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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Juvenile primary lateral sclerosis


Other Names for this Disease

  • JPLS
  • PLS juvenile
  • Primary lateral sclerosis, juvenile
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Symptoms

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What are the signs and symptoms of juvenile primary lateral sclerosis?

Juvenile primary lateral sclerosis is a rare disorder characterized by progressive weakness and stiffness of muscles in the arms, legs, and face. Symptoms of juvenile primary lateral sclerosis begin in early childhood and progress over a period of 15 to 20 years. Early symptoms include clumsiness, muscle spasms, weakness and stiffness in the legs, and difficulty with balance. As symptoms progress, they include weakness and stiffness in the arms and hands, slurred speech, drooling, difficulty swallowing, and an inability to walk.[1]
Last updated: 3/4/2011

The Human Phenotype Ontology provides the following list of signs and symptoms for Juvenile primary lateral sclerosis. If the information is available, the table below includes how often the symptom is seen in people with this condition. You can use the MedlinePlus Medical Dictionary to look up the definitions for these medical terms.

Signs and Symptoms Approximate number of patients (when available)
Gait disturbance 90%
Hemiplegia/hemiparesis 90%
Hyperreflexia 90%
Hypertonia 90%
Incoordination 90%
Muscle weakness 90%
Pseudobulbar signs 90%
Feeding difficulties in infancy 50%
Neurological speech impairment 50%
Abnormality of the urinary system 7.5%
Amyotrophy 7.5%
Autosomal recessive inheritance -
Babinski sign -
Cerebral cortical atrophy -
Childhood onset -
Difficulty in tongue movements -
Dysphagia -
Hyperreflexia -
Juvenile onset -
Pseudobulbar behavioral symptoms -
Saccadic smooth pursuit -
Slow progression -
Spastic dysarthria -
Spastic gait -
Spastic tetraparesis -
Spasticity of facial muscles -
Spasticity of pharyngeal muscles -
Upper motor neuron abnormality -

Last updated: 11/3/2014

The Human Phenotype Ontology (HPO) has collected information on how often a sign or symptom occurs in a condition. Much of this information comes from Orphanet, a European rare disease database. The frequency of a sign or symptom is usually listed as a rough estimate of the percentage of patients who have that feature.

The frequency may also be listed as a fraction. The first number of the fraction is how many people had the symptom, and the second number is the total number of people who were examined in one study. For example, a frequency of 25/25 means that in a study of 25 people all patients were found to have that symptom. Because these frequencies are based on a specific study, the fractions may be different if another group of patients are examined.

Sometimes, no information on frequency is available. In these cases, the sign or symptom may be rare or common.


References
  1. Juvenile primary lateral sclerosis. Genetics Home Reference (GHR). 2007; http://ghr.nlm.nih.gov/condition/juvenile-primary-lateral-sclerosis. Accessed 3/4/2011.


Other Names for this Disease
  • JPLS
  • PLS juvenile
  • Primary lateral sclerosis, juvenile
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.