Benign hereditary chorea (BHC), also known as benign familial chorea, is a rare autosomal dominant neurogenetic syndrome. It typically presents itself in childhood with isolated chorea, with average to below average intelligence. Unlike other neurogenetic causes of chorea such as Huntingtons disease, BHC is not progressive, and not associated with cognitive decline or psychiatric problems in the vast majority of cases.The first description of BHC was reported in 1967 in an African American family from Mississippi. Two brothers reportedly had delayed motor development in childhood and were diagnosed with chorea. These findings were reaffirmed by other families reporting similar traits and an autosomal dominant pattern of inheritance was suggested. However, heterogeneity in the presentations of the affected individuals made confirmation of these diagnoses of BHC difficult to prove. Features reported in these families, including dystonia, tremor, and myoclonus, led researchers to question whether BHC actually represents different diagnoses with similar phenotypes inappropriately grouped together.Further research in 2000 confirmed a connection between a Dutch family reporting similar characteristics of BHC and one of the original families. The investigators identified a linkage to a disease locus on the long arm of chromosome 14 from this connection. Signs and Symptoms
Benign Hereditary Chorea is characterized by early onset of an abnormal gait, speech articulation difficulties, anxiety, and chorea.The clinical spectrum of symptoms resulting from BHC is vast, manifesting as thyroid agenesis to dysarthria to distress syndrome.
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.