Amyotrophic Lateral Sclerosis (HAS)

January 2017

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.


ALS Diagnosis

First symptoms

  • 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.

Genetic inquiry

  • 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.

ALS Evaluation

  • 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

Medicinal therapy

  • Etiologic aim :
    • Administration of riluzole,
      • Increase survival,
      • Delay the use of mechanic ventilation.
  • Symptomatic:
    • Muscular pains:
      • Quinine,
      • Kinesitherapy ,
    • 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

  • Physiotherapy:
    • Orthopedic,
    • Kinesitherapy.
  • Assisting the organisation of the patient's environment:
    • Orthosis,
    • 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.

Sources


Caring about people suffering from amyotrophic lateral sclerosis, HAS, 2006.

Photographic copyright: Oguzaral Dreamstime.com

Related


Sclérose latérale amyotrophique (HAS)
Sclérose latérale amyotrophique (HAS)
Esclerosis lateral amiotrófica o Enfermedad de Charcot
Esclerosis lateral amiotrófica o Enfermedad de Charcot
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