NIH Clinical Center logo     America's Clinical Research Hospital File folder iconExplore the NIH
Clinical Center
Search the Studies - NIH Clinical Research Studies

Protocol Details

Screening and Natural History: Primary Lateral Sclerosis and Related Disorders

This study is currently recruiting participants.

Summary | Eligibility | Citations | Contacts




Sponsoring Institute

National Institute of Neurological Disorders and Stroke (NINDS)

Recruitment Detail

Type: Participants currently recruited/enrolled
Gender: Male & Female
Min Age: 18
Max Age: N/A

Referral Letter Required


Population Exclusion(s)


Special Instructions

Currently Not Provided


Apparently Sporadic Spastic Paraplegia;
Motor Neuron Disease;
Amyotrophic Lateral Sclerosis;
Primary Lateral Sclerosis

Recruitment Keyword(s)

Muscle Stiffness;
Involuntary Muscle Contraction;
Isaacs Syndrome


Primary Lateral Sclerosis

Investigational Drug(s)


Investigational Device(s)




Supporting Site

National Institute of Neurological Disorders and Stroke


The objectives of this protocol are:

to develop and maintain a repository of clinically characterized patients with primary lateral sclerosis for future research protocols,

to characterize the natural history of neurodegenerative disorders with corticospinal neuron degeneration,

to investigate proposed etiologies, risk factors, and biomarkers for the development of these disorders and for disease progression

Study Population:

240 patients with adult-onset progressive Spasticity with a diagnosis of primary lateral sclerosis or related upper motor neuron disorder


Patients who have been referred by physicians for primary lateral sclerosis will undergo a screening evaluation at the first visit. The screening visit will include review of outside medical records, neurological examination, and diagnostic testing to determine possible causes of Spasticity. Patients fulfilling the clinical criteria for primary lateral sclerosis by history or examination will be followed to determine the natural history of this disorder. Measures of motor and cognitive function will be made at baseline and follow-up visits to follow clinical progression. Magnetic resonance imaging will be carried out to determine if imaging changes occur over time. Patients identified in this protocol who are eligible for other research protocols will be invited to participate in additional protocols.

Outcome Measures:

Clinical progression will be documented by measures of finger-tapping, timed gait, speech. The association between clinical progression and MRI measures will be assessed as a secondary outcome.

--Back to Top--



-Age 18 years of age or older

-Adult onset of progressive Spasticity

-No family history of a similar disorder

-Able to provide consent or with a legally-authorized representative who can provide consent


-History of stroke, cerebral palsy, traumatic brain injury or other known etiology of Spasticity

-Non-neurological disorders producing muscle stiffness, such as fasciitis or rheumatological conditions

-Disorders in which pain limits the ability to move muscles, such as fibromyalgia or complex regional pain syndromes

-Profound weakness of voluntary movement

-Inability to travel to NIH

-Anticoagulation will be an exclusion for needle EMG studies

-Implanted devices or metal fragments in the brain or spinal cord will be an exclusion for MRI scanning

--Back to Top--


Benign nocturnal leg cramps Current controversies over use of quinine

Exercise-induced muscle cramp Proposed mechanisms and management

Diseases associated with excess motor unit activity

--Back to Top--


Principal Investigator

Referral Contact

For more information:

Mary Kay Floeter, M.D.
National Institute of Neurological Disorders and Stroke (NINDS)
National Institutes of Health
Building 10
Room 6-5724
10 Center Drive
Bethesda, Maryland 20892
(301) 496-7428

Carol H. Hoffman
National Institute of Neurological Disorders and Stroke (NINDS)
National Institutes of Health
Building 10
Room 1D45
10 Center Drive
Bethesda, Maryland 20892
(301) 496-7428

Patient Recruitment and Public Liaison Office
Building 61
10 Cloister Court
Bethesda, Maryland 20892-4754
Toll Free: 1-800-411-1222
TTY: 301-594-9774 (local),1-866-411-1010 (toll free)
Fax: 301-480-9793

Clinical Trials Number:


--Back to Top--


Contact the Patient Recruitment and Public Liaison Office for:

  • Details on how to participate in a study
  • Details on how to refer a patient to a study

NIH Clinical Studies Information Request

Contact the Office of Communications for:

  • General information about the NIH Clinical Center

Contact the Department Clinical Research Informatics, (DCRI) for:

  • Technical questions about Adobe Acrobat and the PDF format
  • Technical questions about this web server