History

Primary lateral sclerosis (PLS) usually presents with gradual-onset, progressive, lower-extremity stiffness and pain due to spasticity. Onset is often asymmetrical. Affected individuals typically have no family history of similar disorders.

As PLS progresses, patients may develop balance problems and have a tendency to fall. Axial muscle involvement may result in lower back and neck pain, which may aggravate back or neck pain from other causes (eg, degenerative disc disease, osteoporosis).

As the upper extremities become involved, patients may have difficulties with activities of daily living (ADLs). Involvement of the organs of speech may result in spastic dysarthria (which initially may be mild).

Swallowing and breathing may be compromised late in the disease.

Physical Examination

Signs of upper motor neuron dysfunction may include limb and trunk spasticity, pathologic spread of deep tendon reflexes, clonus, pathologic reflexes (such as Babinski sign), and spastic dysarthria.

Signs of involvement of other systems should not be present. In particular, no cerebellar findings, involuntary movements, sensory findings, findings suggesting lower motor neuron dysfunction (such as fasciculations), visual findings, or bladder dysfunction should be observed.

Differential Diagnoses

Younger DS, Chou S, Hays AP, Lange DJ, Emerson R, Brin M, et al. Primary lateral sclerosis. A clinical diagnosis reemerges. Arch Neurol. 1988 Dec. 45(12):1304-7. [QxMD MEDLINE Link].
Pringle CE, Hudson AJ, Munoz DG, Kiernan JA, Brown WF, Ebers GC. Primary lateral sclerosis. Clinical features, neuropathology and diagnostic criteria. Brain. 1992 Apr. 115 ( Pt 2):495-520. [QxMD MEDLINE Link].
Gordon PH, Cheng B, Katz IB, Pinto M, Hays AP, Mitsumoto H, et al. The natural history of primary lateral sclerosis. Neurology. 2006 Mar 14. 66(5):647-53. [QxMD MEDLINE Link].
Tartaglia MC, Rowe A, Findlater K, Orange JB, Grace G, Strong MJ. Differentiation between primary lateral sclerosis and amyotrophic lateral sclerosis: examination of symptoms and signs at disease onset and during follow-up. Arch Neurol. 2007 Feb. 64(2):232-6. [QxMD MEDLINE Link].
Brugman F, Veldink JH, Franssen H, de Visser M, de Jong JM, Faber CG, et al. Differentiation of hereditary spastic paraparesis from primary lateral sclerosis in sporadic adult-onset upper motor neuron syndromes. Arch Neurol. 2009 Apr. 66(4):509-14. [QxMD MEDLINE Link].
Panzeri C, De Palma C, Martinuzzi A, Daga A, De Polo G, Bresolin N, et al. The first ALS2 missense mutation associated with JPLS reveals new aspects of alsin biological function. Brain. 2006 Jul. 129:1710-9. [QxMD MEDLINE Link].
Valdmanis PN, Dupré N, Rouleau GA. A locus for primary lateral sclerosis on chromosome 4ptel-4p16.1. Arch Neurol. 2008 Mar. 65(3):383-6. [QxMD MEDLINE Link].
Ciccarelli O, Behrens TE, Johansen-Berg H, Talbot K, Orrell RW, Howard RS, et al. Investigation of white matter pathology in ALS and PLS using tract-based spatial statistics. Hum Brain Mapp. 2009 Feb. 30(2):615-24. [QxMD MEDLINE Link].
Iwata NK, Kwan JY, Danielian LE, Butman JA, Tovar-Moll F, Bayat E, et al. White matter alterations differ in primary lateral sclerosis and amyotrophic lateral sclerosis. Brain. 2011 Sep. 134:2642-55. [QxMD MEDLINE Link]. [Full Text].
Stark FM, Moersch FP. Primary lateral sclerosis: a distinct clinical entity. J Nervous Mental Disease. 1945. 102:332-337.
Joyce NC, Carter GT. Electrodiagnosis in persons with amyotrophic lateral sclerosis. PM R. 2013 May. 5(5 Suppl):S89-95. [QxMD MEDLINE Link].
Vinceti M, Solovyev N, Mandrioli J, Crespi CM, Bonvicini F, Arcolin E, et al. Cerebrospinal fluid of newly diagnosed amyotrophic lateral sclerosis patients exhibits abnormal levels of selenium species including elevated selenite. Neurotoxicology. 2013 May 31. [QxMD MEDLINE Link].
Dal Bello-Haas V, Florence JM. Therapeutic exercise for people with amyotrophic lateral sclerosis or motor neuron disease. Cochrane Database Syst Rev. 2013 May 31. 5:CD005229. [QxMD MEDLINE Link].
Gibbons C, Thornton E, Ealing J, Shaw P, Talbot K, Tennant A, et al. The impact of fatigue and psychosocial variables on quality of life for patients with motor neuron disease. Amyotroph Lateral Scler Frontotemporal Degener. 2013 May 31. [QxMD MEDLINE Link].

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Author

Carmel Armon, MD, MSc, MHS Chair, Department of Neurology, Assaf Harofeh Medical Center, Tel Aviv University Sackler Faculty of Medicine, Israel

Carmel Armon, MD, MSc, MHS is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Association of Neuromuscular and Electrodiagnostic Medicine, American Clinical Neurophysiology Society, American College of Physicians, American Epilepsy Society, American Medical Association, American Neurological Association, American Stroke Association, Massachusetts Medical Society, Sigma Xi, The Scientific Research Honor Society

Disclosure: Received research grant from: Neuronix Ltd, Yoqnea'm, Israel<br/>Received income in an amount equal to or greater than $250 from: JNS - Associate Editor. UpToDate - Author Royalties.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Nicholas Lorenzo, MD, CPE, MHCM, FAAPL Co-Founder and Former Chief Publishing Officer, eMedicine and eMedicine Health, Founding Editor-in-Chief, eMedicine Neurology; Founder and Former Chairman and CEO, Pearlsreview; Founder and CEO/CMO, PHLT Consultants; Former Chief Medical Officer, MeMD Inc

Nicholas Lorenzo, MD, CPE, MHCM, FAAPL is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, American Association for Physician Leadership

Disclosure: Nothing to disclose.

Acknowledgements

Paul E Barkhaus, MD Professor, Department of Neurology, Medical College of Wisconsin; Director of Neuromuscular Diseases, Milwaukee Veterans Affairs Medical Center

Paul E Barkhaus, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and American Neurological Association

Disclosure: Nothing to disclose.

Neil A Busis, MD Chief, Division of Neurology, Department of Medicine, Head, Clinical Neurophysiology Laboratory, University of Pittsburgh Medical Center-Shadyside

Neil A Busis, MD is a member of the following medical societies: American Academy of Neurology and American Association of Neuromuscular and Electrodiagnostic Medicine

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Primary Lateral Sclerosis Clinical Presentation

History

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