Motor Neuron Disease (Primary Lateral Sclerosis)

Diseases characterized by a selective degeneration of the motor neurons of the spinal cord, brainstem, or motor cortex. Clinical subtypes are distinguished by the major site of degeneration. In AMYOTROPHIC LATERAL SCLEROSIS there is involvement of upper, lower, and brainstem motor neurons. In progressive muscular atrophy and related syndromes (see MUSCULAR ATROPHY, SPINAL) the motor neurons in the spinal cord are primarily affected. With progressive bulbar palsy (BULBAR PALSY, PROGRESSIVE), the initial degeneration occurs in the brainstem. In primary lateral sclerosis, the cortical neurons are affected in isolation. (Adams et al., Principles of Neurology, 6th ed, p1089)
Also Known As:
Primary Lateral Sclerosis; Motor System Disease; Motor Neuron Diseases; Lateral Sclerosis; Motor Neuron Disease, Familial; Anterior Horn Cell Disease; Lower Motor Neuron Disease; Motor Neuron Disease, Secondary; Secondary Motor Neuron Disease; Upper Motor Neuron Disease; Lateral Scleroses; Lateral Scleroses, Primary; Lateral Sclerosis, Primary; Motor System Diseases; Neuron Disease, Motor; Neuron Diseases, Motor; Primary Lateral Scleroses; Scleroses, Lateral; Scleroses, Primary Lateral; Sclerosis, Lateral; Sclerosis, Primary Lateral; Familial Motor Neuron Disease; Motor Neuron Disease, Lower; Motor Neuron Disease, Upper
Networked: 1250 relevant articles (34 outcomes, 108 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Amyotrophic Lateral Sclerosis (Lou Gehrig's Disease)
2. Nervous System Diseases (Neurological Disorders)
3. Parkinson Disease (Parkinson's Disease)
4. Disease Progression
5. Stroke (Strokes)


1. Sobue, Gen: 33 articles (12/2015 - 03/2003)
2. Katsuno, Masahisa: 26 articles (12/2015 - 03/2003)
3. Tanaka, Fumiaki: 26 articles (05/2013 - 06/2004)
4. Adachi, Hiroaki: 18 articles (12/2015 - 03/2003)
5. Trojanowski, John Q: 16 articles (01/2012 - 09/2004)
6. Suzuki, Keisuke: 15 articles (11/2012 - 03/2006)
7. Banno, Haruhiko: 15 articles (11/2012 - 03/2006)
8. Lee, Virginia M-Y: 15 articles (01/2012 - 09/2004)
9. Brown, Robert H: 11 articles (01/2014 - 04/2003)
10. Minamiyama, Makoto: 11 articles (07/2013 - 03/2003)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Motor Neuron Disease:
1. Biological Markers (Surrogate Marker)IBA
2. AntibodiesIBA
3. Ciliary Neurotrophic Factor (CNTF)IBA
4. RiluzoleFDA LinkGeneric
5. Dyneins (Dynein)IBA
6. Brain-Derived Neurotrophic Factor (BDNF)IBA
7. Proteins (Proteins, Gene)IBA
8. Glutamic Acid (Glutamate)FDA Link
9. GangliosidesIBA
10. Histone Deacetylase InhibitorsIBA

Therapies and Procedures

1. Denervation
2. Drug Therapy (Chemotherapy)
3. Transplantation (Transplant Recipients)
4. Muscle Denervation
5. Gastrostomy