Stiff Person Syndrome: A Rare Neurological Disorder, Heterogeneous in Clinical Presentation and Not Easy to Treat

Table 1

Main characteristics of the three SPS patients.

Pt. nr./SPS form	Sex/age (yrs)	Age of onset (yrs)	Symptoms of onset	Distribution of pain and stiffness	Velocity of progression	Ab	EMG findings	Associated diseases/symptoms	SPS treatment
Patient 1/classic SPS	M/45	36	Pain, stiffness, and muscle cramps	Trunk (axial), followed by limbs (proximal) and face	Years	GAD (low titer)	Axonal sensory-motor polyneuropathy; CMUA at rest	Type I DM; hepatic steatosis; agoraphobia; depression	bzd, CBZ and gabapentin; IVIG
Patient 2/paraneoplastic SPS	M/73	72	Pruritus, severe pain, stiffness, and muscle cramps	Left foot, followed by both feet and both legs	Weeks	AMPH	Left peroneal nerve lesion; CMUA at rest	Small-cell lung carcinoma	bzd; morphine; chemo- and radiotherapy of tumor
Patient 3/SLS	M/68	66	Gait difficulty, pain, and stiffness (> after walking)	Legs (≫ right) (> proximal)	Months	GAD (very high titer)	Variable hypertonic activity	Recurrent vomiting; dysarthria; depression	Prednisone and AZA

Pt.: patient; nr.: number; SPS: stiff person syndrome; yrs: years; Ab: antibodies; EMG: electromyography; M: male; GAD: glutamic acid decarboxylase; CMUA: continuous muscular activity; DM: diabetes mellitus; bzd: benzodiazepines; CBZ: carbamazepine; IVIG: intravenous immunoglobulin; AMPH: amphiphysin; SLS: stiff limb syndrome; >: especially; AZA: azathioprine.