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Reducing Excess Stiffness in Stiff-Person Syndrome using CBT: A Case Study

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1396 (Excluding abstract and acknowledgements)

Search Terms

Stiff Person Syndrome, Stiff Man Syndrome, CBT, Psychotherapy




Miss Lottie L. Morris, BSc

Corresponding author

Clinical Psychologist in Training

Department of Clinical Psychology, 6 West, University of Bath, Claverton Down, Bath, BA2 7AY (UK)

Email: lottie.morris@nhs.net

Telephone: 0785 1717 129


Dr Leon Dysch, DClinPsy

Clinical Psychologist

Bath Community Neuro and Stroke Service, St Martin’s Hospital, Clara Cross Lane, Bath, BA2 5RP (UK)

leon.dysch@sirona-cic.org.uk


Prof.Paul M. Salkovskis, DClinPsy

Professor of Clinical Psychology

Department of Clinical Psychology, 6W 0.9, University of Bath, Claverton Down, Bath, BA2 7AY (UK)

p.m.salkovskis@bath.ac.uk




Reducing Excess Stiffness in Stiff-Person Syndrome using CBT: A Case Study
Abstract

BACKGROUND: Stiff Person Syndrome (SPS) is a rare neurological condition, characterised by rigidity in the trunk and limbs. Comorbid anxiety is common and known to exacerbate stiffness.

OBJECTIVE: This case study examines the extent to which psychological treatment of comorbid anxiety alleviated stiffness in a patient whose condition was exacerbated by social anxiety.

METHODS: A patient was treated using cognitive behavioural therapy, focussing on reducing anxiety and therefore stiffness by addressing rumination, self-focussed attention, and distressing cognitions relating to walking in public. The patient’s walking, stiffness, and anxiety were assessed during and post-therapy using questionnaires.

RESULTS: Walking, stiffness, and anxiety improved during treatment. At five months’ follow up, while the improvement in anxiety was maintained, walking and stiffness had deteriorated. The patient and his Neurologist felt that this deterioration was biological, rather than psychological in nature.

CONCLUSIONS: This is the first published case where SPS has been ameliorated (albeit temporarily) using psychological therapy, and has important implications for future research and treatment.

Stiff Person Syndrome

Stiff Person Syndrome (SPS) is a rare neurological condition, affecting the central nervous system. SPS is characterised by fluctuating rigidity in the trunk and limbs, a heightened sensitivity to stimuli such as noise and emotional distress (National Institute of Neurological Disorders and Stroke (NINDS), 2012), and painful spasms of the trunk and legs (Henningsen and Meinck, 2003). After an initial progressive phase, most people with SPS then stabilise, although remain at risk of falls (NINDS, 2012). The cause of SPS has not yet been established definitively, although research suggests an autoimmune aetiology (Duddy and Baker, 2009) and is thought to be a paraneiplastic phenomenom in some patients.Prevalence is estimated to be around one in a million (Yale School of Medicine, 2012). SPS can be diagnosed with a blood test for elevated levels of the antibody to glutamic acid decarboxylase, GAD-65, in around 60% of cases which inhibits the production of this important neurotransmitter (Duddy and Baker, 2009). At present, there is no cure for SPS, although a recent review (Lockman and Burns, 2007) states that symptoms can be managed using benzodiazepines and/or baclofen, muscle relaxants, anti-convulsants, and painkillers. Non-responsive patients may also be offered immunosuppressant medication. Physical and psychiatric treatment is noted in this review to be beneficial.


The Role of Anxiety in SPS

It is well known that muscular tension is an autonomic physiological symptom of anxiety (e.g. Lang and McTeague, 2009) and it is noted in the literature that anxiety may exacerbate stiffness in SPS (Lockman and Burns, 2007). For example, one study (Henningsen and Meinck, 2003) found that 44% of patients had developed task-specific phobia owing to the motor symptoms of SPS. It was noted that these patients were more likely to show an exaggerated startle response, demonstrating the link between anxiety and physical symptoms of SPS. Given this relationship, one can infer that there might also be a relationship between the bodily symptoms of the anxiety-triggered “fight or flight” response, and stiffness in SPS.


