MARQUEURS BIOLOGIQUES
DE LA MALADIE D ALZHEIMER.
par Didier Lefranc
I INTRODUCTION :
Dans l'état actuel de nos connaissances, il n'existe pas "un"
marqueur du diagnostic de MA. Depuis plus d'une décennie, grâce
aux progrès de la biologie et des techniques d'imagerie, un nombre
considérable de travaux ont été publiés dans
ce domaine avec le plus souvent des résultats jugés statistiquement
significatifs. Néanmoins, les résultats montrent un chevauchement
important entre les populations de patients possédant les critères
de maladie d'Alzheimer "probable" et les sujets utilisés
comme contrôles. Ceci est dû, d'une part à la présence
d'environ 20% de "faux diagnostics positifs" dans la population
étudiée (Tierney et al., 1988) et d'autre part, à
la présence dans la population dite "contrôle" de
patients atteints d'une maladie d'Alzheimer à un stade encore asymptomatique.
Ces résultats obtenus sur des groupes sont très significatifs
mais ils ne sont pas applicables à un individu donné.
III. Marqueurs en rapport avec le déficit cholinergique.
Les études biochimiques et neuropathologiques rapportent dans
le cerveau un déficit cholinergique (ACh), une baisse d'activité
de l'acétylcholinesterase (AChE) et de la choline-acétyl-transférase
(ChAT) (Hardy et al., 1985).Les dosages ont ainsi concernés la Choline
et ses dérivés ainsi que l AChE et son activité métabolique
ou celle de la ChAT, tant dans le plasma que dans le liquide céphalorachidien
(LCR). On pouvait s'attendre à une diminution des taux d'enzymes
du système cholinergique ou à une modification de leur activité.
3.1°) Choline acétyl-transférase:
Cette enzyme est présente dans le sang , le LCR en très
faible quantité ce qui rend son dosage difficile. Une étude
cependant rapporte que son taux et son activité ne sont pas modifiés
au cours de la MA. (Johnson et al., 1971).
3.2°) Acétylcholinestérase:
Le LCR humain contient Deux types de cholinestérases: Les acétylcholinestérases
ou «cholinestérases vraies» et les butyrylcholinestérases
(BChE) ou «pseudocholinestérases ou cholinestérases
non spécifiques» dont les proportions respectives sont de
80% et 20%. L origine de l'AChE est neuronale mais celle de la BChE
est mal connue mais serait double: neuronale et gliale. Les dosages d'ACh
ou d'activités de l'AChE dans le liquide céphalo-rachidien
ont donné des résultats discordants (Pour revue, voir Sirviö
et Riekkinen, 1992). Certains auteurs ont rapporté une activité
réduite de l'acétylcholinestérase dans le LCR de patients
atteints de MA(Soininen et al., 1981, 1984; Appleyard et al., 1983; Arendt
et al., 1984, Thal, 1985; Tune et al., 1985; Gomez et al., 1986; Szilagyi
et al., 1987, Reinikainen et al., 1988; Elble et al., 1989, Sirviö
et al. 1989, Urakami et al., 1989, Shen et al., 1992) parfois en parfaite
corrélation avec le statut cognitif des patients (Kopnen et al.,
1994) alors que d'autres n'ont pu le confirmer ( Johnson
et Domino, 1971, Davies et al., 1979, Marquis et al., 1985; Elble et al.,
1987; Appleyard et al., 1987, Hartikainen et al., 1992). Certains auteurs
ont même proposé que cette activité enzymatique serait
significativement plus élevée chez les pateints atteints
de MA et portant au moins 1 allele apoE e4. Néanmoins, bien souvent,
lorsqu'une différence significative était retrouvée
chez les patients atteints de maladie d'Alzheimer, des anomalies comparables
étaient constatées dans d'autres pathologies notamment la
démence par infarctus multiples, la maladie de Parkinson ou dans
la dépression (Thienhaus et al., 1985; Cutler et al., 1988, Appleyard
et al., 1987). Les études qui ont porté sur le plasma ou
les lymphocytes/erythrocytes montrent des variations jugées significative
de l activité de l AChE avec des chevauchements très importants.
