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Improved Gait, Diplopia, Motor Speed, Symptoms, and Cognition in a 60-year-old female Patient with Stiff Person Syndrome

Megan Manno1, Emily Kalambaheti1, 2, Frederick R. Carrick3, 4, 5, 6 and Matthew M. Antonucci1, 2, 3*
  • 1 Plasticity Brain Centers, United States
  • 2 Accelerate Multidisciplinary Neuroscience Research Center, College of Medicine, University of Central Florida, United States
  • 3 Carrick Institute, United States
  • 4 MGH Institute of Health Professions, United States
  • 5 Bedfordshire Centre for Mental Health Research, University of Cambridge, United Kingdom
  • 6 University of Central Florida College of Medicine, United States

Background: Stiff Person Syndrome (SPS) is a type of rare progressive encephalomyelitis affecting autonomic ganglia, the brainstem, cerebellum, and spinal cord (Barker 1998). It is often associated with diabetes, stiffness, ataxia, and cognitive impairment (Ali 2011). This subject of this report was a 60-year-old female, who patient presented to Plasticity Brain Centers for evaluation and treatment of persistent symptoms from a 12-year history of SPS, Type 1 Diabetes, confirmed Anti-GAD (Glutamic Acid Decarboxylase) antibodies, and thyroid cancer. Her primary symptoms were ataxia, diplopia, and “difficulty thinking clearly”. Findings: The patient’s gait was ataxic requiring the use of a cane and assistance entering and exiting her transport vehicle. She had diplopia only in left lateral gaze, which decreased after passive rotation of her head to the right. Head thrust testing revealed a refixation saccade bilaterally. Symptoms were recorded using a graded symptom checklist (GSC) which demonstrated a symptom severity score of 18. During neurocognitive testing utilizing C3Logix, Trail Making Test (TMT-A and TMT-B) times were 44.0 seconds and 74.3 seconds, respectively. Digit-Symbol Matching score was 31 correctly matched symbols to numbers in 120 seconds. She was unable to perform choice reaction time due to confusion. A Comprehensive Assessment of Postural Systems (CAPS®) (Pagnacco 2014) was performed assessing balance and stability, on a solid surface, with eyes opened, eyes closed. An average stability score upon intake was calculated as 74.864% (considered within normal limits). Methods: This patient was treated over a course of 16 weeks, including a 5-day intensive treatment program consisting of ten 1-hour treatment sessions followed by one 1-hour session every other week for 7 weeks, another 5-day intensive treatment program, concluded with one 30-minute treatment sessions every other week for 7 additional weeks. All treatments consisted of multi-modal programs of neurological exercises similar to Carrick 2017, with repetitive peripheral somatosensory stimuli, cognitive exercises, neuromuscular reeducation exercises, balance exercises, vestibular rehabilitation exercises, orthoptic exercises, and off-vertical axis rotation utilizing a multi-axis rotational chair (MARC, ReNeuro Robotics, Dallas, TX, USA). Outcome: Upon discharge, the patient reported greater mental clarity, being able to walk without use of a cane. She was able to enter and exit her transport vehicle unassisted. She no longer had diplopia. Her positive head thrust test improved completely to the left, but the gain remained low on the right. Her symptom severity score decreased to 9 (-50.00%); TMT-A time was 28.1 seconds (-36.14%) and TMT-B time was to 52.4 seconds (-29.48%); Digit-Symbol Matching speed increased to 44 (+41.94%). She was able to perform choice reaction time with a reaction time of 425 milliseconds. Stability scores improved, with the average stability score calculated as 78.008% (+4.2%). Conclusion: This report describes the successful reduction of symptoms, improvement of ataxia, motor speed, diplopia, vestibular function, and cognition, of a female with a long-standing diagnosis of SPS, through an alternating intensive (inpatient-type) program and traditional less intensive (outpatient-type) program of multi-modal neurorehabilitation.

Acknowledgements

We would like to thank the clinical and administrative staff of Plasticity Brain Centers for assistance in this patients care.

References

Ali F, Rowley M, Jayakrishnan B, Teuber S, Gershwin ME, Mackay IR. Stiff-person syndrome (SPS) and anti-GAD-related CNS degenerations: Protean additions to the autoimmune central neuropathies. J Autoimmun. 2011;37:79–87. doi: 10.1016/j.jaut.2011.05.005. 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;65:633–640. doi:http://dx.doi.org/10.1136/jnnp.65.5.633. Carrick FR, Clark JF, Pagnacco G, Antonucci MM, Hankir A, Zaman R and Oggero E (2017) Head–Eye Vestibular Motion Therapy Affects the Mental and Physical Health of Severe Chronic Postconcussion Patients. Front. Neurol. 8:414. doi: 10.3389/fneur.2017.00414 Pagnacco G, Carrick FR, Wright CH, Oggero E. In-situ verification of accuracy, percision and resolution of force and balance platforms. Biomed Sciences instrumentation (2014) 50:171-8

Keywords: Stiff person syndrome (SPS), Neurorehabiliation, vestibular rehabilitaiton, Ataxia, cognitive impaiment, Intensive therapy program, Diplopia

Conference: International Symposium on Clinical Neuroscience, Orlando, United States, 24 May - 26 May, 2019.

Presentation Type: Poster Presentation

Topic: Clinical Neuroscience

Citation: Manno M, Kalambaheti E, Carrick FR and Antonucci MM (2019). Improved Gait, Diplopia, Motor Speed, Symptoms, and Cognition in a 60-year-old female Patient with Stiff Person Syndrome. Front. Neurol. Conference Abstract: International Symposium on Clinical Neuroscience. doi: 10.3389/conf.fneur.2019.62.00078

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Received: 18 May 2019; Published Online: 27 Sep 2019.

* Correspondence: Dr. Matthew M Antonucci, Carrick Institute, Cape Canaveral, Florida, 32920, United States, drnucci@gmail.com