Meta analytic reviews suggest Cognitive Behavioural Therapy is the most effective treatment for anxiety (e.g. National Institute for Clinical Excellence (NICE), 2007), however no studies to date have explored the use of psychological therapy for anxiety in SPS. It is clear from previous research that anxiety is a factor in the level of disability experienced by people with SPS. This paper therefore presents an interesting and novel line of treatment for such people.
Background

Mr A was diagnosed with Stiff Person Syndrome (SPS) by a neurologist following a number of investigations, one of which found his anti-GAD antibodies to be strongly positive (>2000 IU/ml). Screening for an underlying malignancy was negative. Following a course of intravenous immunoglobulin (IvIG) and physiotherapy, Mr Awas referred to the psychology department of a community neurology clinic as his lower limb stiffening was severely aggravated in public situations by social anxiety. Mr A described how, in public situations, his walking would deteriorate severely, sometimes to the point where he would become completely rigid. This had caused several falls and broken bones. Following psychological assessment, a cognitive behavioural psychological model of social anxiety (Clark and Wells, 1995) was applied to treat this problem, focussing on reducing anxiety and therefore excess stiffness by addressing rumination, self-focussed attention, and distressing cognitions relating to walking in public.



Psychological Formulation

According to Clark and Wells’ cognitive behavioural model of social anxiety (1995), social anxiety is brought about and maintained by idiosyncratic negative beliefs about the self or others, which are then maintained by maladaptive coping strategies, and self-focussed attention, which prevent disconfirmation of the negative beliefs. Treatment involves identifying and addressing these maintaining factors.


This model of social anxiety was used to develop a psychological explanation of Mr A’s increased stiffening and walking difficulties in public situations. Prior to social events (i.e. any event where another person might be present) where Mr A would be required to walk, he would experience a high level of anticipatory anxiety, which would cause increased rigidity in the trunk and lower limbs. During the event, Mr A was convinced that other people were making negative judgements about him due to his restricted walking. These beliefs, in addition to an understandable fear of falls, led Mr A to focus all of his attention on walking properly, looking where he was going, and trying to come across well to others. This entirely self-focussed attention led to the feeling of being the centre of attention, and prevented Mr A from noticing that, in fact, no-one was paying attention to him. Additionally, this sense that other people were staring at him increased Mr A’s beliefs that others were making negative judgements of him. All of this led to increased anxiety, and therefore increased stiffening and poorer walking. Mr A would engage in post-event rumination after each situation, which would invariably be a negative appraisal of the event, focussing on the feeling of being stared at, and Mr A’s beliefs that others were making negative judgements of him. His memory for each event would therefore be overwhelmingly negative, and would spring to mind at the prospect of the next event involving walking in public, bringing about a sense of dread, and completing the vicious cycle.

This psychological explanation of Mr A’s increased stiffening in social situations is outlined in figure 1 in diagrammatic format:



¡§Insert Figure1 here¡

Intervention

Mr A received five, hour long sessions of cognitive behavioural therapy (CBT) for social anxiety, in which the above psychological formulation was developed and shared. Mr A and the therapist then designed and conducted various experiments to test his beliefs and coping behaviours.


Outcome measurement

Mr A completed the following measures at two baseline time points, mid-therapy, post-therapy, and at two follow-up time points:



  • The 6-item Social Phobia Weekly Summary Scale (SPWSS) (Clark et al., 2006) was used to measure social anxiety, avoidance, self-focussed attention, anticipatory processing, and post-event rumination.

  • A Visual Analogue Scale (VAS) measuring social anxiety, confidence, walking, and stiffening in social situations.

Qualitative information about therapy process was yielded using the Helpful Aspects of Therapy Questionnaire (HATQ).




Results

Within five weeks of cognitive behavioural therapy, ratings on the SPWSS (figure 2) showed reductions in self-focussed attention, anticipatory anxiety, and post-event rumination.


