lifting of soft palate, but typically remains relatively mild and seldom leads to the loss of oral communication. Signs of dysphagia include difficulty controlling solids, liquids or saliva in the mouth, coughing and choking. As the disease progresses, there is increased risk of aspiration pneumonia, which is the leading cause of death in SBMA patients. Up to half of all patients experience laryngospasm, an uncontrolled contraction (spasm) of the vocal folds, with a sense of choking, and feel that the air cannot enter or exit the airways for long seconds. Laryngospasms are often followed by a high-pitched breathing sound (stridor) due to rapid and vigorous contraction of the laryngeal sphincters. This is a frightening and hugely distressing experience, but rarely escalates to prolonged or serious episodes. In SBMA, deep tendon reflexes are diminished or absent. Sensory involvement results in degeneration of dorsal root ganglion cells with reduced vibratory sensation (distally in the legs), neuropathic pain, and numbness. There are sporadic reports of certain psychological traits (lack of self-confidence, emotional flattening, and poor concentration), but detailed neuropsychological examination of 64 SBMA patients did not detect any abnormalities. Androgen insensitivity Loss of AR function in SBMA patients results in partial androgen insensitivity, including gynecomastia, erectile dysfunction, decreased libido, infertility and testicular atrophy. Patients with complete androgen insensitivity syndrome do not show the neuromuscular symptoms of SBMA, indicating these symptoms are not caused by a loss of AR function. Gynecomastia, excessive enlargement of male breasts, is observed in about three quarters of SBMA patients. It typically becomes apparent after puberty, and is often the first evidence of this disease. Erectile dysfunction, decreased libido, infertility and testicular atrophy are common. One of the most disease-specific endocrine indices of SBMA is the androgen sensitivity index (luteinizing hormone / testosterone) which is elevated in 64% of cases, indicating both endocrine and exocrine testicular dysfunction. Metabolic disturbances Metabolic disturbances have also been reported in SBMA patients, with increased risk of insulin resistance, fatty liver disease, hyperlipidemia, and electrocardiogram (ECG) abnormalities. Impaired glucose homeostasis is a common feature of SBMA, and recent study found a significant correlation between insulin resistance and motor dysfunction in SBMA. In a group of 22 patients with SBMA, evidence of fatty liver disease was detected in all individuals by magnetic resonance spectroscopy. In a second group, liver dome magnetic resonance spectroscopy measurements were increased in participants with SBMA relative to age- and sex-matched