Epilepsies Vestibulaires: Des entités à part ?

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1 Epilepsies Vestibulaires: Des entités à part ?
Fabrice Bartolomei Hôpital de la Timone & Hôpital Henri Gastaut/Centre Saint Paul Université Aix Marseille II Marseille, France

2 Les vertiges paroxystiques épileptiques: une existence contestée !!
Etiologie des Manifestations vertigineuses (from Dietriech et al, J Neurol (2007) 254:559–568) )

3

4 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).

5 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

6 Métanalyse Etudes d’activation en Imagerie Fonctionnelle
Nombreuses régions corticales activées Mais « core » cortex insulaire postérieur et rétroinsulaire Lopez et al, 2012

7 Intracerebral Electrodes Stimulations
1 insular site Kahane et al. 2003

8 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 8

9 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

10 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

11 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” Personnage de Jacques dans la BH serait atteint de vertige épileptique, personnage de Jacques dans la BH

12 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 et souligne le rôle de l’oreille interne Charcot suit la même idée (1874)

13 From selected writings of JH Jackson

14 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

15 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 15

16 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)

17 Vertiginous disturbances in epilepsy - 120 patients (Smith 1960)
rotation linear movement horizontal vertical oblique rocking feeling of floating walls caving in about to spin unsteadiness turning inside head revolving light Kinetic Static (tilting) Vibration Complex 10 20 30 40 50 60 %

18 Crises Pariétales Bartolomei et al, 2011

19 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 19

20 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

21 Karboski, 1989 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

22 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 22

23 Vertiginous (or vestibular) Epilepsy
Dizziness is the sole (main) manifestation of seizures (Alpers, 1960)

24 Diagnostic Criteria Alpers, 1960

25 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

26 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

27 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

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29 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 ) 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..

30 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:

31 EEG

32 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

33 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

34 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%)

35 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

36 Benign TPO junction epilepsy with vestibular disturbances

37 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

38 Subjective Semiology Rotational vertigo: 9/14; 78%

39 Objective, other semiological aspects
GTCS: 4/14 (28%), Fall : 10/14(74%)->LOC 9/14 (64%)

40 EEG

41 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.

42 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% Rotational 47% 78% GTCS 23% 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 D’après Hewett & Bartolomei, in press

43 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..)

44 Diagnostic Différentiel
Vertiges périphériques (paroxystique bénin positionnel) Vertige psychogène Migraines Ataxies Episodiques Primaires ++

45 Ataxies Episodiques Primaires