Amyotrophic Lateral Sclerosis (ALS)
Traditionally, it has been described as a disease manifested by progressive weakness and atrophy of skeletal, spinal and bulbar muscles because of the degeneration of motor neurons in the anterior horn of the spinal cord and the corticospinal system. Generally, the sensory function is maintained. It is accompanied by slurred speech (dysarthria) and difficulty swallowing. Emotional lability also frequently occurs. As in other progressive neurological diseases, its aetiology is yet unknown, although it is attributed to a possible viral, toxic, genetic or endocrine cause.
At present there are no effective drugs that act in the course of the disease, but there are drugs to alleviate the symptoms. Nevertheless, with the participation of different professionals from the earliest stages of the disease, rehabilitation improves the quality of life of these patients.
In more advanced cases, the use of a pacemaker is surgically implanted in the diaphragm muscle, causing it to contract. This provides patients with certain neuromuscular disorders such as ALS, who are dependent on continuous mechanical ventilation, the ability to breathe without mechanical support (after appropriate training). This represents a significant improvement in their quality of life, reducing recurrent respiratory infections and ultimately increasing survival rate. The Institut Guttmann is a pioneer in the implantation of this type of pacemaker in our country, both in adults and in very young children (in children because of upper cervical spinal cord injury), with highly satisfactory results.
References about ALS
- Van den Berg JP, Kalmijn S, Lindeman E et al. Multidisciplinary ALS care improves quality of life in patients with ALS. Neurology. 2005 Oct 25;65(8):1264-7.
- Lunetta C, Lizio A, Sansone VA et al. Strictly monitored exercise programs reduce motor deterioration in ALS: preliminary results of a randomized controlled trial. J Neurol. 2015 Oct 17.
- Hardiman O. Management of respiratory symptoms in ALS. J Neurol. 2011 Mar;258(3):359-65.
- Beeldman E, Raaphorst J, Klein Twennaar M et al. The cognitive profile of ALS: a systematic review and meta-analysis update. J Neurol Neurosurg Psychiatry. 2015 Aug 17.
- F. Orient-López, R. Terré-Boliart, D. Guevara-Espinosa, M. Bernabeu-Guitart Tratamiento neurorrehabilitador de la esclerosis lateral amiotrófica. Rev Neurol 2006; 43 (9): 549-555