuses puissent être d’origine épileptique, rares et le plus souvent dans le cadre d’auras de manifestations épileptiques

Epileptic manifestations with vestibular disturbances

• Aura of various type of partial (Smith , 1960)

• Vestibulogenic Epilepsy (reflex)• Isolated dizziness/vertigo seizures

(“dizziness epilepsy” Kogeorgos et al, 1981; “Vestibular epilepsy”, Alpers, 1959 )

• Epilepsy with Epileptic Nystagmus• Rotatory/Volvular Epilepsy

Review in Brandt, 1999

Dizziness, vertigo as an aura of different types of seizures

• Not rare (25/155 epilepsy Cases for Gowers, 1885)

• « The sense of dizziness, consisting of a feeling of rotation or bodily displacement, may constitute an introductory aura. It is most often associated with other epileptic phenomena in the evolution of an attack (…) »

(Penfield and Kristiensen (1951)Penfield and Jasper, 1954)

Kin

etic

Com

plex

Vertiginous disturbances in epilepsy - 120 patients (Smith 1960)

0 10 20 30 40 50 60

rotation

linear movement

horizontal

vertical

oblique

rocking

feeling of floating

walls caving in

about to spin

unsteadiness

turning inside head

revolving light

Static (tilting)

Vibration

%

Crises Pariétales

Bartolomei et al, 2011

Epileptic manifestations with vestibular disturbances

• Aura of various type of partial (Smith , 1959)

• Vestibulogenic Epilepsy (reflex)

• Isolated dizziness/vertigo seizures (“dizziness epilepsy” Kogeorgos et al, 1981; Vestibular epilepsy, Alpers, 1959 )

• Epilepsy with Epileptic Nystagmus

• Rotatory/Volvular Epilepsy

Vestibulogenic seizures=Sz triggered by vestibular stimuli

(caloric irrigation, rotation..)

• P Marie et J Pierre, 1922, Satauder, 1934, Spiegel, 1934)

• Behram & Wyke, Brain 1958, Orban & Lang, 1963, Cantor FK, 1971

• Karboski, 1970:62 patients épileptiques avec stimulation calorique: 22 cas activation EEG, 2 cas crises déclenchées

• Barac 1968: activation non spécifique d’une forme d’épilepsie

Chez ceux ayant une activation, groupes hétérogènes de patients dont 1 groupe est caractérisé par des crises

vertigineuses et des anomalies dans le carrefour TPO

Karboski, 1989

Epileptic manifestations with vestibular disturbances

• Aura of various type of partial (Smith , 1959)

• Vestibulogenic Epilepsy (reflex)

• Isolated dizziness/vertigo seizures (“dizziness epilepsy” Kogeorgos et al, 1981; Vestibular epilepsy, Alpers, 1959 )

• Epilepsy with Epileptic Nystagmus

• Rotatory/Volvular Epilepsy

Vertiginous (or vestibular) Epilepsy

• Dizziness is the sole (main) manifestation of seizures (Alpers, 1960)

Diagnostic Criteria

Alpers, 1960

Pure vestibular epileptic manifestations

• Pure epileptic vertigo attacks (Gowers, 1885)• Pedersen & Jepsen, Epileptic vertigo, Acta Psych

neurol, 1956 (14pts, 2 of them having pure vestibular symptoms)

• Alpers J. Vertiginous epilepsy, 1960 (1 case)• Kogeorgos et al, 1981 (30 cases): Dizziness

Epilepsy• Hewett et al (2011) (14 cases) Benign TPO

junction epilepsy with vestibular disturbances

A. christian, 26 y old

• High educational level• Familial History: a cousin has an epilepsy (type ?)• No personal medical history, no migraine, no hearing

problem• Onset of manifestation: 15 y• Diurnal, 1/ month• Lasts several seconds• Abrupt onset of dizziness with imbalance (no real

vertigo) with some movement of the environment• 2 episodes with Loss of consciousness (fall,

description ?)• ORL work up and cardiologist work up: normal

Investigations

• Normal MRI

• EEG: slow waves/ sharp in the right TPO electrodes

• Diagnostic: vestibular epilepsy

• Evolution: good AED response under CBZ. Some relapse after treatment interruption

QUI. Nathalie ; 37 y old

• High educational level (researcher)• Consultation because of repeated « vagal malaises »

with rotational movement sensation (vertigo), heat, nausea, blurred vision during several seconds or minutes

• Onset at the age 31 y• No other problems, no familial history• In some occasions (5 episodes between 2008-2009) there

is a loss of consciousness with abrupt fall and tonic-clonic jerks, some post-ictal confusion

• Once she had a shoulder dislocation and once a facial trauma

• She can have this kind of symptoms after seeing blood..

