PSEUDO-DEBILITY IN MENTAL RETARDATION:

A FRAME OF REFERENCE

 Renato COCCHI, neurologist and medical psychologist

 

(Italian translation) //  Testo in italiano

 

Summary

Mental  retardation  is  defining a functional  phenomenon,  i.e.  the  reduced performance  in  intellectual  tasks. This impairment can  be  produced  by  two different  pathological  conditions: 1. a true mental deficiency  originated  by lesion and death of brain neurons; 2. a false deficiency (pseudodebility) mostly due to functional inhibition of brain areas, structures or pathways involved  in intelligent behaviour.

These  two  conditions differ because pseudodebility is fully  reversible  when correctly treated. According  to this point of view it seems that debility and pseudodebility  are opposite  and mutually exclusive conditions: where one stands there is  no  room for the other. Since  in  surely  lesional  mental retardation it is  possible  to  gain  some improvements  following  various  rehabilitation or  educational  programs,  the two-ways  scheme should be modified as follow: pure pseudodebility opposed  to mixed debility / pseudodebility.

In  other  terms,  besides lesional debility there always exists  a  share  of treatable  pseudodebility of primary origin (the same factors that produced  the lesion  produced also functional impairments in the surrounding neurons) and  of secondary  origin (the consciousness of own debility produced more  intellectual inhibition of psychological and relational origin). This  new  frame of reference is more exhaustive as for  the  possibilities  of explaining  why any therapeutic attempt in mentally retarded can produce  some (partial) success, but could suggest new strategies in the biological  treatment of mental retardation.

  Key  words:  Mental  retardation; debility; pseudodebility;  mixed  debility  / pseudodebility; drug therapy.

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 I  have  been using drugs for more than 15 years in the treatment  of  mentally retarded.  In  particular  I have used them for more than  12  years  in  Down’s syndrome children. I have always achieved results on cognitive abilities -  even though they differ from subject to subject. 

This fact asks for a reflection.  Having  seen over than 500 Down subjects, at the moment I will refer  to  these patients   After  a  six  month therapy results can be already noted. The  child  is  more attentive,  more  clever and if he can speak he will speak more,  etc.  In  many cases  such  outcomes  cannot  be produced  by  other  rehabilitation  therapies (psycho-motor  or  speech  therapy) either  because they  were  not  adopted  or because  they  have  been used for a long time  with  poor  results.  

Patients’ parents,  teachers  and  professionals  have  clearly  seen  and  reported  such progress. This  progress  coming out in Down children treated with drugs give rise  to  a very important problem. I will tray to explain it even because it doesn’t only concern Down’s  syndrome individuals.  It might be stated as follows.

What  is the neuropathological basis of such positive results in brain  damaged mentally  retarded,  mainly  when,  after a comparative  short  period  of  drug therapy, we can see evident improvements? For the moment my argument should only be viewed as a very probable explanation since it is the easiest to be developed.  

I shall exclusively make use of well-known data and phenomena already  reported which haven’t yet been set in a general scheme. I think that a reflection on this topic has not yet been made because of a lack of specific experience. It  seems  that nobody has managed to get rid of the idea that  "brain  lesion" means only "neuronal death" that is to say that no therapy is  possible.

If  we  consider damage as not going to go backwards,  Bax’s  inference  (1988) which  deny the chance of treatment or therapy can be accepted as correct,  even written for CP. "The child has a static encephalopathy and the basic neurological  abnormalities are not to going to go away.

Therefore we are no treating these children, nor do we provide a therapy - we are providing care and management and we want it to be as effective as possible". From a two-party conception to a three-party one.  It  is  hard to believe that so many teachers, professionals and  parents  with whom the children treated by me are in touch, may deceive themselves. To act  on mental   retardation   -  a  phenomenon  which  should  have  by  definition   a  biological  basis (mental deficiency) - may be faced other than in education  or behaviour terms.  I honestly  think that  those  who support the opposite   view  (i.e.  the public opinion)  did not have the chance to observe the problem  from another  point  of view.

As for mental deficiency, I wrote some years ago (Cocchi 1985) that beside  the brain  lesion,  in  the same subject had to be suspected  a  component  of  cell dysfunction,  to which a share of the overall deficit should be due.    We  must get  rid of the concept of full opposition (a neuronal cell may be either  dead, or alive  and healthy). I think that  the three-party  scheme  (a neuron  may be either   dead,  or  alive  but sick, or alive and healthy) is more  useful  (see Fig. 3).   Fig. 1: the three party scheme: The mental debility ---> false m3ntal debility

 

    

Then  it  is  easy  to understand  that  a treatment  or a  proper  therapy  on alive but  impaired  cells (because  they  are  "sick")  might exist. In such  a  way   these  cells  may  be improved. 

