Copyright
©The Author(s) 2019.
World J Clin Cases. Oct 6, 2019; 7(19): 2942-2952
Published online Oct 6, 2019. doi: 10.12998/wjcc.v7.i19.2942
Published online Oct 6, 2019. doi: 10.12998/wjcc.v7.i19.2942
Table 1 Clinical features of patients with stiff-person syndrome
Characteristic | Total number | Classical SPS | SLS |
Number of patients (n) | 14 | 12 | 2 |
Male (%) | 9 (64.3) | 7 (58.3) | 2 (100) |
Age of onset (yr) | 39.3 (28.0-54.0) | 35 (26.0-56.0) | 46.7 |
Follow-up duration (yr) | 6 (2.7-13.3) | 9 (2.1-14.9) | 4.3 |
Clinical feature | |||
Low back stiffness and spasm (n) | 12 | 12 | 0 |
Lower limb stiffness and spasm (n) | 14 | 12 | 2 |
Lumbar lordosis (n) | 8 | 8 | 0 |
Neck stiffness and spasm (n) | 9 | 9 | 0 |
Upper limbs stiffness and spasm (n) | 10 | 10 | 0 |
Abdominal wall stiffness and spasm (n) | 11 | 11 | 0 |
Falls (n) | 10 | 8 | 2 |
Exacerbating factors | |||
Cold, noises, and emotional stress (n) | 14 | 12 | 2 |
Excessive startle (n) | 9 | 8 | 1 |
Neurological/psychiatric feature | |||
Seizure/epilepsy (n) | 2 | 2 | 0 |
Gait disturbances (n) | 12 | 10 | 2 |
Parkinsonism (n) | 2 | 2 | 0 |
Alzheimer’s disease (n) | 2 | 2 | 0 |
Depression (n) | 5 | 4 | 1 |
Anxiety (n) | 8 | 7 | 1 |
Panic disorder (n) | 2 | 2 | 0 |
Table 2 Autoimmune diseases and autoantibodies associated with stiff-person syndrome
Variable | Classical SPS | SLS |
Number of patients (n) | 12 | 2 |
Associated disease | ||
≥2 coexisting autoimmune diseases (n) | 1 | 0 |
Type 1 diabetes mellitus (n) | 2 | 0 |
Latent autoimmune diabetes in adults (n) | 1 | 0 |
Autoimmune thyroid disease (n) | 2 | 0 |
Sjögren syndrome (n) | 1 | 0 |
Myasthenia graves (n) | 1 | 0 |
Malignancy1 (n) | 1 | 0 |
Thymoma (n) | 1 | 0 |
Other disease (n) | 12 | nil |
Associated autoantibody (n/N)3 | ||
GAD antibody | 3/7 | 0/2 |
IA-2 antibody | 0/2 | nil |
Anti-TPO | 2/5 | nil |
ATA | 1/1 | nil |
Ach-R antibody | 1/1 | nil |
ANA | 1/1 | nil |
Anti-Ro antibody | 1/1 | nil |
Table 3 Correlation of associated diseases and glutamic acid decarboxylase autoantibodies among stiff-person syndrome patients
Subgroup | Seropositive | Seronegative | Unknown | |||
Classical | SLS | Classical | SLS | Classical | SLS | |
Patient number | 3 | 0 | 4 | 2 | 5 | 0 |
Type 1 diabetes mellitus | 1 | 0 | 0 | 0 | 1 | 0 |
Latent autoimmune diabetes in adults | 1 | 0 | 0 | 0 | 0 | 0 |
Autoimmune thyroid disease | 2 | 0 | 0 | 0 | 0 | 0 |
Sjögren syndrome | 0 | 0 | 1 | 0 | 0 | 0 |
Myasthenia graves | 0 | 0 | 1 | 0 | 0 | 0 |
Malignancy1 | 0 | 0 | 1 | 0 | 0 | 0 |
Thymoma | 0 | 0 | 1 | 0 | 0 | 0 |
Type 2 diabetes mellitus | 0 | 0 | 0 | 1 | 0 | 0 |
Table 4 Summary of two stiff person syndrome cases associated with autoimmune diabetes
Case 1: T1DM | Case 2: LADA | |
Gender | Female | Male |
Age of diagnosis of DM | 43 | 30 |
Age of diagnosis of SPS | 32 | 33 |
Age of requirement for insulin | 43 | 42 |
HbA1c at diagnosis of DM (%) | 13.2 | 13.1 |
Basal C-peptide (ng/mL) | 0.31 | 0.26 |
Glucagon-stimulated C-peptide (ng/mL) | 0.6 | 0.41 |
Initial GAD Ab (U/mL) (Ref < 1 units)1 | 1496 (before T1DM) | 110 (before LADA) |
GAD-Ab titer before receiving plasmapheresis (U/mL) (Ref < 5 units)2 | > 2000 (after T1DM) | > 2000 (after LADA) |
GAD-Ab titer after receiving plasmapheresis (U/mL) (Ref < 5 units)2 | > 2000 | > 2000 |
IA2 antibody (U/mL) (Ref < 7.5) | 0.97 | 0.65 |
Anti-TPO (IU/mL) (Ref < 5.6) | < 1.0 | 18.81 |
Medications | Diazepam, clonazepam, baclofen, lamotrigine, azathioprine | Diazepam, clonazepam, baclofen, tizanidine |
IVIG | No | Yes (IVIG 0.4/kg/d) |
Plasmapheresis | Yes, 10 times | Yes, 15 times |
Table 5 Coexisting diseases and related autoantibodies in stiff-person syndrome patients
Autoimmune disease | Associated antibody | Neoplasm | Associated antibody |
Type 1 DM | Anti-GAD Anti-IA2 | Breast cancer | Gephyrin |
LADA | Lung cancer | Amphiphysin | |
Hashimoto’s thyroiditis | Anti-TPO, ATA, anti-TSHR | Thyroid cancer | ATA |
Graves’ disease | Colon cancer | ||
Pernicious anemia | Anti-parietal cells | Renal cell carcinoma | |
Celiac disease | Anti-transglutaminase antibodies (anti-tTG), anti-endomysial antibodies | Thymoma | Anti-AchR |
Myasthenia graves | Anti-AchR | Neuroendocrine tumor | |
Sjögren syndrome | ANA, anti-Ro | Hodgkin lymphoma | |
Systemic lupus erythematosus | ANA, ds-DNA | Multiple myeloma | |
Vitiligo | |||
Autoimmune polyglandular Syndrome type 1 or type 2 or type 3 |
- Citation: Lee YY, Chen IW, Chen ST, Wang CC. Association of stiff-person syndrome with autoimmune endocrine diseases. World J Clin Cases 2019; 7(19): 2942-2952
- URL: https://www.wjgnet.com/2307-8960/full/v7/i19/2942.htm
- DOI: https://dx.doi.org/10.12998/wjcc.v7.i19.2942