Investigations

• ORL: negative, normal hearing and vestibular functions

• Cardio: positive tilt test attesting vagal hyperactivity, no rythmic problems

• She was initially considered to have both vagal syncopes and psychogenic manifestations (paroxetine treatment no effect)

• MRI: normal• Standart EEG: normal, Nap EEG:

EEG

• 30 pts (15-65 y,mean 35) age at onset: 25

• 18F, 17M

• Dizziness: transient sense of disequilibrium with or without feeling of rotation as the sole or main reason for referral

Symptoms

• Last Few seconds

• Feeling of rotation: 47%

• with frequent nausea

• No triggering factor (movement etc..)

• Frequency ranged from once a week to daily occurrence

• Mean age of onset: 25 y

Other manifestations

• Generalized convulsions in 7(23%) pts• « absences » noted in 15pts (brief episodes of

LOC)• Other neurosensorial symptoms could be

occasionnaly reported (epigastric discomfort, headaches, nausea, auditory hallucinations..)

• Febrile Sz in two pts (7%)• Epilepsy family history in 6 (20%)

Diagnosis/Treatment

• EEG: posterior temporal or temporal sharp or slow waves, sometimes generalized discharges

• Sdt or sleep recordings• CT Scan: normal• Prominent on the left side (15/30) Right (7) or

bilateral (6) Nl in two• CBZor PHT: good outcome in all but one patient

Benign TPO junction epilepsy with vestibular disturbances

Results

• 14 patients (10 males, 4 females) having recurrent

episodes of paroxysmal vestibular symptoms,

including true vertigo and loss of balance;

• Mean age at onset was 26.5 (range: 12–59) and

average age at diagnosis was 30.5

(range: 14–59) (~4y diagnostic delay)

• 4/14(28%) patients had a family history of epilepsy

• All patients had normal intellectual development and

normal neurological examination.

• MRI was normal

Subjective Semiology

Rotational vertigo: 9/14; 78%

Objective, other semiological aspects

GTCS: 4/14 (28%), Fall : 10/14(74%)->LOC 9/14 (64%)

EEG

Outcome

• Two patients refused treatment• Seizures in 8 patients are currently controlled on

monotherapy and in 4 patients on dual therapy• Follow-up has ranged from 1 to 11 years (mean

follow-up=3.9 years); • All patients remain seizure free including the 6

patients followed up for more than 3 years.

En résumé: 2 séries très proches  Kogeorgos et al

(1980)Hewett et al

(2011)Age at onset (mean,years)

25 26

Sexe ratio M/F 1/1 2/1

Familial History 20% 28%

Febrile Sz 7% -Vestibular Symptoms 100% 100%

Rotational 47% 78%GTCS 23% 28%

Other Clinical Pictures "absences" neurocardiogenic syncopes, falls

Neuroimaging N (CT-scan) N (MRI)

EEG Temporal posterior TPO junction

Side predominance Left right

Therapeutic response good good

D’après Hewett & Bartolomei, in press

Epilepsie Bénigne du carrefour TPO : une épilepsie spécifique des systèmes

vestibulaires corticaux ?

• Epilepsie débutant chez l’adolescent ou l’adulte jeune (12-30 ans)

• Sémiologie vestibulaire prédominante, souvent prise pour une pathologie cardiaque (syncopes) ou ORL

• Anomalies EEG bilaterales ou unilatérales prédominant sur les régions postérieures

• Imagerie normale

• Réponse thérapeutique excellente

• Rôle de facteurs génétiques ? (ATCD familiaux..)

Diagnostic Différentiel

• Vertiges périphériques (paroxystique bénin positionnel)

• Vertige psychogène

• Migraines

• Ataxies Episodiques Primaires ++

Ataxies Episodiques Primaires