Mr A reported on the VAS (figure 2) that his stiffening in public situations had reduced from 10/10 to 2/10, his walking in public had improved from 4/10 to 8/10, social anxiety had reduced from 6/10 to 2/10, and self-confidence had risen from 5/10 to 8/10. As social anxiety began to decrease, so did the frequency and intensity with which Mr A stiffened up whilst walking in public. At the same time, his walking rating and self-confidence in social situations increased. Mr A indicated in the HATQ that developing a psychological explanation for his increased stiffening had been particularly helpful. Mr A stated that shifting his attention from self-focussed to externally focussed, and learning that other people weren’t noticing him or judging him as much as he predicted had helped to reduce his anxiety.

¡§Insert Figure2 here¡
These improvements were maintained at 2.5 weeks follow-up. Unfortunately, while Mr A’s social anxiety continued to improve at 5 months follow-up, his neurological condition had worsened. Mr A and his Neurologist feel that this deterioration was biological, rather than psychological in nature.
Discussion

The case of Mr A demonstrates how useful CBT can be for reducing anxiety-related stiffness in SPS. This study has obvious limitations, namely the lack of a definitive reason for the deterioration in stiffening seen at follow-up; single case study design; and lack of control of extraneous variables, particularly medication. However, it does seem that by addressing negative beliefs, self-focussed attention, anticipatory and post-event rumination, Mr A’s anxiety decreased, resulting in reduced stiffness and improved walking in public, while his neurological condition was stable.


The present case report provides initial evidence to suggest that CBT is likely to be an effective treatment for social anxiety in SPS. In addition to the results of this case report, it is reasonable to assume that, as CBT reduces anxiety in the general population (Acaturk et al., 2008), it should also reduce anxiety and therefore excess stiffening for people with SPS. Given that previous literature has demonstrated that anxiety worsens stiffness and therefore increases disability in SPS, it is important that further research is carried out to explore the utility of CBT for social anxiety and other anxiety disorders in this population. This is the first published case where SPS has been ameliorated using psychological therapy, and has important repercussions for future research and treatment.
Acknowledgements, competing interests, and funding

The authors would like to thank Mr A for consenting to the publication of this paper. The study was completed as part of the corresponding author’s doctoral training in Clinical Psychology, therefore there are no funding or competing interests to be disclosed.




Reference list

Acarturk, C., Cuijpers, P., van Straten, A., & de Graaf, R. (2008). Psychological treatment of social anxiety disorder: a meta analysis. Psychological Medicine, 39, 241-254.


Clark, D. M., Ehlers, A., Hackmann, A., McManus, F., Fennell, M., and Grey, N. (2006). Cognitive therapy versus exposure and applied relaxation in social phobia: A randomised controlled trial. Journal of Consulting and Clinical Psychology, 74: 568-578.
Clark, D. M. & Wells, A. (1995).A cognitive model of social phobia. In R. Heimberg, M. Liebowitz, D.A. Hope, & F.R. Schneier (Eds.) Social Phobia: Diagnosis, assessment and treatment. (pp. 69-93). New York: Guilford Press.

Duddy, M. E., & Baker, M. R. (2009). Immune-mediated neuromuscular diseases: Stiff Person Syndrome. Frontiers of Neurology and Neuroscience, 26, 147-165.


Henningsten, P., & Meinck, H. M. (2003). Specific phobia is a frequent non-motor feature in stiff man syndrome. Journal of Neurology, Neurosurgery, and Psychiatry, 74, 462-465.
Lang, P. J. & McTeague, L. M. (2009). The anxiety disorder spectrum: Fear imagery, physiological reactivity, and differential diagnosis. Anxiety Stress Coping, 22(1):5-25.
Lockman, J., & Burns, T. M. (2007).Stiff-Person Syndrome.Current Treatment Options in Neurology, 9, 234-240.
National Institute of Neurological Disorders and Stroke (2012).NINDS Stiff-Person Syndrome Information Page. [online] Available at: [Accessed 4 September 2012]
National Institute of Clinical Excellence (2007). Anxiety: management of anxiety (panic disorder, with or without agoraphobia, and generalised anxiety disorder) in primary, secondary and community care. NICE: London.
Yale School of Medicine (2012).Stiff-Man Syndrome. Yale School of Medicine [online] Available at: http://medicine.yale.edu/neurology/divisions/neuromuscular/sms.aspx [Accessed 12 September 2012].


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