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. 2016 Mar 4;6:340. doi: 10.7916/D85M65GD

Table 1. Subjective and Objective Findings in Reported Cases of Autoimmune Stiff Person Syndrome and Its Variants.

Symptoms History or Examination Findings Antibody Testing Other Testing
Reference Reported Diagnoses at Presentation Age and Sex at Presentation Number Of Symptomatic Months Before Presentation Bulbar (Excluding Taste Changes) Taste Changes Genitourinary Dysfunction Difficulty Changing Position Difficulty Moving Limb Falls Gait Difficulties (Including Ataxia) Pain Pruritus Axial Stiffness or Hyperlordosis Autonomic Instability (Besides Genitourinary Dysfunction) Concomitant Autoimmunity (Other Than Diabetes) Cramps/Spasms Diabetes Extraocular Movement Abnormalities Hepatitis C Encephalopathy or Psychiatric/Behavioral Changes Hyperekplexia Hyperreflexia Limb Posturing/Dystonia Limb Stiffness/Rigidity Malignancy Myoclonus Seizures Weakness Serum Anti-GAD Antibodies Serum Anti-Glyr Antibodies Other Serum Antibodies Detected CSF Anti-GAD Antibodies CSF Anti-Glyr Antibodies Other CSF Antibodies Detected CSF Oligoclonal Bands EEG (Findings Consistent With Seizures) Electromyography Findings Consistent With SP MRI Brain Abnormalities MRI Spine Abnormalities To Explain The Patient’s Symptoms
Lobo et al.44 SP 41F 84 + + + + – + – + +
Scavone et al.24 SP 66M + + + + + – + + + – + AT, AG
Awad et al.23 A 48F + + P – + + + AI, ANA, AP, SSA + + – –
Castelnovo et al.45 SL 63F 36 + + – P + + + + + + – – – –
Cuturic et al.46 SP, ED 35F 24 + + + + + – + – – –
Ehler et al.6 SP 61M <1 + + + + + + + + + K K – + – –
Gnanapavan et al.47 SP 45M 60 + + + + + + + +
Goldkamp et al.48 SP 27F + +
Mas et al.37 E, PERM 60M <1 + + + + + + + + + + + + – + – + – + –
PERM 48M 2 + + + + + + + + + – + – + – – – –
SL, SP 33F 3 + + + + T + + + + + – + – – + – –
Piotrowicz et al.49 PERM 58M >1 + + + + + + + – + + – + – + + + –
Turner et al.27 PERM 28M 1 + + + + + + + + + + – + AN + – –
Witherick et al.28 SP 69M + MG, P + + + + +
Anagnostou et al.50 SL 40F 108 + + + + + + + + + – –
Amyradakis et al.51 SP F <1 + + + + + + +
Fekete and Jankovic52 SP 12M 84 + + + + + + + + + + + – – –
Fernandes et al.53 SP 50F 48 + + + + + – + + – –
A, E 52F <1 + + + + + – – –
Iizuka et al.25 PERM 61F 1.5 + + + + + + + + + + AG, AX + + + + –
Lorenzoni et al.33 SL 10F + + + + + + + +
SP 40M + + + + + + +
SP 42M + + + + + + + +
Najjar, at al.22 S 19F – + + – – –
Peeters et al.36 PERM 37F 1 + + + + + + + + + – + + + – + – + – + + + – –
Qureshi et al.54 SP 56M 72 + + + + + – + – + + + + – + – – –
Tsai et al.55 SP 66M 4 + + + + + + + + –a + + + – – –
Baroncini et al.26 A, LE 44F 36 + + + + T + + + + + – + + AP, AX + + – + +
Clardy et al.14 SP 8F <12 V + – + + + –
SP 26M 168 + + V + + + –
SP 51F 552 + – – + + – + –
SP 49M 528 + G + – + + – –
SL 14F 156 T + + + + – – –
ST 17M 36 + – – + – + +
PERM 13F <12 + + + + + + + – –
De la Casa-Fages et al.56 SP 59F 10 + + + P, V + + + – +
SP 48M 240 + + + V + + + + + – +
Damasio et al.57 PERM 1F <1 + + + + + + + + + + + + + + – + – –
Marinovic et al.58 SP 51F + + + + + – + –
Nakane et al.59 SP +
SP +
O’Toole et al.60 SP 72F 12 + + T + + + + – + +
Sidransky et al.61 SP 34 6 – + + + +
Sengupta et al.62 A, SP F +
Vetrugno et al. 201363 OT, SP 77F 1 + + + + + + + – + – – –
OT, SP 55F 12 + + + + T, V + + + – + –
Bordelon et al.64 SP 60F 108 + + + + + + + + – + –
Enuh et al.65 SP 20F 12 + + + + T + + + + + – + + + – –
Fourlanos et al.66 SP 78F 300 + + + + + + + + + – –
SP 72F 6 + + + + + + + – – –
Georgieva et al.67 A, E 45M + + + + + + + + + + –
Ho et al.68 SP 43F + + + + + + + + + – – + – – + – –
Jung et al.69 SP 55F 12 + + + + – + + + – + +
SP 58F 15 + + P + + + + + – – –
E, SP 49F 10 + + + + – + + + –
Pagano et al.30 SP 45M + – +
A, SP 65F + – +
SP 48F + – +
SP 61F – + AA
SP 34F + – +
A, SP 50F – +
Rana et al.70 SP 50F 30 + + + V + +
Sanders et al. 71 SP 48F + + + + + + – + – – –
SP 30F 24 + + + + +
Stern et al.72 PERM 40M <1 + + + + + + + – + – + – + – –
Wuerfel et al.73 B, E 2M 12 + + + – + – – – + – + – – – + –
Bowen et al.29 C, SP 49F 24 + + + + + – + – + – – + + + – –
C, SP 68F 84 + + + + + – + + + + –
Farooqi et al.74 E 23F 48 – – – – + + + +

Abbreviations: A, Ataxia; AA, Anti-amphiphysin Antibodies; AG, Anti-thyroglobulin; AI, Anti-intrinsic Factor; AN, Anti-NMDA Receptor Antibodies; ANA, Anti-nuclear Antibody; AP, Anti-parietal Cell Antibody; AT, Anti-thyroid Microsomal; AX, Anti-thyroid Peroxidase Antibody; B, Behavioral Changes; C, Corticobasal Syndrome; CSF, Cerebrospinal Fluid; E, epilepsy; ED, Eating Disorder; F, Female; G, Graves’ Disease; K, Antibodies against Tick-borne Meningoencephalitis; LE, Limbic Encephalitis; M, Male; MG, Myasthenia Gravis; MRI, Magnetic Resonance Imaging; OT, Orthostatic Tremor; P, Pernicious Anemia; PERM, Progressive Encephalomyelitis with Rigidity and Myoclonus; T, Autoimmune Thyroid Disease; S, Schizophrenia; SL, Stiff Limb Syndrome; SP, Stiff Person Syndrome; SSA, SS-A; ST, Stiff Trunk Syndrome; V, Vitiligo.

a

This patient had no evidence of active malignancy at the time of symptom presentation; but had a remote history of treated lymphoma and thymoma.