Session Information
Date: Sunday, October 7, 2018
Session Title: Other
Session Time: 1:45pm-3:15pm
Location: Hall 3FG
Objective: A complicated case presented with trauma history and insidious onset of stiff person syndrome.
Background: Stiff person syndrome is a rare neurological disorder characterized by truncal stiffness, limb rigidity, frequent muscle spasms and awkward gait. cervical spine injury is a common cause of gait problem when patient presented with bizarre gait.
Methods: A 67 year-old man presented with bilateral foot drop for years with insidious four limb stiffness and gait difficulties for four years. He had one operation of odentoid process fracture ten years ago. There was no history of congenital disorders, strokes or traumatic event. He walked in a bizarre with steppage gait, decreased lumbar curve and both upper limbs floating floating. In addition, bilateral distal limb atrophy, mild weakness in foot plantar flexion, generalized hyperreflexia (3+) and rigidity were also noted. HLA-B27, Vitamin B12, tumor markers were within normal limits. Both brain MRI and cerebrospinal fluid study revealed non-specific finding.
Results: Needle electromyography (EMG) study showed increase muscle activity in L3/L4 paraspinal muscle and left vastus medialis muscle. The level of anti-glutamic acid decarboxylase antibody was 7.3U/ml. After a therapeutic trial with methylprednisolone therapy and benzodiazepines, he had significant improvement on the symptoms of muscle stiffness and gait difficulty.
Conclusions: This patient presented with bizarre gait, stiffness after C-spine surgery for years . Stiff person syndrome (SPS) was diagnosed under EMG finding and strongly positive of serum anti-GAD antibody level. Meanwhile, his gait difficulty was significant improvement by MTP therapy. it is important to include stiff person syndrome as a differential diagnosis of bizarre gaits.
References: 1. Baizabal-Carvallo JF, Jankovic J Stiff-person syndrome: insights into a complex autoimmune disorder J Neurol Neurosurg Psychiatry Published Online First: 15 December 2014. doi: 10.1136/jnnp-2014-309201. 2. Sadalage G, Karim A, Jacob S CLINICAL FEATURES AND TREATMENT RESPONSE IN A LARGE UK COHORT OF STIFF PERSON SYNDROME PATIENTS J Neurol Neurosurg Psychiatry 2015;86:e4.
To cite this abstract in AMA style:
K.W. Chen, K.L. Ho, A.C. Chen, J.J. Lin. C-spine injury with progressive gait difficulty, drop foot, bizarre gait and serum positive stiff person syndrome: A case report [abstract]. Mov Disord. 2018; 33 (suppl 2). https://www.mdsabstracts.org/abstract/c-spine-injury-with-progressive-gait-difficulty-drop-foot-bizarre-gait-and-serum-positive-stiff-person-syndrome-a-case-report/. Accessed September 7, 2024.« Back to 2018 International Congress
MDS Abstracts - https://www.mdsabstracts.org/abstract/c-spine-injury-with-progressive-gait-difficulty-drop-foot-bizarre-gait-and-serum-positive-stiff-person-syndrome-a-case-report/