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Stiff-Person Syndrome and PERM

Picture of progressive encephalomyelitis, rigidity and myoclonus.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Nöroloji department. Book Appointment →

What is Stiff-Person Syndrome and PERM?

Stiff-person syndrome is a rare autoimmune disease characterized by progressive rigidity in the trunk and extremities, painful spasms and antibodies against glutamic acid decarboxylase (GAD65).

The PERM form (progressive encephalomyelitis with rigidity and myoclonus) is a more severe variant with brainstem and medullary involvement.

Treatment combines GABAergic agents, immunotherapies and physical rehabilitation.

Symptoms

Excessive muscle stiffness in the trunk
Spasms triggered by sudden noise or touch
Falling attacks
Accentuated lumbar lordosis
Myoclonus and ataxia in PERM form
Respiratory and swallowing problems
Severe anxiety and agoraphobia

Risk Factors

Female sex
Type 1 diabetes mellitus
Other autoimmune endocrinopathies
Malignancy (paraneoplastic form)
Family history of autoimmunity

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Truncal stiffness and falls
  • Shortness of breath
  • Speech and swallowing problems
  • Altered consciousness
  • Severe panic attacks
  • Increased frequency of spasms

Treatment Methods

01
High-dose diazepam and baclofen
02
IVIG immunotherapy
03
Plasma exchange
04
Rituximab
05
Intensive care support in PERM form
06
Pain and anxiety management
07
Physical medicine and rehabilitation

Which Department to Visit?

You can visit our Nöroloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Let us help you

You can make an appointment with our specialists or contact us for your concerns.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.