Stiff-Person Syndrome (Stiff Man 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)
Also Known As:
Stiff Man Syndrome; Syndrome, Stiff-Man; Stiffman Syndrome; Moersch Woltmann Syndrome; Stiff Person Syndrome; Syndrome, Moersch-Woltmann; Syndrome, Stiff-Person; Syndrome, Stiffman; Moersch-Woltmann Syndrome; Stiff-Man Syndrome
Networked: 378 relevant articles (21 outcomes, 24 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Dermatomyositis (Dermatopolymyositis)
2. Myasthenia Gravis
3. Lambert-Eaton Myasthenic Syndrome (Lambert-Eaton Syndrome)
4. Polyneuropathies (Polyneuropathy)
5. Multiple Sclerosis


1. Dalakas, Marinos C: 19 articles (02/2015 - 05/2004)
2. Hampe, Christiane S: 10 articles (01/2015 - 12/2005)
3. Graus, Francesc: 7 articles (08/2015 - 05/2003)
4. Geis, Christian: 7 articles (03/2015 - 11/2010)
5. Vincent, Angela: 7 articles (08/2014 - 06/2003)
6. Graus, F: 7 articles (06/2012 - 07/2000)
7. Saiz, A: 7 articles (06/2012 - 07/2000)
8. Toyka, Klaus V: 6 articles (03/2015 - 04/2005)
9. Weishaupt, Andreas: 6 articles (03/2015 - 04/2005)
10. Sommer, Claudia: 6 articles (03/2015 - 04/2005)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Stiff-Person Syndrome:
1. Intravenous Immunoglobulins (IVIG)FDA Link
2. AntibodiesIBA
3. BaclofenFDA LinkGeneric
4. Immunoglobulins (Immunoglobulin)IBA
5. AutoantibodiesIBA
6. Glutamate Decarboxylase (Decarboxylase, Glutamate)IBA
7. Diazepam (Valium)FDA LinkGeneric
8. Immunoglobulin M (IgM)IBA
05/01/1997 - "In other controlled or open-label trials and case reports, IVIg produced improvement in several patients with the Lambert-Eaton myasthenic syndrome and myasthenia gravis but had a variable, mild, or unsubstantiated benefit in some patients with inclusion-body myositis, paraproteinemic IgM demyelinating polyneuropathy, certain intractable childhood epilepsies, polymyositis, multiple sclerosis, optic neuritis, and the stiff-man syndrome. "
05/01/1995 - "The neurological diseases with definite or putative immune pathogenesis include myasthenia gravis; Lambert-Eaton myasthenic syndrome; IgM monoclonal anti-myelin-associated glycoprotein-associated demyelinating polyneuropathy; Guillain-Barré syndrome; chronic inflammatory demyelinating polyneuropathy; multifocal motor neuropathy with or without GM1 antibodies; multiple sclerosis; inflammatory myopathies; stiff-man syndrome; autoimmune neuromyotonia; paraneoplastic neuronopathies and cerebellar degeneration; and neurological diseases associated with systemic autoimmune conditions, vasculitis, or viral infections. "
04/01/2007 - "Recommendations for use of IVIG were made for 14 conditions, including acute disseminated encephalomyelitis, chronic inflammatory demyelinating polyneuropathy, dermatomyositis, diabetic neuropathy, Guillain-Barré syndrome, Lambert-Eaton myasthenic syndrome, multifocal motor neuropathy, multiple sclerosis, myasthenia gravis, opsoclonus-myoclonus, pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections, polymyositis, Rasmussen's encephalitis, and stiff person syndrome; IVIG was not recommended for 8 conditions including adrenoleukodystrophy, amyotropic lateral sclerosis, autism, critical illness polyneuropathy, inclusion body, myositis, intractable childhood epilepsy, paraproteinemic neuropathy (IgM variant), and POEMS syndrome. "
11/01/1994 - "These are; ple21 in limbic encephalitis; PCD17, CDR62, CDR34, and CZF in paraneoplastic cerebellar degeneration; one of the anion transporters band 3 in chorea-acanthocytosis; visinin-like substance in cancer-associated retinopathy (CAR syndrome); myelin basic protein (MBP) and proteolipid protein (PLP) in acute disseminated encephalomyelitis; MBP, PLP and myelin-oligodendrocyte glycoprotein (MOG) in multiple sclerosis; glutamic acid decarboxylase in stiff-man syndrome; GM1 ganglioside in amyotrophic lateral sclerosis; peripheral nerve K+ channel in Isaacs syndrome; synaptotagmin in Lambert-Eaton syndrome; acetylcholine receptor in myasthenia gravis; GM1 ganglioside in Guillain-Barré syndrome; GQ1b ganglioside in Fisher syndrome; myelin-associated glycoprotein in IgM paraproteinemic neuropathy; HuD in paraneoplastic sensory neuropathy; and tRNA and HSP65 in polymyositis."
9. etiracetam (levetiracetam)FDA LinkGeneric
10. Antineutrophil Cytoplasmic Antibodies (ANCA)IBA

Therapies and Procedures

1. Plasmapheresis
2. Plasma Exchange
3. Drug Therapy (Chemotherapy)
4. Immunotherapy
5. Aftercare (After-Treatment)