Neurológia pre prax 6/2016

Amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by progressive degeneration of motor neurons in the primary motor cortex, corticospinal trats, brainstem and spinal cord. Progressive muscular paralysis with fasciculations represent a typical clinical presentation. Initially, only one extremity is usually affected with further progression and generalisation. Is some patients (approximately 25 %), bulbar syndrome with dysartria and dysfagia is a dominant feature at the beginning of the disease course with later generalisation of the process. The diagnosis is clinically based and is performed according so-called revised El Escorial criteria. Electromyography is useful particularly for confirmation of the neurodegenerative process in subclinicaly affected regions. The management of ALS is multidisciplinary, supportive and mainly paliative. Riluzole (glutamate inhibitor) is the only drug known to extend survival in ALS patients.

Keywords: Amyotrophic lateral sclerosis, motor neuron disease, nerve degeneration, paresis, bulbar palsy, respiratory insufficiency