Table 4. Reported Cases of Cryptogenic Stiff Person Syndrome and Its Variants.
Symptoms | History or Examination Findings | Testing | Treatment | Outcome | ||||||||||||||||||||||||||||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Reference | Reported Diagnosis | Age And Sex at Presentation | Number Of Symptomatic Months Before Presentation | Bulbar Dysfunction | Genitourinary Dysfunction | Difficulty Moving Limb | Falls | Gait Difficulties (Including Ataxia) | Pain | Axial Stiffness Or Hyperlordosis | Autonomic Instability (Besides Genitourinary Dysfunction) | Concomitant Autoimmunity | Cramps/Spasms | Encephalopathy Or Psychiatric/Behavioral Changes | Hyperekplexia | Hyperreflexia | Limb Posturing/Dystonia | Limb Stiffness/Rigidity | Myoclonus | Seizures | Weakness | Documentation Of Autoantibody Testing | Serum Anti–Gad Or Anti–Glyr Antibodies | CSF Oligoclonal Bands | Electroencephalographic Abnormalities | Electromyography Findings Consistent With SP | MRI Brain Abnormalities (Incidental Findings Excluded) | Antiepileptics | Baclofen | Benzodiazepines | Botulinum Toxin Injections | Intravenous Immune Globulin | Other agents or interventions | Plasmapheresis | Steroids | Steroid–sparing immunosuppression | Surgical intervention | Improvement or resolution | 1 or more relapses | Stabilization without improvement |
Ughratdar et al.32 | SL, SP | 44M | 168 | + | + | + | + | + | – | + | + | + | + | + | + | |||||||||||||||||||||||||
Hegyi.40 | SP | 24F | 9 | + | + | + | – | + | + | + | + | + | – | + | + | + | ||||||||||||||||||||||||
Iwata et al.34 | SL | 29M | 24 | + | + | + | + | + | + | + | + | – | + | – | + | – | + | + | + | |||||||||||||||||||||
Prasad89 | SP | 51M | 6 | + | + | – | + | + | + | + | + | – | – | – | + | + | + | + | ||||||||||||||||||||||
Sanefuji et al.90 | SP | 7F | <1 | + | + | + | + | + | – | – | + | – | – | + | + | + | + | + | + | + | + | |||||||||||||||||||
Schreiber et al.41 | SP | 75F | 36 | + | + | + | + | + | – | + | + | + | + | – | – | – | + | + | + | + | ||||||||||||||||||||
Lorenzoni et al.33 | SP | 43M | + | + | + | T, V | + | + | + | + | + | – | + | + | + | + | ||||||||||||||||||||||||
Clardy et al.14 | SP | 13M | 2 | + | – | + | – | + | – | + | + | + | + | |||||||||||||||||||||||||||
Newton et al.42 | SP | 48M | + | + | + | + | + | – | + | + | – | + | + | + | + | |||||||||||||||||||||||||
Vicente–Valor et al.43 | SP | 40M | 72 | + | + | + | + | + | + | + | + | + | + | – | + | + | + | + | + | + | + | + | + | |||||||||||||||||
Sharma et al. 91 | SP | 65M | 6 | + | + | + | + | + | – | + | + | – | + | + | + | + | + | |||||||||||||||||||||||
Pakeerappa et al.92 | SP | 48M | 17 | + | + | + | + | – | + | + | + | + | + | |||||||||||||||||||||||||||
Ueno et al.35 | PERM | 48F | <1 | + | + | + | + | + | + | + | + | + | + | + | + | – | – | + | + | + | + | + | + | + | + | + | + |