Disease Information for Primary lateral sclerosis

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Clinical Manifestations
Signs & Symptoms
Akinetic rigid frozen Posture
Dysphagia Swallowing Difficulties
Drooling
Muscle Atrophy
Muscle stiffness/rigidity
Stiff muscles/spasms legs/feet
Stiffness
Ataxia
Awkward Uncoordinated Child
Basal ganglion signs
Clumsiness/poor coordination
Delayed walking milestone/child
Development Motor Skills (Milestones) Delayed
Dysarthria
Intelligence normal
Limb ataxia/clumsiness child
Progressive neurological disorder/signs
Slurred speech
Slurred speech/Chronic
Spasms in Both Legs
Staggering Gait
Difficulty Talking
Balance problems
Walking difficulties
Weakness
Disease Progression
Course/Chronic disorder
Course/Chronic only
Course/Progressive
Course/Twenty years survival/from onset
Onset/Childhood
Demographics & Risk Factors
Population Group
Child
Infant
Population/Pediatrics population
Young Adult
Sex & Age Groups
Population/Child
Population/Children/all
Population/Infant
Population/Young adult
Diagnostic Test Results
Pathology
BX/Muscle Biopsy Abnormal
BX/Muscle biopsy/denervation effect
Electrodiagnosis
EMG/Abnormal findings
Associated Diseases & Rule outs
Rule Outs
Amyotrophic lateral sclerosis
Associated Disease & Complications
Motor neuron disease
Parkinsonism, secondary
Primary lateral sclerosis
Akinetic Rigid Syndrome
Ataxia Disorder
Disease Mechanism & Classification
Class
CLASS/Primary organ/system disorder (ex)
CLASS/Motor neuron/unit disorder (ex)
CLASS/Neurologic (category)
Pathophysiology
Pathophysiology/CTSA (Cathepsin A) Gene Mutation
Pathophysiology/Maternal Chromosome mutation
Pathophysiology/ALS2 Gene mutation
Pathophysiology/Aslin Protein Gene mutation
Process
PROCESS/Autosomal recessive disorder (ex)
PROCESS/Genetic disorder/Spontaneous mutations/sporadic
PROCESS/Hereditofamilial (category)
PROCESS/INCIDENCE/Rare disease (ex)
PROCESS/Reference organ/system (category)
PROCESS/Use/Age/atrophic disorder (category)
PROCESS/Developmental degenerative neurological disorder (ex)
Synonyms
Synonym
Lateral Scleroses, Lateral Scleroses Primary, Lateral Sclerosis, Lateral Sclerosis Primary, Primary Lateral Scleroses, Primary lateral sclerosis, Primary lateral sclerosis (disorder), Scleroses Lateral, Scleroses Primary Lateral, Sclerosis Lateral, SCLEROSIS PRIMARY LATERAL, Synonym/Juvenile Primary Lateral Sclerosis, Synonym/PLSJ (JPLS)
Definition

Primary lateral sclerosis (PLS) is a rare neuromuscular disease characterized by progressive muscle weakness in the voluntary muscles. PLS belongs to a group of disorders known as motor neuron diseases. Motor neuron diseases develop when the nerve cells that control voluntary muscle movement degenerate and die, causing weakness in the muscles they control. Onset of PLS usually occurs after age 50. Symptoms may include difficulty with balance, weakness and stiffness in the legs, and clumsiness. Other symptoms may include spasticity (sudden, involuntary muscle spasms) in the hands, feet, or legs; foot dragging, and speech problems due to involvement of the facial muscles. The disorder usually begins in the legs, but it may also start in the tongue or the hands. The disease-which scientists believe is not hereditary-progresses gradually over a number of years, or even decades. In PLS, there is no evidence of the degeneration of spinal motor neurons or muscle wasting (amyotrophy) that occurs in amyotrophic lateral sclerosis or ALS (Lou Gehrig's disease), which it resembles. Diagnosis of PLS is often delayed because it is mistaken for ALS.

Is there any treatment?

Treatment for individuals with PLS is symptomatic. Baclofen and tizanidine may reduce spasticity. Quinine or phenytoin may decrease cramps. Physical therapy often helps prevent joint immobility. Speech therapy may be useful for those with involvement of the facial muscles.

What is the prognosis?

PLS is not fatal. There is no cure, and the progression of symptoms varies. Some people may retain the ability to walk without assistance, but others eventually require wheelchairs, canes, or other assistive devices.

Wikipedia.org 2008

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External Links Related to Primary lateral sclerosis
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Medscape (eMedicine)
Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)
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