Stiff-Person Syndrome and Psychiatric Comorbidities: A Systematic Review

Caffrey, Deirdre; Finn, Christine; Song, Seo Ho; Burton, Frederick; Arsan, Cybele

doi:10.1016/j.psym.2020.08.005

Cited by 16 publications

(18 citation statements)

References 32 publications

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“…Among the 20 patients included in our case series, 65% were prescribed antidepressants, and approximately half of those assessed for depression and anxiety reported clinically significant symptoms. This is consistent with prior studies (3,28,29), and a recent systematic review which found that the relative risk of psychiatric comorbidity in SPS was higher than that of the general population (7). Mood and anxiety disorders are associated with deficits in learning and memory, executive function, and attention-areas also impaired in SPSD and anti-GAD65 associated diseases (8,9).…”

Section: Discussionsupporting

confidence: 90%

“…Furthermore, the presence of anti-GAD65 antibody has been associated with cognitive impairment in patients with neurological conditions (4), type 2 diabetes (5), and in animal models (6). In addition to cognitive dysfunction, patients with SPSD are also more likely than the general population to report anxiety and depressive symptoms, and to regularly use prescription benzodiazepines and muscle relaxants (7), all of which may contribute to poor performance on cognitive testing (8)(9)(10). To our knowledge, only two prior studies have assessed cognitive symptoms in patients with SPSD (3,11).…”

Section: Introductionmentioning

confidence: 99%

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Cognitive and Mood Profiles Among Patients With Stiff Person Syndrome Spectrum Disorders

et al. 2022

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An emerging body of evidence suggests that changes in cognitive and emotional function are common aspects of stiff person spectrum disorders (SPSD). We sought to examine the pattern of cognitive impairment and psychiatric symptoms in SPSD.MethodsA retrospective review of medical records was conducted for patients seen at the Johns Hopkins Stiff Person Syndrome (SPS) center from 1997 to January 1st, 2020. Individuals who had received formal cognitive testing as part of routine clinical care for patient-reported cognitive changes were included. Demographics, prevalence of cognitive impairment, psychoactive medication use, and clinically significant psychiatric symptoms were described.ResultsOut of 205 patients screened, 20 completed cognitive testing (75% female, mean age 47.4 years). The most common domains of impairment were verbal learning and recall memory (n = 14, 70%), verbal fluency (n = 10, 50%), processing speed (n = 8, 40%), and attention (n = 8, 40%). 9/11 patients assessed for depression reported clinically significant symptoms, and 4/9 patients assessed for anxiety reported clinically significant symptoms.ConclusionsScreening for cognitive impairment in SPSD should utilize testing that assesses verbal learning and recall, phonemic verbal fluency, attention, and processing speed. Moreover, it is important to evaluate for co-existing depression and anxiety symptoms, as these are common in SPSD.

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Section: Discussionsupporting

confidence: 90%

Section: Introductionmentioning

confidence: 99%

Cognitive and Mood Profiles Among Patients With Stiff Person Syndrome Spectrum Disorders

et al. 2022

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“…Anxiety and depression found in more than half of the patients were present in similar frequencies between SPSD and non‐SPSD. While chronic pain syndromes and functional neurologic diagnoses might predict a higher prevalence of anxiety and depression, it is well recognized that anxiety and phobias are prevalent among patients with GAD65‐IgG‐positive SPSD 4,28,34 …”

Section: Discussionmentioning

confidence: 99%

“…SPSD diagnosis is often delayed 4,25 . To date, issues relating to SPSD diagnosis have focused on missing the diagnosis 26–28 . However, we have identified many patients with alternative diagnoses misdiagnosed with SPSD and unnecessarily exposed to immunotherapies.…”

Section: Introductionmentioning

confidence: 99%

Stiff person spectrum disorder diagnosis, misdiagnosis, and suggested diagnostic criteria

