Amyotrophic lateral sclerosis: Part I. Clinical features, pathology, and ethical issues in management

R. Tandan, Walter G Bradley

Research output: Contribution to journalArticle

316 Citations (Scopus)

Abstract

Amyotrophic lateral sclerosis (ALS) is a progressive degenerative disease of the motor sysem in adults that occurs in sporadic, familial, and Western Pacific forms. Involvement of non-motor pathways has been increasingly recognized, both clinically and pathologically. Although the usual course is relentlessly progressive with death in half the cases within three years from onset, it can sometimes be protracted. Degeneration and loss of large motor neurons in the cerebral cortex, brainstem, and cervical and lumbar spinal cord are characteristic. Marked reduction in the number of large myelinated fibers is notable in the cervical and lumbar ventral roots. Peripheral nerves show reduced numbers of large myelinated fibers, acute axonal degeneration at all levels, and distal axonal atrophy. Motor end-plates reveal small or absent nerve terminals. Subclinical non-motor system involvement includes neuronal loss in Clarke's nucleus and dorsal root ganglia, degeneration of non-motor tracts in the spinal cord, loss of receptors in the dorsal horns of the spinal cord, and myelinated fiber loss with segmental demyelination in sensory and mixed nerves. The serious implications of the diagnosis of ALS make it mandatory to exclude similar potentially treatable disorders. Management should be multidisciplinary, and discussions with the patient and family members should be frank and frequent. Discussions about ventilatory support should take place early in the disease so that death from respiratory failure can be prevented, when that is desired, and conversely to obviate the discontent and anger that accompany involuntary life on a ventilator.

Original languageEnglish
Pages (from-to)271-280
Number of pages10
JournalAnnals of Neurology
Volume18
Issue number3
StatePublished - Jan 1 1985
Externally publishedYes

Fingerprint

Clinical Pathology
Amyotrophic Lateral Sclerosis
Ethics
Spinal Cord
Motor Endplate
Spinal Nerve Roots
Anger
Spinal Ganglia
Motor Neurons
Demyelinating Diseases
Mechanical Ventilators
Peripheral Nerves
Respiratory Insufficiency
Cerebral Cortex
Brain Stem
Atrophy
Spinal Cord Dorsal Horn
Cervical Cord

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Amyotrophic lateral sclerosis : Part I. Clinical features, pathology, and ethical issues in management. / Tandan, R.; Bradley, Walter G.

In: Annals of Neurology, Vol. 18, No. 3, 01.01.1985, p. 271-280.

Research output: Contribution to journalArticle

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