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Neurodegenerative Disorders Division

Division Director: Russ Swerdlow, MD
Associate Director Doug Wright, PhD

What is a Neurodegenerative disease?

Neurodegenerative diseases are characterized clinically by their insidious onset and chronic progression. Particular parts of the brain, spinal cord, or peripheral nerves functionally fail and the neurons of the dysfunctional region die. Neuroanatomically localizable functional impairment and neurodegeneration associate with recognizable syndromes that are ideally distinct, although in clinical and even neuropathologic practice substantial syndromic overlap exists. Neurodegenerative disease clinical syndromes are often categorized by whether they initially affect cognition, movement, strength, coordination, sensation, or autonomic control. Frequently, however, patients will present with symptoms and signs referable to more than one system. Either involvement of several systems can occur concomitantly, or else by the time the patient has functionally declined enough to seek medical attention multiple systems have become involved. Diagnosing neurodegenerative diseases can prove particularly intimidating to clinicians, because many times the diagnosis cannot be critically “confirmed” by a simple test.

Neurodegenerative diseases are complicated on other levels. While the term “neurodegenerative” implies it is the loss of neurons that cause disease, it is possible neuronal demise is merely the final stage of a preceding period of neuron dysfunction. It is difficult to know whether clinical decline associates with actual neuron loss, or with a period of neuron dysfunction that precedes neuron loss. Also, particular neurodegenerative diseases are etiologically heterogeneous. The manifestations of human nervous system failure, not surprisingly, are limited. This brings up some very real questions of lumping versus splitting.

In addition to syndromically defining neurodegenerative diseases by what neuroanatomical system is involved, these disorders are broken down along other clinical lines. Early (childhood, young adulthood, or middle aged adulthood) versus late (old age) onset is an important distinction. Some clinically similar neurodegenerative diseases are sub-categorized by their age of onset, despite the fact at the molecular level different forms of a particular disease may have very little in common. Sporadic onset versus Mendelian inheritance constitutes another important distinction, and many named neurodegenerative diseases have both sporadic (Mendelian inheritance is not recognizable) and Mendelian subtypes.

A brief list of well characterized neurodegenerative diseases. This list is not inclusive, and diseases can present in ways in which they have not been listed. ANS/PNS=autonomic nervous system/peripheral nervous system.

Cognition	Movement	Strength	Coordination	PNS/ANS	Myelin
Alzheimer’s disease Frontotemporal dementia Dementia with Lewy bodies Corticobasal degeneration Progressive supranuclear palsy Prion disorders	Parkinson’s disease Frontotemporal dementia Dementia with Lewy bodies Corticobasal degeneration Progressive supranuclear palsy Huntington’s disease Multiple system atrophy	Amyotrophic lateral sclerosis Frontotemporal dementia Hereditary spastic paraparesis	Spinocerebellar atrophies Friedreich’s ataxia Prion disorders	Amyloidoses Friedreich’s ataxia Metabolic (diabetes) related Toxin related	Multiple Sclerosis Charcot Marie Tooth

Cognition

Movement

Strength

Coordination

PNS/ANS

Myelin

Alzheimer’s disease

Frontotemporal dementia

Dementia with Lewy bodies

Corticobasal degeneration

Progressive supranuclear palsy

Prion disorders

Parkinson’s disease

Frontotemporal dementia

Dementia with Lewy bodies

Corticobasal degeneration

Progressive supranuclear palsy

Huntington’s disease

Multiple system atrophy

Amyotrophic lateral sclerosis

Frontotemporal dementia

Hereditary spastic paraparesis

Spinocerebellar atrophies

Friedreich’s ataxia

Prion disorders

Amyloidoses

Friedreich’s ataxia

Metabolic (diabetes) related

Toxin related

Multiple Sclerosis

Charcot Marie Tooth

Organizational Structure of the Neurodegenerative Disorders Division

The Neurodegenerative Disorders Division has these subdivisions:

Degenerative Disorders of the Brain
Degenerative Disorders of the Nerves
Disorders of Myelin

Degenerative Disorders of the Brain

Degenerative disorders of the brain can present as memory loss, personality change, problems with movement, weakness, or poor balance.

These neurodegenerative diseases can present with memory loss or personality change: Alzheimer’s disease, Frontotemporal Dementias, Dementia with Lewy Bodies, Prion diseases.

These neurodegenerative diseases can present as problems with movements: Parkinson’s disease, Huntington’s disease, Progressive Supranuclear Palsy, Corticobasal Degeneration, Mutiple System Atrophy.

These neurodegenerative diseases can present as weakness: amyotrophic lateral sclerosis, inclusion body myositis, degenerative myopathies.

These neurodegenerative diseases can present as poor balance: the spinocerebellar atrophies.

Degenerative Disorders of the Nerves

Degenerative disorders of nerves can present as problems with sensation, strength, pain, or autonomic control

Disorders of the peripheral nerves include diabetic neuropathy, other metabolic neuropathies, endocrine neuropathies, orthostatic hypotension

Disorders of Myelin

Disorders of Myelin include multiple sclerosis, Charcot-Marie-Tooth disease