* Immunosuppressive Treatment for Retinal Degeneration in Juvenile Neuronal Ceroid Lipofuscinosis (Juvenile Batten Disease).
- Juvenile Neuronal Ceroid Lipofuscinosis (JNCL) presents with progressive vision loss at 4-7 years of age. Blindness results within 2 years, followed by inexorable neurologic decline and death. There is no treatment or cure. Neuroinflammation is postulated to play a role in the neurodegeneration. The JNCL mouse model demonstrated decreased neuroinflammation and improved motor skills with immunosuppression. Based on this work, a short-term human clinical trial of mycophenolate mofetil has begun, however longer term effects, and whether immunosuppression modulates vision loss, have not been studied. We report a JNCL patient treated with immunosuppressive therapy in whom visual function was comprehensively characterized over 2 years.
Overview of verb demonstrate
The verb demonstrate has 4 senses (first 4 from tagged texts)
1. (24) show, demo, exhibit, present, demonstrate -- (give an exhibition of to an interested
audience; "She shows her dogs frequently"; "We will demo the new software in Washington")
2. (22) prove, demonstrate, establish, show, shew -- (establish the validity of something, as by an
example, explanation or experiment; "The experiment demonstrated the instability of the compound";
"The mathematician showed the validity of the conjecture")
3. (2) attest, certify, manifest, demonstrate, evidence -- (provide evidence for; stand as proof of;
show by one's behavior, attitude, or external attributes; "His high fever attested to his illness";
"The buildings in Rome manifest a high level of architectural sophistication"; "This decision
demonstrates his sense of fairness")
4. (1) demonstrate, march -- (march in protest; take part in a demonstration; "Thousands
demonstrated against globalization during the meeting of the most powerful economic nations in
Seattle")
Overview of adj demonstrated
The adj demonstrated has 1 sense (first 1 from tagged texts)
1. (1) demonstrated -- (having been demonstrated or verified beyond doubt)
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