Ainsi, la mesure de l activité de l AChE ne semble pas être
exploitable en tant que marqueur de la MA. De même, l'activité
de la BChE dans le LCR a été considéré comme
potentiel outil diagnostic. Cependant, là encore, les résultats
sont très contradictoires. En effet, certains ont décrits
des activités de BChE diminuées (Szilgyi et al., 1987; Appleyard
et al., 1987; Huff et al., 1986; Sirviö et al., 1989) ou augmentées
(Arendt et al., 1984) De plus, si l'activité de la butyrylcholinesterase
semble plus basse dans le LCR des patients atteints de la maladie d'Alzheimer,
un chevauchement important existe avec les sujets âgés contrôles
(Huff et al., 1986). Certains auteurs ont proposé d'exprimer les
résultats de mesure d'activité enzymatique sous la forme
du rapport Activité AChE/ activité BChE (Arendt et al., 1984;
Marquis et al., 1985; Huff et al., 1986; Szilagyi et al., 1987; Sirviö
et al, 1989). Ce rapport a été rapporté diminué,
inchangé ou augmenté au cours de la MA. Nous constatons donc
que ni la mesure de la BChE , ni l expression des résultats sous
frome AChE/ BChE n'ont permis de clarifier les résultats obtenus
avec les mesures d'activité de l'AChE. Plus prometteur que la mesure
de l'activité enzymatique est le résultat de Navaratnam et
al. (1991), décrivant par isoelectrofocalisation une isoforme supplémentaire
d'AchE dans le LCR des patients Alzheimers, avec une sensibilité
proche de 80% et une spécificité de 100%. Une confirmation
par des équipes indépendantes est cependant nécessaire
et n a toujours pas été rapportée.
3.3°) La choline et ses dérivés:
La choline est le substrat principal de la ChAT, avec l'acétate
fourni par le Coenzyme A, pour effectuer la synthèse de l'acétylcholine.
Les recherches dans cette voie sont aussi équivoques. En effet,
les premiers travaux ont portés sur des analyses cinétiques
de la choline et montrent des résultats peut concordant et surtout
inapplicable dans le cadre de mis en place d'un outil diagnostic. Les travaux
récents de mesures dans le LCR des concentrations de choline et
d'acétylcholine souligne la complexité de ce type de dosage.
En effet, dans une étude portant sur 33 patients atteints de différents
troubles neurologiques, Yamada et al. (1994) ne constatent aucune variation
significative en relation avec la MA, alors que dans le contre-temps, Tohgi
et al. (1994) constatent eux une diminution importante de la concentration
d'acétylcholine dans le LCR de patients atteints de MA. Ces derniers
proposent même (Tohgi et al., 1996) un dosage simultané de
la choline et de l'acétylcholine qui permettrait de distinguer les
sujets sains ,les patients atteinst de MA et ceux ayant une démence
vasculaire.
4. Marqueurs en rapport avec d'autres déficits neurochimiques.
Certains travaux dont ceux de Hardy et al. (1985) Reinikainen et al.
(1990) montrent une atteinte des plusieurs systèmes de neurotransmetteurs
incluant, outre les sytèmes cholinergiques, les voies dopaminergiques,
sérotoninergiques et neuropeptidergiques. Ces observations ont conduit
à l'analyse dans le LCR et le plasma des modifications des différents
métabolites impliqués dans ces systèmes neurochimiques
(Hartikainen et al., 1992)
4.1°) Système noradrénergique:
Mann et al. (1984) ont réalisé une étude montrant
une atteinte du locus coeruleus suggérant une atteinte des vois
noradrénergiques. Dans le LCR, les taux de noradrénaline,
dont l'origine est centrale en majorité et de son principal métabolite,
le 3-méthoxy-4-hydroxy-phényl-glycol (MHPG) dont l'orignie
est mixte ont fait l'objet de dosages reflétant les perturbations
des systèmes noradrénergique et dopaminergique essentiellement.
Les variations dans le dosage de la noradrénaline (Raskind et al.,
1984) proviendrait du degré de démence des sujets inclus
dans l'étude et de la durée de l'alitement précédant
la ponction lombaire. Le HMPG, lui, est augmenté pour certains (Gibson
et al., 1985), inchangé pour d'autres (Beal et al., 1986) et diminué
pour Martignoni et al. (1992). De même, l'etude de la Dopamine-hydroxylase,
enzyme qui permet la transformation de la dopamine en noradrénaline
ne présentent pas de résultats concordant (Nakamura et al.,
1984; Soininen et al., 1984b). L'étude du système noradrénergique
périphérique semble donc peu utile comme marqueur de la MA.
