Stiff-person syndrome in a female patient with type 1 diabetes, dermatitis herpetiformis, celiac disease, microcytic anemia and copper deficiency

Bilić, Ervina; Bilić, Ernest; Šepec, Branimir Ivan; Vranješ, Davorka; Žagar, Marija; Butorac, Visnja; Čerimagić, Denis

doi:10.1016/j.clineuro.2009.04.006

Cited by 7 publications

(5 citation statements)

References 4 publications

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“…Stiff person syndrome (SPS) is characterised by the increased tone of axial and limb muscles, with superimposed muscle spasms leading to lumbar hyperlordosis, impaired gait, falls, and autonomic dysfunction associated with anti-GAD and/or other autoantibodies [ 69 ]. This syndrome has a strong concurrence with other autoimmune entities [ 70 , 71 , 72 ]. SPS has an estimated prevalence of 1–2 cases per million with an incidence of one case per million per year [ 73 ].…”

Section: Resultsmentioning

confidence: 99%

Movement Disorders Related to Gluten Sensitivity: A Systematic Review

et al. 2018

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Gluten related disorders (GRD) represent a wide spectrum of clinical manifestations that are triggered by the ingestion of gluten. Coeliac disease (CD) or gluten sensitive enteropathy is the most widely recognised, but extra-intestinal manifestations have also been increasingly identified and reported. Such manifestations may exist in the absence of enteropathy. Gluten sensitivity (GS) is another term that has been used to include all GRD, including those where there is serological positivity for GS related antibodies in the absence of an enteropathy. Gluten ataxia (GA) is the commonest extraintestinal neurological manifestation and it has been the subject of many publications. Other movement disorders (MDs) have also been reported in the context of GS. The aim of this review was to assess the current available medical literature concerning MDs and GS with and without enteropathy. A systematic search was performed while using PubMed database. A total of 48 articles met the inclusion criteria and were included in the present review. This review highlights that the phenomenology of gluten related MDs is broader than GA and demonstrates that gluten-free diet (GFD) is beneficial in a great percentage of such cases.

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Section: Resultsmentioning

confidence: 99%

Movement Disorders Related to Gluten Sensitivity: A Systematic Review

et al. 2018

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“…According to the literature, SPS has been reported to develop insidiously in the middle ages, and especially after emotional stress. It has an incidence of 1/1 000 000, with a female/male ratio of 2/1 7. While SPS is usually seen with autoimmune disorders, it may appear as a paraneoplastic condition in 5% of the cases 8.…”

Section: Discussionmentioning

confidence: 99%

The horses are the first thought but one must not forget the zebras even if they are rare: Stiff person syndrome associated with malignant mesothelioma

et al. 2014

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Stiff person syndrome (SPS) is a rare condition that causes rigidity in the muscles of the body and extremities, difficulty in walking, episodic spasms and progressive disability. SPS is generally seen together with autoimmune disorders such as diabetes mellitus, thyroiditis, vitiligo and pernicious anaemia. Rarely, it may develop as a paraneoplastic condition. SPS cases associated with breast cancer, small cell lung carcinoma, thymoma, Hodgkin's lymphoma and colorectal cancer have been reported in the literature. We present a case of a 58-year-old female patient who had malignant mesothelioma-associated SPS. Patients who have muscle spasms and difficulty in movement of joints should be evaluated for SPS before diagnosis of Parkinson's or other neurological disorders, and possible underlying malignancies should be excluded.

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“…Cu deficiency was independent of Hb and SF, a finding that is difficult to explain considering that Fe and Cu share transporters and a number of proteins in their metabolism and that Cu is needed for the formation of Hb molecules (38,39); the low number of cases with Cu deficiency may explain at least part of this result. Chronic Cu deficiency without anemia is relevant because, being asymptomatic, it may remain undiagnosed and patients may develop neurological symptoms in the long term; this can be treated, but there is evidence that at least part of the damage is not reversible (12,14,40). Zn status measured as serum Zn concentration revealed that cases had a higher frequency of deficient values than controls, but the differences did not reach statistical significance; significant differences in serum Zn concentration by serological status also were not found, a finding that is consistent with data recently published by Ho ¨gberg et al (41).…”

Section: Discussionmentioning

confidence: 99%