For the present, education  or  behaviour therapies,  though   with  poor  results, seem to be satisfactory,  failing  all else. Otherwise,  if  we want to obtain a result which has always also  a  biological side,  a  proper drug therapy can work shorter. Such a therapy can  be  verified with no difficulties and it can be compared with any other type of  intervention used to improve the cognitive abilities of mentally retarded children. 

Moreover it is fully compatible with education or rehabilitation programs. The definition itself of mental retardation is hereby specified. On the basis of the three-party conception, mental retardation may be:

- caused by pure dysfunction (full mental pseudo-debility);

·   partly  caused by a mental deficiency (lesion) and partly  by  a dysfunction (mental pseudo-debility).

Both of them coexist in the same subject.

Dysfunction of brain cells: myth or reality? The  proposed  presence of some alive but impaired neurons in  cognitive  brain areas, may be the key to solve the contradiction between the facts (recovery  of some cognitive abilities) and the usual frame of reference. This latter is up to now stating that  i. a dead neuron cannot resurrect; ii. certainly the sprouting of new neurons, if possible, shall never be a short-term process. 

Do  we have any evidence of the existence of alive neurons, which  are  "sick" and then with a dysfunction?  The answer is: yes we do. By now the fact that  an epileptic  focus  is made by alive cells but sick, often around the  lesion,  is well  known fact. Under the effect of special stimuli, which are  all  stressors (physical, chemical, biological or psychological stressors) these cells start up an anomalous discharge. This is the starting-point of an epileptic fit.

Although these cells can be also part of a brain area such as the temporal region,  whose links with intelligence certainly exist but are not well defined, one can object that mental retardation and epilepsy are different and not comparable phenomena.

Two  cases, early diagnosed as autism, then were correctly revised  as  temporal epilepsy,  following  an  EEG control. Treated as such they  had  an  impressive cognitive recovery (Gillberg & Schaumann, 1983).  In  epilepsy, temporary post-seizure paralysis, also called  Todd’s  paralysis, call for a dysfunction which can cause a serious motor disorder. Yet such a dis- order goes quickly to an end. It can be said that also this example is not really convincing since its  cause is not linked to the outcome of  a  brain lesion.  Besides recovery times are definitely short (hours or, at most, one day). 

It is however urgent  to point out another fact. In the forms of depression so-called  "inhibited"  a  cognitive  slowing down is  often  put  into  light.  In childhood  depression  this  happens in 2 out of 3 cases  (Nissen,  1973).  This process  concerns  the  greater  number of mental  pseudo-deficiency   forms  in children (Cocchi, 1985; Cordella 1988). On the other hand psychodrug therapy  is to be seen, or at least it should be, a therapy of the neuronal dysfunction.  It is therefore easier to relate the partial recover of cognitive impairment to cell dysfunction, reversible as such.

At the moment we do not exactly know what such a neuronal dysfunction is.   The most  probable  hypothesis refers to reduction of dendrites  and  then  synaptic decrease,  as  a result of neurotoxic effect of  glucocorticoids  and  glutamate (Cordella  1988, 1989a and 1989b). Such a reduction is preserved by  the  stress  condition  that every chronic disease (mental retardation included)  imposes  on the organism. In acute forms, such as in cognitive slowing down in cases of pure depression, the dysfunction would be fully caused by stress condition,  probably without any dendrites or synapses reduction but with decrease of receptor sites. 

In such a way it is possible to find out a single explanation to mental  pseudo-debility, pseudo-dementia and also to motor pseudo-debility.  Otherwise  we may run the risk of needing of more explicative hypothesis.  This would  violates Occam’s razor principle,  the exact logical counterpart  of  the principle of economy at biological level. In  my  opinion,  it is easier to suppose that a biological  problem  could  be solved by biological means rather than by any other means (psychological  and/or educational). 

Previous argumentation might be accepted in order to explain  any improvement which could be obtained in  mentally retarded children with  evident brain  lesions.  All the more reason, the same argument may be considered  valid for the reduction of mental retardation in those subjects where is not  possible to  determine a specialised lesion. This is odd to the cognitive or other  brain areas. For  convenience  and owing to a long personal experience, I will  continue  to refer to Down’s syndrome children.

