Disease Information for Stiff-man syndrome

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Clinical Manifestations
Signs & Symptoms
Akinetic rigid frozen Posture
Abdominal tenderness
Abdominal wall pain
Rigidity/Muscle/movement
Acute bilateral Hand muscle spasms
Bilateral Shoulder Muscle pain/Weakness
Carpopedal spasms
Cramping in Extremities
Fluctuating rigidity/axial/limb muscles
Muscle cramps/spasms
Muscle Pain
Muscle spasticity
Muscle stiffness/rigidity
Myalgias
Stiff muscles/spasms legs/feet
Stiffness
Symmetrical muscle involvement/stiffness
Triggered muscle spasms/sensory input
Twitchy/muscle spasms
Gait disturbance/abnormality
Noise Triggers Spasms
Passive stretch resistance increased
Spasms in Both Legs
Startle response/trigger
Stiff neck/Nuchal rigidity
Waddling Trendelenberg Gait
Bilateral shoulder pain
Neck and Arm Pain
Neck and upper back musculoskeletal pain
Neck/Shoulder pain/stiffness
Rigid back posture/spasm
Rigidity of back
Skeletal problems/symptoms/signs
Stiffness upper limb girdle
Falling
Otherwise well Isolated problem
Disease Progression
Course/Chronic disorder
Course/Chronic only
Course/Progressive
Course/Progressive/slowly chronic illness
Onset/insidious
Demographics & Risk Factors
Population Group
Child
Man
Population/Pediatrics population
Sex & Age Groups
Population/Adult Aged Only
Population/Adult/all
Population/Child
Population/Children/all
Population/Male
Population/Man patient
Laboratory Tests
Abnormal Lab Findings (Non Measured)
Leukopenia
Abnormal Lab Findings - Decreased
WBC
WBC/White Blood Cell Count/Leukocytes (Lab)
Abnormal Lab Findings - Increased
Anti-GAD antibodies
Eosinophiles (LAB)
Diagnostic Test Results
Pathology
BX/Muscle Biopsy Abnormal
BX/Muscle biopsy/Increased sarcolemma nuclei
BX/Muscle biopsy/Lymphorrhages/aggregates
BX/Muscle biopsy/muscle fiber degeneration
Electrodiagnosis
EMG/Abnormal findings
EMG/Continuous activity
Associated Diseases & Rule outs
Rule Outs
Tetanus
Associated Disease & Complications
Eosinophilia Hypereosinophilia
Kyphoscoliosis
Kyphosis
Pathological/stress fractures from spasms
Skeletal dysplasia
Skeletal/bone malformations
Stiff-man syndrome
Subluxations
Tetany
Akinetic Rigid Syndrome
Disease Mechanism & Classification
Class
CLASS/Primary organ/system disorder (ex)
CLASS/Muscle disorder (ex)
CLASS/Muscle/tendon/extremities (category)
CLASS/Shoulder girdle/upper extremity involvement/disorder (ex)
CLASS/Neurologic (category)
CLASS/Axial skeletal involvement/disorder (ex)
CLASS/Skeletal (category)
Pathophysiology
Pathophysiology/Skeletal defomities/acquired
Process
PROCESS/Congenital/developmental (category)
PROCESS/Idiopathic/unclassified/unknown (category)
PROCESS/INCIDENCE/Rare disease (ex)
PROCESS/Vegetative-Autonomic/Endocrine (category)
Synonyms
Synonym
Gamma neuron overactivity syndrome, Moersch Woltman syndrome, Moersch Woltmann Syndrome, MUSCULAR RIGIDITY AND SPASM PROGRESSIVE, Progressive encephalomyelitis with rigidity, SPS, stiff man syndrome, Stiff man syndrome (disorder), Stiff Person Syndrome, STIFF TRUNK SYNDROME, Stiffman Syndrome, Subacute myoclonic spinal neuronitis, Syndrome Moersch Woltmann, syndrome stiff man, Syndrome Stiff Person, Syndrome Stiffman, Synonym/Moersch-Woltmann syndrome, Synonym/Muscular rigidity-progressive spasm, Synonym/SMS, Synonym/Stiff Person Syndrome
Treatment
Drug Therapy - Indication
RX/Diazepam (Valium)
RX/Gabapentin (Neurontin)
RX/Gamma globulin (intravenous, high dose)
RX/Immunoglobulin infusion
RX/Tiagabine (Gabitril)
Definition

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 advance cases; (Adams et al, Principles of Neurology, 6th ed, p1492; Neurology 1998 Jul;51(1):85-93)--------------------------------------------------Stiff-man syndrome is characterized by the insidious onset of progressive stiffness in the trunk and abdomen and, to a lesser degree, in the legs and arms; Patients are otherwise normal, and examination detects only muscle hypertrophy and stiffness; Electromyography shows only the electrical activity of normal contraction; The syndrome may be autoimmune; autoantibodies to glutamic acid decarboxylase have been found; Only symptomatic therapy is available; Diazepam is the only drug that consistently relieves muscle stiffness; Results of plasmapheresis are conflicting--------[Merck Manual 17th]--------------------

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External Links Related to Stiff-man syndrome
Google
Wikipedia
Merck
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PubMed (National Library of Medicine)
NGC (National Guideline Clearinghouse)
Medscape (eMedicine)
Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)
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