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Symptoms
By Mayo Clinic staffSigns and symptoms of primary lateral sclerosis typically take years to progress. They include:
- Stiffness, weakness and spasticity in your legs
- Tripping, difficulty with balance and clumsiness as the leg muscles weaken
- Weakness and stiffness progressing to your trunk, then your arms, hands, tongue and jaw
- Hoarseness, reduced rate of speaking, slurred speech and drooling as the facial muscles weaken
- Difficulties with swallowing and breathing late in the disease
Less commonly, PLS begins in the tongue or hands, and then progresses down the spinal cord to the legs.
When to see a doctor
Make an appointment to see your doctor if you have persistent problems with stiffness or weakness in your legs, or with swallowing or speaking.
If your child develops involuntary muscle spasms or seems to be losing balance more often than usual, make an appointment with a pediatrician for an evaluation.
References
- NINDS primary lateral sclerosis information page. National Institute of Neurological Disorders and Stroke. http://www.ninds.nih.gov/disorders/primary_lateral_sclerosis/primary_lateral_sclerosis.htm. Accessed Aug. 17, 2010.
- Juvenile primary lateral sclerosis. National Institutes of Health Genetics Home Reference. http://ghr.nlm.nih.gov/condition=juvenileprimarylateralsclerosis. Accessed Aug. 16, 2010.
- Murray B, et al. Disorders of upper and lower motor neurons. In: Bradley WG, et al. Neurology in Clinical Practice. 5th ed. Philadelphia, Pa.: Butterworth-Heinemann Elsevier; 2008. http://www.mdconsult.com/book/player/book.do?method=display&type=bookPage&decorator=header&eid=4-u1.0-B978-0-7506-7525-3..50116-3&displayedEid=4-u1.0-B978-0-7506-7525-3..50116-3--cesec19&uniq=215117994&isbn=978-0-7506-7525-3&sid=1040472768. Accessed Aug. 16, 2010.
- Brugman F, et al. Adult-onset primary lateral sclerosis is not associated with mutations in the ALS2 gene. Neurology. 2007;69:702.
- Tartaglia MC, et al. Differentiation between primary lateral sclerosis and amyotrophic lateral sclerosis: Examination of symptoms and signs at disease onset and during follow-up. Archives of Neurology. 2007;64:232.
- Gordon PH, et al. Clinical features that distinguish PLS, upper motor neuron-dominant ALS, and typical ALS. Neurology. 2009;72:1948.
- Hadano S, et al. Molecular and cellular function of ALS2/alsin: Implication of membrane dynamics in neuronal development and degeneration. Neurochemistry International. 2007;51:74.
- Motor neuron diseases fact sheet. National Institute of Neurological Disorders and Stroke. http://www.ninds.nih.gov/disorders/motor_neuron_diseases/detail_motor_neuron_diseases.htm. Accessed Aug. 17, 2010.
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