The  cognitive  disease  of  these  subjects  may  be  defined  as  a   general retardation. Each central component of it, necessary to realise the cognitive action, partly contributes  to the disease, as a result of diffuse  micro-lesions. Besides,  on the basis of one of my research, these children may develop some shield  against anoxic-ischemic damage, of pre-,peri-, and neo-natal origin (Cocchi 1987; Cocchi 1988),  at  least for the CP outcomes.  The increased incidence of CP  in  post-natal  time  (Cocchi,  1990) seems to be related to the starting  of  a  diffuse deterioration  which  moves faster just after the birth (Cocchi  1990).  

In  any case, if the drug therapy gets evident results in a short time, it can only  act through  the reduction of part of the mental pseudo-debility caused by  neuronal dysfunction.  This portion is connected to the true mental deficiency and it  is jointly  responsible  for the overall mental retardation.  As it has  been  said before  (Cocchi,  1991)  this  neuronal dysfunction  in  mentally  retarded  can generally be preserved by the bio-psycho-social stress which the disease  itself implies.  Yet a metabolic endogenous stress, induced by the acceleration of  all metabolisms  which  are controlled by genes placed on the  chromosome  21,  also persists in Down children.  Such  metabolisms  have  increased  of 50%  (the  so-called  "dosage  effect", experimentally  verified). Because of the presence of chromosome 21 they are  no longer controlled by two genes (100%) but by three genes (150%). 

This  metabolic stress was the principal cause of a negative influence  on  the brain  cognitive areas since their differentiation, because it starts  from  the conception, or little after, in mosaic forms. Final remarks The  most probable explanation to a more or less wide improvement of  cognitive debility  in mentally retarded, at least in my opinion lies in a part of  mental pseudo-debility  caused  by  a neuronal cell dysfunction.   Such  a  dysfunction develops when stress, any kind of stress, is taking place.  In mentally retarded without  any  evident  neurological lesion, such as Down subjects,  a  share  of mental  pseudo-debility,  elicited  by the results of the therapy,  in  a  broad sense,  seems  partly to be the cause of the reduced  cognitive  abilities. 

The treatment of this part of mental pseudo-debility with drugs in such subjects has obtained positive results in short time.  This fact opens  the  way  to  further practical and theoretical prospects in the field of cognitive diseases and their treatment.  

 

References.

Bax  M.:  Controlled trial of physical therapy at John Hopkins.  Develop.  Med. Child Neurol. 1988, 30: 285-286.

Cocchi R.: La pseudoinsufficienza mentale. In: Strutture e dinamiche  neuropsi-copatologiche in eta’ evolutiva. Montefeltro, Urbino 1985.   

Cocchi  R.: Presenza di scavengers e incidenza di paralisi cerebrali  infantili da  prematurita’  e  basso peso alla nascita in 381 soggetti  Down  allevati  in famiglia.  Giorn. Neuropsich. Eta’ Evol. 1987, 7: 317-323.  

Cocchi R.: The birth as a second breakpoint in the biological balance of  Down’ syndrome  subjects: Confirmation and implications.  It. J. Intellect.  Impair.  1990, 3: 179-183.

Cocchi  R.: Paralisi cerebrali infantili in bambini Down; 3 casi.   Riv.  It. Disturbo Intellet. 1990, 3: 327-330.

Cordella L.: Mental pseudo-debility: An expanding concept. It. J.  Intellect. Impair. 1988, 1: 3-8. Cordella  L.: Stress, glucocorticoidi e handicap: il danno pre- e  perinatale.. Riv. It. Disturbo Intellet. 1989, 2: 75-83.

 Cordella L.: Stress, glucocorticoidi e handicap. II. Le influenze post-natali. Riv. It. Disturbo Intellet. 1989, 2: 93-101.

 Gillberg  C., Schaumann H.: Epilepsy presenting as Infantile Autism?  Two  case studies. Neuropediatrics 1983, 14: 206-212.  

Nissen  G.: La depression masquee chez l'enfant et l'adolescent. In:  Kielholz P. (ed) La depression masquee, Huber, Berne 1973: 135-151.  

Savoldi  F.,  Zerbi F., Cocchi R.: Sull'entita' clinica di  pseudodemenza.  In: L'invecchiamento tra paura e desiderio. Vol. I. Idelson, Napoli 1986: 251-256.  

 

Paper presented during the IX World Congress of the IASSMD. Brisbane / Broadbench,  1992.

Printed on It. J. Intellect. Impair. 1992, 5: 137-142

 

Author's address : dr Renato COCCHI, via Rabbeno, 3

42100 Reggio Emilia (Italy)

renatococchi@libero.it

 

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Mental retardation

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