Biomaterial Database

Stiff-man syndrome. Report of a case. 1994

D Tarsy, and E K Miyawaki

Division of Neurology, New England Deaconess Hospital, Boston, Mass.

Stiff-man syndrome is a well-described, but rare and often overlooked, neuromuscular syndrome of rigidity, spasm, and gait abnormality that is associated with several endocrinologic and autoimmune disorders. A patient exhibiting many typical features of stiff-man syndrome had intermittent symptoms for 22 years before the correct diagnosis was made. Similar to many described patients, she was diabetic, hyperthyroid, and had elevated islet cell, antithyroid, and glutamic acid decarboxylase antibody levels. The high frequency of diabetes mellitus among patients with stiff-man syndrome is emphasized, as is increasing evidence to suggest that elaboration of anti-glutamic acid decarboxylase and anti-islet cell antibodies may play a role in the pathophysiologic state of the disorder.

UI	MeSH Term	Description	Entries
D006980	Hyperthyroidism	Hypersecretion of THYROID HORMONES from the THYROID GLAND. Elevated levels of thyroid hormones increase BASAL METABOLIC RATE.	Hyperthyroid,Primary Hyperthyroidism,Hyperthyroidism, Primary,Hyperthyroids
D008875	Middle Aged	An adult aged 45 - 64 years.	Middle Age
D003922	Diabetes Mellitus, Type 1	A subtype of DIABETES MELLITUS that is characterized by INSULIN deficiency. It is manifested by the sudden onset of severe HYPERGLYCEMIA, rapid progression to DIABETIC KETOACIDOSIS, and DEATH unless treated with insulin. The disease may occur at any age, but is most common in childhood or adolescence.	Diabetes Mellitus, Brittle,Diabetes Mellitus, Insulin-Dependent,Diabetes Mellitus, Juvenile-Onset,Diabetes Mellitus, Ketosis-Prone,Diabetes Mellitus, Sudden-Onset,Diabetes, Autoimmune,IDDM,Autoimmune Diabetes,Diabetes Mellitus, Insulin-Dependent, 1,Diabetes Mellitus, Type I,Insulin-Dependent Diabetes Mellitus 1,Juvenile-Onset Diabetes,Type 1 Diabetes,Type 1 Diabetes Mellitus,Brittle Diabetes Mellitus,Diabetes Mellitus, Insulin Dependent,Diabetes Mellitus, Juvenile Onset,Diabetes Mellitus, Ketosis Prone,Diabetes Mellitus, Sudden Onset,Diabetes, Juvenile-Onset,Diabetes, Type 1,Insulin Dependent Diabetes Mellitus 1,Insulin-Dependent Diabetes Mellitus,Juvenile Onset Diabetes,Juvenile-Onset Diabetes Mellitus,Ketosis-Prone Diabetes Mellitus,Sudden-Onset Diabetes Mellitus
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D001323	Autoantibodies	Antibodies that react with self-antigens (AUTOANTIGENS) of the organism that produced them.	Autoantibody
D016750	Stiff-Person Syndrome	A condition characterized by persistent spasms (SPASM) involving multiple muscles, primarily in the lower limbs and trunk. The illness tends to occur in the fourth to sixth decade of life, presenting with intermittent spasms that become continuous. Minor sensory stimuli, such as noise and light touch, precipitate severe spasms. Spasms do not occur during sleep and only rarely involve cranial muscles. Respiration may become impaired in advanced cases. (Adams et al., Principles of Neurology, 6th ed, p1492; Neurology 1998 Jul;51(1):85-93)	Moersch-Woltmann Syndrome,Stiff-Man Syndrome,Congenital Stiff-Man Syndrome,Congenital Stiff-Person Syndrome,Familial Hyperekplexia,Hereditary Hyperekplexia,Startle Syndrome,Stiff-Baby Syndrome,Stiff-Trunk Syndrome,Stiffman Syndrome,Congenital Stiff Man Syndrome,Congenital Stiff-Man Syndromes,Congenital Stiff-Person Syndromes,Familial Hyperekplexias,Hereditary Hyperekplexias,Hyperekplexia, Familial,Hyperekplexia, Hereditary,Hyperekplexias, Familial,Hyperekplexias, Hereditary,Moersch Woltmann Syndrome,Startle Syndromes,Stiff Man Syndrome,Stiff Person Syndrome,Stiff Trunk Syndrome,Stiff-Baby Syndromes,Stiff-Man Syndrome, Congenital,Stiff-Man Syndromes, Congenital,Stiff-Person Syndrome, Congenital,Stiff-Person Syndromes, Congenital,Stiff-Trunk Syndromes,Syndrome, Congenital Stiff-Man,Syndrome, Congenital Stiff-Person,Syndrome, Moersch-Woltmann,Syndrome, Startle,Syndrome, Stiff-Baby,Syndrome, Stiff-Man,Syndrome, Stiff-Person,Syndrome, Stiff-Trunk,Syndrome, Stiffman,Syndromes, Congenital Stiff-Man,Syndromes, Congenital Stiff-Person,Syndromes, Startle,Syndromes, Stiff-Baby,Syndromes, Stiff-Trunk