The spelling of "tabes hereditaria" can be a bit confusing to those unfamiliar with Latin. The word is pronounced /ˈteɪ.biːz hɛrɪˈdɪtɛrɪə/ and translates to "hereditary wasting disease." The first word, "tabes," is pronounced with a long "a" sound and a soft "s" at the end. The second word, "hereditaria," is pronounced with the emphasis on the second syllable and a rolled "r" sound at the end. This term is used in medical contexts to describe a condition where there is a genetic predisposition to muscle wasting and weakness.
Tabes hereditaria, also known as hereditary tabes dorsalis, is a rare, inherited neurodegenerative disease characterized by the progressive deterioration of the sensory nerves of the spinal cord. It is considered a form of locomotor ataxia that primarily affects individuals belonging to families with a history of the condition. The term "tabes hereditaria" is derived from the Latin word "tabes," meaning wasting away, and "hereditaria," meaning hereditary or inherited.
The onset of tabes hereditaria is typically observed during adulthood, with symptoms gradually worsening over time. Patients may experience various manifestations including unsteady gait, loss of coordination, difficulty in maintaining balance, and a characteristic swaying movement of the trunk. Sensory disturbances commonly occur, such as reduced sensation to touch, temperature, and pain, with affected individuals often reporting a sensation of "walking on cushions." Moreover, the disease may cause muscle weakness, typically affecting the lower extremities initially and eventually progressing to involve the upper body.
Tabes hereditaria is believed to arise from a mutation in the gene responsible for the production of a specific protein involved in the function and maintenance of nerve cells. This genetic alteration leads to the slow deterioration of nerve fibers, particularly those responsible for transmitting sensory information. The condition is often diagnosed based on clinical presentation, family history, and additional diagnostic tests, such as electromyography and nerve conduction studies.
Although there is currently no cure for tabes hereditaria, treatment mainly consists of managing symptoms and providing supportive care. Patients may benefit from physical therapy to improve gait and balance, pain management strategies, and assistive devices to aid mobility. Genetic counseling is also recommended for affected individuals and their families to assess the risk of inheritance in future generations.
Friedreich's, or hereditary, ataxia.
A practical medical dictionary. By Stedman, Thomas Lathrop. Published 1920.
The term tabes hereditaria has Latin roots. Tabes means wasting or decay, while hereditaria is derived from hereditarius, meaning related to inheritance or hereditary. Therefore, tabes hereditaria can be interpreted as hereditary wasting or hereditary decay.