The neuropsychologist and his role with ataxic patients
by Thérèse Botez-Marquard
Neuropsychologist
“What is a neuropsychologist?”, one often asks this question and with reason.
The definition can be summarized thus:
Human neuropsychology is the study of neurological mechanisms (anatomical, physiological and neurochemical) which underlie human behaviour. The discipline is based on a systematic analysis of consecutive behavioural troubles to the alterations of the activity of the normal brain by the disease (degenerative, inflammatory, viral, intoxication, etc.), the damage (stroke, tumour, skull trauma, etc.) or an alteration (removal of a tumour or brain tissue).
(Reference: M. I. Botez, Clinical neuropsychologist and behavioural neurology, PUM and Masson, 1987)
The clinical neuropsychologist has for his (her) task to measure and to analyze in humans the changes in their intellectual, sensorial, mnemonic (memory), attentive and executive (anticipation, planning elaboration of mental strategies) as well as changes in personality which can be manifested following a brain problem.
As for the neurology of behaviour, it goes back to the beginning of medical science (Egypt 2500 – 3000 before Jesus Christ) and gives evidence of the practice of autopsies of the skull and of trepidation suggesting the recognition of the links between the brain and behaviour. It is behavioural neurology which gave birth to clinical neuropsychology and to experimental neuropsychology at the beginning of the 20th century. Behavioural neurology is an approach which takes place in general at the bedside of the patient and which studies individual cases by organizing test situations which allow one to distinguish the abnormal deviations from the normal functioning. This type of test is given most of the time by the neurologist or the neuropsychiatrist.
The Ataxia Clinic at the Hôtel-Dieu of Montreal
In the hospital milieu, it is the attending physician, that is to say the neurologist, who requests the neuropsychological assessment. The latter has as its goal:
of the sensory-motor and cognitive abilities as well as a profile of affect;
assessment can, in fact, be repeated, especially if there is reason to believe that the
cognitive functions are deteriorating.
The assessment of cognitive functions in ataxic patients (Friedreich, Charlevoix-Saguenay, and the SCA 2, 3, 4, 6, 8 etc.)allows a better understanding of the functions of the cerebellum in the modulation of neuronal projections toward the encephalon and the cortex of the brain. Formerly, it was believed that the function of the cerebellum was limited to the motor functions and balance. Since then studies of the cerebellum benefited from an unbelievable scientific development and continue today to display the cerebellar mechanisms which intervene with the emotions and cognitive functions.
At our ataxic clinic, the neuropsychological assessment helps to qualify the different symptoms and to demonstrate the stability or the evolution of the disease.
I would like to proclaim, loud and clear, that it is thanks to the ataxic patients who participated in these studies with enthusiasm and curiosity, here and elsewhere in the world, that science has progressed so much in this field.
Today great efforts are exhibited in different research centres for advancement in regards to medication and genetics.
One must not fail to mention the exemplary efforts which have been deployed as regards to the taking charge of ataxic patients (here in Montreal: Lucie-Bruneau Rehabilitation Centre and Hôpital Marie-Enfant; and of course elsewhere).
The neuropsychologist and his role with ataxic patients
The neuropsychologist has first of all the privilege and the duty to establish a relationship of confidence with the patient. One also brings along a primordial interest in the emotions of the person who is consulting him, his well-being, his behavioural troubles reported by the patient or his entourage and which are often secondary to the situation in which the patient finds himself. The neuropsychologist can hereby also be brought to intervene as for:
The neuropsynchologist can also easily become a listener of quality for the patient. He can furthermore intervene on the level of social services in order to elucidate the situation with the social worker, for example. He can also reply to the patient’s questions and inform him in a satisfactory way by inducing reasonable hopes, but not false hopes. One can learn to come to terms with the disease without really accepting it. Besides, who would accept it? Without doubt, no one. But we have observed over several years that the patients who continue to fight keep their head high, socialize and get instructed, and come out of it in general much better.
The parents of ataxic children, just as the brothers and sisters of ataxic adults, should also receive all the information necessary to understand that which their young children or their close ones live. The neuropsychologist can intervene to fulfill this need of consultation; and eventually direct the persons toward the appropriate resources.
Permit me to close on a personal note since I have been working in the clinic for ataxic patients since 18 years and I hold these patients dear. I feel very privileged, having learned enormously on the personal, professional, and scientific levels and I continue to work in this clinic hoping that the young generation will soon take over from the neuropsychological point of view.