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Psychiatry may be consulted, especially when symptoms of depression or anxiety are prominent. The psychiatrist should be made aware of the pathophysiology of stiff-person syndrome and that the anxiety symptoms may be directly related to the presence of glutamic acid decarboxylase antibodies in the central nervous system. If possible, consult a psychiatrist that has shown interest in the disease.
Although early intervention can reduce long-term disability, approximately 50% of SPS patients eventually have to use a wheelchair as a result of pain and immobility.
The patient’s fear of falling and startle can lead to rational agoraphobia versus true agoraphobia.
Depression due to loss of quality of life is seen in over half of the patients. The anxiety and fear response can be mistakenly viewed as psychosomatic illness. Due to the level of medications required to treat the spasms, patients are sometimes mislabeled as drug-seeking or malingering.
Untreated or under-treated patients are at a high risk of suicide. Sudden withdrawal of treatment causes a rapid escalation of symptoms which can be life threatening.
In a study of twenty-four patients to determine effects on quality of life in the UK, it was found that SPS patients showed reduced scores on all aspects of a Short Form Health Survey (SF-36) and disability correlated with the degree and location of stiffness.
Depression affected half of the respondents according to the Beck Depression Inventory.
2. Ameli R, Snow J, Rakocevic G, Dalakas MC. A neuropsychological assessment of phobias in patients with stiff-person syndrome. Neurology 2005;64:1961. Link to article.
A neuropsychological assessment was performed in 10 patients with stiff person syndrome (SPS) to determine whether their anxiety and phobic symptoms precede stiffness and spasms or represent a reaction to disability. No neurocognitive dysfunction was noted. Patients perceived fears and anxiety as realistic and caused by SPS rather than due to an inherent phobic neurosis.
3. Black JL, Barth EM, Williams DE, Tinsley JA. Stiff-man syndrome. Results of interviews and psychologic testing. Psychosomatics. 1998 Jan-Feb;39(1):38-44. Link to article.
Thirteen patients with stiff-man syndrome (SMS) were studied with the Minnesota Multiphasic Personality Inventory (MMPI), the Self-Administered Alcoholism Screening Test (SAAST), the State-Trait Anxiety Inventory (STAI) profiles, and by telephone interviews. The authors hypothesize that SMS patients have a gamma-aminobutyric acid deficiency or GABAergic neuron dysfunction that leads to psychiatric symptoms, including depression and chemical abuse. Clinicians treating patients with SMS must be alert to the possible presence of comorbid psychiatric illnesses in this patient population.
4. Culav-Sumić J, Bosnjak I, Pastar Z, Jukić V. Anxious depression and the stiff-person plus syndrome. Cogn Behav Neurol. 2008 Dec;21(4):242-5. doi: 10.1097/WNN.0b013e318185e6d2. Link to article.
Before the onset of typical SPS symptoms, psychiatric symptoms (like depression and anxiety) may be prominent and as such misleading, resulting in the diagnosis of a psychiatric condition.
5. Cuturic M, Harden LM, Kannaday MH, Campbell NN, Harding RK. Stiff-person syndrome presenting as eating disorder: a case report. Int J Eat Disord. 2011 Apr;44(3):284-6. doi: 10.1002/eat.20794. Link to article.
SPS has not been previously reported in the context of eating disorders, although it has been linked to other psychiatric disorders. Often the psychiatrist may be the first physician to diagnose SPS. We present this case to alert practitioners to the potential co-morbidity and symptom overlap between SPS and eating disorders, to aid in early recognition and appropriate treatment of this rare illness.
6. Drake ME Jr. Stiff-man syndrome and dementia. Am J Med 1983;74:1085-1087. Link to article.
An association between stiff-man syndrome and dementia has not been previously described. Increased muscle tone and muscular rigidity is frequently encountered in patients with dementia, however, and pathologic reflexes involving neck and proximal musculature have been described in dementia. It is possible that this patient represents an exaggerated form of such motor disturbances in dementia, and that clinical and electromyographic features of stiff-man syndrome may be present with increased incidence in patients with dementia.
7. Henningsen P, Clement U, Kuchenhoff J, et al. Psychological factors in the diagnosis and pathogenesis of stiff-man syndrome. Neurology 1996;47:38. Link to article.
Eight patients were initially misdiagnosed as having psychogenic movement disorder. We conclude that the common misdiagnosis of SMS as a psychogenic movement disorder is due to the compelling association of a set of salient psychological features, bizarre and fluctuating motor symptoms, and lack of approved neurologic signs.
8. Henningsen P, Meinck HM. Specific phobia is a frequent non-motor feature in stiff man syndrome. J Neurol Neurosurg Psychiatry 2003;74:462. Link to article
Specific phobia is a frequent non-motor symptom of stiff man syndrome. Early recognition is an important aid to correct diagnosis. The aetiology of phobia in stiff man syndrome is unknown. There is no evidence of a direct pathogenic role of autoantibodies directed against glutamic acid decarboxylase in the development of phobia.
9. Ho CS, Ho RC, Wilder-Smith EP. Stiff person syndrome masquerading as panic attacks. Lancet. 2014 Feb 15;383(9917):668. doi: 10.1016/S0140-6736(14)60127-6. Link to article.
10. Tinsley A, Barth EM, Black JL, Williams DE. Psychiatric consultations of stiff-man syndrome. J Clin Psychiatry 1997;58:444. Link to article
We speculate that the GABA system is involved in both the neurologic and psychiatric
symptoms of these patients. Psychiatrists have a significant role in the management
of patients with stiff-man syndrome and may be expected to manage anxiety, depression,
and substance misuse.
11. Dinnerstein E, Collins D, Berman SA. A patient with post-traumatic stress disorder developing stiff person syndrome: is there a correlation? Cogn Behav Neurol. 2007 Jun;20(2):136-7. Link to article.
Here, we present a case report of a patient diagnosed with post-traumatic stress disorder (PTSD) acquired by sexual trauma and by exposure to the severely wounded soldiers she attended as a nurse. Subsequently, she developed SPS confirmed by serology. The possibility of an association between PTSD and SPS is theorized, given their relationship to the GABAergic system. Further studies examining the relation between PTSD and SPS should be initiated.
12. Rodrigues de Cerqueira AC, Ferreira Bezerra JM, Rozenthal M, Egídio Nardi A. Stiff-Person syndrome and generalized anxiety disorder. Arq Neuropsiquiatr 2010;68(4):659-661 Link to article.
This case report highlights the importance of the recognition of anxiety disorders in patients with SPS. Although the exact mechanism of such an association remains unknown, it has been postulated that it could be the result of a reduced or impaired GABAergic inhibition induced by the anti-GAD antibodies, which may predisposed the development of anxiety symptoms in these patients. This hypothesis is supported by the fact that drugs that enhance GABAergic inhibitory transmission such benzodiazepines are effective for treatment in both conditions. In conclusion, the early recognition of psychiatric symptoms in these patients is particularly important to develop treatment strategies to help them manage their disease more successfully.