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Previous Volume 340:1970-1980 June 24, 1999 Number 25 Next

	Since this article has no abstract, we have provided an extract of the first 100 words of the full text and any section headings.

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Neurodegenerative disorders, which are chronic and progressive, are characterized by selective and symmetric loss of neurons in motor, sensory, or cognitive systems. Delineation of the patterns of cell loss and the identification of disease-specific cellular markers have aided in nosologic classification: senile plaques, neurofibrillary tangles, neuronal loss, and acetylcholine deficiency define Alzheimer's disease^1,2; Lewy bodies and depletion of dopamine characterize Parkinson's disease³; cellular inclusions and swollen motor axons are found in amyotrophic lateral sclerosis⁴; and -aminobutyric acid–containing neurons of the neostriatum are lost in Huntington's disease.⁵

Mendelian inheritance can be demonstrated in many of these disorders (. . . [Full Text of this Article]

Alzheimer's Disease

Pathogenesis

The Amyloid Hypothesis

Transgenic Mice and Alzheimer's Disease

Role of Tau in the Formation of Neurofibrillary Tangles

Frontotemporal Dementia Associated with Mutations in Tau

Disorders of Trinucleotide Repeats

Huntington's Disease

Functions of the Proteasome

Spinocerebellar Ataxias

Friedreich's Ataxia

Parkinson's Disease

The Role of -Synuclein in Other Neurologic Disorders

Amyotrophic Lateral Sclerosis and Other Motor-Neuron Disorders

Amyotrophic Lateral Sclerosis

Spinal Muscular Atrophy

Spinal and Bulbar Muscular Atrophy

Familial Spastic Paraparesis

Conclusions

Source Information

From Harvard Medical School, Boston.

Address reprint requests to Dr. Martin at Harvard Medical School, 25 Shattuck St., Rm. A111, Boston, MA 02115, or at joseph_martin@hms.harvard.edu.

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