Stiff person syndrome (SPS), with a prevalence of one to two per million, is an extremely rare neurological condition that is characterized by axial muscle stiffness and rigidity along with intermittent painful muscle spasms. It is often associated with psychiatric co-morbidities such as anxiety and depression. The pathophysiology, although poorly understood, is widely believed to be autoimmune in nature due to the association of anti-glutamic acid decarboxylase-65 (anti-GAD 65) antibodies with this condition. There is also a paraneoplastic variant that is more commonly associated with anti-ampiphysin antibodies. It occurs most commonly in patients with breast cancer followed by colon cancer. Most of the practising neurologists encounter just one or two cases of SPS in their entire careers, hence this condition remains underdiagnosed, leading to significant disability and distress to the patient. In this case report we describe a postmenopausal female who presented initially with symptoms of vertigo and dizziness and was hospitalized multiple times before the diagnosis was reached. Through this article, we attempt to increase awareness about this condition among practising physicians so as to increase the likelihood of earlier diagnosis and treatment.

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Background Neuropsychiatric symptoms (NPS) in AD, though more prevalent and severe in advanced disease, can occur as early features, with case reports suggesting more robust NPS in autosomal dominant Alzheimer disease (ADAD). The characterization of NPS in person with ADAD and their comparison to late‐onset Alzheimer’s disease (LOAD) enhances our ability to anticipate such symptoms and provides insight into differences in the underlying pathology of the two forms of the disease. Method Informants of 19 participants with mutations known to cause ADAD (mean age 45.58, 36.8% male) and 115 participants with LOAD (mean age 74.71, 42.6% male) were administered the full Neuropsychiatric Inventory (NPI) to assess the presence, frequency, and severity of 12 NPS, namely delusions, hallucinations, agitation, depression, anxiety, elation, apathy, disinhibition, irritability, aberrant motor behavior, sleep, and eating disorders. All participants had Clinical Dementia Rating (CDR) scores of 0.5. Chi‐square tests were used to compare the prevalence of NPS between ADAD and LOAD patients using both NPI screening and subquestions. Result ADAD and LOAD groups did not significantly differ in terms of MMSE score, CDR Sum of Boxes, gender, or years of education though ADAD subjects were more likely to be of Latinx origin (84% vs. 23%, p<0.001). ADAD and LOAD subjects did not differ on any qualifying questions, except for apathy, which was more common in ADAD mutation carriers (20.9% of LOAD subjects vs. 44.4% of ADAD subjects, p=0.039). Positive responses to subquestions regarding whether the participant was less affectionate and less interested in family were more commonly endorsed in ADAD. Conclusion Although apathy was more prevalent in ADAD subjects, the difference was not large and was not maintained when controlling for multiple comparisons. We therefore failed to identify robust differences in NPS between ADAD and LOAD. Our findings are limited by the small sample size of ADAD subjects but suggest that NPS are not markedly different in the early stages of ADAD and LOAD.

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