Familial encephalopathy with neuroserpin inclusion bodies (FENIB) is a progressive disorder of the nervous system that is characterized by a loss of intellectual functioning (dementia) and seizures. At first, affected individuals may have difficulty sustaining attention and concentrating. Their judgment, insight, and memory become impaired as the condition progresses. Over time, they lose the ability to perform the activities of daily living, and most people with this condition eventually require comprehensive care.The signs and symptoms of familial encephalopathy with neuroserpin inclusion bodies vary in their severity and age of onset. In severe cases, the condition causes seizures and episodes of sudden, involuntary muscle jerking or twitching (myoclonus) in addition to dementia. These signs can appear as early as a persons teens. Less severe cases are characterized by a progressive decline in intellectual functioning beginning in a persons forties or fifties.Mutations in the SERPINI1 gene cause familial encephalopathy with neuroserpin inclusion bodies. The SERPINI1 gene provides instructions for making a protein called neuroserpin. This protein is found in nerve cells, where it plays a role in the development and function of the nervous system. Neuroserpin helps control the growth of nerve cells and their connections with one another, which suggests that this protein may be important for learning and memory. Mutations in the gene result in the production of an abnormally shaped, unstable version of neuroserpin. Abnormal neuroserpin proteins can attach to one another and form neuroserpin inclusion bodies or Collins bodies within nerve cells.
Lactation suppression refers to the act of suppressing lactation by medication or other non pharmaceutical means. The breasts may become painful when engorged with milk if breastfeeding is ceased abruptly, or if never started. This may occur if a woman never initiates breastfeeding, or if she is weaning from breastfeeding abruptly. Historically women who did not plan to breastfeed were given diethylstilbestrol and other medications after birth to suppress lactation. However, its use was discontinued, and there are no medications currently approved for lactation suppression in the US and the UK. Dopamine agonists are routinely prescribed to women following a stillbirth in the UK under the NHS. Reasons
After birth, some women may desire to stop the production of breast milk, for example when the mother decides to bottle feed from birth, or in the case when the infant dies or is surrendered at birth. Additionally, women who are breastfeeding may need to stop breastfeeding abruptly, for instance if she is taking medication contraindicated for breastfeeding or undergoes surgery. The abrupt weaning process may lead to severe engorgement, extremely painful breasts, and possibly mastitis. Up to one third of women who do not breast-feed and who use a brassiere or binder, ice packs, or analgesics may experience severe breast pain. Specific studies of nonpharmacologic methods of lactation suppression were limited and inconclusive. Available data suggest that many women using currently recommended strategies for treatment of symptoms may nevertheless experience engorgement or pain for most of the first postpartum week.