In the past, it was used at even higher doses of 10 to 40 every 1 to 4 weeks for estrogen replacement. Estradiol valerate is usually used in the treatment of advanced prostate cancer in men at a dosage of 30 mg or more every 1 to 2 weeks by intramuscular injection. In transgender women, estradiol valerate given by intramuscular injection is usually used at a dosage of 5 to 20 mg, but up to 30 to 40 mg, once every 2 weeks. Estradiol valerate has also been used at a dose of 10 to 40 mg by intramuscular injection to limit bleeding in women with hemorrhage due to dysfunctional uterine bleeding. : 318 : 60

Available forms
Estradiol valerate is and has been available in the form of vials and ampoules of oil solution for intramuscular injection in concentrations of 4, 5, 10, 20, and 40 mg/mL and in the form of oral tablets at doses of 0.5, 1, 2, and 4 mg per tablet. In the United States, it is specifically available in formulations of 10, 20, and 40 mg/mL in oil solution (as Delestrogen, as well as generics). Aside from estradiol valerate, the only other injectable estrogen formulations that remain available in the United States are estradiol cypionate (5 mg/mL in oil solution) and conjugated estrogens (25 mg/vial in solution).
Spinal muscular atrophy with progressive myoclonic epilepsy (SMA-PME), sometimes called Jankovic–Rivera syndrome, is a very rare neurodegenerative disease whose symptoms include slowly progressive muscle (atrophy), predominantly affecting proximal muscles, combined with denervation and myoclonic seizures. Only 12 known human families are described in scientific literature to have SMA-PME.SMA-PME is associated with a missense mutation (c.125C→T) or deletion in exon 2 of the ASAH1 gene and is inherited in an autosomal recessive manner. SMA-PME is closely related to a lysosomal disorder disease called Farber lipogranulomatosis. As with many genetic disorders, there is no known cure for SMA-PME. The condition was first described in 1979 by American researchers Joseph Jankovic and Victor M. Rivera. ASAH1 gene
The ASAH1 gene codes for acid ceramidase, an enzyme found in lysosomes. The lysosome breaks down acid ceramidase; the fatty acid component  is then used to produce myelin. Myelin is an insulating coating around the neurons in the body which helps to contain bioelectrical signals along a nerve cells axon and increase transmission rate. In patients with SMA-PME, the ceramidase function decreases to 33.33% effective. The lack of myelin resulting from the lack of acid ceramidase breakdown leads to nerve cell dysfunction. See also
Spinal muscular atrophies
Progressive myoclonus epilepsy

References
Further reading
National Institutes of Health (December 2013). "Genetics Home Reference: Spinal muscular atrophy with progressive myoclonic epilepsy". == External links ==