Psychosomatics 43:243-244, June 2002
© 2002 The Academy of Psychosomatic Medicine
Stiff-Man Syndrome: A Case Report and Review of the Literature
Carol R. Kiriakos, M.D., and
Kathleen N. Franco, M.D.
Received January 18, 2002; revised February 28, 2002; accepted March 5, 2002. From the Department of Psychiatry and Psychology, Cleveland Clinic Foundation, Cleveland, Ohio. Address correspondence and reprint requests to Dr. Franco, Department of Psychiatry and Psychology—P57, 9500 Euclid Avenue, Cleveland, OH 44195; E-mail; francok{at}ccf.org
Key Words: Stiff-Man Syndrome
Stiff-man syndrome (SMS) is a rare autoimmune neurological condition first described a half decade ago and only rarely spotted on consultation-liaison services. It is characterized by involuntary rigidity and painful spasm of the axial muscles, resembling a chronic form of tetanus. 1–10 Patients are likened to "tin soldiers," 2 with the following criteria: (1) initial stiffness and rigidity in the axial muscles; (2) slow progression of stiffness to include proximal limb muscles, making volitional movements and ambulation difficult; (3) a fixed deformity of the spine, as in lordosis, and with some patients a restriction of hip movement; (4) the presence of superimposed episodic spasms precipitated by sudden movements, jarring noise, and emotional upset; (5) normal findings on motor and sensory nerve examinations; (6) normal intellect; and (7) electromyographic findings typical of continuous motor activity that can be abolished by intravenous or orally administered diazepam. 3,4 Antiglutamic acid decarboxylase (anti-GAD65) autoantibodies disrupt the function of gamma-aminobutyric acid (GABA) 7 and may explain why SMS, a relapsing and remitting disease, only partially responds to diazepam and baclofen, with 50% of patients becoming wheelchair bound. 2 GABA agonists, such as benzodiazepines, alcohol, and barbiturates, in high doses can greatly alleviate psychiatric and neurological symptoms. 5,10,11
We describe a patient diagnosed by neurological symptoms of SMS who also met criteria for generalized anxiety, major depression, and panic disorder.
Case Report
A 36-year-old woman with severe pain arrived at the hospital to change her intrathecal pump (ITP) medication from cyclobenzaprine (a skeletal muscle relaxant closely related to tricyclic antidepressants) with morphine to cyclobenzaprine with hydromorphone. She reported inadequate pain relief with increasing spells of nausea, vomiting, and constipation believed to be secondary to morphine. Her current treatment included oxycodone 30–35 mg/day, lansoprazole 30 mg/day, amitriptyline 75 mg/day, odansetron 24 mg/day, diazepam 15 mg/day, and ITP with cyclobenzaprine 2000 U/mL and morphine 6 mg/mL. Four months into treatment with cyclobenzaprine, she experienced more frequent episodes of stiffness and spasms spreading to her hands, face, and mouth and forcing her to take a 4-month leave of absence from her supervisory position.
During her hospitalization, a psychiatric consultation was requested by the anesthesia-pain management team for assistance with her depression, anxiety, and inadequate pain control. Notes from the neurology service described the patient's anger and her frequent demands on the staff. The patient continually verbalized frustration at the way her pain was being managed but was resistant to any psychiatric intervention. Having tried everything else, she wanted a definitive answer as to whether she would be a candidate for intravenous immunoglobulin treatment to improve relief.
The patient's history revealed 3 years of chronic back spasms and abdominal pain aggravated by standing, walking, and other activities of daily life. The cascade of symptoms would begin with her "back going out and pulling [her] down." Additionally, the patient remarked that "pain moved up and down [her] spine to the left hip." Despite increasing doses of benzodiazepines, stiffness in her lower extremities became so severe that she could not walk and needed a cane or even a wheelchair intermittently over the past 18 months. She had seen multiple physicians during the 3-year period, including a chiropractor, an otolaryngologist, a neurosurgeon, and several neurologists. Two years earlier, she underwent a lumbar laminectomy without much relief. Her current neurologist of 2 years had diagnosed SMS and tried to support her with regularly scheduled appointments. However, she frequently called both the neurologist and the anesthesia pain specialist with increasing demands and eventually acknowledged that she admitted herself under the guise of a referral from another doctor.
