Jerking Stiff Man Syndrome

 

Course: chronic

 

Cause: autoimmune, idiopathic, possibly paraneoplastic (unlikely in a chronic survivor)

 

Clnical features:

(1) florid muscle rigidity involving all 4 extremities that may occur in paroxysms

(2) absence of long tract signs

(3) brainstem signs with myoclonus

(4) involvement of breathing muscles, which can lead to asphyxiation

 

Laboratory features: anti-GAD, anti-acetylcholine receptor and other antibodies reported

 

Differential diagnosis:

(1) seizure disorder

 

Management:

(1) tracheostomy with ventilation may be required in severe cases

(2) immunosuppression with plasmapheresis may be necessary