Chia

McKeon

Dalakas

et al. 2023

Ann Clin Transl Neurol

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Background: Stiff person spectrum disorder (SPSD) is heterogeneous, and accurate diagnosis can be challenging. Methods: Patients referred for diagnosis/ suspicion of SPSD at the Mayo Autoimmune Neurology Clinic from July 01, 2016, to June 30, 2021, were retrospectively identified. SPSD diagnosis was defined as clinical SPSD manifestations confirmed by an autoimmune neurologist and seropositivity for high-titer GAD65-IgG (>20.0 nmol/L), glycinereceptor-IgG or amphiphysin-IgG, and/or confirmatory electrodiagnostic studies (essential if seronegative). Clinical presentation, examination, and ancillary testing were compared to differentiate SPSD from non-SPSD. Results: Of 173 cases, 48 (28%) were diagnosed with SPSD and 125 (72%) with non-SPSD. Most SPSD were seropositive (41/48: GAD65-IgG 28/41, glycine-receptor-IgG 12/41, amphiphysin-IgG 2/41). Pain syndromes or functional neurologic disorder were the most common non-SPSD diagnoses (81/125, 65%). SPSD patients more commonly reported exaggerated startle (81% vs. 56%, p = 0.02), unexplained falls (76% vs. 46%, p = 0.001), and other associated autoimmunity (50% vs. 27%, p = 0.005). SPSD more often had hypertonia (60% vs. 24%, p < 0.001), hyperreflexia (71% vs. 43%, p = 0.001), and lumbar hyperlordosis (67% vs. 9%, p < 0.001) and less likely functional neurologic signs (6% vs. 33%, p = 0.001). SPSD patients more frequently had electrodiagnostic abnormalities (74% vs. 17%, p < 0.001), and at least moderate symptomatic improvement with benzodiazepines (51% vs. 16%, p < 0.001) or immunotherapy (45% vs. 13% p < 0.001). Only 4/78 non-SPSD patients who received immunotherapy had alternative neurologic autoimmunity. Interpretation: Misdiagnosis was threefold more common than confirmed SPSD. Functional or non-neurologic disorders accounted for most misdiagnoses. Clinical and ancillary testing factors can reduce misdiagnosis and exposure to unnecessary treatments. SPSD diagnostic criteria are suggested.

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“…SPS can also manifest psychiatric symptoms like depression, anxiety, and obsessive‐compulsive disorder. Up to 50% of patients with SPS may have psychiatric comorbidities 242,243 . While specific information regarding a direct relationship between SPS and HSV is not readily evident in the available literature, it's worth noting that certain studies have explored the connection between AE and HSV‐1 244 .…”

Section: Role Of Hsv Infection In Inflammatory Neurological Diseasesmentioning

confidence: 99%

Immunopathology of herpes simplex virus‐associated neuroinflammation: Unveiling the mysteries

Hussain,

Gupta,

Samuel

et al. 2023

Reviews in Medical Virology

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The immunopathology of herpes simplex virus (HSV)‐associated neuroinflammation is a captivating and intricate field of study within the scientific community. HSV, renowned for its latent infection capability, gives rise to a spectrum of neurological expressions, ranging from mild symptoms to severe encephalitis. The enigmatic interplay between the virus and the host's immune responses profoundly shapes the outcome of these infections. This review delves into the multifaceted immune reactions triggered by HSV within neural tissues, intricately encompassing the interplay between innate and adaptive immunity. Furthermore, this analysis delves into the delicate equilibrium between immune defence and the potential for immunopathology‐induced neural damage. It meticulously dissects the roles of diverse immune cells, cytokines, and chemokines, unravelling the intricacies of neuroinflammation modulation and its subsequent effects. By exploring HSV's immune manipulation and exploitation mechanisms, this review endeavours to unveil the enigmas surrounding the immunopathology of HSV‐associated neuroinflammation. This comprehensive understanding enhances our grasp of viral pathogenesis and holds promise for pioneering therapeutic strategies designed to mitigate the neurological ramifications of HSV infections.

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Stiff-Person Syndrome and Psychiatric Comorbidities: A Systematic Review

Cited by 16 publications

References 32 publications

Cognitive and Mood Profiles Among Patients With Stiff Person Syndrome Spectrum Disorders

Cognitive and Mood Profiles Among Patients With Stiff Person Syndrome Spectrum Disorders

Stiff person spectrum disorder diagnosis, misdiagnosis, and suggested diagnostic criteria

Immunopathology of herpes simplex virus‐associated neuroinflammation: Unveiling the mysteries

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