most often the onset is sudden and usually without any awareness signal, and the pathology is focused in the basilar tronc ( stroke or trauma. ) , the mortality is very high.
the caracteristics of this syndrome
1- motor quadriplegia except eye movement
2- mutisme
3- conscious markedly awake
4- sensibility present in all the body
5- cognitive skills are normal
6- lesion of lower cranial nerves especilly X, XI, XII.
there are some accessory signs less caracteristic
1- unvolontary respiration
2- unvolontary deglutition
3- inefficience mastication
4- atrophy of neck muscles
5- gastric dilatation
Cranial nerves
I intact
II intact
III intact
IV intact
V intact
VI intact
VII partial lesion
VIII intact
IX partial lesion
X partial lesion
XI partial lesion
XII totally paralyze
Cerebral scan normal
Vertebral arteriography severe spasme on right
Naso-gastric tube removed after four months
Urinary tube removed after one month
Tracheostomy tube removed after ten years
Familial hyperlipemia.
this state isn’t simple and leaves the patient in tragic situation, I call the tétraplégic state “super handicap” but the locked-in syndrome “poly-super handicap”, and if the first state demands a great service daily and needs four assistants permanently at home the second one needs eight assistants which means it’s much more complicated than tetraplegic state.
when we see a victim of LIS, immediately the diagnosis is made even before the paraclinic’s results, it’s similar a hurricane destroying and swallowing everything leaving behind a desert land in disastrous state.
the words of any language are incapable to fully describe this state, in one word “I live hell on earth since January 1985 ” !
I remember, the priest who still visit me, when he came for the first time ( in february 1990 ) and saw me he was shocked and the words were strangulated in his throat, then he run away to his home where he cried for several hours.
If we take an example “the meals”, tetraplegic patients can eat normal foods because they have a well controlled muscle tongue, but patients with LIS have a paralysed one and the mastication is ineffective and need semi-mixed foods ( because mixed foods loose their taste! ) three times every day? ! in the begining it seems too simple and fast but after twenty years it’s very hard and too heavy, especilly if the assitant is ill without any one to take his place, it’s a major problem for the patient which overwhelm the others problems and marks the syndrome in manner the patient needs permanently and without ceasing a persone to prepare semi-mixed foods to him
adding frustration over isolation with total incapacity leaving the patient in desesperate state.
After twenty one years carrying the cross on my soulders with unceasing flagellation, I feel my body becomes too heavy like an iron diving bell which compresses the thorax and strangulates me. In the last years I begged the lord to reduce my suffering days, but instead of this I had eight heart attacks in the last thirty months from which I recover successfully.
No one can triumph over handicap especially the catastrophic state (LIS ) without considering the divine will like his own will, otherwise every single symptome of this syndrome is capable of destroying the patient.
Dr Nabil Daoud
January 25/2006
There is a medical report given by the physician who treated me:
Rapport Medical
le Dr Nabil DAOUD, né en 1955, a été victime le 25 Janvier 1985 d’un accident vasculaire cerebral, dû a une hyperlipémie familiale moderée, au niveau du tronc cerebral. Apres un séjour de plus d’un an a l’Hôtel-Dieu, il quitte avec un tableau “Locked-in syndrom”. Tétraplégie avec paralysie des muscles de la nuque, communique uniquement avec les mouvements des yeux et des paupieres. Jusqu’a ce jour, il est soigné a domicile, son état reste inchangé et la trachéotomie a pu être fermée en 94, il a une respiration normale, une conscience parfaite, capable d’activités intellectuelles superieures, il manipule son ordinateur grâce a un mouvement d’abduction du pouce gauche.
il mange des repas mous, la deglution est correte, la position assise, soutenue, est tout a fait possible.
Prof. Nabil Okaïs
chef de service de Neuro-chir.
Hôtel Dieu-Beyrouth
Mars /14/2002