On examination, her speech was soft and childlike, her affect restricted, and her mood depressed. Tearfully, she admitted fears that her doctors would not want to take care of her. The patient described early morning awakening, decreased appetite with a 10-lb weight loss over 6 months, anhedonia, and feelings of hopelessness. In addition, she reported frequent crying episodes, anxious feelings in her stomach, and muscle tightness in her back and legs. She also described feeling "out of control" with heart palpitations, rapid breathing, and a sense of doom with any new physical discomfort. Even the patient wondered if depression and anxiety exacerbated physical pain and body spasms associated with her SMS. Further history revealed that the patient met criteria for major depression, generalized anxiety, and panic disorder. She denied any personal or family history of alcohol, marijuana, or street drug abuse now or in the past.
The psychiatric team recommended starting the patient on mirtazapine for depression and gabapentin for pain relief, generalized anxiety, and panic attacks. Biofeedback and relaxation training were also recommended to encourage the patient's participation in reducing her anxiety and pain.
Discussion
As emotional distress precipitates painful spasms, psychogenic movement disorder is a frequent initial diagnosis. 4,6 Patient anger, as in our case, results when individuals feel they are being told "it is in your head." Anxiety, depression, and alcohol abuse may precede physical symptoms as a result of a major life stressor or be a sequelae of SMS. 4,6 In our patient, panic attacks, hopeless and helpless feelings, and fear of physician abandonment repeatedly seemed to initiate and intensify her symptoms. She met criteria for major depression, panic disorder, and generalized anxiety disorder and demonstrated substance tolerance. Over 60% of patients with SMS have at least one psychiatric diagnosis, and of these, half either abused or were dependent on alcohol. 7 The role of secondary gain must also be considered in this instance and included time away from work, financial disability assistance, and help with child care.
Concurrent psychiatric disorder and SMS share the pathogenesis of exaggerated activity of suprasegmental centers. Autoantibodies attack glutamic acid decarboxylase (GAD), limiting the production of GABA and lowering the threshold for anxiety disorders and muscle spasms. 2, 7,10 GABA agonists, such as diazepam and lansoprazole, brought initial relief to our patient but later very little pain control and may have increased her depression. GABA-ergic dysfunction leading to SMS would also predict a higher incidence of chemical abuse. Tolerance and dependence on GABA agonists and narcotics became quite problematic for our patient and increased physician frustration. Besides tolerance and addiction, increased narcotic use at the time of admission led to more nausea, requiring additional antiemetic use. Both medications can induce akasthesia and dyskinesia and potentially contribute to her movement disorder. There was real fear on the part of the patient and her caretakers that her pain might become unrelievable. Believing she had little control over pain, her anticipatory anxiety increased the frequency of attacks. Henningsen et al. hypothesized that this further activates autoantibodies including the long-term vulnerability of the immune system and particularly targeting limbic neurons. Currently, antianxiety and antidepressant medication along with relaxation training may offer some relief. Psychotherapy is useful to treat emotional distress and to explore the role of possible secondary gain in maintaining symptoms. Chemical dependency treatment is also important for appropriate patients. GABA-ergic medication such as gabapentin may be safely given without worry of dependency. Prednisone and immunoglobulin offer hope to control or limit changes in the immune system that currently predict a prolonged course. 11
Perhaps most crucial is close collaboration among primary care physicians, neurologists, anesthesiologists, and psychiatrists to provide clear and nonconflicting explanations and presenting "team supported" recommendations for these troubled patients.
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P Henningsen and H-M Meinck
Specific phobia is a frequent non-motor feature in stiff man syndrome
J. Neurol. Neurosurg. Psychiatry,
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[Abstract]
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