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Institute for Neurological Disorders
Institute for Neurological Disorders

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

 

Organizational Structure of the Neurodegenerative Disorders Division

The Neurodegenerative Disorders Division has these subdivisions:

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

Neurodegenerative Disorders Division Investigators

Name Affiliation
Agbas, Abdulbaki Department of Biochemistry - KCMB
Agbas, Dora Molecular and Integrative Physiology - KUMC
Anderson, Heather Neurology - KUMC
Barohn, Richard Neurology - KUMC
Belousov, Andrei Molecular and Integrative Physiology - KUMC
Bruce, Amanda Hoglund Brain Imaging Center - KUMC
Bruses, Juan Anatomy & Cell Biology - KUMC
Burns, Jeff Neurology - KUMC
Butler, Merlin Psychiatry & Behavior Science - KUMC
Choi, In-Young Hoglund Brain Imaging Center - KUMC
Dimachkie, Mazen Neurology - KUMC
Dobrowsky, Richard Pharmacology, Toxicology & Therapeutics - KUMC
Dubinsky, Richard Neurology - KUMC
Farassati, Faris Gastroenterology/Hepatology/Motility - KUMC
Festoff, Barry Neurology and Pharmacology - VAMC
Geiger, Paige Molecular and Integrative Physiology - KUMC
Grant, John Neurosurgery - KUMC
Hammond, Nancy Neurology - KUMC
Honea, Robin Alzheimer and Memory Program - KUMC
Johnson, Michael Chemistry - KU
Kluding, Patricia SAH Physical Therapy Rehabilitation Sciences - KUMC
Krise, Jeff Pharmaceutical Chemistry - KU
Krumlauf, Robb Anatomy & Cell Biology - KUMC
Lee, Sang-Pil Hoglund Brain Imaging Center - KUMC
Levine, Steven Molecular and Integrative Physiology - KUMC
Liu, Wen SAH Physical Therapy Rehabilitation Sciences - KUMC
Lynch, Sharon Neurology - KUMC
Lyons, Kelly Neurology - KUMC
McCarson, Kenneth Pharmacology, Toxicology & Therapeutics - KUMC
McVey, April Neurology - KUMC
Michaelis, Eli Pharmacology, Toxicology & Therapeutics - KUMC
Michaelis, Mary Pharmacology, Toxicology & Therapeutics - KUMC
Muma, Nancy Pharmacology, Toxicology & Therapeutics - KUMC
Nazzaro, Jules Surgery Neurosurgery - KUMC
Newell, Kathy Pathology - KUMC
Osorio, Ivan Neurology - KUMC
Pawah, Rajesh Neurology - KUMC
Pollack, Ania Neurosurgery - KUMC
Pasnoor, Mamatha Neurology - KUMC
Reeves, Alan Radiology - KUMC
Salacz, Michael Neuro-Oncology, Medical Oncology - KUMC
Savage, Cary Hoglund Brain Imaging Center - KUMC
Smith, Peter KIDDRC - KUMC
Stanford, John Molecular and Integrative Physiology - KUMC
Swerdlow, Russell

Obstetrics and Gynecology - KUMC

Taylor, Sarah  
Wang, Fen  
Wang, WenFang SAH Physical Therapy Rehabilitation Sciences - KUMC
Wang, Yunxia Neurology - KUMC
Wright, Doug Anatomy & Cell Biology - KUMC
Yu, Ron Anatomy & Cell Biology - KUMC
Zhu, Hao SAH Physical Therapy Rehabilitation Sciences - KUMC