4.2°) Systèmes dopaminergique et sérotoninergique:
Il semblerait qu'il existe une diminution des taux de dopamine et de
serotonine dans le tissu cérébral de patients atteints de
MA (Hardy et al., 1985). Certains auteurs tel Mann et al.(1984) ont décrit
une atteinte du noyau du raphé capable d'expliquer une atteinte
et une implication des voies serotoninergiques dans la MA. Dans le LCR,
les dosage de l'acide homovanillique (HVA, un produit de catabolisme de
la dopamine) et de l'acide 5-hydroxy-indol acétique (5-HIA, métabolite
de la sérotonine) se montrent également très équivoques
(Palmer et al., 1984, Kay et al., 1984). Certains auteurs comme Nyback
et al. (1987) concluent que ce type de dosage ne peut constituer une aide
dans l'évaluation du statut cognitif des patients atteints de MA
, ni dans leur distinction vis à vis des sujets sains, mais soulignerait
plutôt l'hétérogénéïté de
la MA (Parnetti et al., 1992).
4.3°) Les neuropeptides:
Beal et al. (1987) ont rapporté une diminution du taux de somatostatine
dans le cortex et l'hippocampe de patients atteints de MA. Des résultats
divergents ont également été rapportés quant
au dosage de la somatostatine dans le LCR (pour revue voir Bissette et
Myers, 1992), trouvée soit abaissée soit équivalente
à celle d'une population contrôle (Raskind et al. 1986; Sunderland
et al. 1987; Edvisson et al., 1993) ou encore abaissée par rapport
à une population contrôle mais non par rapport à des
patients souffrant d'autres causes de démence (Beal et al. 1986).
D'autres candidats ont fait l'objet d'analyses et de dosages. Ainsi, citons
la neurophysine (North et al., 1992), le neuropeptide Y (Martignoni et
al., 1992; Litvan et al., 1992; Maeda et al., 1994; Heilig et al., 1995),
la Galanine (Litvan et al., 1992), la corticotropine releasing hormone
et la tyrotropin-releasing hormone (Banki et al., 1992, Suemaru et al.,
1993), la pancréastatine (Sekiya et al., 1994), la vasopressine,
l'oxytocine et la b-endorphine (Raskind et al., 1986; Mazurek et al., 1986).
Tous font l'objet de variations jugées statistiquement significatives
mais présentent de recouvrement très important entre les
différentes populations et ne permettent pas de discriminer éfficacement
les patients des sujets contrôles, les rendant inutilisables en tant
que marqueur de la MA
4.4°) Les acides aminés et acides aminés excitateurs:
Les travaux de Olney (1989) montraient un role potentiel des acides
aminés exctitateurs dasn des pathologies dégénératives
telles que la MA ou la maladie de Parkinson.. D'autre part, des modèles
animaux ont permis de montrer que ces mêmes acides aminés
jouaient un rôle important dans les processus de mémorisation
et d'apprentissage et donc pourrait être impliqués dans la
MA (Greenamyre et Young, 1989). Enfin des quantités diminuées
de glutamate et de son recepteur (N-methyl-D-aspartate pour NMDA) ont été
rapportée par Procter et al. (1988) et Greenamyre et al. (1985)
Pomara et al. (1992), Ferrarese et al. (1993) et Csernansky et al. (1996)
décrivent une quantité augmentée de glutamate qui
supporte l'hypothèse d'une toxicité éventuelle mais
ne peut être utilisé en tant que marqueur de la MA. Il s'agit
surtout d'un marqueur de l'activité du glutamate ce qui explique
que l'on retrouve des quantités anormalement élevée
de Glutamate chez des patients atteints de 'motor neurone disease' (Shaw
et al., 1995). L'analyse des autres acides aminés s'avère
être aussi peu discriminative (Martinez et al., 1993; Fisher et al.,
1994; Mochizuki et al., 1996).
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