Benign Hereditary Choreas, also referred to as benign hereditary chorea or BHC, is a rare genetic disorder characterized by involuntary, purposeless movements, known as chorea. The term "benign" is used to indicate that the condition does not typically cause life-threatening complications or progression to other neurological disabilities.
The condition is hereditary, meaning it is passed down from one generation to the next through genes. It is usually autosomal dominant, meaning that an affected individual only needs to inherit one copy of the mutated gene from one parent to develop the disorder. However, instances of autosomal recessive inheritance have also been reported.
Symptoms of benign hereditary choreas typically manifest during childhood or adolescence. The chorea movements primarily affect the face, limbs, and trunk, often involving jerky, unpredictable, and uncontrollable motions, which may increase during times of stress or excitement. Additional symptoms may include muscle weakness, speech difficulties, and emotional or behavioral problems.
Although the condition is generally considered benign, it can cause significant impairment in daily functioning and quality of life for affected individuals. Treatment options aim to manage symptoms and may include medications that help to reduce involuntary movements or address other associated symptoms.
Due to the rarity of Benign Hereditary Choreas, it is crucial for affected individuals and their families to seek medical advice and support from healthcare professionals experienced in the management of genetic movement disorders. Genetic counseling is often recommended to help individuals understand the inheritance pattern and potential risks associated with the disorder.
