Biomaterial Database

Stiff-leg syndrome: a focal form of stiff-man syndrome. 1998

A Saiz, and F Graus, and F Valldeoriola, and J Valls-Solé, and E Tolosa

Service of Neurology, Department of Medicine, Hospital Clínic i Provincial, University of Barcelona, Spain.

We report on 2 patients who presented stiffness and spasms similar to those of stiff-man syndrome (SMS) that were limited to one leg for up to 11 years. Patients had serum glutamic acid decarboxylase (GAD) autoantibodies in high titer, clinical evidence of organ-specific autoimmunity, and electromyographic pattern of continuous motor unit activity with abnormally enhanced exteroceptive reflexes. The clinical and immunological profile suggests that this disorder may be a focal form of SMS.

UI	MeSH Term	Description	Entries
D007866	Leg	The inferior part of the lower extremity between the KNEE and the ANKLE.	Legs
D008875	Middle Aged	An adult aged 45 - 64 years.	Middle Age
D012018	Reflex	An involuntary movement or exercise of function in a part, excited in response to a stimulus applied to the periphery and transmitted to the brain or spinal cord.
D004576	Electromyography	Recording of the changes in electric potential of muscle by means of surface or needle electrodes.	Electromyogram,Surface Electromyography,Electromyograms,Electromyographies,Electromyographies, Surface,Electromyography, Surface,Surface Electromyographies
D005260	Female		Females
D005968	Glutamate Decarboxylase	A pyridoxal-phosphate protein that catalyzes the alpha-decarboxylation of L-glutamic acid to form gamma-aminobutyric acid and carbon dioxide. The enzyme is found in bacteria and in invertebrate and vertebrate nervous systems. It is the rate-limiting enzyme in determining GAMMA-AMINOBUTYRIC ACID levels in normal nervous tissues. The brain enzyme also acts on L-cysteate, L-cysteine sulfinate, and L-aspartate. EC 4.1.1.15.	Glutamate Carboxy-Lyase,Glutamic Acid Decarboxylase,Acid Decarboxylase, Glutamic,Carboxy-Lyase, Glutamate,Decarboxylase, Glutamate,Decarboxylase, Glutamic Acid,Glutamate Carboxy Lyase
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
D018482	Muscle, Skeletal	A subtype of striated muscle, attached by TENDONS to the SKELETON. Skeletal muscles are innervated and their movement can be consciously controlled. They are also called voluntary muscles.	Anterior Tibial Muscle,Gastrocnemius Muscle,Muscle, Voluntary,Plantaris Muscle,Skeletal Muscle,Soleus Muscle,Muscle, Anterior Tibial,Muscle, Gastrocnemius,Muscle, Plantaris,Muscle, Soleus,Muscles, Skeletal,Muscles, Voluntary,Skeletal Muscles,Tibial Muscle, Anterior,Voluntary Muscle,Voluntary Muscles