In 2006, the HAS published a conference report concerning the Amyotrophic Lateral Sclerosis (ALS), or maladie de Charcot. This disease is characterised by a paralysis of the labial, glottal, pharyngeal, laryngeal limbs and muscles resulting from progressive degeneration of neurons.
- This HAS document emphasizes that symptoms lie on the combination of symptoms involving the peripheral motor neurons and the central motor neurons :
- Motor deficiency of one or several limbs;
- Muscular atrophy and pain;
- Cramps ;
- Fasciculation (involuntary muscular contractions) ;
- Difficulties to walk, stiffness, repetitive sprains ;
- Decrease of muscular tonus ;
- Swallowing disorders, dysphonia and dysarthria ;
- Salivation stasis;
- Spasmodic laughter or cry, phonation, swallowing disorders, exaggeration of nausea reflexes, yawing
- Chin clonus, automatic voluntary dissociation of the velum palatinum.
- Other signs :
- Sensatory disorders,
- Oculo-motors paralysis,
- Sphincterian disorders,
- Marked cognitive disorders.
- Differential diagnosis according to the clinical and familial context, clinical presentation and related pathologies.
- A genetic inquiry is recommended in case of:
- An hereditary form including at least 2 cases even over several generations,
- Unusual clinical form (Kennedy disease, spinal amyotrophy for adult, juvenile ALS before the age of 20)
- Dementia related ALS.
- This HAS document states that it must :
- Be regular,
- State the physical condition of the patient and its requests,
- Allow forseeing the evolution of the disability.
- It includes:
- Interview: family, professional, social status and place of living;
- Assessment of motor and psychological incapacities,
- Manual muscular testing;
- Orthophonic check-up: dysarthria, swallowing, communication ;
- Supervising nutrition status: BMI, weight, fat mass;
- Impairment of sleeping quality and quantity;
- Dyspnoea, orthopnea , bronchial congestion.
Therapy and follow up
- Etiologic aim :
- Administration of riluzole,
- Increase survival,
- Delay the use of mechanic ventilation.
- Muscular pains:
- Salivation disorders:
- Stasis: tricyclic, scopolamine, A botulinum toxin or radiotherapy;
- Modification of salivation consistency: beta adrenergic blocking agent;
- Spasticity : baclofen, tizanidine, dantrolen or benzodiazepines;
- Emotional lability: tricyclic or inhibitors of the serotonin recapture;
- Sleeping disorders: antihistaminic or hypnotic.
Non medicinal therapy
- Assisting the organisation of the patient's environment:
- Health aid,
- Appropriate medical equipments.
- Hygiene and comfort care, feeding assistance.
- Psychological support of the patient and his relatives.
- Substitution to breathing and nutrition fonctions.
Caring about people suffering from amyotrophic lateral sclerosis
, HAS, 2006.
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Sclérose latérale amyotrophique (HAS)
Esclerosis lateral amiotrófica o Enfermedad de Charcot