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LETTER TO EDITOR
Year : 2014  |  Volume : 62  |  Issue : 1  |  Page : 76-77

Anti-GAD negative stiff person syndrome with a favorable response to intravenous methylprednisolone: An experience over evidence


Department of Neurology, SMS Medical College, Jaipur, Rajasthan, India

Date of Submission04-Jan-2014
Date of Decision13-Jan-2014
Date of Acceptance30-Jan-2014
Date of Web Publication7-Mar-2014

Correspondence Address:
Kadam Nagpal
Department of Neurology, SMS Medical College, Jaipur, Rajasthan
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.128332

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How to cite this article:
Sharma B, Nagpal K, Prakash S, Gupta P. Anti-GAD negative stiff person syndrome with a favorable response to intravenous methylprednisolone: An experience over evidence. Neurol India 2014;62:76-7

How to cite this URL:
Sharma B, Nagpal K, Prakash S, Gupta P. Anti-GAD negative stiff person syndrome with a favorable response to intravenous methylprednisolone: An experience over evidence. Neurol India [serial online] 2014 [cited 2019 Sep 18];62:76-7. Available from: http://www.neurologyindia.com/text.asp?2014/62/1/76/128332


Sir,

A 65-year-old male patient presented with 6 months duration of insidious onset progressive stiffness in both lower limbs followed by upper limbs, trunk and neck rendering him non-ambulatory within 5 weeks. He used to experience severe painful spasms, getting aggravated during the emotional stress. These symptoms were less during sleep. Rest of the history was normal. Examination revealed marked difficulty in lying straight supine. Higher functions and cranial nerves were intact. Motor examination revealed distal amyotrophy in upper limbs with fasciculations in upper and lower limbs both distally and proximally. Along with this patient had a "woody" or "plank like" rigid abdomen muscles [Figure 1]a. He had lead pipe rigidity in all the limbs, neck, and axial musculature. Deep tendon jerks were normal and plantar response was bilaterally flexor. Sensory and cerebellar systems examination were non remarkable. He had exaggerated lumbar lordosis with increased flexion posturing at neck. Extensive workup including screening for malignance was negative. Considering possibility of "stiff person syndrome" (SPS) both serum and cerebrospinal fluid samples were subjected to anti-glutamic acid decarboxylase (GAD) antibody testing; which were negative. Electromyography revealed presence of continuous motor unit activity in paraspinals, rectus abdominis [Figure 1]b and c, biceps brachii and vastus lateralis muscles with fasciculations and doublets seen in bilateral first dorsal interossei. The spontaneous activity subsided on administering intravenous diazepam [Figure 1]d which further substantiated the diagnosis. Patient was put on high doses of baclofen (40 mg/day) and diazepam (50 mg/day). Due to financial constraint intravenous immunoglobulins could not be administered to the patient. Instead intravenous methylprednisolone (1 g/day Χ 5 days) was given. Patient showed remarkable relief from painful spasms and rigidity [Video 1]. Follow-up at 2 months, pain was alleviated with diazepam and baclofen.
Figure 1: (a) Patient having exaggerated forward fl exion of neck with "plank-like" rigidity of abdominal muscles. (b) Needle electromyography of the patient in rectus abdominis muscle right side showing spontaneous motor unit activity. (c) Continuous hypersynchronous electromyography activity seen during spasms. (d) Improvement in the electromyography activity after diazepam

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SPS is a rare immunological disorder characterized by progressive rigidity, painful spasms and continuous motor activity. [1] The hallmark of SPS is persistent muscular stiffness due to continuous co-activation of agonist and antagonist muscles, particularly "core muscles" that is the paraspinal and abdominal muscles. [2] Treatment ideally should involve symptom relief by baclofen, diazepam, and immunomodulation by intravenous immunoglubulin, plasmapheresis or in refractory cases by rituximab; but head to head trials are still lacking. [3] Corticosteroids have been used in cases which do not respond to high doses of baclofen. This was supported by results of Meinck [4] which stated a satisfactory response can be achieved by corticosteroids used as pulse therapy and as long-term maintenance in SPS.

 
  References Top

1.Moersch FP, Woltman HW. Progressive fluctuating muscular rigidity and spasm ("stiff-man" syndrome); report of a case and some observations in 13 other cases. Proc Staff Meet Mayo Clin 1956;31:421-7.  Back to cited text no. 1
    
2.Barker RA, Revesz T, Thom M, Marsden CD, Brown P. Review of 23 patients affected by the stiff man syndrome: Clinical subdivision into stiff trunk (man) syndrome, stiff limb syndrome, and progressive encephalomyelitis with rigidity. J Neurol Neurosurg Psychiatry 1998;65:633-40.  Back to cited text no. 2
    
3.Holmøy T, Geis C. The immunological basis for treatment of stiff person syndrome. J Neuroimmunol 2011;231:55-60.  Back to cited text no. 3
    
4.Meinck HM. Stiff man syndrome. CNS Drugs 2001;15:515-26.  Back to cited text no. 4
    


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