What is amyotrophic lateral sclerosis ALS

Amyotrophic lateral sclerosis (ALS) is a progressive degenerative disorder of motor neurons in the spinal cord, brainstem, and motor cortex manifested clinically by muscular weakness, atrophy, and corticospinal tract involvement.

Clinical presentation typically includes atrophic weakness of hands and forearms, slight spasticity of the legs, and generalized hyperreflexia. Other findings may include hand and finger stiffness, cramping, fasciculations, and atrophy and weakness of tongue, pharyngeal, and laryngeal muscles. There is no sensory loss. The disease is characterized by middle life presentation and death is usually within 2 to 6 years. Diagnosis is made on the basis of history and neurologic examination and electromyography (EMG)/nerve conduction studies. Riluzole is a medication to treat ALS and may improve the neurologic function and survival. Its mechanism is not well understood. Physical therapy, occupational therapy, and speech therapy are necessary treatments. Symptomatic treatment for depression, secretion control, pain, fatigue, muscle spasms, and constipation are supportive measures. The disease is also called Lou Gehrig's disease, named for the New York Yankee's baseball player who died from this disorder.

Key Points

1. The differential diagnosis of a spinal cord tumor is determined by the anatomic compartment in which it occurs (i.e. extradural, intradural-extramedullary, or intramedullary).

2. Syringohydromyelia is an abnormal fluid cavity within the spinal cord, which may cause progressive neurologic dysfunction.

3. Spinal dysraphism is broadly classified into two forms: spina bifida occulta and spina bifida cystica.

4. Myelomeningocele is the most common significant spinal birth defect and results from disruption of the process of neurulation between days 24 and 28 following conception.

Websites

Chiari malformation: http://emedicine.medscape.com/article/1483583-overview Intramedullary spinal cord tumors: http://emedicine.medscape.com/article/251133-overview Neural tube defects: http://emedicine.medscape.com/article/1177162-overview Spinal cord disorders: http://neuromuscular.wustl.edu/spinal.html

Spinal dysraphism and myelomeningocele: http://emedicine.medscape.com/article/413899-overview Syringomyelia: http://emedicine.medscape.com/article/1151685-overview

Bibliography

1. Batjer HH, Loftus C, editors. Textbook of Neurological Surgery: Principles and Practice. Philadelphia: Lippincott Williams and Wilkins; 2002.

2. Gebauer GP, Farjoodi P, Sciubba DM, et al. Magnetic resonance imaging of spine tumors: Classification, differential diagnosis, and spectrum of disease. J Bone Joint Surg 2008;90A:146-62.

3. Lew SM, Kothbauer KF. Tethered cord syndrome: an updated review. Pediatr Neurosurg 2007;43:236-48.

4. Sutton LN, Schwartz DM. Congenital anomalies of the spinal cord. In: Herkowitz HN, Garfin SR, Eismont FJ, Bell GR, Balderston RA, editors. Rothman-Simeone The Spine. 5th ed. Philadelphia: Saunders; 2006. p. 675-707.

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