Abstract
Motor neurons disorders: amyotrophic lateral sclerosis (ALS), spinal bulbar muscular atrophy (SBMA or Kennedy’s disease), and spinal muscular atrophy (SMA) are characterized by the degeneration of cortical motor neurons, lower motor neurons, or both.
ALS is invariably fatal, even if its clinical phenotypes have different prognostic characteristics. Median survival is 2–3 years.
SBMA is adult-onset, X-linked recessive, due to expansion of a CAG repeat. Patients’ long-term survival is only slightly reduced.
SMA is caused by a homozygous deletion of the survival motor neuron 1 gene. Death occurs within 2 years (type I); within the second decade (type II). Patients are ambulant until adult life, and sometimes have favorable prognosis quoad vitam (type III and type IV).
Hirayama disease (monomelic amyotrophy), a cervical myelopathy formerly classified as motor neuron disease, has a progressive course for 2–6 years followed by steadiness of symptoms.
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Mora, G., Chiò, A. (2015). Disorders of Upper and Lower Motor Neurons. In: Sghirlanzoni, A., Lauria, G., Chiapparini, L. (eds) Prognosis of Neurological Diseases. Springer, Milano. https://doi.org/10.1007/978-88-470-5755-5_21
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DOI: https://doi.org/10.1007/978-88-470-5755-5_21
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